【醫師專欄-耳科】神經耳科學的各種症候群 (含人名姓氏的體徵/現象/症/病/方法)　The syndromes of the neurotology (Last name sign/phenomenon/syndrome/disease/method)－:: Dr.Wu-Vertigo

文章封面圖_症候群 v2020-04-02

‧美國哈佛大學‧麻州眼耳醫院‧研究員‧中國醫藥大學附設醫院‧耳鼻喉頭頸外科‧主任吳志賢醫師‧

～～相傳百年的眩暈科醫學諺語～～

～～ Proverbs of Vertigo Medicine ～～

～～眩暈不是疾病，而是一種症狀，如同發燒一樣，有數百種疾病會發生眩暈的情況～～

～～ Vertigo is not a disease but a symptom, like fever. There are hundreds diseases that can cause vertigo. ～～

～～人類的平衡系統主要有四個輸入與整合系統：視覺，內耳前庭，本體感覺，小腦。任何一個系統出現干擾，整個平衡系統就會出現紊亂，產生眩暈、頭暈、不平衡、步態不穩以及噁心、嘔吐、恐慌感～～

～～ The human balance system mainly has four input and integration systems: vision, inner ear vestibule, proprioception and cerebellum. If any one of the systems occur distaurbance, the entire balance system will be disordered, resulting in vertigo, dizziness, imbalance, unsteady gait, and nausea, vomiting, panic sensation. ～～

～～頭暈一詞可細分成四種症狀：眩暈、接近昏厥、不平衡、只是頭暈～～

～～ The term dizziness can be broken down into four categories of symptoms: vertigo, near syncope, imbalance, just dizzy ～～

～～眩暈症可歸因為三類疾病：末稍性眩暈、中樞性眩暈、反射性眩暈～～

～～ Vertigo can be classified into three categories of diseases: peripheral vertigo, central vertigo, reflex vertigo ～～

～～要選擇哪一科的醫生，就診原則就是找「具有眩暈專長的醫生」，能把眩暈治好的就是好醫生～～

～～ Which department doctor should be selected, the principle of seeing a doctor is to find "a doctor with vertigo expertise". Whoever cures vertigo is a good doctor. ～～

～～眩暈治癒的關鍵就是：詳細檢查，確認病因，追根究柢，對症下藥～～

～～ The key to cure vertigo is to make a detailed inspection, confirm the cause, follow up evidence, and take appropriate management. ～～

～～經常眩暈/頭暈是不是腦部出問題了？如有以下症狀一定要立即就醫：臉歪嘴斜，手腳無力；臉麻手麻，說話不清楚；胸痛，心悸，呼吸急促；頭痛，昏厥，意識不清～～

～～ Often vertigo / dizzy, is there a brain problem? Seek medical treatment immediately if you have the following symptoms: face crooked, mouth slanted, paralyzed limbs; facial numbness, hand numbness, unclear speech; chest pain, palpitation, short of breath; headache, fainting, unclear consciousness ～～

【醫師專欄-耳科】神經耳科學的各種症候群 (含人名姓氏的體徵/現象/症/病/方法)　The syndromes of the neurotology (Last name sign/phenomenon/syndrome/disease/method)

Sort alphabetically 按英文字母排序

● Abadie's sign of tabes dorsalis 脊髓癆的阿巴迪體徵：

可能共濟失調。neurosyphilis 神經梅毒。第三期梅毒通常發生於最初的感染的1-10年後，在某些情況下，可能需要長達50年。這一階段的特點是形成gumma，gumma是柔軟，瘤樣的球，組織學上為炎症肉芽腫，可發生於幾乎任何組織，包括骨骼。其他特點包括未經處理的第三期梅毒會導致神經性關節病，這是因喪失感覺(包括本體感覺)引起的關節面損傷。更嚴重的表現形式，包括神經性梅毒和心血管梅毒。在一研究中，未治療的梅毒，10％的患者發展為心血管梅毒，16％形成gumma，7％發展為神經性梅毒。神經併發症，在這一階段可以是多樣的。在一些病人，表現為全身性麻痺的瘋狂(generalized paresis of the insane)，這會導致人格改變，情緒變化，過度反射和Argyll-Robertson pupil。Argyll-Robertson pupil是一個診斷標誌，在聚焦時，瞳孔會產生不規則收縮，但是對光反射卻沒有此一現象。脊髓癆(Tabes Dorsalis)，也被稱為局部運動共濟失調(locomotor ataxia)，常常導致shuffling gait。心血管併發症包括梅毒性大動脈炎、主動脈瘤、主動脈竇動脈瘤和主動脈瓣閉鎖不全。也會導致左心室肥大，引發心衰竭。A medical sign of tabes dorsalis, a late consequence of neurosyphilis. It is elicited by compressing the Achilles tendon, which normally causes pain. A positive Abadie's sign is defined by the absence of pain. It may occur in any disease that damages the dorsal column–medial lemniscus pathway, which transmits sensory information from the periphery to the brain. It is named for Joseph Louis Irenée Jean Abadie, a French neurologist.

＜阿巴迪是法國神經科醫生. Joseph Louis Irenée Jean Abadie (15 December 1873, Tarbes – 1934) was a French neurologist.＞

● Adair-Dighton's syndrome 阿黛爾-戴通症 (= Van der Hoeve's syndrome 凡德霍夫症, Osteogenesis imperfecta type 1 第一型成骨不全症)：

三聯徵：藍鞏膜 + 晚發性成骨不全 + 耳硬化症。成骨不全症(Osteogenesis Imperfecta, OI)是一種罕見的遺傳疾病，其特徵為患者的骨骼強度耐受力變差、骨質脆弱而常發生骨折。大約有90%的患者，其致病原因為形成第一型膠原纖維(collagen type I)相關的基因COL1A1和COL1A2產生突變而引起。傳統上，成骨不全症可分為四型，但是近年的研究顯示，依患者的臨床症狀和基因上的發現，目前可將成骨不全症分為七型。在第五型和第六型患者之COL1A1和COL1A2基因上找不到突變、第一型膠原纖維也沒有異常，致病的基因目前仍未知；而第七型患者導因於CRTAP (cartilage-associated protein)基因的突變，CRTAP與膠原纖維的後修飾(post-translation)有關；第八型則與LEPRE基因的突變有關。Triad: Blue sclera + Osteogenesis imperfecta tarda + Otosclerosis. Osteogenesis imperfecta, or brittle bones syndrome, occurs in four types. Of the several applicable eponyms choice usually depends upon those abnormalities that are most conspicuous. This is a dominant form of the syndrome, characterized by a combination of blue sclerae, brittle bones, and conductive deafness due to otosclerosis-like changes in the temporal bone, although one condition may be absent. The course is progressive, but many patients live to an advanced age. Osteogenesis imperfecta is one of the most frequent of the heritable disorders of connective tissue. The Ekman-Lobstein syndrome is an adult variant.

＜阿黛爾-戴通是英國耳鼻喉科醫生. Charles Adair-Dighton (1885 - died) was an English otolaryngologist.＞

＜凡德霍夫是荷蘭眼科醫生. Jan van der Hoeve (1878 - 1952) was a Dutch ophthalmologist.＞

● Alagille's syndrome 阿拉吉歐症：

可能聽損。一種體染色體顯性疾病，多數因第20號染色體上的JAG1基因發生突變或缺失所造致。有5大臨床表現是特殊面容、膽汁鬱積、先天性心血管病、骨骼異常、角膜異常等。A genetic disorder that affects primarily the liver and the heart. Problems associated with the disorder generally become evident in infancy or early childhood. The disorder is inherited in an autosomal dominant pattern, and the estimated prevalence of Alagille syndrome is 1 in every 100,000 live births. It is named after the French pediatrician Daniel Alagille, who first described the condition in 1969. The severity of the disorder can vary within the same family, with symptoms ranging from so mild as to go unnoticed, to severe heart and/or liver disease that requires transplantation. It is uncommon, but Alagille syndrome can be a life-threatening disease with a mortality rate of 10%. The majority of deaths from ALGS are typically due to heart complications or chronic liver failure.

＜丹尼爾·阿拉吉歐是法國醫生. Daniel Alagille (24 January 1925 – 8 November 2005) was a French physician who specialized in pediatric hepatology.＞

● Alexander’s law 亞歷山大定律：

急性單側前庭自發性眼振，將眼振的強度分三級。若眼振方向(快速相方向)向右，第一度，向右看才有眼振；第二度，向右看、向前看都有眼振；第三度，向快慢相看、向前看都有眼振。Refers to spontaneous nystagmus that occurs after an acute unilateral vestibular loss. It was first described in 1912 and has three elements to explain how the vestibulo-ocular reflex responds to an acute vestibular insult. The first element says that spontaneous nystagmus after an acute vestibular impairment has the fast phase directed toward the healthy ear. The direction of the nystagmus, by convention, is named for the fast phase, so the spontaneous nystagmus is directed toward the healthy ear. The second element says nystagmus is greatest when gaze is directed toward the healthy ear, is attenuated at central gaze and may be absent when gaze is directed toward the impaired ear. The third element says that spontaneous nystagmus with central gaze is augmented when vision is denied. This became apparent with the implementation of electrographic testing. Alexander's law states that in individuals with nystagmus, the amplitude of the nystagmus increases when the eye moves in the direction of the fast phase (saccade). It is manifested during spontaneous nystagmus in a patient with a vestibular lesion. The nystagmus becomes more intense when the patient looks in the quick-phase than in the slow-phase direction. The law was named after Gustav Alexander who described it in 1912.

＜古斯塔夫·亞歷山大是奧地利耳鼻喉科醫生. Gustav Alexander (1873 – 12 April 1932) was an Austrian otolaryngologist.＞

● Alzheimer's disease 阿茲海默症：

可能頭暈。又稱老年痴呆症，是一種常見的痴呆症(Dementia)，多半發生在 65 歲以上的老年人身上。患者會有大腦認知與記憶功能退化的症狀。目前阿茲海默症還無法被治癒。2006 年時，全球約有 266 萬人患有阿茲海默症，估計在在 2050 時每 85 個人中便有 1 人患有阿茲海默症。阿茲海默症大致可分為四個階段。隨著時間推進，患者的大腦神經細胞會遭到破壞，初期以海馬迴與杏仁核為主要的目標（這也是為什麼患者在記憶功能及情緒上會受到影響），最終則會擴展至整個大腦，導致大腦萎縮，使得認知與記憶功能會逐步惡化。神經細胞的死亡也會造成神經傳導物質的流失，包含乙醯膽鹼(ACh - Acetylcholine)、多巴胺(Dopamine)、谷氨酸(Glutamate)、正腎上腺素(Norepinephrine)、血清素(Serotonin)等等，對記憶的形成、學習、肢體動作、情緒等等皆會造成嚴重的影響。阿茲海默症的病程可能歷經 2 到 20 年的時間，死亡通常發生在初次發病後的 8 到 10 年之間。疾病發展的過程因人而異，變化幅度相當大。A chronic neurodegenerative disease that usually starts slowly and gradually worsens over time. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, not managing self-care, and behavioural issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the typical life expectancy following diagnosis is three to nine years.

＜愛羅斯·阿茲海默是德國精神科醫生與神經病理學家. Aloysius Alzheimer (June 14, 1864 – December 19, 1915) was a German psychiatrist and neuropathologist.＞

● Alport's syndrome 亞伯症 (= Progressive hereditary nephritis and deafness 進行性遺傳性腎炎耳聾, Hereditary Oto-Oculo-Renal Syndrome 遺傳性耳眼腎症)：

會造成聽損。一種遺傳性腎炎(Herditary nephropathy)。此為遺傳性腎炎佔最多的一種症候群，在小孩的腎炎患者中，佔三％；在歐美接受腎移植患者中，佔一至二％。基因出現的頻率在美國約為百萬分之一，遺傳的形式變異性大，性染色體連鎖性遺傳者，佔八十％，另十五至二十％為自體染色體顯性遺傳，也可發現父傳子之遺傳模式。典型亞伯症候群的表現，包括：血尿、進行性腎衰竭、耳聾、眼病變和典型腎絲球基底膜之變厚，通常男性的患者比女性患者較嚴重。在小孩時，即可表現出：(1)在腎臟方面：最初期症狀往往為血尿，初期並沒有蛋白尿，而年紀愈大愈明顯，男孩約四十％可出現腎病症候群；若有蛋白尿，則預後不好。(2)在聽力方面：病人有非特異性的高音感覺神經性耳聾，聽力受損可能輕微，只能用聽力檢查才能發現；但嚴重患者，可完全耳聾；聽力受損在男性的患者較為嚴重，且常為腎衰竭的預告。(3)在眼睛方面：出現機率較多，約有十五至四十％，主要的變化在晶狀體和視網膜；前圓錐形晶狀體是亞伯症候群最特異的表現，常出現在有耳聾男性患者；少數人亦會患有高脯氨酸血症、巨大血小板及血小板缺乏症、抗甲狀腺抗體等。A genetic disorder affecting around 1 in 5,000-10,000 children, characterized by glomerulonephritis, end-stage kidney disease, and hearing loss. Alport syndrome can also affect the eyes, though the changes do not usually affect sight, except when changes to the lens occur in later life. Blood in urine is universal. Proteinuria is a feature as kidney disease progresses. The disorder was first identified in a British family by the physician Cecil A. Alport in 1927. Alport Syndrome once also had the label hereditary nephritis, but this is misleading as there are many other causes of hereditary kidney disease and 'nephritis'. Alport syndrome is caused by an inherited defect in type IV collagen—a structural material that is needed for the normal function of different parts of the body. Since type IV collagen is found in the ears, eyes, and kidneys, this explains why Alport syndrome affects different seemingly unrelated parts of the body (ears, eyes, kidneys, etc.).

＜亞瑟·塞西爾·亞伯是南非醫生.Arthur Cecil Alport, M.D. (1880 – 1959) was a South African physician.＞

● Arnold-Chiari malformation 阿諾德-奇阿里畸形：

可能眩暈。一種先天性後腦異常(hindbrain abnormality)。第二型奇阿里畸形(type II Chiari malformation)又叫作阿諾德-奇阿里畸形。奇阿裡畸形是一種先天性後腦異常，主要表現是小腦組織落入上頸椎管。1883年由John Cleland醫師在解剖上觀察到，1891年由德國病理醫師Hans Chiari詳細描述，分成四種類型，其中，第二型最常見。第二型奇阿里畸形常在幼年時就出現症狀，影像學特徵是後顱窩窄小，伴有腦幹、小腦蚓部及第四腦室落入上頸椎管，影響後顱窩及腦室的發育。常合併腦積水、胼胝體發育不良、脊髓脊膜膨出及脊髓空洞積水的發生。Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull). CMs can cause headaches, difficulty swallowing, vomiting, dizziness, neck pain, unsteady gait, poor hand coordination, numbness and tingling of the hands and feet, and speech problems. Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis. This can sometimes lead to non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid (CSF) outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. The malformation is named for Austrian pathologist Hans Chiari.

＜朱利葉斯·阿諾德是德國病理學家. Julius Arnold (August 19, 1835 – February 3, 1915) was a German pathologist.＞

＜漢斯·奇阿里是奧地利病理學家. Hans Chiari (4 September 1851 − 6 May 1916) was an Austrian pathologist.＞

● Aschan's classification of positional nystagmus 頭位眼振的阿斯肯分類:

Aschan將頭位眼振分成三型：

Type I: Nystagmus persistent, direction changing (commonly reversing in opposite head positions)

Type II: Nystagmus persistent, direction fixed in all head positions.

Type III: Nystagmus transitory. The commonest form is the benign paroxysmal type of Dix and Hallpike (1952) though Stahle and Terins (1965) have properly pointed out that other forms of paroxysmal positional nystagmus may be found.

Aschan's modification (1961) of Nylen's earlier classification (1950) is clinically usefull, as it takes into acount varying durations of positional nystagmus under different circumstances, as well as other prominent features. Positional nystagmus is one of the valuable vestibular signs for the diagnosis of vertiginous diseases. However, the classification of positional nystagmus has been modified since Nylen's initial classification in 1950. Positional nystagmus may be persistent or transitory. Of the persistent forms, type I refers to nystagmus changing direction in different head positions and type II to nystagmus beating in a single direction. Both types may denote either peripheral or central abnormality. Transitory, or paroxysmal, positional nystagmus is classified as type III, and the history and physical and electronystagmographic features of its accompanying vertigo and nystagmus permit subdivision into typical and atypical forms. Guidelines are given for the differentiation of typical and atypical forms. In the great majority of cases type III positional nystagmus denotes a harmless inner ear disturbance, but the occasional serious intracranial lesion is also causative.

● Barre-Lieou's syndrome 巴利-劉症 (= Cervical syndrome 頸症)：

頸源性眩暈(cervical vertigo)的一個舊稱，目前已經不被使用。頸源性眩暈是一個籠統的病名，這個病名目前也漸漸被揚棄，改用數個精準的頸部疾病。A traditional medical diagnosis that is not utilized frequently in modern medicine. It is a complex combination of symptoms, amounting to a headache syndrome, that was originally hypothesized to be due to cervical spondylosis. Damage to the posterior cervical sympathetic chain due to the degeneration of the cervical vertebra was theorized to play a role in this syndrome by the prolapsing of disc in the mid-cervical spine. However, the medical theory as was originally postulated was found to contain inconsistencies. The Barré–Liéou syndrome is regarded by many current medical researchers as synonymous with cervicogenic headache. Thus, the original works of Barré and Liéou were foundational in identifying a crucial feature which distinguishes cervicogenic headache from other headache syndromes—the concept that the pain originates from a structural abnormality in the cervical spine.
＜巴利是法國神經科醫生. Jean Alexandre Barré (25 May 1880, Nantes – 26 April 1967, Strasbourg) was a French neurologist.＞

<劉是中國醫生. Yong-Cheon Lieou was a Chinese physician.>

● Behçet's disease 貝塞特病：

可能眩暈。貝塞特症是一種慢性、反覆發作的發炎性疾病，可能是自體免疫性疾病。西元 1937 年，由土耳其的皮膚科醫師 Hulusi Behcet 提出詳細描述，故以其名命名之。此病可侵犯全身許多器官，臨床的表現具多樣性，且易反覆發作。主要的臨床特徵為口腔潰瘍(recurrent oral ulceration)、反覆性生殖器潰瘍(recurrent genital ulceration)及眼睛葡萄膜炎(uveitis)，除前述三種典型症狀外，尚可侵犯關節、中樞神經、呼吸系統、心血管系統、消化系統及泌尿系統等，當引發嚴重的併發症時，可能會危及生命。A type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful mouth sores, genital sores, inflammation of parts of the eye, and arthritis. The sores typically last a few days. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often the symptoms come and go. The cause is unknown. It is believed to be partly genetic. Behçet's is not contagious. Diagnosis is based on at least three episodes of mouth sores in a year together with at least two of the following: genital sores, eye inflammation, skin sores, a positive skin prick test.

＜貝塞特是土耳其皮膚科醫生. Hulusi Behçet (20 February 1889, in Istanbul – 8 March 1948) was a Turkish dermatologist and scientist.＞

● Bell's palsy 貝爾麻痺 (= Idiopathic facial paralysis, IFP 原發性顏面神經麻痺)：

一種急性、周邊性、原因未明的顏面神經麻痺，發生率約是每年每十萬人有13到34個案例，性別、種族、地區的差異對發生率沒有影響，但在孕婦及糖尿病的病人身上出現機率較高，2012年的研究也發現糖尿病病人出現貝爾麻痺時症狀會較為嚴重。A type of facial paralysis that results in an inability to control the facial muscles on the affected side. Symptoms can vary from mild to severe. They may include muscle twitching, weakness, or total loss of the ability to move one or rarely both sides of the face. Other symptoms include drooping of the eyelid, a change in taste, pain around the ear, and increased sensitivity to sound. Typically symptoms come on over 48 hours. The cause of Bell's palsy is unknown. Risk factors include diabetes, a recent upper respiratory tract infection, and pregnancy. It results from a dysfunction of cranial nerve VII (the facial nerve). Many believe that this is due to a viral infection that results in swelling. Diagnosis is based on a person's appearance and ruling out other possible causes. Other conditions that can cause facial weakness include brain tumor, stroke, Ramsay Hunt syndrome type 2, myasthenia gravis, and Lyme disease. The condition normally gets better by itself with most achieving normal or near-normal function. Corticosteroids have been found to improve outcomes, while antiviral medications may be of a small additional benefit. The eye should be protected from drying up with the use of eye drops or an eyepatch. Surgery is generally not recommended. Often signs of improvement begin within 14 days, with complete recovery within six months. A few may not recover completely or have a recurrence of symptoms.

＜查爾斯·貝爾是蘇格蘭外科醫生，解剖學家，生理學家，神經學家，藝術家和哲學神學家. Charles Bell (12 November 1774 – 28 April 1842) was a Scottish surgeon, anatomist, physiologist, neurologist, artist, and philosophical theologian.＞

● Bezold's abscess 貝佐德膿腫：

中耳炎的顱外併發症。1881年德國耳科學家Friedrich von Bezold (1824～1908)最早介紹了一種耳源性顱外併發症－頸部膿腫。後來，這種疾病以Bezold的名字命名，稱為貝佐德膿腫 (Bezold’s abscess)。在 Bezold的年代，中耳炎中大約50％發生乳突炎，其中20％的乳突炎患者發生骨膜下膿腫即貝佐德膿腫，到1959年融合性乳突炎的發病率下降到0.4％。從1975～2000年，全世界的英語文獻中報道貝佐德膿腫的僅有24例。隨著醫療條件的改善以及抗生素的廣泛應用等原因，因慢性化膿性中耳炎引起的貝佐爾德膿腫已經非常罕見。An abscess deep to the sternocleidomastoid muscle where pus from mastoiditis erodes through the cortex of the mastoid part of the temporal bone, medial to the attachment of sternocleidomastoid, extends into the infratemporal fossa, and deep to the investing layer of the deep cervical fascia. It is a rare complication of acute otitis media.

＜佛里德希·貝佐德是德國耳科醫生，也是慕尼黑大學的教授. Friedrich Bezold (February 9, 1842 – October 5, 1908) was a German otologist and professor at the University of Munich.＞

● Bickerstaff's brainstem encephalitis 畢克斯達夫腦幹腦炎：

可能頭暈。畢克斯達夫腦幹腦炎是吉巴症(GBS)的一個變型(variant)，並不常見。米勒費雪症候群(Miller Fisher syndrome)及畢克斯達夫腦幹腦炎(Bickerstaff brainstem encephalitis)都屬於Guillian-Barre症候群的鑑別診斷之一。是一種常見在感染後發生的神經根發炎及腦幹發炎的疾病。相較於米勒費雪症候群，畢氏腦炎較為少見且較為嚴重。兩者都會出現運動失調(ataxia)，眼外肌麻痺(ophthalmoplegia)等症狀。由於影響到腦幹，畢氏腦炎患者可能會出現意識障礙(impaired consciousness)或反射增強(hyper-reflexia)的現象。米勒費雪症候群及畢氏腦炎的病患，大多於發病前一周會出現前驅的感染症狀，且以上呼吸道感染為主。空腸弧菌(Campylobactar jejuni)是常見的相關感染病菌之一。A rare inflammatory disorder of the central nervous system, first described by Edwin Bickerstaff in 1951. It may also affect the peripheral nervous system, and has features in common with both Miller Fisher syndrome and Guillain–Barré syndrome. In order to diagnose Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present. These are also diagnostic features of Miller Fisher syndrome, and so Bickerstaff's is only diagnosed if other features are present which exclude Miller Fisher syndrome. These may include drowsiness, coma or hyperreflexia. When the condition is defined in this way, a number of other features are commonly but not always found: among these are weakness of the limbs, the face, and/or the bulbar muscles; abnormalities of the pupils; and absent reflexes. Like some other autoimmune diseases, the condition usually follows a minor infection, such as a respiratory tract infection or gastroenteritis.

＜愛德溫·羅伯特·比克斯達夫是英國神經科醫生. Edwin Robert Bickerstaff (20 November 1920 – 23 November 2008), was a British neurologist.＞

● Bill's bar 比爾脊：

內聽道上表面的垂直脊，分隔開前側顏面神經、後側上前庭神經。後表面上的水平脊(transverse crest)，則分隔開上、下前庭神經。

The opening to the meatus is called the porus acusticus internus or internal acoustic opening. It is located inside the posterior cranial fossa of the skull, near the center of the posterior surface of the petrous part of the temporal bone. The size varies considerably. Its outer margins are smooth and rounded. The canal which comprises the internal auditory meatus is short (about 1 cm) and runs laterally into the bone. The lateral (outer) aspect of the canal is known as the fundus.[1] The fundus is subdivided by two thin crests of bone to form three separate canals, through which course the facial and vestibulocochlear nerve branches. From medial to lateral, the transverse (falciform) crest first divides the meatus into superior and inferior sections; a second vertical crest (Bill's bar, named by William F. House) then divides the upper passage into anterior and posterior sections. Although there are three osseous canals, the fundus is conceptually divided more commonly into four quadrant areas according to the four major nerve branches of the inner ear: (1) anterior superior - facial nerve area (contains facial nerve and nervus intermedius). (2) anterior inferior - cochlear nerve area (contains cochlear nerve). (3) posterior superior - superior vestibular area (contains superior division of vestibular nerve). (4) posterior inferior - inferior vestibular area (contains inferior division of vestibular nerve). The cochlear and vestibular branches of cranial nerve VIII separate according to this schema and terminate in the inner ear. The facial nerve continues traveling through the facial canal, eventually exiting the skull at the stylomastoid foramen.

＜威廉·福斯·豪斯是美國耳科醫生，美國洛杉磯豪斯耳研所(HEI)是全世界的顳骨研磨手術、人工電子耳手術、側顱底手術的教學研究重鎮，豪斯更被稱為美國神經耳科之父. William Fouts House (December 1, 1923 in Kansas City, Missouri – December 7, 2012 in Aurora, Oregon) was an American otologist, physician and medical researcher who developed and invented the cochlear implant. The cochlear implant is considered to be the first invention to restore not just the sense of hearing, but any of the absent five senses in humans. Dr. House also pioneered approaches to the lateral skull base for removal of tumors, and is considered "the Father of Neurotology."＞

● Bing's test 並測驗：

骨傳道的一種音叉測驗，透過耳球蓋跟不蓋住外耳道，區分是否為骨傳導聽損。A test of bone conduction. Examine the effect of occlusion of ear canal on hearing. Vibrating tuning fork is placed on the mastoid process while examiner alternatively closes and open the ear canal by pressing tragus inward. A tuning fork test in which the vibrating fork is held against the mastoid process and the auditory meatus is alternately occluded and left open; an increase and decrease in loudness (positive Bing) is perceived by the normal ear and in sensorineural hearing loss, whereas lack of a difference in loudness (negative Bing) is the perception in conductive hearing loss.

● Bruininks-Oseretsky's test of motor proficiency (BOTMP) 布魯茵克斯-歐索瑞斯基動作精熟度測試：

使用的年齡層適於4歲半到14歲半的兒童，主要的施測用意於評估小朋友的動作技巧，採用ㄧ對ㄧ施測。此量表可提供教育者,臨床和實驗者豐富的資訊,。此種量表分為兩種形式：a) 完整的量表: 內含8項次項目，總共有46個不同的施測類別，施測所需的時間大約為45-60分鐘。八項次項目分別為快速短跑能力，平衡能力，雙側整合，肌力，上肢的協調，反應速度，視覺動作控制，上肢的使用與速度品質。b) short-form：內含上述八種次項目中的14種測驗, 此種量表僅用於大約的評估, 完成的時間為15到20分鐘。The Bruininks-Oseretsky Test of Motor Proficiency Second Edition (BOT™-2) delivers the most precise and comprehensive measure of motor skills, both gross and fine. It contains subtests and challenging game-like tasks and is very easy to administer. Age range: 4:0-21:11.

● Bruns' nystagmus 伯朗斯眼振：

一種特異眼振。向右看出現向右眼振，向左看出現向左眼振，左右還不對稱，一側振幅大頻率小，一側振幅小頻率大，振幅大之側為病灶側。很可能是腦幹病灶造成，尤其是小腦橋腦角病灶。An unusual type of bilateral nystagmus most commonly occurring in patients with cerebellopontine angle tumours. It is caused by the combination of slow, large amplitude nystagmus (gaze paretic nystagmus) when looking towards the side of the lesion, and rapid, small amplitude nystagmus (vestibular nystagmus) when looking away from the side of the lesion. It occurs in 11% of patients with vestibular schwannoma, and occurs mainly in patients with larger tumours (67% of patients with tumours over 3.5 cm diameter). Bruns nystagmus is also associated with an increased incidence of balance disturbance in patients with vestibular schwannoma. It may be caused by the compression of both flocculi, the vestibular part of the cerebellum, and improvement in both the nystagmus and balance problems occur commonly after removal of the tumour. Bruns nystagmus is named for Ludwig Bruns (1858 – 1915).

＜路德維希·伯朗斯是德國神經科醫生. Ludwig Bruns (25 June 1858 – 9 November 1916) was a German neurologist.＞

● Bruns' syndrome 伯朗斯症：

MPPV = Bruns syndrome ＋ Das akute Unterwurm syndrome。1971年日本順天堂大學耳鼻喉科Sakata坂田英治教授命名MPPV (malignant paroxysmal positional vertigo)惡性陣發性姿勢性眩暈，有別於BPPV，同時包含函了Bruns' syndrom伯朗斯症及Das akute Unterwurm's syndrome昂特溫症。An insidious percentage of paroxysmal positional vertigo appears to be intractable with canalith repositioning maneuver and also is not self-limiting. This type of positional vertigo is sustained by the action of intracranial tumors that mimics the clinical aspects of benign paroxysmal positional vertigo.Aim of this study is to clarify the features of these forms of positional vertigo, which we indicate as malignant paroxysmal positional vertigo.

＜路德維希·伯朗斯是德國神經科醫生. Ludwig Bruns (25 June 1858 – 9 November 1916) was a German neurologist.＞

● Carhart's notch 卡哈凹槽：

耳硬化症的骨傳導在1k或2k Hz會出現聽損，聽力圖看起來呈現一個凹槽。Otosclerosis is a disease that stiffens the stapes annular ligament, resulting in air-conducted (AC) hearing impairment. In addition, it produces a bone-conducted (BC) hearing impairment of approximately 20 dB near 2 kHz, which is called Carhart’s notch (Carhart, 1950). While Carhart attributed this phenomenon to “mechanical factors associated with stapedial fixation” (Carhart, 1971), Tonndorf (1971) suggested that the Carhart’s notch at 2 kHz is caused by the loss of the middle-ear inertia due to the otosclerotic condition close to the resonance point of the ossicular chain. According to Stenfelt and Goode (2005), five factors potentially affect BC hearing, with inertia of the cochlear fluid and middle-ear mass, as well as bone compression among the major factors. We hypothesized that Carhart’s notch is affected by bone compression as well as by the inertia of the cochlear fluid and middle-ear mass. In this study, we investigated the effects on BC hearing of each factor separately, inertia and bone compression, in addition to the effects of the two factors in combination.

＜雷蒙德·卡哈是一位美國語言/語言病理學家. Raymond T Carhart (1912-1975) was an American Speech/Language Pathologist.＞

● Chimani Moose's test 奇曼尼‧摩斯測驗 (= Weber’s lateralization test 韋伯定向測驗)：

一種音叉測驗，韋伯測驗的變異型。A variation of Weber's test. Normally in Weber's test, the patient hears the best in the occluded ear. In malingering, the patient will not accept to hear better in the occluded ear.

● Cogan's syndrome 寇甘症：

會造成眩暈。一種罕見的自體免疫疾病，屬細胞性自體免疫，好發於白種人，男女比例相同。臨床特徵是可逆性的間質性角膜炎，不可逆性的漸進性耳蝸前庭症狀(聽損、耳鳴、眩暈)，陰性梅毒血清反應。可能合併全身性血管炎，或其他免疫疾病，例如紅斑性狼瘡、類風濕性關節炎。Cogan syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Cogan syndrome can lead to vision difficulty, hearing loss and dizziness. The condition may also be associated with blood-vessel inflammation (called vasculitis) in other areas of the body that can cause major organ damage in 15% of those afflicted or, in a small number of cases, even death. It most commonly occurs in a person's 20s or 30s. The cause is not known. However, one theory is that it is an autoimmune disorder in which the body's immune system mistakenly attacks tissue in the eye and ear. A rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D.G. Cogan in 1945.

＜大衛·格倫登寧·冠甘是美國眼科醫生. David Glendenning Cogan was an American ophthalmologist.＞

● Corti's orgran 柯蒂氏器：

內耳聽覺受器。柯蒂氏器由支持細胞和感覺細胞(outer, inner hair cells)組成，並包含了內側面的邊緣細胞(border cells)以及外側面的Hensen細胞(cells of Hensen)。Rhe receptor organ for hearing and is located in the mammalian cochlea. This highly varied strip of epithelial cells allows for transduction of auditory signals into nerve impulses' action potential. Transduction occurs through vibrations of structures in the inner ear causing displacement of cochlear fluid and movement of hair cells at the organ of Corti to produce electrochemical signals. Italian anatomist Alfonso Giacomo Gaspare Corti (1822–1876) discovered the organ of Corti in 1851. The structure evolved from the basilar papilla and is crucial for mechanotransduction in mammals.

＜柯蒂是義大利解剖學家. Alfonso Giacomo Gaspare Corti (22 June 1822 – 2 October 1876) was Italian anatomist.＞

● Costen's syndrome 柯斯騰症：

可能聽損、頭暈。一種顳顎關節位置異常或機能不全疾病，使鄰近的組織及神經受壓迫所致。Costen described the syndrome as consisting of partial deafness, stuffy sensation in the ears (especially during eating), tinnitus, clicking and snapping of the temporomandibular joint, dizziness, headache, and burning pain in the ears, throat, and nose. Costen ascribed the symptoms to dental malocclusion. Later investigators disprove Costen’s anatomical and clinical conclusions. According to later studies, the syndrome consists mainly of temporomandibular crepitation, decreased temporomandibular mobility, preauricular and auricular pain, pain on movement, headache, tenderness of the jaws on palpation, and, sometimes, head and nasopharyngeal symptoms.

＜詹姆斯-伯雷-柯斯騰是美國耳鼻喉科醫生. James Bray Costen (1895 - 1962) was an American otorhinolaryngologist.＞

● Crohn’s disease 克隆病：

可能眩暈。一種發炎性腸病。發炎性腸道疾病(inflammatory bowel disease, IBD)主要是指克隆病(Crohn's disease, CD)及潰瘍性結腸炎(ulcerative colitis, UC)兩種疾病，臨床上的特點是容易復發並形成慢性以及無法預期的病程。克隆病為一慢性、全壁式(transmural)的發炎性疾病，可侵犯由口至肛門的任何部位，約半數會有直腸病變，且常合併有許多腸道外表現。潰瘍的特點是鵝口瘡般(aphthoid)、縱走式或融合式的型態，且邊緣組織接近正常；病變則是跳躍及全壁式侵犯。初期黏膜的表現只是水腫，但不斷進行全壁性發炎，造成纖維化，形成緊縮的瘢痕(scar)。其致病機轉迄今雖未十分明瞭，但與免疫反應失控關係十分密切。罹患克隆病在性別上並無明顯差異，好發年齡為15-35歲，高峰期是20歲左右。臨床症狀包括陣發性右下腹痛、腹瀉或便祕、體重減輕、偶會發燒。此外，較嚴重者甚至會導致腸阻塞、腸道廔管、巨結腸、腸穿孔等。藥物治療以sulfasalazine、類固醇(steroids)等為主。A type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract from the mouth to the anus. Symptoms often include abdominal pain, diarrhea (which may be bloody if inflammation is severe), fever, and weight loss. Other complications outside the gastrointestinal tract may include anemia, skin rashes, arthritis, inflammation of the eye, and tiredness. The skin rashes may be due to infections as well as pyoderma gangrenosum or erythema nodosum. Bowel obstruction may occur as a complication of chronic inflammation, and those with the disease are at greater risk of bowel cancer.

＜伯瑞爾·伯納德·克隆是美國胃腸病學家. Burrill Bernard Crohn (June 13, 1884 – July 29, 1983)[1] was an American gastroenterologist.＞

● Crouzon's syndrome 克魯松症：

可能聽損、眩暈。因第十號及第四號染色體上的FGFR基因出現錯誤而引致的骨骼、軟骨的形成異常的罕見遺傳疾病。An autosomal dominant genetic disorder known as a branchial arch syndrome. Specifically, this syndrome affects the first branchial (or pharyngeal) arch, which is the precursor of the maxilla and mandible. Since the branchial arches are important developmental features in a growing embryo, disturbances in their development create lasting and widespread effects. This syndrome is named after Octave Crouzon, a French physician who first described this disorder. First called "craniofacial dysostosis" ("craniofacial" refers to the skull and face, and "dysostosis" refers to malformation of bone), the disorder was characterized by a number of clinical features which can be described by the rudimentary meanings of its former name. This syndrome is caused by a mutation in the fibroblast growth factor receptor 2 (FGFR2), located on chromosome 10. The developing fetus's skull and facial bones fuse early or are unable to expand. Thus, normal bone growth cannot occur. Fusion of different sutures leads to abnormal patterns of growth of the skull.

＜克魯松是法國神經科醫生. Louis Edouard Octave Crouzon (1874–1938), was a French neurologist.＞

● Dandy's syndrome 丹第症：

會造成眩暈。一種視覺識別障礙性眩暈。Dandy's Syndrome, or bilateral vestibular hypofunction and oscillopsia, may cause chronic disequilibrium aggravated by head movement or in the presence of reduced light. It may be secondary to ototoxicity, central nervous system tumors, Meniere's Syndrome, infections or trauma, or may be idiopathic.

＜沃爾特·愛德華·丹第是美國神經外科醫生和科學家. Walter Edward Dandy (1886-1946) was an American neurosurgeon.＞

● Das akute Unterwurm's syndrome 昂特溫症：

MPPV = Bruns syndrome ＋ Das akute Unterwurm syndrome。1971年日本順天堂大學耳鼻喉科Sakata坂田英治教授命名MPPV (malignant paroxysmal positional vertigo)惡性陣發性姿勢性眩暈，有別於BPPV，同時包含函了Bruns's syndrom及Das akute Unterwurm's syndrome。An insidious percentage of paroxysmal positional vertigo appears to be intractable with canalith repositioning maneuver and also is not self-limiting. This type of positional vertigo is sustained by the action of intracranial tumors that mimics the clinical aspects of benign paroxysmal positional vertigo.Aim of this study is to clarify the features of these forms of positional vertigo, which we indicate as malignant paroxysmal positional vertigo.

＜路德維希·伯朗斯是德國神經科醫生. Ludwig Bruns (25 June 1858 – 9 November 1916) was a German neurologist.＞

● DiGeorge's syndrome 狄喬治症 (= 22q11.2 deletion syndrome)：

可能聽損。一種遺傳疾病，病因為第22對染色體上的22q11.2基因座上有約30~40個基因的刪除。A syndrome caused by the deletion of a small segment of chromosome 22. While the symptoms can vary, they often include congenital heart problems, specific facial features, frequent infections, developmental delay, learning problems and cleft palate. Associated conditions include kidney problems, hearing loss and autoimmune disorders such as rheumatoid arthritis or Graves' disease. DiGeorge syndrome is typically due to the deletion of 30 to 40 genes in the middle of chromosome 22 at a location known as 22q11.2. About 90% of cases occur due to a new mutation during early development, while 10% are inherited from a person's parents. It is autosomal dominant, meaning that only one affected chromosome is needed for the condition to occur. Diagnosis is suspected based on the symptoms and confirmed by genetic testing. Although there is no cure, treatment can improve symptoms. This often includes a multidisciplinary approach with efforts to improve the function of the potentially many organ systems involved. Long-term outcomes depend on the symptoms present and the severity of the heart and immune system problems. With treatment, life expectancy may be normal. DiGeorge syndrome occurs in about 1 in 4,000 people. The syndrome was first described in 1968 by American physician Angelo DiGeorge. In late 1981, the underlying genetics were determined.

＜安吉洛·馬里奧·狄喬治是美國醫生和小兒內分泌學家. Angelo Mario DiGeorge[1] (April 15, 1921 – October 11, 2009) was an Italian American physician and pediatric endocrinologist.＞

● Dix-Hallpike's test 戴克斯-霍爾派克測驗:

屬於誘發型眼振(induced nystagmus)中的頭位變換性眼振測驗(positioning nystagmus test)的一種。是診斷良性陣發性姿勢性眩暈(benign paroxysmal positional vertigo, BPPV)的常用測驗。The Dix–Hallpike test is a diagnostic maneuver used to identify benign paroxysmal positional vertigo (BPPV). Positive test result: A positive test is indicated by patient report of a reproduction of vertigo and clinician observation of nystagmus (involuntary eye movement). Limitations: The negative predictive value of this test is not 100%. Some patients with a history of BPPV will not have a positive test result. The estimated sensitivity is 79%, along with an estimated specificity of 75%.

＜瑪格麗特·露絲·戴克斯是英國神經耳科醫生. Margaret Ruth Dix (1902 – 9 December 1991) was a British neuro-otologist.＞

＜查爾斯·斯金納·霍爾派克是英國耳科醫生. Charles Skinner Hallpike, CBE, FRS (19 July 1900 – 26 September 1979) was an English otologist.＞

● Down's syndrome 唐氏症 (trisomy 21 三染色體21症)：

可能聽損、眩暈。唐氏症是最常見的染色體異常疾病，多為偶發，並非來自遺傳。由於唐寶寶比一般人多出部分或完整的第21對染色體，這些多出來的遺傳物質，使得唐寶寶具有特殊的外表特徵、身材較矮小、生長發育遲緩及某些先天結構異常(如先天性心臟病)，並影響中樞神經系統，可導致低張力、智能障礙、發展遲緩、痛覺不敏感、癲癇、認知功能減退、失智症及行為問題；患者也常有免疫缺陷及感染症，亦可能有血液疾病 (如暫時性骨髓增生、白血病)、甲狀腺功能異常、腸胃道疾病，及視力、聽力、皮膚、骨骼、肥胖、睡眠、心理及老化方面的問題。A genetic disorder caused by the presence of all or part of a third copy of chromosome 21. It is usually associated with physical growth delays, mild to moderate intellectual disability, and characteristic facial features. The average IQ of a young adult with Down syndrome is 50, equivalent to the mental ability of an 8- or 9-year-old child, but this can vary widely. The parents of the affected individual are usually genetically normal. The probability increases from less than 0.1% in 20-year-old mothers to 3% in those of age 45. The extra chromosome is believed to occur by chance, with no known behavioral activity or environmental factor that changes the probability. Down syndrome can be identified during pregnancy by prenatal screening followed by diagnostic testing or after birth by direct observation and genetic testing. Since the introduction of screening, pregnancies with the diagnosis are often terminated. Regular screening for health problems common in Down syndrome is recommended throughout the person's life. There is no cure for Down syndrome. Education and proper care have been shown to improve quality of life. Some children with Down syndrome are educated in typical school classes, while others require more specialized education. Some individuals with Down syndrome graduate from high school, and a few attend post-secondary education. In adulthood, about 20% in the United States do paid work in some capacity, with many requiring a sheltered work environment.Support in financial and legal matters is often needed. Life expectancy is around 50 to 60 years in the developed world with proper health care. Down syndrome is the most common chromosome abnormality in humans. It occurs in about 1 in 1,000 babies born each year. In 2015, Down syndrome was present in 5.4 million individuals globally and resulted in 27,000 deaths, down from 43,000 deaths in 1990. It is named after British doctor John Langdon Down, who fully described the syndrome in 1866. Some aspects of the condition were described earlier by French psychiatrist Jean-Étienne Dominique Esquirol in 1838 and French physician Édouard Séguin in 1844. The genetic cause of Down syndrome was discovered in 1959.

＜約翰·朗頓·唐是英國醫生. John Langdon Haydon Down (18 November 1828 – 7 October 1896) was a British physician.＞

● Edwards' syndrome 愛德華症 (trisomy 18 三染色體18症)：

會造成聽損。愛德華症即三染色體18症，發生率約為每7,000個活產當中會出現一例，女性患者遠多於男性。主要的臨床表徵為出生體重不足、肌肉張力異常(先是低肌肉張力，而後逐漸發展成肌肉張力過高)、長頭畸形、枕部凸顯、眼瞼縫短小、內眥贅皮、耳朵畸形、耳位低下、小嘴小下巴、部分患兒合併有脣　裂，手指常呈交疊握拳狀、有半數以上的患兒會有先天性心臟病(圖3-9、彩圖一)，其大腳趾短且向上翹起、搖椅底樣腳並且常合併仰趾外翻足、骨盆狹窄、大關節攣縮。大多數患兒在嬰兒期即夭折，少數存活者皆有極重度的智能不足。染色體檢查是唯一可以確認診斷的方法，大多數患兒的父母的細胞核型(染色體)均為正常。A genetic disorder caused by the presence of a third copy of all or part of chromosome 18. Many parts of the body are affected. Babies are often born small and have heart defects. Other features include a small head, small jaw, clenched fists with overlapping fingers, and severe intellectual disability. Most cases of Edwards syndrome occur due to problems during the formation of the reproductive cells or during early development. The rate of disease increases with the mother's age. Rarely, cases may be inherited from a person's parents. Occasionally, not all cells have the extra chromosome, known as mosaic trisomy, and symptoms in these cases may be less severe. An ultrasound during pregnancy can increase suspicion for the condition, which can be confirmed by amniocentesis. Treatment is supportive. After having one child with the condition, the risk of having a second is typically around one percent. It is the second-most common condition due to a third chromosome at birth, after Down syndrome. Edwards syndrome occurs in around 1 in 5,000 live births. Some studies suggest that more babies that survive to birth are female. Many of those affected die before birth. Survival beyond a year of life is around 5–10%. It is named after English geneticist John Hilton Edwards, who first described the syndrome in 1960.

＜愛德華是英國醫學遺傳學家. John Hilton Edwards (26 March 1928 – 11 October 2007) was a British medical geneticist.＞

● Epley's maneuver 埃普利手法：

治療BPPV的的一種耳石復位術。1992 年 Epley 發表耳石復位術 (Canalith repositioning procedure)，即熟知的 Epley Maneuver，徒手快速改變患者之頭部與身體姿勢 270 度，藉由頭部位置的變換與重力的交互作用，來將漂流的耳石從後半規管導入橢圓囊。Repositioning maneuver is a maneuver used by medical professionals to treat one common cause of vertigo, benign paroxysmal positional vertigo (BPPV) of the posterior or anterior canals of the ear. It works by allowing free-floating particles from the affected semicircular canal to be relocated, using gravity, back into the utricle, where they can no longer stimulate the cupula, therefore relieving the patient of bothersome vertigo.[2][3] This maneuver was developed by Dr. John Epley and first described in 1980. A version of the maneuver called the "modified" Epley does not include vibrations of the mastoid process originally indicated by Epley, as they have since been shown not to improve the efficacy of the treatment.

＜埃普利是美國耳科醫生. John M. Epley (1930-) is an American otologist.＞

● Eustachian's tube 歐氏管 (= Auditory tube or pharyngotympanic tube 耳咽管)：

連接鼻咽腔與鼓室腔的管路。A tube that links the nasopharynx to the middle ear. It is a part of the middle ear. In adult humans the Eustachian tube is approximately 35 mm (1.4 in) long and 3 mm (0.12 in) in diameter. It is named after the sixteenth-century Italian anatomist Bartolomeo Eustachi. In humans and other land animals the middle ear (like the ear canal) is normally filled with air. Unlike the open ear canal, however, the air of the middle ear is not in direct contact with the atmosphere outside the body. The Eustachian tube connects from the chamber of the middle ear to the back of the nasopharynx. Normally, the Eustachian tube is collapsed, but it gapes open both with swallowing and with positive pressure. When taking off in an airplane, the surrounding air pressure goes from higher (on the ground) to lower (in the sky). The air in the middle ear expands as the plane gains altitude, and pushes its way into the back of the nose and mouth. On the way down, the volume of air in the middle ear shrinks, and a slight vacuum is produced. Active opening of the Eustachian tube is required to equalize the pressure between the middle ear and the surrounding atmosphere as the plane descends. A diver also experiences this change in pressure, but with greater rates of pressure change; active opening of the Eustachian tube is required more frequently as the diver goes deeper into higher pressure.

＜巴托羅梅奧·埃烏斯塔基恩是文藝復興時期歐洲教皇醫生，是義大利解剖學家. Bartolomeo Eustachi (1500 or 1514 – 27 August 1574) was an Italian anatomist.＞

● Ewald's law埃瓦德三定律：

內耳半規管的生理學。1. 對內耳某一對半規管刺激時，眼振方向(快速相方向)會指向剌激效果大的內淋巴流動。2. 在水平半規管內，比起離壺腹的(ampullofugal)內淋巴流動方向，向壺腹的(ampullopetal)內淋巴流動方向會產生較大的刺激。3. 在垂直的前、後半規管內，產生的結果剛好和第二定律相反。Ewald is best remembered for his research of the vestibular system of the inner ear, which largely involved experiments performed on the semicircular canal system of pigeons. From these studies the so-called "Ewald laws" are derived, which deal with the effects of endolymph motion on body, head and eye movements and also on the phenomena of excitation-inhibition asymmetries in the vestibular system. Ewald's first law: "The axis of nystagmus parallels the anatomic axis of the semicircular canal that generated it". Ewald's second law: "Ampullopetal endolymphatic flow produces a stronger response than ampullofugal flow in the horizontal canal". Ewald's third law: "Ampullofugal flow produces a stronger response than ampullpetal flow in the vertical canals (anterior and posterior semicircular canals).

＜恩斯特·朱利葉斯·理查德·埃瓦德是德國生理學家. Ernst Julius Richard Ewald (14 February 1855 – 22 July 1921) was a German physiologist.＞

● Frenzel's glass 法藍佐眼鏡：

評估眼振用的放大鏡眼鏡，病患戴上無法聚焦凝視，施測者可以清楚看到放大的眼球運動。A diagnostic tool used in ophthalmology, otolaryngology and audiovestibular medicine for the medical evaluation of involuntary eye movement (nystagmus). They are named after Hermann Frenzel, a German otolaryngolotist. The purpose of the goggles is to disable the patient's ability to visually fixate on an object while at the same time allowing the examiner to adequately visualize the eye. This is done by using high-powered (+20 diopters) magnifying glasses with an illumination system. With such a high-powered lens, it is unlikely that the patient can adequately focus and visually fixate on an object to suppress nystagmus.

＜赫曼‧法藍佐是德國耳鼻喉科醫生. Hermann Frenzel (16. May 1895 - December 1967) was a German otolaryngolotist.＞

● Friedreich's ataxia 佛里德里希共濟失調：

一種共濟失調，可能頭暈。一種神經系統進行性損傷的遺傳疾病，它可導致肌無力、言語困難或心臟病等，首發症狀通常為行走困難，許多患此運動失調的患者最終需要使用輪椅。該病以Nikolaus Friedreich醫生的名字命名。他在1860年代首次介紹了此病。運動失調是指協調困難和步態不穩，佛里德里希共濟失調表現為控制上臂和腿運動的神經和脊髓發生了退行性變化。脊髓變得更薄，神經細胞也失去了部分絕緣層(即髓鞘)。這層絕緣層幫助神經傳導衝動。An autosomal recessive genetic disease that causes difficulty walking, a loss of sensation in the arms and legs and impaired speech that worsens over time. Symptoms can start between 5 and 15 years of age. Many develop hypertrophic cardiomyopathy and will require a mobility aid such as a cane, walker or wheelchair in their teens. As the disease progresses, people lose their sight and hearing. Other complications include scoliosis and diabetes mellitus. The condition is caused by mutations in the FXN gene on the chromosome 9. The FXN gene makes a protein called frataxin. Degeneration of nerve tissue in the spinal cord causes the ataxia; particularly affected are the sensory neurons essential for directing muscle movement of the arms and legs through connections with the cerebellum. The spinal cord becomes thinner and nerve cells lose some myelin sheath. The condition is named after the German physician Nikolaus Friedreich, who first described it in the 1860s.

＜尼古拉斯·佛里希是德國病理學家和神經科醫生，也是佛里希家族的第三代醫師. Nikolaus Friedreich was a German pathologist and neurologist, and a third generation physician in the Friedreich family.＞

● Fukuda's stepping test 福田氏踏步測驗：

檢測前庭功能受損的一種踏步測驗。要求患者閉眼的情況下踏步前進50步，患者將旋轉到前庭較弱的那一側。The stepping test is a valuable part of the neurological examination that is used to localize labyrinthine pathology. The test is known by two eponyms: the “Fukuda Test” in the United States and Asia and the “Unterberger Test” in Europe. Some controversy exists as to which name is correct. Siegfried Unterberger was an Austrian otolaryngologist who initially described the test in 1939. It was modified in 1959 by Tadashi Fukuda, a Japanese otolaryngologist, who introduced a method to better quantify the test results. Thus, the test should be called the “Unterberger Test,” unless Fukuda's methods for measurement are used.

＜福田是日本耳鼻喉科醫生. Tadashi Fukuda (1910–1987) was a Japanese otolaryngolotist.＞

● Fukuda's vertical writing test 福田氏垂直書寫測驗 (= Vertical writing with eyes coved, Blindfolded vertical writing test 蒙眼垂直書寫測驗)：

VSR的一種測驗。左手放膝上，開眼，右手寫四個字，閉眼，右手寫四個字。偏斜度大於10度，表示異常。右側聽神經瘤者，敏感度89%，左側聽神經瘤者，敏感度58%。

A new test of vestibulo-spinal reaction is prsented whitch consists of observing deviation in a letters written vertically with eyes covered and with the arm kept free of any contact with the desk, and which is characterized by pure objectivity of its results.

＜福田是日本耳鼻喉科醫生. Tadashi Fukuda (1910–1987) was a Japanese otolaryngolotist.＞

● Gelle's test 蓋利測驗：

骨傳導的一個音叉檢查，透過外耳道正壓造成鼓膜與聽小骨內推且固定，檢測有無聽小骨固著，已經被Tympanometry鼓室圖取代。A test of bone conduction. Examinate the effect of increased air pressure in ear canal. Performed by vibrating fork on the mastoid process. By increasing the pressure in the EAC with a Siegel's speculum, it pushes the TM and ossicles inwards, raises the intra-labyrinthine pressure and causes immobility of basilar membrane and decreased hearing, but no change in hearing when ossicles are fixed. This test is replaced with Tympanogetry.

＜蓋利是法國醫生. Marie Ernst Gellé (1834–1923) was a French physician.＞

● Goldenhar's syndrome 高登哈症：

會造成聽損。又名眼、耳、脊椎發育異常症候群。這是一種臉部發育異常的先天性缺陷，此缺陷通常只侷限在一側的顏面部，其特徵包括：1.macpostoma：不對稱的大嘴，嘴巴開口只向一側的耳朵延伸。2.hypoplasia：臉部肌肉、顴骨及皮膚發育不良。3.小的或不成形的耳朵4.prfauficular：在耳朵前緣與嘴巴開口連線的皮膚上，會出現凹洞或贅肉。5.hemivertebre：單側脊椎發育異常。6.另外、有些病患可能會有眼睛發育異常、耳聾、兔唇、顎裂或合併其他心臟、腎臟或肢體的問題。A rare congenital defect characterized by incomplete development of the ear, nose, soft palate, lip and mandible on usually one side of the body. Common clinical manifestations include limbal dermoids, preauricular skin tags and strabismus. It is associated with anomalous development of the first branchial arch and second branchial arch. The term is sometimes used interchangeably with hemifacial microsomia, although this definition is usually reserved for cases without internal organ and vertebrae disruption. It affects between 1 in 3,500 and 1 in 5,600 live births, with a male-to-female ratio of 3:2.

＜莫里斯·戈登哈爾是美國眼科醫生. Maurice Goldenhar (January 15, 1924 – September 11, 2001) was an American ophthalmologist.＞

● Goodpasture's syndrome 古德巴斯捷症 (= Anti-glomerular basement membrane disease 抗腎小球基底膜抗體病)：

會造成聽損。一種罕見的自體免疫性疾病，其中抗體攻擊肺和腎臟，導致從肺及腎功能衰竭出血。A rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture's antigen. Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with medications that suppress the immune system such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood. The disease was first described by an American pathologist Ernest Goodpasture of Vanderbilt University in 1919 and was later named in his honor.

＜歐內斯特·威廉·古德巴斯捷是美國病理學家和醫生. Ernest William Goodpasture (October 17, 1886 – September 20, 1960) was an American pathologist and physician.＞

● Gowers' syndrome 高爾斯症 (= Situational syncope 情境昏厥, Vaso-inhibitory syncope syndrome 血管抑制性晕厥)：

會造成眩暈、昏厥。血管迷走性暈厥(vasovagal syncope, VVS)是指各種刺激通過迷走神經介導反射，導致內臟和肌肉小血管擴張及心動過緩，表現為動脈低血壓伴有短暫的意識喪失，能自行恢復，而無神經定位體征的壹組綜合征。由於除心臟外，全身其他部位的血管不受迷走神經支配，近年有些學者認為使用“神經心源性暈厥”可能更為恰當。臨床主要表現為反復發作的暈厥。血管迷走性暈厥綜合征於1907年由Gowers描述，因此又稱Gowers症。血管迷走性暈厥多見於學齡期兒童，女孩多於男孩，通常表現為立位或坐位起立時突然發生暈厥，起病前可有短暫的頭暈、註意力不集中、面色蒼白、視、聽覺下降，惡心、嘔吐、大汗、站立不穩等先兆癥狀，嚴重者可有10-20s的先兆。如能警覺此先兆而及時躺下可緩解或消失。初時心跳常加快，血壓尚可維持，以後心跳減慢，血壓漸下降，收縮壓較舒張壓下降明顯，故脈壓差縮小，當收縮壓下降至80mmHg時，可出現意識喪失數秒或數分鐘，少數患者可伴有尿失禁，醒後可有乏力、頭昏等不適，嚴重者醒後可有遺忘、精神恍惚、頭痛等癥狀，持續1-2天癥狀消失。Gower (situational syncope): The temporary loss of consciousness in particular kinds of situations. (Syncope is temporary loss of consciousness or, in plain English, fainting). The situations that trigger this reaction are diverse and include having blood drawn, straining while urinating or defecating or coughing. The reaction also can be due to the emotional stress of fear or pain. With Gower syndrome, people often become pale and feel nauseated, sweaty, and weak just before they lose consciousness. Gower syndrome is caused by a reflex of the involuntary nervous system called the vasovagal reaction. The vasovagal reaction leads the heart to slow down (bradycardia) and, at the same time, it leads the nerves to the blood vessels in the legs to permit those vessels to dilate (widen). The result is that the heart puts out less blood, the blood pressure drops, and what blood is circulating tends to go into the legs rather than to the head. The brain is deprived of oxygen and the fainting episode occurs. A vasovagal reaction is also called a vasovagal attack. Gower syndrome is synonymous with situational syncope, vasovagal syncope, and vasodepressor syncope. Sir William Richard Gower (1845-1915) was a famous English neurologist whose name is also associated with a sign, a solution, another syndrome, and a tract in the central nervous system.

＜威廉·理查德·高爾斯爵士是英國的神經學家. Sir William Richard Gowers FRS (20 March 1845 – 4 May 1915) was a British neurologist.＞

● Gradenigo's syndrome 格拉代尼戈症 (= Gradenigo-Lannois syndrome格拉代尼戈-拉諾斯症, apical petrositis 岩骨尖端發炎)：

一種中耳炎或乳突炎的併發症，岩骨尖端化膿性感染會造成三徵：同側外旋神經麻痺、三叉神經痛、耳流膿。

A complication of otitis media and mastoiditis involving the apex of the petrous temporal bone. It was first described by Giuseppe Gradenigo in 1904. Components of the syndrome include: (1) retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve (fifth cranial nerve), (2) abducens nerve palsy (sixth cranial nerve), (3) otitis media. Other symptoms can include photophobia, excessive lacrimation, fever, and reduced corneal sensitivity. The syndrome is classically caused by the spread of an infection into the petrous apex of the temporal bone.

＜格拉代尼戈是義大利耳科醫生. Giuseppe Gradenigo (1859–1926) was an Italian otologist.＞

● Gufoni's maneuver 古佛尼手法：

水平半規管BPPV的一種代替性手法。Gufoni Maneuver was first described in 1998. For geotropic nystagmus: Patient quickly lies down on the side of the unaffected ear and remains in this position for 1 to 2 minutes, until the evoked nystagmus subsides. The head is then quickly rotated 45 degrees toward the floor and kept in this position for another 2 minutes, after which the patient resumes an upright position. For apogeotropic nystagmus: Patient quickly lies down on the affected side and remains in this position for 1 to 2 minutes after the nystagmus has stopped or has been markedly reduced. Then the head is quickly turned 45 degrees toward the ceiling and is held in this position for 2 minutes, after which the patient slowly resumes the sitting position. For the geotrophic variant: one starts on the unaffected side (side of weaker nystagmus), and then proceeds to 45 degree nose down. For the ageotrophic variant: one starts on the affected side (side of weaker nystagmus), and proceeds to 45 degree nose up.

＜古佛尼是義大利耳鼻喉科醫生. Gufoni M is an Italian otorhinolaryngologist.＞

● Guillain-Barre syndrome (GBS) 吉蘭-巴利症：

可能眩暈。急性發炎性脫髓鞘多神經病變 (Acute inflammatory demyelinating polyneuropathy, AIDP)的一種亞型。一種急性、單一病程、侵犯運動、感覺及自主神經系統，並且以發炎及脫髓鞘為主要病理變化的疾病。A rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. The initial symptoms are typically changes in sensation or pain along with muscle weakness, beginning in the feet and hands, often spreading to the arms and upper body, with both sides being involved. The symptoms may develop over hours to a few weeks. During the acute phase, the disorder can be life-threatening, with about 15 percent of people developing weakness of the breathing muscles and, therefore, requiring mechanical ventilation. Some are affected by changes in the function of the autonomic nervous system, which can lead to dangerous abnormalities in heart rate and blood pressure.

＜喬治·查爾斯·吉蘭是法國神經科醫生.Georges Charles Guillain (3 March 1876 – 29 June 1961) was a French neurologist.＞

＜巴利是法國神經科醫生. Jean Alexandre Barré (25 May 1880, Nantes – 26 April 1967, Strasbourg) was a French neurologist.＞

● Halmagyi head thrust test (HTT) 哈瑪吉頭脈衝測試 (= Head impulse test, HIT 頭脈衝測驗)：

半規管的測驗方法。Micheal Halmagyi and Ian Curthoys described this simple and reliable bedside test that could be used for detecting persons with unilateral peripheral vestibular deficits in 1988. It is also known as the Head Thrust Test. Purpose: This test works by testing the Vestibulo Ocular Reflex (VOR). This reflex is helpful to maintain corrective eye position during any change in head position and to correct the eye movement rapidly so that vision remains on the target. Advantages of the test: - Relatively quick. - Can be used even on patients with acute Vertigo. - Can be repeated within a short time. Technique: Position of the Tester: Sitting. Position of the Subject: Sitting in front of the tester with eyes fixed on the examiner's nose or a distant target. Alternatively testing position: the tester can stand or sit behind the subject, but there needs to be a way to record the eye movement. Precautions: The tester must ensure that the subject doesnot have any neck issues like Vertebro basilar insufficiency and neck range of motion is adequate. Expectation of the subject: The subject needs to keep their eyes focussed on the target during the testing procedure and avoid premature eye closure. Examiner action: The examiner moves the head quickly and unpredictably to 10 to 15 degrees of neck rotation[3], care needs to be ensured to avoid Cervical spine manipulation during the testing. Normal response: Eyes remain on the target after the examiners movement. Abnormal response: Eyes are dragged off the target by the turning of the head, followed by a corrective saccade back to the target after the turning of head.

＜哈瑪吉是澳洲神經科醫生. Gabor Michael Halmagyi (1947-) is an Australian neurologist.＞

● Hand-Schuller-Christian (HSC) disease 漢德-敘勒-克莉絲汀病：

會造成聽損，可能眩暈。一種中耳與顳骨腫瘤，屬於蘭格罕細胞組織球增生症(Langerhans cell histiocytosis)，會造成頭殼破個洞。Associated with multifocal Langerhans cell histiocytosis.　It is associated with a triad of exophthalmos, lytic bone lesions (often in the skull), and diabetes insipidus (from pituitary stalk infiltration). It is named for the American pediatrician Alfred Hand Jr., the Austrian neurologist and radiologist Arthur Schüller, and the American internist Henry Asbury Christian, who described it in 1893, 1915/16 and 1919.
＜漢德是美國兒科醫生. Alfred Hand Jr was an American pediatrician.＞
＜敘勒是奧地利神經科與放射科醫生. Arthur Schüller was an Austrian neurologist and radiologist.＞
＜克莉絲汀是美國內科醫生. Henry Asbury Christian was an American internist.＞

● Heerfordt's Syndrome 海爾福德症 (= Uveoparotid fever 葡萄膜腮腺熱)：

會造成眩暈。一種類肉瘤病。A rare manifestation of sarcoidosis. The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever, and in some cases, palsy of the facial nerves.

＜海爾福德是丹麥眼科醫生. Christian Frederick Heerfordt (1871-1953) was a Danish ophthalmologist.＞

● Hennebert's sign 赫內伯體徵:

一種瘻管(fistula)測驗的結果，甚至可以與視頻眼振圖(VNG)一併測驗。內耳外淋巴液瘻管(fistula)的一種體徵，外耳道壓力改變會誘發眩暈、頭暈、噁心或眼振。在上半規管裂損症(superior semicircular canal dehiscence syndrome, SSCD syndrome)、外淋巴瘻管(perilymph fistula)中會發生，在先天性梅毒(congenital syphilis)、美尼爾病(Meniere's disease)(一種內淋巴水腫)也可能出現陽性。The Hennebert sign describes a positive fistula test without clinical evidence of middle ear or mastoid disease. It is associated with congenital syphilis and may also be present in Ménière disease. It has been postulated that the vestibular stimulation is mediated by fibrous bands between footplate of stapes and the vestibular membranous labyrinth. Another explanation is that the vestibular response is due to an excessively mobile footplate. Nystagmus associated with Hennebert sign is usually more marked when negative pressure is applied. Camille Hennebert (1867–1954), was a Belgian otolaryngologist who originally noted the sign in cases of congenital syphilis.

＜卡米爾‧赫內伯是比利時耳鼻喉科醫生. Camille Hennebert (1867-1954) was a Belgian otolaryngologist.＞

● Hennebert's syndrome 赫內伯症 (= Congenical syphilic otogenic nystagmus 先天性梅毒性耳源性眼振)：

會造成眩暈。兒童先天性梅毒眼振。首先由比利時醫生Hennebert (1905)介紹，故以其姓命名此病，是鼓膜完整情況下用鼓氣耳鏡壓縮或減壓時出現眩暈及眼震，即“ 臨床表現為眩暈及前庭性眼震，迷路瘻管試驗陽性，康瓦反應陽性。後來有人發現這一綜合徵出現於美尼爾病，卡他性和滲出性中耳炎，耳硬化症，粘連性中耳炎，甚至中耳正常者亦有此徵，臨床上遇到鼓膜完整之Hennebert綜合症發病機制不清者，應追查病因採取適當的治療措施。若聽力正常可無需治療。The phenomenon of spontaneous nystagmus, of otitic origin, in children the subjects of hereditary syphilis, is one that is well known to the continental otologists. It is constantly referred to by them in their writings and lectures, but does not appear in the English literature devoted to paediatrics, otology or syphilology. Hen.nebertl drew attention to the syndrome, and it goes by the aponym 'Hennebert's syndrome,' or ' fistel symptom ohne fistel ' (fistula symptom, without fistula). Alexander3 and Ruttin have subsequently recorded their observations.

＜卡米爾‧赫內伯是比利時耳鼻喉科醫生. Camille Hennebert (1867-1954) was a Belgian otolaryngologist.＞

● Hitselberger's sign 希索伯格體徵：

聽神經瘤的表現，當侵犯到第七對腦神經時，外聽道後側的皮膚會感覺遲鈍或喪失。In the Hitselberger test the patient has to estimate the magnitude of the tactile part of a compound audio-tactile stimulus. From this point of view it is doubtful, that the sign is a simple peripheral phenomenon based on the impairment of the facial nerve by tumor pressure. To investigate the process of audiotactile perception we developed a device which delivers a calibrated compound audio-tactile stimulus. In the tested 70 patients with different sensorineural lesions there was no significant difference between the percentage values of positive Hitselberger signs in the various diagnostic groups. Evaluation of the different factors influencing the Hitselberger test results in a new concept of audiotactile perception.

＜希索伯格是美國神經外科醫生. William E. Hitselberger (1930-2014) was an American neurosurgeon.＞

● Horner's syndrome 霍納症 (= Oculosympathetic paresis 眼交感神經麻痺症)：

霍納症的症狀包括縮瞳、眼球陷沒、瞬膜脫出及眼瞼下垂，是因交感神經對眼睛及附屬器的神經控制喪失所致，交感神經的神經控制主要負責眼窩及眼瞼平滑肌張力的維持，也包括瞬模(第三眼瞼)在內，交感神經也藉由控制虹膜開大肌來對抗副交感神經所控制的虹膜縮肌，任何沿著交感神經路徑出現的病兆都有可能會引發霍納症。A combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhydrosis (decreased sweating), with apparent enophthalmos (inset eyeball). The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a division of the autonomic (or involuntary) nervous system. Once the syndrome has been recognized, medical imaging and response to particular eye drops may be required to identify the location of the problem and the underlying cause. Signs that are found in people with Horner's syndrome on the affected side of the face include the following: 1.ptosis (drooping of the upper eyelid), 2.anhidrosis (decreased sweating), 3.miosis (constriction of the pupil), 4.sinking of the eyeball into the face, 5.inability to completely close or open the eyelid, 6.facial flushing, 7.headaches, 8.loss of ciliospinal reflex, 9.bloodshot conjunctiva, depending on the site of lesion, 10.unilateral straight hair (in congenital Horner's syndrome); the hair on the affected side may be straight in some cases, 11.heterochromia iridum (in congenital Horner's syndrome).

＜約翰·佛里德里希·霍納是瑞士眼科醫生. Johann Friedrich Horner (27 March 1831 – 20 December 1886) was a Switzerland ophthalmologist.＞

● Hutchinson's triad 哈傾森三聯徵：

會造成聽損。先天性梅毒的晚期表現。A common pattern of presentation for congenital syphilis, and consists of three phenomena: interstitial keratitis, malformed teeth (Hutchinson incisors and mulberry molars), and eighth nerve deafness. There may also be a deformity on the nose known as saddle nose deformity.

＜哈傾森是英國外科醫生. Sir Jonathan Hutchinson (23 July 1828 – 23 June 1913) was an English surgeon.＞

● JLN (Jervell-Lange-Nielsen) syndrome JLN症 (= Congenital QT prolong syndrome, 先天性QT延長症)：

會造成聽損。可能突然昏厥、猝死。Jervell and Lange-Nielsen syndrome is caused by mutations in the KCNE1 and KCNQ1 genes. A type of long QT syndrome associated with severe, bilateral sensorineural hearing loss. Long QT syndrome causes the cardiac muscle to take longer than usual to recharge between beats. If untreated, the irregular heartbeats, called arrhythmias, can lead to fainting, seizures, or sudden death. It was first described by Anton Jervell and Fred Lange-Nielsen in 1957. The symptoms of this condition are: Congenital deafness, Syncope, Seizures, Palpitations, Sudden cardiac death. The clinical signs are: Prolonged QTc on the ECG, Bilateral sensorineural deafness.

＜安東·傑維爾是挪威醫生. Anton Jervell (June 1901 – December 1987) was a Norwegian physician.＞

＜弗雷德·朗格·尼爾森是挪威醫生. Fred Lange-Nielsen (September 1919 – December 1989) was a Norwegian doctor and jazz musician (bass, vocals).＞

● Kartagener's syndrome 卡塔格症 (= Primary ciliary dyskinesias, PCD 原發性纖毛運動不全症)：

可能聽損。一種罕見的遺傳疾病，有三聯徵：內臟轉位、慢性鼻竇炎(鼻息肉)、支氣管擴張。Kartagener syndrome is characterized by the clinical triad of : (1) situs inversus, (2) chronic sinusitis and/or nasal polyposis, (3) bronchiectasis. A rare, ciliopathic, autosomal recessive genetic disorder that causes defects in the action of cilia lining the respiratory tract (lower and upper, sinuses, Eustachian tube, middle ear), fallopian tube, and flagellum of sperm cells. The phrase "immotile ciliary syndrome" is no longer favored as the cilia do have movement, but are merely inefficient or unsynchronized. Respiratory epithelial motile cilia, which resemble microscopic "hairs" (although structurally and biologically unrelated to hair), are complex organelles that beat synchronously in the respiratory tract, moving mucus toward the throat. Normally, cilia beat 7 to 22 times per second, and any impairment can result in poor mucociliary clearance, with subsequent upper and lower respiratory infection. Cilia also are involved in other biological processes (such as nitric oxide production), currently the subject of dozens of research efforts.

＜卡塔格是瑞士內科醫生. Manes Kartagener (1897 - 1975) was a Swiss internist.＞

● Kearns-Sayre syndrome (KSS) 科恩-賽莉症 (= Oculocraniosomatic disorder, Oculocranionsomatic neuromuscular disorder with ragged red fibers 眼腦神經肌肉症合併殘紅纖維)：

會造成聽損。一種粒線體疾病，最早是在1958年，由Kearns及Sayre所提出。多數為偶發(sporadic)案例，大多數的病患是因粒線體DNA上發生單一大片段缺失(deletion)，或是粒線體MTTL1基因(mitochondrial tRNA (leucine)-1 gene)發生突變(mutation)所導致而成。粒線體DNA缺失症候群大致上可分為三種類型：一、Pearson syndrome：此型最為嚴重，幾乎大部分的組織中都可發現粒線體基因的缺失，患者常合併有全部血球減少(pancytopenia)、貧血、胰臟衰竭(exocrine pancreatic failure)。二、Kearns-Sayre Syndrome：受影響的範圍較小，僅在某些組織中找到缺失的粒線體基因，這些缺失大多位於肌肉和中樞神經系統。患者最常見的症狀即為外眼肌麻痺及眼底色素沉積。三、慢性漸進性外眼肌麻痺(Chronic progressive external ophthalmoplegia; CPEO)：此疾病受影響的範圍僅限於肌肉組織，所造成的症狀多為外眼肌麻痺、雙側眼瞼下垂，及輕微的近側肌肉病變(mild proximal myopathy)。KSS患者通常在幼年(＜20歲)即因眼外肌麻痺及視網膜色素變性，而影響到視力，並常合併有多個系統；包括：中樞神經、心臟及內分泌等系統的異常。由於一開始的症狀表現並不明顯，患者常因此延誤就醫及診斷。A mitochondrial myopathy with a typical onset before 20 years of age. KSS is a more severe syndromic variant of chronic progressive external ophthalmoplegia (abbreviated CPEO), a syndrome that is characterized by isolated involvement of the muscles controlling movement of the eyelid (levator palpebrae, orbicularis oculi) and eye (extra-ocular muscles). This results in ptosis and ophthalmoplegia respectively. KSS involves a combination of the already described CPEO as well as pigmentary retinopathy in both eyes and cardiac conduction abnormalities. Other symptoms may include cerebellar ataxia, proximal muscle weakness, deafness, diabetes mellitus, growth hormone deficiency, hypoparathyroidism, and other endocrinopathies. In both of these diseases, muscle involvement may begin unilaterally but always develops into a bilateral deficit, and the course is progressive. This discussion is limited specifically to the more severe and systemically involved variant.

＜科恩是美國神經眼科醫生. Thomas P. Kearns (1922 - died) was an American neuroophthalmologist.＞

＜賽莉是美國眼科醫生. George Pomeroy Sayre (1911- died) was an American ophthalmologist.＞

● Klippel-Feil (KF) syndrome KF症 (= Cervical vertebral fusion syndrome 先天性頸椎融合症)：

可能眩暈。先天性頸椎融合畸形也稱頸椎分節不良，此病於1912年最初由Klippel和Feil報導，故稱為Klippel-Feil綜合徵。為兩個或兩個以上頸椎融合性畸形，表現為頸椎間裂減少，頸項縮短，頭部位移運動，並常伴有其他部位的畸形，少數患者可伴有神經系統障礙。A rare congenital condition characterized by the abnormal fusion of any two of the seven bones in the neck (cervical vertebrae). It results in a limited ability to move the neck and shortness of the neck, resulting in the appearance of a low hairline. The syndrome is difficult to diagnose, as it occurs in a group of patients affected with many different abnormalities who can only be unified by the presence of fused or segmental cervical vertebrae. KFS is not always genetic and not always known the day of the birth. The disease was initially reported in 1884 by Maurice Klippel and André Feil from France. In 1919, in his Doctor of Philosophy thesis, André Feil suggested another classification of the syndrome, encompassing not only deformation of the cervical spine, but also deformation of the lumbar and thoracic spine.

＜莫里斯·克利普是法國醫生. Maurice Klippel (30 May 1858 – 20 July 1942) was a French physician.＞

＜費爾是法國醫生. André Feil (1884–1955) was a French physician.＞

● Koerner's septum 科納間隔 (= Petro squamosal lamina 岩骨鱗狀間隔)：

是位於乳突(mastoid)內鱗部小房(squamous air cell)和岩部小房(petrous air cell)之間的薄骨板。An anatomic boundary in the temporal bone formed by the petrosquamous suture between the petrous and squamosal portions of the mastoid air cells, at the anatomic level of the antrum. Along with the middle ear ossicles, it is usually eroded in middle ear cholesteatomas. Superiorly, this continues as the petrosquamous suture, a normal anatomic structure that can be mistaken for fractures on temporal bone CT. The Koerner septum is a thin bridge of bone which divides the petrous and squamous portion of the mastoid air cells at the level of the mastoid antrum. It is commonly eroded by middle ear cholesteatomas. Koerner's septum is an important anatomic handicap predisposing the individual to chronic otitis media, particularly when it is characterized by attic retraction pockets and cholesteatoma, and adhesive otitis media, because Koerner's septum contributes to attic blockage. This statement is in accordance with the original articles written by Cheatle (1910, 1923) and Williams (1966), and recently published data related to supratubal recess and the cog.

● Korsakoff's syndrome 高沙可夫症：

可能眩暈。 Wernicke’s encephalopathy未治療造成。高沙可夫症，又稱健忘綜合徵，為一種大腦缺乏硫胺(維生素B1)而引起的精神障礙。其疾病由高血壓神經病學家塞吉·高沙可夫最先發現而命名。高沙可夫症表現為選擇性的認知功能障礙，包括近事遺忘，時間及空間定向障礙。An amnestic disorder caused by thiamine (vitamin B1) deficiency associated with prolonged ingestion of alcohol. There is a similar condition seen in non-alcoholic Korsakoff syndrome. The syndrome and psychosis are named after Sergei Korsakoff, the Russian neuropsychiatrist who discovered it during the late 19th century. This neurological disorder is caused by a lack of thiamine in the brain, and is also exacerbated by the neurotoxic effects of alcohol. When Wernicke encephalopathy accompanies alcoholic Korsakoff syndrome the combination is called Wernicke–Korsakoff syndrome; however, a recognized episode of Wernicke encephalopathy is not always obvious.

＜謝爾蓋·謝爾蓋耶維奇·高沙可夫，俄國神經精神學家. Sergei Sergeievich Korsakoff (January 22, 1854 – May 1, 1900) was a Russian neuropsychiatrist.＞

● Laurence-Moon-Biedl-Bardet's (LMBBS) syndrome 勞-蒙-畢-巴症：

會造成聽損。又稱性幼稚-多指畸形症，是一種遺傳性疾病，患者出生後會有肥胖、多指、視網膜萎縮、性腺發育不全、腎臟畸形及學習困難等問題。但除視網膜外，大部分症狀都具有高度的異質性，即便在同一家族，患者臨床表現的差異也極為明顯。A ciliopathic human genetic disorder that produces many effects and affects many body systems. It is characterized principally by obesity, retinitis pigmentosa, polydactyly, hypogonadism, and kidney failure in some cases. Historically, slower mental processing has also been considered a principal symptom but is now not regarded as such.

＜勞倫斯是英國眼科醫生. John Zachariah Laurence (1829 – 18 July 1870) was an English ophthalmologist.＞

＜蒙是英國眼科醫生. Robert Charles Moon (1844–1914) was an English ophthalmologist.＞

＜畢多是匈牙利病理學家. Arthur Biedl (4 October 1869 – 26 August 1933) was a Hungarian pathologist.＞

＜巴德是法國醫生. Georges Louis Bardet (1885–1966) was a French physician.＞　

● Lempert's endaural incision 蘭伯特切開法：

顳骨手術的一種經耳內皮膚切開法。A technique which provides a pedicle skin graft for the lining of the mastoid cavity in surgery of the temporal bone.

＜茱莉‧蘭伯特是美國耳科醫生. Julius Lempert (1891–1968) was an American Otolotist.＞

● Lermoyez's syndrome 拉莫葉滋症：

會造成眩暈。美尼爾病的變異型，和美尼爾病一樣都是內耳內淋巴水腫造成，但比較良性，不太會殘留困擾的耳鳴。在眩暈發作前會先出現耳鳴症狀，然後突然出現眩暈症狀，眩暈出現後耳鳴就消失了，可能是因為耳蝸內淋巴壓力獲得了釋放。Lermoyez's syndrome is usually regarded as a rare variant of Meniere's disease which is characterized by the disappearance of aural symptoms after vertiginous attacks. It is recognized as an uncommon paroxysmal clinical syndrome caused by pathological endolymphatic change in the labyrinth.

＜馬塞爾·拉莫耶滋是法國耳鼻喉科醫生和外科醫生. Marcel Lermoyez (24 July 1858 – 1 February 1929) was a French otolaryngologist and surgeon.＞

● Letterer–Siwe (LS) disease 李特勒-西韋病：

會造成聽損，可能眩暈。一種中耳與顳骨腫瘤，屬於蘭格罕細胞組織球增生症(Langerhans cell histiocytosis)，會造成頭殼破個洞，預後最差。One of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH). It causes approximately 10% of LCH disease and is the most severe form. Prevalence is estimated at 1:500,000 and the disease almost exclusively occurs in children less than three years old. The name is derived from the names of Erich Letterer and Sture Siwe.

＜李特勒是德國病理科醫生. Erich Letterer (30. Juni 1895 - 26. Mai 1982) war a Germany pathologist.＞

＜西韋是瑞典兒科醫生. Sture August Siwe (23 januari 1897 - 24 april 1966) was a Swedish pediatrician.＞

● Listing’s law 李斯廷定律：

眼球運動的基本原理。Named after German mathematician Johann Benedict Listing (1808–1882), describes the three-dimensional orientation of the eye and its axes of rotation. Listing's law has been shown to hold when the head is stationary and upright and gaze is directed toward far targets, i.e., when the eyes are either fixating, making saccades, or pursuing moving visual targets. Listing's law (often abbreviated L1) has been generalized to yield the binocular extension of Listing's law (often abbreviated L2) which also covers vergence.

＜约翰‧班尼迪克‧李斯廷是德國數學家. Johann Benedict Listing (25 July 1808 – 24 December 1882) was a German mathematician.＞

● Luscher test and Sisi- oder：

一種聽力檢查。The diagnosis of stapedial ankylosis.

SISI試驗（短增量敏感指數）是一個聽力的方法，耳，鼻，咽喉藥，檢查用的小體積波動的知名度。由於音量波動超過聽力閾值，因此SISI測試是閾值聽力測試的一部分。該測試可以得出關於招聘的存在的結論，從而得出關於聽力損失情況下損害的位置的結論。該測試基於Lüscher測試與正常聽力相比，在耳蝸失聰的情況下（相對於聽力閾值）小強度波動的可識別性提高。與Lüscher檢驗相比，該檢驗是一種方法上的簡化，大大簡化了被檢查者和檢查者的測試過程。該測試由James Jerger及其同事在1959年開發。

Lüscher的測試是一種聽力方法的的耳，鼻，喉，檢查用的小體積變化可檢測性。該測試可以得出關於招聘的存在的結論，從而得出關於聽力損失情況下損害的位置的結論。該測試由位於巴塞爾的ErhardLüscher和Josef Zwislocki開發，並於1948年進行了描述。該測試後來經過修改和簡化，並以SISI測試的形式廣泛使用。

● Lyme disease 萊姆病 (= Lyme borreliosis 萊姆疏螺旋體病)：

可能眩暈。萊姆病(Lyme Disease)是由伯氏疏螺旋體(Borrelia burgdorferi)感染的蜱(tick，俗稱壁蝨)叮咬而傳播的人畜共通傳染病，多發生於哺乳類動物，包括人、犬、貓、牛及馬等。臨床症狀包括在皮膚組織、循環系統、神經系統及肌肉骨骼系統出現的異常症狀。感染初期症狀類似感冒，會有頭痛、發燒、全身疲勞、寒顫、噁心、嘔吐、頭部僵硬、肌肉疼痛及淋巴腺腫脹等症狀。在被蜱叮咬後 3～30 日(平均約 7 日)出現遊走性紅斑(erythema migrans)，其特徵為紅斑性環狀丘疹且中央泛白、局部灼熱，通常無痛感，此紅斑會逐漸擴散而成牛眼狀，約有 70%至 80%的病患會出現此種病徵。遊走性紅斑患者若未經適當治療，皮疹多在 3～4 週後消退，而在數週或數月後可能引發心臟或神經系統的症狀，以及骨骼方面的症狀(如：關節炎)。一般說來，關節炎在美國較常見，而歐洲則是神經性的合併症較多見。若再經過數月或數年後，病患的主要症狀包括關節變形、關節痛及關節炎，數年後可復發；神經方面之症狀有腦膜炎(發燒、頸部僵硬及劇烈頭痛)、顏面神經麻痺(通常為單側)及神節根痛；心臟方面則有傳導缺損及心包膜炎。
An infectious disease caused by the Borrelia bacterium which is spread by ticks. The most common sign of infection is an expanding area of redness on the skin, known as erythema migrans, that appears at the site of the tick bite about a week after it occurred. The rash is typically neither itchy nor painful. Approximately 70–80% of infected people develop a rash. Other early symptoms may include fever, headache and tiredness. If untreated, symptoms may include loss of the ability to move one or both sides of the face, joint pains, severe headaches with neck stiffness, or heart palpitations, among others. Months to years later, repeated episodes of joint pain and swelling may occur. Occasionally, people develop shooting pains or tingling in their arms and legs. Despite appropriate treatment, about 10 to 20% of people develop joint pains, memory problems, and tiredness for at least six months. Lyme disease was diagnosed as a separate condition for the first time in 1975 in Old Lyme, Connecticut.

＜舊萊姆是一個位於美國康乃狄克州新倫敦郡的城鎮. Old Lyme is a coastal town in New London County, Connecticut, United States.＞

● MacEwen's triangle 麥克尤恩三角 (= Suprameatal triangle 外耳道上三角)：

乳突切除術的動要標誌。兩端顴骨根後下緣與外耳道口後上緣之間的三角區。相當於鼓竇之外頂部。In the temporal bone, between the posterior wall of the external acoustic meatus and the posterior root of the zygomatic process is the area called the suprameatal triangle, suprameatal pit, mastoid fossa, foveola suprameatica, or Mc Ewan's triangle, through which an instrument may be pushed into the mastoid antrum. In the adult, the antrum lies approximately 1.5 to 2 cm deep to the suprameatal triangle. This is an important landmark when performing a cortical mastoidectomy. The triangle lies deep to the cymba conchae.

＜威廉·麥克尤恩爵士是蘇格蘭外科醫生. Sir William Macewen, CB, FRS, FRCS (/məˈkjuːɪn/; 22 June 1848 – 22 March 1924) was a Scottish surgeon.＞

● Marfan's syndrome 馬凡症：

可能眩暈。造成前庭導水管異常。馬凡症的病患，往往給人第一眼的印象便是又高又瘦。古時的劉備(傳言其雙手過膝)，及高瘦的美國總統林肯均被懷疑為馬凡症候群之病患。除了身材的特徵外，深度的近視，眼內水晶體脫垂，甚至造成視網膜剝離而雙眼失明亦為常見之症狀。然而，其最嚴重之併發症為心臟的升主動脈(Aorta ascendens)剝離破裂，而造成病人的瘁死；因其為體顯性遺傳，故此病往往在某些家族成為揮不去的夢靨。A genetic disorder of the connective tissue. The degree to which people are affected varies. People with Marfan tend to be tall and thin, with long arms, legs, fingers and toes. They also typically have flexible joints and scoliosis. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. Other commonly affected areas include the lungs, eyes, bones and the covering of the spinal cord. MFS is caused by a mutation in FBN1, one of the genes that makes fibrillin, which results in abnormal connective tissue. It is an autosomal dominant disorder. About 75% of the time, the condition is inherited from a parent, while 25% of the time it is a new mutation. Diagnosis is often based on the Ghent criteria.

＜安托萬·伯納德·讓·馬凡是法國兒科醫生. Antoine Bernard-Jean Marfan (June 23, 1858 – February 11, 1942) was a French pediatrician.＞

● Melnick-Fraser's syndrome 梅爾尼克-費拉雪症 (= BOR (Branchio-Oto-Renal) Syndrome 鰓耳腎發育不良症)：

會造成聽損。為一體染色體顯性遺傳基因且有高的穿透力與具有多變的表現型，突變基因已被發現是位於第八對染色體的長臂上。特徵表現十分多變，有鰓裂、耳、腎臟、其他等畸形，有些患者很可能只有其中一項或兩項特徵而已。An autosomal dominant genetic disorder involving the kidneys, ears, and neck. It often has also been described as Melnick-Fraser syndrome. The symptoms and/or signs of branchio-oto-renal syndrome are consistent with underdeveloped (hypoplastic) or absent kidneys with resultant chronic kidney disease or kidney failure. Ear anomalies include extra openings in front of the ears, extra pieces of skin in front of the ears (preauricular tags), or further malformation or absence of the outer ear (pinna). Malformation or absence of the middle ear is also possible, individuals can have mild to profound hearing loss. People with BOR may also have cysts or fistulae along the sides of their neck. In some individuals and families, renal features are completely absent. The disease may then be termed Branchio-oto Syndrome (BO syndrome).

＜費拉雪是加拿大醫學遺傳學家. Frank Clarke Fraser (1920 - died) was a Canadian medical geneticist.＞

● Meniere's disease 美尼爾病：

常見的周邊性眩暈症。美尼爾病又叫作耳水不平衡，侵犯內耳的迷路(耳蝸與前庭)，與內淋巴水腫有關，是一種周邊前庭型的陣發性眩暈疾病，經常伴隨波動性聽覺症狀(感音性聽損、耳鳴、耳悶脹)，症狀可能反覆發作，疾病可能不斷進展直至耳蝸前庭無功能，甚至進展至雙耳。A disorder of the inner ear that is characterized by episodes of feeling like the world is spinning (vertigo), ringing in the ears (tinnitus), hearing loss, and a fullness in the ear. Typically, only one ear is affected initially; however, over time both ears may become involved. Episodes generally last from 20 minutes to a few hours. The time between episodes varies. The hearing loss and ringing in the ears can become constant over time.The cause of Ménière's disease is unclear but likely involves both genetic and environmental factors. A number of theories exist for why it occurs including constrictions in blood vessels, viral infections, and autoimmune reactions. About 10% of cases run in families. Symptoms are believed to occur as the result of increased fluid build up in the labyrinth of the inner ear. Diagnosis is based on the symptoms and, frequently, a hearing test. Other conditions that may produce similar symptoms include vestibular migraine and transient ischemic attack. Ménière's disease was first identified in 1861 by Prosper Ménière.

＜柏斯貝·美尼爾是法國耳科醫生，巴黎聾啞研究所所長. Prosper Menière (18 June 1799 – 7 February 1862) was a French doctor who first identified that the inner ear could be the source of a condition combining vertigo, hearing loss and tinnitus, which is now known as Ménière's disease.＞

● Meniere's syndrome 美尼爾症：

歷史上，在美尼爾病的研究上，曾經一度將美尼爾症(syndrome)定義為具有這些典型臨床表現，但是沒有內淋巴水腫的證實；將美尼爾病(disease)定義為具有這些典型臨床表現，且有內淋巴水腫的證實。然而，美尼爾症這樣的定義已經不再使用。

● Michel's aplasia 蜜雪兒未發育 (= Complete labyrinthine aplasia, CLA 迷路完全未發育)：

會造成聽損，可能眩暈。內耳異常可分為五種：1) 蜜雪兒未發育(迷路完全未發育)；2)蒙迪尼異常(不完全劃分)；3) 耳蝸未發育；4)耳蝸發育不良；5) 共同腔。據信蜜雪兒未發育是胚胎第三週時內耳發育障礙所致，此類病患出生即無聽覺，且常合併內聽道異常——內聽道內僅有顏面神經而無耳蝸前庭神經，因此不適合裝置人工電子耳。Inner ear anomalies can be classified into ﬁve (5) groups: 1) Michel aplasia (complete labyrinth aplasia); 2) Mondini anomaly (incomplete division); 3) Cochlear aplasia; 4) Cochlear hypoplasia; 5) Common cavity. A congenital abnormality of the inner ear. It is characterized by the bilateral absence of differentiated inner ear structures and results in complete deafness (anacusis). Michel aplasia should not be confused with michel dysplasia. It may affect one or both ears. Aplasia is the medical term for body parts that are absent or do not develop properly. In Michel aplasia, the undeveloped (anaplastic) body part is the bony labyrinth of the inner ear. Other nearby structures may be underdeveloped as well.

● Miller Fisher's syndrome 米勒費雪症 (= Fisher's syndrome 費雪症)：

可能頭暈。米勒費雪症是吉巴症(GBS)的一個變型(variant)，並不常見。此症以1956年首次報告個案的C. Miller Fisher醫生命名，亦稱為費雪症(Fisher’s syndrome)。此症有三大病徵：1.共濟失調(ataxia)：患者的肌肉協調異常，影響平衡，以致步履笨拙。2.眼肌癱瘓(ophthalmoplegia)：造成視物重影或模糊。3.神經反射消失(areflexia)。米勒費雪症及畢氏腦幹腦炎(Bickerstaff brainstem encephalitis)都屬於Guillian-Barre syndrome的鑑別診斷之一，是一種常見在感染後發生的神經根發炎及腦幹發炎的疾病。相較於米勒費雪症，畢氏腦炎較為少見且較為嚴重。兩者都會出現運動失調(ataxia)，眼外肌麻痺(ophthalmoplegia)等症狀。Miller Fisher syndrome is a rare, acquired nerve disease that is considered to be a variant of Guillain-Barré syndrome. It is characterized by abnormal muscle coordination, paralysis of the eye muscles, and absence of the tendon reflexes. Like Guillain-Barré syndrome, symptoms may be preceded by a viral illness. Additional symptoms include generalized muscle weakness and respiratory failure. The majority of individuals with Miller Fisher syndrome have a unique antibody that characterizes the disorder.

＜查爾斯·米勒·費雪是加拿大神經科醫生. Charles Miller Fisher (December 5, 1913 – April 14, 2012) was a Canadian neurologist.＞

● Mobius's syndrome 莫比烏斯症：

可能聽損、眩暈。Moebius症候群於西元1888年首次被神經科醫師保羅•朱利葉斯•莫比烏斯發現,是一種極為罕見的先天性神經系統疾病。它主要會影響患者第6和第7對腦神經，使得患者無法具有臉部動作(包括了不能微笑，皺眉，吸吮，做鬼臉或眨眼睛），也無法橫向地移動眼睛；除此之外，Moebius症候群也可能影響患者其他對跟語言、咀嚼跟吞嚥有關的腦神經，這些腦神經的異常造成患者面部肌肉無力或麻痺。患者的骨骼可能會受到影響，進而造成手、腳功能異常或畸形足。患者有可能會有呼吸困難，言語和吞嚥障礙，視力障礙，感覺統合失調，睡眠障礙，上肢無力等臨床表徵。Moebius症候群患者擁有正常的智力。

A rare congenital neurological disorder which is characterized by facial paralysis and the inability to move the eyes from side to side. Most people with Möbius syndrome are born with complete facial paralysis and cannot close their eyes or form facial expressions. Limb and chest wall abnormalities sometimes occur with the syndrome. People with Möbius syndrome have normal intelligence, although their lack of facial expression is sometimes incorrectly taken to be due to dullness or unfriendliness. It is named for Paul Julius Möbius, a German neurologist who first described the syndrome in 1888.

＜保羅·朱利葉斯·莫比烏斯是德國神經科醫生. Paul Julius Möbius (January 24, 1853 – January 8, 1907) was a German neurologist.＞

● Mondini's dysplasia 蒙迪尼發育不良 (= Mondini malformation 蒙迪尼畸形, Mondini defect 蒙迪尼缺陷)：

會造成聽損，可能眩暈。內耳異常可分為五種：1) 蜜雪兒未發育(迷路完全未發育)；2)蒙迪尼異常(不完全劃分)；3) 耳蝸未發育；4)耳蝸發育不良；5) 共同腔。胚胎第六至第八週時耳蝸完成兩圈半間隔發育。此時阻礙導致耳蝸間隔無法完成兩圈半發育，形成僅有一圈半耳蝸。此類異常即是一般所謂蒙狄尼發育不良(Mondini dysplasia)，是可依影像學檢查診斷之內耳異常最常見者。聽力損失程度不一。由中至重度均有可能。Inner ear anomalies can be classified into ﬁve (5) groups: 1) Michel aplasia (complete labyrinth aplasia); 2) Mondini anomaly (incomplete division); 3) Cochlear aplasia; 4) Cochlear hypoplasia; 5) Common cavity. An abnormality of the inner ear that is associated with sensorineural hearing loss. This deformity was first described in 1791 by Mondini after examining the inner ear of a deaf boy. The Mondini dysplasia describes a cochlea with incomplete partitioning and a reduced number of turns, an enlarged vestibular aqueduct and a dilated vestibule. A normal cochlea has two and a half turns, a cochlea with Mondini dysplasia has one and a half turns; the basal turns being normally formed with a dilated or cystic apical turn to the cochlear. The hearing loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth. Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early adult life. Some children may pass newborn hearing screen to lose hearing in infancy but others present with a hearing loss at birth. Hearing loss is often progressive and because of the associated widened vestibular aqueduct may progress in a step-wise fashion associated with minor head trauma. Vestibular function is also often affected. While the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. The Mondini dysplasia can occur in cases of Pendred Syndrome and Branchio-oto-renal syndrome and in other syndromes, but can occur in non-syndromic deafness.

＜卡爾羅‧蒙迪尼是義大利解剖學家與醫生. Carlo Mondini (1729 – 1803) was an Italian anatomist and physician.＞

● Morquio's syndrome 莫奎歐症 (= Mucopolysaccharidosis Type IV, MPS IV 黏多糖症第四型)：

可能聽損。莫奎歐症便是黏多醣症之第四型；顧名思義當初發現此病的醫生就是莫奎歐醫師(Dr.Morquio)。他是一位烏拉圭的醫生，在西元1929年時他發表了一篇報告，在同一個家庭中有四個小孩皆有此病症；在同一年英國的布雷斯福醫生(Dr.Brailsford)也有相同的報告發表，所以此病有時亦稱為Morquio-Brailsford disease。而黏多醣症我們通常簡稱為MPS 。A rare metabolic disorder in which the body cannot process certain types of sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides). In Morquio syndrome, the specific GAG which builds up in the body is called keratan sulfate. This birth defect, which is autosomal recessive, is a type of lysosomal storage disorder. The buildup of GAGs in different parts of the body causes symptoms in many different organ systems. In the US, the incidence rate for Morquio is estimated at between 1 in 200,000 and 1 in 300,000 live births.

＜路易斯·莫奎歐是烏拉圭內科醫生和教授. Luis Morquio (September 24, 1867 - June 19, 1935) was a Uruguayan physician and professor.＞

● Norrie's disease 諾里病：

會造成聽損，可能眩暈。諾里病於1927年首次報導，是一種極為罕見的遺傳性疾病，目前很少有其發病率的報導。因此當你搜索百度的時候，很少有相關的報導。1992年Berger和Chen等通過定位克隆分離出了諾里病的附加基因NDP基因。諾里病是一種罕見的由視網膜神經退行發生和增生性改變導致的X連鎖隱性遺傳性疾病，以先天性失明為主要臨床特徵，偶發智力障礙，感音神經性耳聾和精神行為異常等。諾里病主要發生於足月產嬰兒中，患者以男性為主。引起該病的基因定位於Xp11.1，由3個外顯子組成並編碼含有133個氨基酸的Norrin蛋白，Norrin是一總蛋白的作用機理尚不明確。A rare disease and genetic disorder that primarily affects the eyes and almost always leads to blindness. It is caused by mutations in the Norrin cystine knot growth factor (NDP) gene, which is located on the X chromosome. In addition to the congenital ocular symptoms, the majority of patients suffer from a progressive hearing loss starting mostly in their 2nd decade of life, and some may have learning difficulties among other additional characteristics. Patients with Norrie disease may develop cataracts, leukocoria (a condition where the pupils appear white when light is shone on them), along with other developmental issues in the eye, such as shrinking of the globe and the wasting away of the iris. Around 30 to 50% of them will also have developmental delay/learning difficulties, psychotic-like features, incoordination of movements or behavioral abnormalities. Most patients are born with normal hearing; however, the onset of hearing loss is very common in early adolescence. About 15% of patients are estimated to develop all the features of the disease. Due to the X-linked recessive pattern of inheritance, Norrie disease affects almost entirely males. Only in very rare cases, females have been diagnosed with Norrie disease; cases of symptomatic female carriers have been reported. It is a very rare disorder that is not associated with any specific ethnic or racial groups, with cases reported worldwide (including cases in North America, South America, Europe, Asia and Australasia). While more than 400 cases have been described, the prevalence and incidence of the disease still remains unknown.

＜戈登-諾里是丹麥外科醫生與眼科醫生. Gordon Norrie (May 6 1855 - October 11 1941 ) was a Danish surgeon and ophthalmologist.＞

● Osler-Weber-Rendu disease 奧斯勒-韋伯-倫杜病 (= Hereditary hemorrhagic telangiectasia, HHT 遺傳性出血性血管擴張病)：

可能聽損。遺傳性出血性血管擴張病(hereditary hemorrhagic telangiectasia, HHT)是由Osler、Weber及Rendu三位學者先後加以描述的一種遺傳疾病，故亦稱為Osler-WeberRendu syndrome。它是由於基因缺陷所導致的一種顯性遺傳疾病，目前已知與造成血管生成及修補異常有關，非血液凝固問題或凝血因子缺乏所造成，是屬於一種血管畸形病變。患者血管缺乏彈性、血管壁薄、血管易捲曲變形，輕微碰撞即可能造成毛細血管破裂而出血。此疾病主要盛行於白種人，亞洲人則較為罕見。A rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain. It may lead to nosebleeds, acute and chronic digestive tract bleeding, and various problems due to the involvement of other organs. Treatment focuses on reducing bleeding from blood vessel lesions, and sometimes surgery or other targeted interventions to remove arteriovenous malformations in organs. Chronic bleeding often requires iron supplements and sometimes blood transfusions. HHT is transmitted in an autosomal dominant fashion, and occurs in one in 5,000–8,000 people in North America. The disease carries the names of Sir William Osler, Henri Jules Louis Marie Rendu, and Frederick Parkes Weber, who described it in the late 19th and early 20th centuries.

＜威廉·奧斯勒是加拿大醫生，也是約翰·霍普金斯醫院的四位創始教授之一. William Osler (July 12, 1849 – December 29, 1919) was a Canadian physician and one of the four founding professors of Johns Hopkins Hospital.＞

＜佛雷德里克·帕克斯·韋伯是英國的皮膚科醫生. Frederick Parkes Weber (8 May 1863 – 2 June 1962) was an English dermatologist.＞

＜亨利·朱爾斯·路易斯·瑪麗·倫杜是法國醫生. Henri Jules Louis Marie Rendu (24 July 1844 – 16 April 1902) was a French physician.＞

● Paget's disease of bone 帕杰特骨病 (= osteitis deformans 原發性變形性骨炎)：

可能眩暈。是一群因不同缺陷基因(heterogenous)所致的骨骼代謝異常疾病，分為成年型1-6型與青少年型，主要因活化的蝕骨細胞(activated osteoclasts)過度進行骨骼再吸收及合成而導致骨骼病變，使骨骼變得更大、更易脆，讓患者的骨骼可能會變形、容易骨折，任何種類的骨頭都可能受到影響，特別是脊椎、骨盆、頭骨或腿部的骨骼。此症常於成年人發生，通常會在中年或更晚以後才會發病，但也有些青年發病的類型。A condition involving cellular remodeling and deformity of one or more bones. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone formation. These structural changes cause the bone to weaken, which may result in deformity, pain, fracture or arthritis of associated joints. The exact cause is unknown, although leading theories indicate both genetic and acquired factors (see Causes). Paget's disease may affect any one or multiple bones of the body (most commonly pelvis, tibia, femur, lumbar vertebrae, and skull), but never the entire skeleton, and does not spread from bone to bone. Rarely, a bone affected by Paget's disease can transform into a malignant bone cancer. As the disease often affects people differently, treatments of Paget's disease can vary. Although there is no cure for Paget's disease, medications (bisphosphonates and calcitonin) can help control the disorder and lessen pain and other symptoms. Medications are often successful in controlling the disorder, especially when started before complications begin. Paget's disease affects from 1.5 to 8.0 percent of the population, and is most common in those of British descent. It is primarily diagnosed in older people and is rare in people less than 55 years of age. Men are more commonly affected than women (3:2). The disease is named after English surgeon Sir James Paget, who described it in 1877.

＜詹姆斯·佩吉特爵士是英國外科醫生和病理學家. Sir James Paget, 1st Baronet FRS HFRSE (11 January 1814 – 30 December 1899) was an English surgeon and pathologist.＞

● Parinaud's syndrome 帕里諾症：

由中腦上丘的眼球垂直同向運動皮質下中樞病變而導致的眼球垂直同向運動障礙，累及上丘的破壞性病灶可導致兩眼向上同向運動不能。An inability to move the eyes up and down. It is caused by compression of the vertical gaze center at the rostral interstitial nucleus of medial longitudinal fasciculus (riMLF). The eyes lose the ability to move upward and down. It is a group of abnormalities of eye movement and pupil dysfunction. It is caused by lesions of the upper brain stem and is named for Henri Parinaud (1844–1905), considered to be the father of French ophthalmology.

＜亨利-帕里諾是法國眼科醫生與神經科醫生. Henri Parinaud (1 May 1844 – 23 March 1905) was a French ophthalmologist and neurologist.＞

● Parkinson's disease 巴金森氏病：

可能眩暈/頭暈。一種漸進性的中樞神經退化疾病。常見症狀為身體四肢會不自主地顫抖，尤其在肢體休息時，顫抖會特別明顯，同時病人的肌肉力量也逐漸減退，走路時身體有前傾、駝背的情形，並且走起路來，剛開始時是緩慢的步伐，會逐漸變成碎步，向前直衝。A long-term degenerative disorder of the central nervous system that mainly affects the motor system. As the disease worsens, non-motor symptoms become more common. The symptoms usually emerge slowly. Early in the disease, the most obvious symptoms are shaking, rigidity, slowness of movement, and difficulty with walking. Thinking and behavioral problems may also occur. Dementia becomes common in the advanced stages of the disease. Depression and anxiety are also common, occurring in more than a third of people with PD. Other symptoms include sensory, sleep, and emotional problems. The main motor symptoms are collectively called "parkinsonism", or a "parkinsonian syndrome". The cause of Parkinson's disease is unknown, but is believed to involve both genetic and environmental factors. Those with a family member affected are more likely to get the disease themselves. There is also an increased risk in people exposed to certain pesticides and among those who have had prior head injuries, while there is a reduced risk in tobacco smokers and those who drink coffee or tea.[4][10] The motor symptoms of the disease result from the death of cells in the substantia nigra, a region of the midbrain. This results in not enough dopamine in this region of the brain. The cause of this cell death is poorly understood, but it involves the build-up of proteins into Lewy bodies in the neurons. Diagnosis of typical cases is mainly based on symptoms, with tests such as neuroimaging used to rule out other diseases. There is no cure for Parkinson's disease. Treatment aims to improve the symptoms.

＜詹姆斯·巴金森是一位英國外科醫生，地質學家，古生物學家，社會活動家. James Parkinson FGS (11 April 1755 – 21 December 1824) was an English surgeon, apothecary, geologist, palaeontologist, and political activist＞

● Parry-Romberg syndrome 帕里-隆伯格症 (= Progressive facial hemiatrophy, PFHA 進行性半側顏面萎縮)：

可能聽損。一種少見的，以單側皮膚、皮下組織及骨結構萎縮為特徵命名的疾病。這種病發病率非常低，嚴重者患病部位的骨頭都會遭到侵蝕。進行性半側顏面萎縮，又稱帕-隆症(Parry-Romberg syndrome)，由Parry在1825年首次報道，1846年Romberg對此病進行詳細描述，故以兩人的名字命名。1871年Enlenburg首次提出了進行性顏面萎縮癥(Progressive facial hemiatrophy, PFHA)這一名稱。A rare disease characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy) but occasionally extending to other parts of the body. An autoimmune mechanism is suspected, and the syndrome may be a variant of localized scleroderma, but the precise cause and pathogenesis of this acquired disorder remains unknown. It has been reported in the literature as a possible consequence of sympathectomy. The syndrome has a higher prevalence in females and typically appears between 5 – 15 years of age. In addition to the connective tissue disease, the condition is sometimes accompanied by neurological, ocular and oral symptoms. The range and severity of associated symptoms and findings are highly variable.

＜帕里是英國醫生. Caleb Hillier Parry (21 October 1755 – 9 March 1822) was an English physician.＞
＜隆伯格是德國醫生. Moritz Heinrich Romberg (11 November 1795 – 16 June 1873) was a Germany physician.＞

● Patau's syndrome 巴陶症 (= Trisomy 13 三染色體13症)：

會造成聽損。

染色體三倍體症之一，少見，患者多在出生後一年內死亡。13-三體症候群早在1657年便由丹麥醫學家托馬斯·巴托林發現，但其染色體性質是1960年由德裔美國遺傳學家Klaus Patau博士確認，故以其姓氏命名。臨床表徵：智能不足，生長遲緩，小頷畸形，耳畸形，手指變曲，短胸骨畸形，心室中隔缺損。A syndrome caused by a chromosomal abnormality, in which some or all of the cells of the body contain extra genetic material from chromosome 13. The extra genetic material disrupts normal development, causing multiple and complex organ defects. This can occur either because each cell contains a full extra copy of chromosome 13 (a disorder known as trisomy 13 or trisomy D), or because each cell contains an extra partial copy of the chromosome or because of mosaic Patau syndrome. Full trisomy 13 is caused by nondisjunction of chromosomes during meiosis (the mosaic form is caused by nondisjunction during mitosis). Like all nondisjunction conditions (such as Down syndrome and Edwards syndrome), the risk of this syndrome in the offspring increases with maternal age at pregnancy, with about 31 years being the average. Patau syndrome affects somewhere between 1 in 10,000 and 1 in 21,700 live births.

＜克勞斯-巴陶是美國遺傳學家. Klaus Patau (1908–1975) was an American geneticist.＞

● Pendred's syndrome 彭德雷氏症：

會造成聽損，可能眩暈。一種先天性隱性遺傳疾病，會合併先天性聽損與甲狀腺腫。聽損多為中度至重度，高頻較為嚴重，甲狀腺腫多發生在青春期之後。A genetic disorder leading to congenital bilateral (both sides) sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism (decreased thyroid gland function). There is no specific treatment, other than supportive measures for the hearing loss and thyroid hormone supplementation in case of hypothyroidism. It is named after Dr Vaughan Pendred (1869–1946), the British doctor who first described the condition in an Irish family living in Durham in 1896. It accounts for 7.5% to 15% of all cases of congenital deafness.

＜沃恩‧彭德雷是英國醫生. Vaughan Pendred (1869–1946) was an English doctor.＞

● Pierre Robin syndrome 皮爾-羅賓症 (= Robin sequence 羅賓後遺症)：

可能聽損。是由法國牙醫Pierre Robin於1923年所命名，以此形容合併小下巴、下巴後縮、顎裂、舌後垂症狀的患者。此疾病會單獨發生，也可能與其他症候群一起出現，例如：Stickler syndrome、Treacher Collins syndrome、velocardiofacial syndrome、酒精性胎兒畸形(fetal alcohol syndrome)、胎兒內醯胺症候群(fetal hydantoin syndrome )等。此疾病為胚胎時期下顎發育不良所致，正常狀況下，胎兒於母親妊娠7~10週時，下顎會快速發展，讓舌頭能置於上下顎間，如下顎無法正常生長而過小，使舌頭無法安置於正確位置，會塞在口腔中，阻礙顎蓋的關閉，發生顎裂的情形；導致出生後，過小的下顎會使舌頭擠在口腔後方，阻礙呼吸，嚴重者會造成呼吸困難。造成皮爾-羅賓症的原因很多，與下顎發育有關的染色體或基因突變為最常見，除此之外，環境問題而產生畸胎因子亦會造成，例如母親於妊娠期抽煙、喝酒、不當用藥等皆可能導致此一疾病；子宮內有纖維瘤或有雙角子宮等也可能干擾胎兒下顎之發展。A congenital defect observed in humans which is characterized by facial abnormalities. The three main features are micrognathia (abnormally small mandible), which causes glossoptosis (downwardly displaced or retracted tongue), which in turn causes breathing problems due to obstruction of the upper airway. A wide, U-shaped cleft palate is commonly also present. PRS is not merely a syndrome, but rather it is a sequence—a series of specific developmental malformations which can be attributed to a single cause.

＜皮爾-羅賓是法國口腔顎面外科醫生. Pierre Robin (1867–1949) was a French oral and maxillofacial surgeon.＞

● Politzer's bag 波利澤瘻管測驗打氣袋：

Politzer bag也成為後世醫生用作經耳道做瘻管測驗(fistula test)的檢查工具。

＜阿達姆·波利澤是匈牙利和奧地利的內科醫生，也是耳科學的先驅和創始人之一. Ádám Politzer (1 October 1835 – 10 August 1920) was a Hungarian and Austrian physician and one of the pioneers and founders of otology.＞

● Politzer's maneuver 波利澤中耳通氣手法 (= Politzer's method 波利澤方法, Politzerization 波利澤中耳通氣術)：

波利澤是耳科學始祖，出版耳科學第一本教科書，催生耳科學第一本雜誌。1861年波利澤首次發表Politzer maneuver (中耳通氣法)，就是將空氣經鼻灌入耳咽管到達中耳，以解決病患耳悶的治療方式。Politzer bag也成為後世醫生用作經耳道做瘻管測驗(fistula test)的檢查工具。A medical procedure that involves inflating the middle ear by blowing air up the nose during the act of swallowing. It is often performed to reopen the Eustachian tube and equalise pressure in the sinuses. The procedure was derived from a medical experiment first performed by Ádám Politzer of Vienna that involved studying the air movement through the Eustachian tube by connecting a manometer to the external auditory canal meatus and another manometer in the pharynx. His first results on the technique were published in 1861 and he introduced a pear-shaped rubber air-bag for performing the procedure in 1863, which came to be known as a Politzer bag. This system was far more practical and less difficult for the patient than catheterizing the Eustachian tube and brought fame to Politzer.

● Porschl view of resoncstructive CT 電腦斷層掃描的重組性波秋平面：

適用於診斷SSCD syndrome上半規管裂損症的CT平面包括Porschl's波秋、Stenver's view史坦弗平面。顳骨CT除了標準的三個平面，axial、saggital、coronal views，也可以透過重組成Stvenver's, Porschl's view。顳骨解剖結構複雜，並且由於相關結構的小尺寸和三維定向而進一步複雜化。電腦斷層掃描(CT)徹底改變了顳骨成像。32切，64切和現在的128切CT掃描儀的最新進展允許獲取高分辨率的體積數據，從而可以在任何平面上重建圖像。除了傳統的軸向和冠狀平面外，在Stenvers和Poschl投影中也可以獲得重建。Stenvers投影是平行於岩狀骨長軸的平面，而Poschl投影是平行於岩狀骨短軸的平面。這些額外的平面對於評估中耳和內耳的結構非常有用，而在標準的軸向和冠狀平面上可能看不到這些結構。各種各樣的病理可能會影響顳骨的相對較小的解剖區域。在顳骨引起的病理實體中，臨床症狀常常重疊。因此，良好的臨床病史與專用顳骨CT的結合有助於評估顳骨是否存在細微或不太細微的疾病。這個基於網絡的交互式顳骨解剖模塊為用戶提供了一個機會，可以查看正常的顳骨解剖和異常病例，這些異常病例涉及影響顳骨的四種病理類型：先天性畸形，炎性疾病，創傷，腫瘤和腫瘤樣疾病。還有一個自我評估測驗，涉及與這四個一般類別有關的問題。Temporal bone anatomy is complex, and further complicated by the small size and three-dimensional orientation of associated structures. Computed tomography (CT) has revolutionized imaging of the temporal bone. Recent advances in 32, 64 and now 128-slice CT scanners allow the acquisition of high-resolution, volumetric data that allows image reconstruction in any plane. In addition to the traditional axial and coronal planes, it has become routine to also obtain reconstructions in the Stenvers and Poschl projections. The Stenvers projection is the plane parallel to the long axis of the petrous bone and the Poschl projection is the plane parallel to the short axis of the petrous bone. These additional planes are extremely useful for evaluating the structures of the middle and inner ear that may not be as well seen on the standard axial and coronal planes. A wide array of pathology may affect the relatively small anatomical region of the temporal bone. Often there is overlap of clinical symptoms in pathological entities arising from the temporal bone. Thus, the combination of a good clinical history in conjunction with a dedicated temporal bone CT facilitates evaluation of the temporal bone for subtle or not-so subtle disease. This interactive web-based temporal bone anatomy module provides the user an opportunity to review normal temporal bone anatomy and abnormal cases involving four general categories of pathology affecting the temporal bone: congenital malformations, inflammatory conditions, trauma and tumor and tumor-like conditions. There is also a self-assessment quiz with questions pertaining to these four general categories.

● Potter's syndrome 波特症 (= Potter sequence 波特後遺症)：

會造成聽損。波特症是指因羊水過少引起的子宮內壓力造成的典型身體外觀，經典情況下是由於雙側腎臟發育不全(BRA)引起的，但也可能在其他情況下發生，包括嬰兒多囊腎，腎臟發育不全和阻塞性尿毒症。一些人認為波特後遺症是更合適的術語，因為並非每個患有該症的人都有完全相同的症狀和體徵，而是他們共享由不同原因觸發的共同事件鏈，從而導致羊水減少或缺乏的相同終點流體。The atypical physical appearance of a baby due to oligohydramnios experienced when in the uterus. It includes clubbed feet, pulmonary hypoplasia and cranial anomalies related to the oligohydramnios.[clarification needed] Oligohydramnios is the decrease in amniotic fluid volume sufficient to cause deformations in morphogenesis of the baby. Oligohydramnios is the cause of Potter sequence but there are many things that can lead to oligohydramnios. It can be caused by renal diseases such as bilateral renal agenesis (BRA), atresia of the ureter or urethra causing obstruction of the urinary tract, polycystic or multicystic kidney diseases, renal hypoplasia, amniotic rupture, toxemia, or uteroplacental insufficiency from maternal hypertension. The term Potter sequence was initially intended to only refer to cases caused by BRA;[citation needed] however, it is now commonly used by many clinicians and researchers to refer to any case that presents with oligohydramnios or anhydramnios regardless of the source of the loss of amniotic fluid.

＜伊迪絲·路易斯·波特是美國的醫生和科學家，創立了圍產期病理學領域. Edith Louise Potter (1901 - March 22, 1993) was an American physician and scientist who established the field of perinatal pathology.＞

● Prussak's space 普魯薩克間隙 (= Lateral epitympanic recess 側上鼓室隱窩)：

位於鼓膜鬆弛部與鎚骨頸之間，是鼓膜鬆弛部膽脂瘤的好發部位。The small middle ear recess, bordered laterally by the flaccid part of Shrapnell's membrane, superiorly by the scutum (a sharp bony spur that is formed by the superior wall of the external auditory canal) and lateral malleal ligament, inferiorly by the lateral process of the malleus, and medially by the neck of the malleus. From the neck of the malleus, the anterior malleolar fold and the anterior ligament arise, demarcating Prussak's space anteriorly. Ventilation of Prussak's space is only possible posteriorly above the posterior malleus fold. It communicates with the posterior pouch of von Troltsch. It is named after the Russian otologist Alexander Prussak (1839-1897).

＜亞歷山大·普魯薩克是俄羅斯耳科醫生. Alexander Prussak (1839 – 20 January 1897, in St. Petersburg) was a Russian otologist.＞

● Purkinje's cell 浦肯野細胞 (= Purkinje neurons 浦肯野神經元)：

小腦的丙胺基丁酸神經元。小細胞肺癌的腫瘤伴隨症(paraneoplastic syndrome)，容易攻擊小腦的浦肯野細胞，導致小腦退化，造成步態異常。A class of GABAergic neurons located in the cerebellum. In humans, Purkinje cells can be harmed by a variety causes: toxic exposure, e.g. to alcohol or lithium; autoimmune diseases; genetic mutations causing spinocerebellar ataxias, gluten ataxia, Unverricht-Lundborg disease, or autism; and neurodegenerative diseases that are not known to have a genetic basis, such as the cerebellar type of multiple system atrophy or sporadic ataxias.
＜楊·伊萬傑列斯塔·浦肯野是捷克解剖學家和生理學家. Jan Evangelista Purkyně (also written Johann Evangelist Purkinje) (17 or 18 December 1787 – 28 July 1869) was a Czech anatomist and physiologist.＞

● Ramsay Hunt syndrome 侖謝亨特症：

可能眩暈。第二型侖謝亨特症又稱之為耳帶狀疱疹，三聯徵為同側耳痛、疱疹、顏面神經麻痺。Three different neurological syndromes carry the name of Ramsay Hunt syndrome. Their only connection is that they were all first described by the famous neurologist James Ramsay Hunt (1872–1937). Ramsay Hunt syndrome type 1, also called Ramsay Hunt cerebellar syndrome, is a rare form of cerebellar degeneration which involves myoclonic epilepsy, progressive ataxia, tremor, and a dementing process. Ramsay Hunt syndrome type 2 is the reactivation of herpes zoster in the geniculate ganglion. It is sometimes called herpes zoster oticus, and has variable presentation which may include a lower motor neuron lesion of the facial nerve, deafness, vertigo, and pain. A triad of ipsilateral facial paralysis, ear pain, and vesicles on the face, on the ear, or in the ear is the typical presentation. Ramsay Hunt syndrome type 3 is a less commonly referenced condition, an occupationally induced neuropathy of the deep palmar branch of the ulnar nerve. It is also called Hunt's disease or artisan's palsy.

＜詹姆斯·侖謝·亨特是美國神經科醫生. James Ramsay Hunt (1872 – July 22, 1937) was an American neurologist.＞

● Refsum syndrome 雷夫敘姆症：

會造成運動失調。一種體染色體隱性遺傳神經系統疾病，會造成細胞與組織裡過度堆積植烷酸。會造成不平衡、運動失調，幾乎不會產生眩暈、頭暈。雷夫敘姆症也稱之為運動失調-多發性神經炎症、遺傳性運動失調多發性神經炎症、Boussy-Levy症、植烷酸氧化酸缺乏病。其特徵為呈AR遺傳，臨床主要表現為色素性視網膜炎、多發性神經炎、小腦運動失調、皮膚損害和心臟傳導障礙。An autosomal recessive neurological disease that results in the over-accumulation of phytanic acid in cells and tissues. It is one of several disorders named after Norwegian neurologist Sigvald Bernhard Refsum (1907–1991). Refsum disease typically is adolescent onset and is diagnosed by above average levels of phytanic acid. Humans obtain the necessary phytanic acid primarily through diet. It is still unclear what function phytanic acid plays physiologically in humans, but has been found to regulate fatty acid metabolism in the liver of mice.

＜雷夫敘姆是挪威神經科醫生. Sigvald Bernhard Refsum (8 May 1907, Gransherad – 8 July 1991, Oslo) was a Norwegian neurologist.＞

● Reissner's membrane 雷斯納膜 (= Vestibular membrane 前庭膜)：

雷斯納膜分隔前庭階與中間階。人類的耳蝸形似蝸牛殼，由底端(basal)至頂端(apikal)螺旋環繞二又八分之五周，展開長度約為35 mm。耳蝸是一個骨質結構。如右圖耳蝸由三個內部充滿淋巴液的空腔組成。這三個空腔由上到下依次為：前庭階/前庭管(scala vestibuli)，內含外淋巴(perilymph)液體，中間階/蝸管(Scala media)，內含內淋巴(eEndolymph)液體，鼓室階/鼓管(scala tympani)，內含外淋巴(perilymph)液體。前庭階在底端中止於卵圓窗(oval window)，是鐙骨施力的部位。鼓室階在底端中止於圓窗(round window)，毗鄰中耳腔，是聲壓釋放的窗口。雷斯納氏膜(Reissner's membrane)分隔前庭階和中間階，基底膜(basilar membrane)分隔中間階和鼓階。聽覺轉導器官柯蒂氏器(Organ of Corti)坐落於基底膜之上、中間階內部。前庭階和鼓室階在蝸孔(helicotrema)相通。聽神經的纖維通過基底膜與內毛細胞和外毛細胞形成突觸連接。其細胞體位於在耳蝸中心部的螺旋神經節(spiral ganglion)。A membrane inside the cochlea of the inner ear. It separates the cochlear duct from the vestibular duct. Together with the basilar membrane it creates a compartment in the cochlea filled with endolymph, which is important for the function of the spiral organ of Corti. It primarily functions as a diffusion barrier, allowing nutrients to travel from the perilymph to the endolymph of the membranous labyrinth. Histologically, the membrane is composed of two layers of flattened epithelium, separated by a basal lamina. Its structure suggests that its function is transport of fluid and electrolytes. Reissner's membrane is named after German anatomist Ernst Reissner (1824-1878).

＜斯特·雷斯納是德國解剖學家. Ernst Reissner (September 24, 1824 – September 16, 1878) was a Baltic German anatomist.＞

● Rinne's test 林內測驗：

又稱氣骨導對比試驗，是比較同側氣導和骨導的一種檢查方法。先測骨導聽力，當聽不到音叉聲時，立即測同側氣導聽力，也可反之。The Rinne test is used primarily to evaluate loss of hearing in one ear. It compares perception of sounds transmitted by air conduction to those transmitted by bone conduction through the mastoid. Thus, one can quickly screen for the presence of conductive hearing loss. A Rinne test should always be accompanied by a Weber test to also detect sensorineural hearing loss and thus confirm the nature of hearing loss. The Rinne test was named after German otologist Heinrich Adolf Rinne (1819–1868); the Weber test was named after Ernst Heinrich Weber (1795–1878).

＜海因里希·阿道夫·林內是德國耳科醫生. Heinrich Adolf Rinne (January 24, 1819 – July 26, 1868) was a German otologist.＞

● Romberg's test 隆伯格測驗 (= Romberg's sign, 體徵 Romberg maneuver 手法)：

一種本體感覺的神經學檢查，請病人站著，雙腳併攏然後閉上雙眼，若閉眼後會搖晃，無法站好，代表本題感覺有問題。這不是小腦檢查，人體保持平衡的三大系統分別為：視覺，本體感覺，與前庭覺，只要有兩個系統正常運作，人體就能保持某種程度的平衡，Romberg test就是把視覺關掉，看剩下的兩個是否能保持平衡, 閉上眼睛站不穩就是陽性，不需要推病人，從來沒有一本文獻或教科書說要推病人。實際上Romberg test測的是本體感覺，Romberg test陽性代表的，就是本體感覺路徑失常造成的不平衡。至於小腦平衡不在測量的範圍裡面，因為這是sensory檢查，不負責coordination，cerebellar ataxia連開眼站穩都沒辦法，自然無法執行Romberg test，小腦功能要用motor coordination來測量。A test used in an exam of neurological function for balance, and also as a test for driving under the influence of an intoxicant. The exam is based on the premise that a person requires at least two of the three following senses to maintain balance while standing: proprioception (the ability to know one's body position in space); vestibular function (the ability to know one's head position in space); and vision (which can be used to monitor and adjust for changes in body position). A patient who has a problem with proprioception can still maintain balance by using vestibular function and vision. In the Romberg test, the standing patient is asked to close his or her eyes. An increased loss of balance is interpreted as a positive Romberg's test. The Romberg test is a test of the body's sense of positioning (proprioception), which requires healthy functioning of the dorsal columns of the spinal cord. The Romberg test is used to investigate the cause of loss of motor coordination (ataxia). A positive Romberg test suggests that the ataxia is sensory in nature, that is, depending on loss of proprioception. If a patient is ataxic and Romberg's test is not positive, it suggests that ataxia is cerebellar in nature, that is, depending on localized cerebellar dysfunction instead. It is used as an indicator for possible alcohol or drug impaired driving and neurological decompression sickness. When used to test impaired driving, the test is performed with the subject estimating 30 seconds in their head. This is used to gauge the subject's internal clock and can be an indicator of stimulant or depressant use.

＜莫里茨·海因里希·隆伯格是德國醫生. Moritz Heinrich Romberg (11 November 1795 – 16 June 1873) was a Germany physician.＞

● Rosenmuller's fossa 羅森穆勒隱窩 (= Pharyngeal recess 咽隱窩)：

耳咽管咽口後方的凹窩，是鼻咽癌好發的部位。鼻咽(nasopharynx)位在鼻腔的後方，向前經鼻後孔通鼻腔，其側壁正對下鼻甲後方，有一耳咽咽口(Eustachian tube orifice)，通中耳鼓室，在耳咽管咽口前、上、後方有弧形的隆起稱咽鼓管圓枕(tubal torus)，其後方與咽後壁之間的縱形深窩，稱咽隱窩(pharyngeal recess；fossa of Rosenmüller)，是鼻咽癌的好發部位。在鼻咽後上壁的黏膜內有豐富的淋巴組織，稱咽扁桃體(pharyngeal tonsil)，自三至四歲以後快速成長，通常在青春期前消退，鼻咽部可經由豐富之淋巴管與咽後淋巴結及其他頸部淋巴結相通，因此鼻咽癌病人極易產生頸部淋巴結的轉移。Behind the ostium of the eustachian tube (ostium pharyngeum tuba auditiva) is a deep recess, the pharyngeal recess (fossa of Rosenmüller). At the base of this recess is the retropharyngeal lymph node (the Node of Rouvier). This is clinically significant in that it may be involved in certain head and neck cancers, notably Nasopharyngeal cancer.

＜約翰·克里斯蒂安·羅森穆勒是德國解剖學家. Johann Christian Rosenmüller (May 25, 1771 – February 28, 1820) was a German anatomist.＞

● Scarpa’s ganglion 史卡帕神經節 (= Vestibular ganglion 前庭神經節)：

前庭系統的一級神經元在內聽道(IAC)的底部形成前庭神經節(vestibular ganglion)，又叫作史卡帕神經節(Scarpa’s ganglion)，屬於雙極神經元，從三個半規管、球囊上半部分、球囊大部分、橢圓囊來的神經纖維會合成上、下前庭神經，穿過內聽道，再重新分支，到達各自的前庭神經核。The vestibular ganglion (also called Scarpa's ganglion) is the ganglion of the vestibular nerve. It is located inside the internal auditory meatus. The ganglion contains the cell bodies of bipolar neurons whose peripheral processes form synaptic contact with hair cells of the vestibular sensory end organs. These include hair cells of the cristae ampullares of the semicircular duct and macula in the utricle and saccule. It is named for Antonio Scarpa. At birth, it is already close to its final size.

＜安東尼-史卡帕是義大利解剖學家. Antonio Scarpa (9 May 1752 – 31 October 1832) was an Italian anatomist.＞

● Scheibe's dysplasia 沙伊貝發育不良 (= Cochleosaccular dysplasia 耳蝸球囊發育不良)：

會造成聽損，可能眩暈。為常染色體隱性遺傳，是最輕的內耳畸形，畸形僅限於蝸管和球囊，故又稱耳蝸球囊型畸形。診斷：主要根據先天性耳聾和MRI檢查。治療：此病可選擇性地行人工耳蝸植入術。主要是耳蝸管(耳蝸的膜性迷路)與前庭的球囊(saccule)發育不全。一般相信這是隱性遺傳性先天聽障最多的異常。聽力大多集中在極重度或全聾，但在低頻偶會發現有殘存聽力。裝置電子耳一般會有不錯的效果。因為電子耳正是取代耳蝸管的功能，直接刺激聽神經。Scheibe dysplasia represents the mildest end of the spectrum of vestibulocochlear dysplasias and consist of membranous abnormalities primarily of the cochlea with involvement of the saccule. It is also referred to as cochleosaccular dysplasia. In contrast to Bing-Siebenmann dysplasia, the membranous utricle and SCCs are fully formed and functioning. The petrous bone and the bony cochlea and vestibule are fully developed, making radiologic diagnosis impossible. The organ of Corti is often rudimentary with very few sensory cells, and the stria vascularis is degenerate with relatively few vessels. The canal of the cochlea is collapsed and the techtorial membrane is formed but flattened down over the organ of Corti and its supporting cells. In cases where the canal of the cochlea is dilated rather than collapsed, the immaturity of the sensory endorgan is atrributed to a degenerative process rather than an arrest in development. The degree of development can vary throughout the cochlea, and some areas of the cochlea function sufficiently well to provide some level of hearing.

● Schwartz's sign 史瓦茲體徵 (= Flemingo's flush sign or Rising sun sign)：

耳硬化的一種體徵，不常見，10%出現於耳硬化症的早期。火紅體徵(火鶴潮紅) 或稱史瓦茲體徵，透過耳膜可見耳岬(promontory)及前窗裂(Fissula ante fenestram)因海棉樣骨變化而血管增生，產生火紅體徵。A diagnostic indicator for otosclerosis, a disease of the bones of the middle or inner ear. In clinical examination of the ear drum, increased vascularity of the promontory may be seen through the ear drum. This sign is known as Flemingo's flush sign or Schwartz's sign. This indicates otospongiosis (active otosclerosis). In about 10% of cases of otosclerosis, there is a redness of the promontory of the cochlea seen through the tympanic membrane due to prominent vascularity associated with an otospongiotic focus. Be aware of the similar, Brown's sign. This is a red retro-tympanic bulge that blanches on pressure via pneumatic otoscopy. This is secondary to paragangliomata of the middle ear.

＜赫曼‧史瓦茲是德國耳科醫生. Hermann Schwartze (1837-1910) was a German otologist.＞

● Semont's maneuver 西蒙手法：

後半規管BPPV的一種治療手法，比起Epley's maneuver，Semont's maneuver較少被使用。Aims to cure benign paroxysmal positional vertigo (BPPV). Repeated treatments may be required and doctors often teach their patients the techniques if they get recurrent episodes. These maneuvers are designed to reposition otoliths in the posterior Semicircular canals. There are different maneuvers for those in the lateral (horizontal) canals. Instructions: a) Turn your head 45 degrees horizontally toward the unaffected ear. b) Then tilt 105 degrees so that you are lying on the side of the affected ear with your head hanging and your nose pointed upward. After dizziness stops, hold this position for 5 minutes. c) Then move quickly via the seated position, holding your head in place, until you are lying on the side of the unaffected ear with your nose pointed to the ground. After dizziness stops, hold this position for 5 minutes. d) Then slowly move back to the seated position.

● Shprintzen-Goldberg syndrome (SGS) 什普林森-哥德堡症 (= velocardiofacial syndrome 顎心臉症, Marfanoid-craniosynostosis syndrome 類馬凡顱縫合症)：

可能聽損。什普林森-哥德堡症又叫類馬凡-顱縫合症，患者具有明顯的面部、骨骼及神經功能異常。一個常見特征是顱縫早閉，即某些頭骨過早融合。這種早期融合阻止了頭骨的正常生長。患者具有獨特的面部特征，包括頭部長而窄；眼睛間距大；眼球突出；眼睛外角下斜；上顎高而窄；下顎小。低垂的耳朵向後旋轉。患有此症的人通常被認為有馬凡症(Marfan's syndrome)的特徵。他們的身體特徵與患有馬凡症這種基因病的人相似。例如，他們可能手指細長，四肢長，胸部凹陷或突出，脊柱側彎或側凸。患有此症的人可能有其他骨骼異常，例如一個或多個一直彎曲的手指，以及具有異常的活動範圍的關節。患有此症的人往往發育遲緩，具有輕度到中度的智力障礙。其他常見特征是心臟或大腦異常、嬰兒期的肌肉張力弱，肚臍周圍或下腹部有向外突出的軟袋。此症的臨床特征與馬凡症和另一種稱為Loeys-Dietz's syndrome的遺傳疾病相似。然而，智力殘疾在此症中更常見，在另外這這兩種病中並不常見。此外，在馬凡症和Loeys-Dietz's syndrome中，心臟異常更常見，而且通常更嚴重。A multiple anomaly syndrome that has craniosynostosis, multiple abdominal hernias, cognitive impairment, and other skeletal malformations as key features. Several reports have linked the syndrome to a mutation in the FBN1 gene, but these cases do not resemble those initially described in the medical literature in 1982 by Shprintzen and Goldberg, and Greally et al. in 1998 failed to find a causal link to FBN1. At this time, the cause of Shprintzen–Goldberg syndrome remains uncertain. The syndrome is rare with fewer than 50 cases described in the medical literature to date.

● Siegel's pneumatic Speculum 席耶格打氣式耳鏡：

Siegel's pneumatic speculum席耶格打氣耳鏡，不含光源。Pneumatic otoscopy打氣光源耳鏡，含光源。The pneumatic otoscope is the standard tool used in diagnosing otitis media. In addition to the pneumatic (diagnostic) head, a surgical head also is useful. The pneumatic head contains a lens, an enclosed light source, and a nipple for attachment of a rubber bulb and tubing. The head is designed so that when a speculum is attached and fitted snugly into the patient’s external auditory canal, an air-tight chamber is produced. In some cases, the addition of a small sleeve of rubber tubing at the end of the plastic speculum or use of a rubber-tipped speculum helps to avoid trauma and improve the air-tight seal. Gently squeezing and releasing the rubber bulb in rapid succession permits observation of the degree of eardrum mobility in response to both positive and negative pressure.

● Sjögren syndrome 修格蘭症：

可能聽損、眩暈。俗稱乾燥症，是一種病因尚未完全明瞭的外分泌腺自體免疫疾病。外分泌腺受到淋巴球浸潤後長期引起發炎，最後導致乾口症、乾燥性角膜結膜炎與唾液腺腫大。少數病人會併發腺體外自體免疫症狀。A long-term autoimmune disease that affects the body's moisture-producing glands. Primary symptoms are a dry mouth and dry eyes. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Those affected are also at an increased risk (5%) of lymphoma. While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacteria. It can occur independently of other health problems (primary Sjögren syndrome) or as a result of another connective tissue disorder (secondary Sjögren syndrome). The inflammation that results progressively damages the glands. Diagnosis is by biopsy of moisture-producing glands and blood tests looking for specific antibodies. On biopsy there are typically lymphocytes within the glands. Treatment is directed at the person's symptoms. For dry eyes artificial tears, medications to reduce inflammation, punctal plugs, or surgery to shut the tear ducts may be tried. For a dry mouth, chewing gum (preferably sugar-free), sipping water, or a saliva substitute may be used. In those with joint or muscle pain, ibuprofen may be used. Medications that can cause dryness, such as antihistamines, may also be stopped. The disease was described in 1933 by Henrik Sjögren, after whom it is named; however, a number of earlier descriptions of people with the symptoms exist. Between 0.2% and 1.2% of the population are affected, with half having the primary form and half the secondary form. Females are affected about ten times as often as are males. Though the disease commonly begins in middle age, anyone can be affected. Among those without other autoimmune disorders, life expectancy is unchanged.

＜修格蘭是瑞士眼科醫生. Henrik Samuel Conrad Sjögren (23 July 1899 – 17 September 1986) was a Swedish ophthalmologist.＞

● Stenger's principle史丹爵原理：

史丹爵測驗基於聽覺現象“ 史丹爵原理”，用於懷疑患者患有聽力障礙的聽力障礙時使用的測試方法。此原理指出，只有同時出現在兩隻耳朵上的兩個相似音調中的較大聲才會被感知。通常，建議僅在單側聽力損失或明顯不對稱(至少20dB)的情況下執行此測驗。The Stenger test is based on the auditory phenomenon “The Stenger Principle” and is used when a patient is suspected malingering a hearing loss. The Stenger Principle states that only the louder of two similar tones presented to both ears at the same time will be perceived. Generally, it is recommended only to perform the Stenger test in cases of unilateral hearing losses or significant asymmetries (at least 20dB).

● Stenger's test 史丹爵測驗：

該測試可以在臨床環境中使用音叉完成。在測試開始之前，該人被蒙住了雙眼。敲擊兩個相同頻率的音叉，並與每個耳朵保持25 cm的距離。當被問及時，該人將聲稱可以通過正常的耳朵聽到它。然後，將音叉放在假耳朵附近8 cm處，同時使音叉在正常側保持相同的距離。如果他/她是騙子，則該人將拒絕聽到任何聲音。真正耳聾的人應繼續從正常一側聽到聲音。另外，使用純音調信號的兩通道聽力計可以更準確地執行此測試。The test can be done using tuning forks in the clinical setting. The individual is blindfolded before the test starts. Two tuning forks of the same frequency are stricken and kept at a distance of 25 cm from each ear. When asked, the individual will claim to hear it in the normal ear. Then, the tuning fork is brought as close as 8 cm near the feigned ear while maintaining the tuning fork at the normal side at the same distance. The individual will deny hearing anything if he/she is a malingerer. An individual with true deafness should continue to hear the sound on the normal side. Alternatively, this test can be performed more accurately using two-channel audiometer using pure tone signals.

● Stenger's test of positioning nystagmus 頭位變換眼振的史丹爵測驗：

把頭部連同軀幹，作自動或被動之急速變換，而誘發出之眼振稱為頭位變換眼振，主要有二法：Stenger's method 史丹爵法：坐位，頭朝正前方，快速躺下，頭懸垂位，若誘發出純垂直性眼振，即中樞性病變，若誘發出純迴旋性眼振，即迷路病變。Dix-Hallpike's method 戴克斯-霍爾派克法：坐位，頭轉左方或右方，快速躺下，頭懸垂位，若是BPPV疾病則會出現counter-rolling nystagmus時鐘反轉眼振，也就是懸垂位若出現順時鐘迴轉眼振，當快速坐起時則出現逆時鐘迴轉眼振，這是BPPV特有的時鐘反轉眼振，因為耳石在半規管中的滾動方向。Positional and positioning vertigo and nystagmus syndromes can be attributed to either peripheral or central vestibular dysfunction. The most common form is benign paroxysmal positioning vertigo which is caused by cupulolithiasis into the posterior semicircular canal. Other labyrinthine manifestations such as positional alcohol nystagmus, positional nystagmus with macroglobulinaemia and "heavy water" or glycerol ingestion occur because of a specific gravity differential between the cupula and the endolymph (buoyancy mechanism). Neurovascular compression of the vestibular nerve may be a causative factor for "disabling positional vertigo" which is an insufficiently described entity. Hesitation is highly justifiable since retromastoid craniectomy for microvascular decompression is the recommended management. Central positional vertigo is either induced by head movements which result in a transient ischaemia of the ponto-medullary brainstem, or by a change in head position relative to the gravitational vector. The latter is comprised of at least three forms: positional downbeat nystagmus (nodulus), positional nystagmus without concurrent vertigo, and positional vertigo with nystagmus. The site of the lesion is always near the fourth ventricle and the vestibular nuclei. The most probable explanation for the positional response is a vestibular tone imbalance caused by disinhibition of the vestibular reflexes on perception, eye, head and body position.

● Stenver's view of X-ray 史坦弗角度X光片攝影：

在放射學中，對顱骨進行傾斜觀察，以建立對岩狀骨，骨性迷路和內耳道的更好觀察。In radiology, oblique view of skull, to establish better views for petrous bone, bony labyrinth and internal auditory canal. Stenvers projection taken to demonstrate internal auditory canal and temporal bones.anterior projections. 12 degree cephalad angulation, with head rotated 45 degrees from AP. With patient head in RPO position, CR is directed below left zygoma and centered to mastoid. Bilateral views done. Older editions of Merrill positioning books will have full description as well as other references. Newer editions tend to lack mastoids views because of the predominance of CT imaging for this study today. Named after the physician Hendrik Willem Stenvers (1889–1973) from Utrecht.

＜亨德里克·威廉·史丹弗是荷蘭神經科醫生. Hendrik Willem Stenvers ( Deventer , February 16 1889 - Zeist , August 26 1973 ) was a Dutch neurologist.＞

● Stickler's syndrome 史蒂克勒症 (= hereditary progressive arthro-ophthalmodystrophy 遺傳性漸進性關節眼營養不良)：

會造成聽損，可能眩暈。一種結締組織病，包括近視，白內障，視網膜脫離的眼部表現；傳導性和感音神經性聽力損失；面中部發育不良和顎裂(單獨存在或作為羅賓併發症的一部分)；輕度脊椎骨骼發育不良和/或早發性關節炎。此症在家系內和家系間均可出現表型變異；家系間的變異性可以部分以位點和等位基因異質性解釋。A group of very rare genetic disorders affecting connective tissue, specifically collagen. Stickler syndrome is a subtype of collagenopathy, types II and XI. Stickler syndrome is characterized by distinctive facial abnormalities, ocular problems, hearing loss, and joint and skeletal problems. It was first studied and characterized by Gunnar B. Stickler in 1965.

＜史蒂克勒是德國兒科醫生. Gunnar B. Stickler (13 June 1925 – 4 November 2010) was a Germany pediatrician.＞

● Susac's syndrome 蘇薩克症 (= retinocochleocerebral vasculopathy 視網膜耳蝸腦血管病)：

可能眩暈/頭暈。一種極為罕見的微血管疾病，會造成腦病變、分枝性視網膜靜脈阻塞和聽覺障礙。此疾病以John Susac博士(1940–2012)的名字命名，他在1979年首次發現此疾病。A very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. The cause is unknown but the current thinking is that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness. Despite this being an extremely rare disease, there are 4 registries collecting data on the illness; two are the United States, one in Germany, and one in Portugal. Susac's syndrome is named for Dr. John Susac (1940–2012), of Winter Haven, Florida, who first described it in 1979. Susac's syndrome is a very rare disease, of unknown cause, and many persons who experience it do not display the bizarre symptoms named here. Their speech can be affected, such as the case of a female of late teens who suffered speech issues and hearing problems, and many experience unrelenting and intense headaches and migraines, some form of hearing loss, and impaired vision. The problem usually corrects itself, but this can take up to five years. In some cases, subjects can become confused. The syndrome usually affects women around the age of 18 years, with female to male ratio of cases of 2:1. William F. Hoyt was the first to call the syndrome "Susac syndrome" and later Robert Daroff asked Dr. Susac to write an editorial in Neurology about the disorder and to use the eponym of Susac syndrome in the title, forever linking this disease with him.

＜約翰‧蘇薩克是美國神經科醫生. John O. Susac, MD (1940–2012) was a American neurologist.＞

● Schwabach's test 史瓦巴赫測驗：

在施瓦巴赫聽力測試中，將患者的骨傳導與檢查者的骨傳導進行比較(假定檢查者的聽力正常)。In Schwabach's Test for hearing, the patient's bone conduction is compared with that of the examiner (presuming that the examiner's hearing is normal). Procedure: A vibrating tuning fork is placed on the mastoid process of the patient. Once the patient signals that he/she can no longer hear the sound (ie, the sound fades completely), the examiner places the tuning fork on his/her mastoid process. Interpretation: a) Normal Schwabach - If the examiner and patient hears the sound equally well, then the patient's hearing is normal. b) Prolonged Schwabach - If the bone conduction of the patient is better than the examiner (ie, patient can hear the sound for a longer period due to the absence of masking effect of environmental noise), patient is suffering from conductive deafness. c) Diminished Schwabach - If the bone conduction of the patient is worse compared to the examiner (ie, patient stops hearing the sound sooner than the examiner), patient is suffering from sensorineural deafness.

＜史瓦巴赫是德國耳科醫生. Dagobert Schwabach (1846 - 1920) was a Germany otologist.＞

● Teal's test 替爾測驗：

Chimani, Moos, Stengers and Teals test are very useful in detecting non-organic hearing loss. Teals test is used in patients who claim to hear only by bone conduction. Tuning fork is first placed on the mastoid process and the person hears, then non vibratory fork is placed on the mastoid and vibratory fork in front of the ear to confuse the patient about the source of the sound.

● Treacher-Collins syndrome 崔契爾-柯林斯症：

可能聽損。一種先天性臉頰骨及下頷骨發育不全疾病，又稱為「下頷骨顏面發育不全」。此疾病屬於體染色體顯性遺傳疾病，致病原因為位於第五對染色體上的TCOF1基因(5q32-33.1)發生缺陷所致，大部分患者是因此基因偶發性突變而罹病，另一部分患者具有家族史，意即遺傳自父母親其中一人之缺陷基因。此疾病之臨床症狀差異很大，許多成人患者症狀十分輕微不易診斷出來，待生育此病症之患童才發現自身亦為患者。A genetic disorder characterized by deformities of the ears, eyes, cheekbones, and chin. The degree to which a person is affected, however, may vary from mild to severe. Complications may include breathing problems, problems seeing, cleft palate, and hearing loss. Those affected generally have an average intelligence. TCS is usually autosomal dominant. More than half the time it occurs as a result of a new mutation rather than being inherited from a person's parents. The involved genes may include TCOF1, POLR1C, or POLR1D. Diagnosis is generally suspected based on symptoms and X-rays, and potentially confirmation by genetic testing. Treacher Collins syndrome is not curable. Symptoms may be managed with reconstructive surgery, hearing aids, speech therapy, and other assistive devices. Life expectancy is generally normal. TCS occurs in about one in 50,000 people. The syndrome is named after Edward Treacher Collins, an English surgeon and ophthalmologist, who described its essential traits in 1900.

＜愛德華·崔契爾·柯林斯是英國的外科醫生和眼科醫生. Edward Treacher Collins (28 May 1862 – 13 December 1932) was an English surgeon and ophthalmologist.＞

● Trotter's syndrome 特羅特症：

會造成聽損。鼻咽癌晚期可能出現的一組神經症狀。A cluster of symptoms associated with certain types of advanced nasopharyngeal carcinoma. The cause of pain is the mandibular nerve of the foramen ovale, through which the tumor enters the calvarium. Symptoms include the following: (1) Unilateral conductive deafness due to middle ear effusion, (2) Trigeminal neuralgia due to perineural spread, (3) Soft palate immobility, (4) Difficulty opening mouth.

● Tullio's phenomenon 圖利歐現象:

內耳外淋巴液瘻管(fistula)的一種體徵，聲響誘發眩暈、頭暈、噁心或眼振。在上半規管裂損症(superior semicircular canal dehiscence syndrome, SSCD syndrome)中會發生。Sound-induced vertigo, dizziness, nausea or eye movement (nystagmus) was first described in 1929 by the Italian biologist Prof. Pietro Tullio. (1881–1941) During his experiments on pigeons, Tullio discovered that by drilling tiny holes in the semicircular canals of his subjects, he could subsequently cause them balance problems when exposed to sound. The cause is usually a fistula in the middle or inner ear, allowing abnormal sound-synchronized pressure changes in the balance organs. Such an opening may be caused by a barotrauma (e.g. incurred when diving or flying), or may be a side effect of fenestration surgery, syphilis or Lyme disease. Patients with this disorder may also experience vertigo, imbalance and eye movement set off by changes in pressure, e.g. when nose-blowing, swallowing or when lifting heavy objects. Tullio phenomenon is also one of the common symptoms of superior canal dehiscence syndrome (SCDS), first diagnosed in 1998 by Dr. Lloyd B. Minor, The Johns Hopkins University, Baltimore, United States.

＜皮耶特羅‧圖利歐是義大利生物學家. Pietro Tullio. (1881–1941) was a Italian biologist.＞

● Tumarkin's otolith crisis 杜馬丁耳石危象 (= Drop attacks of Tumarkin 杜馬丁跌倒發作)：

一種美尼爾病的晚期症狀，無預警突然跌倒。內淋巴水腫高壓造成耳石器(球囊、橢圓囊)的膜性迷路破裂，會突然跌倒，但不會眩暈，無預警的嚴重跌倒可能致殘。女性多於男性，在美尼爾病的發生率為 5 ~ 72 %不等，是美尼爾病患者產生生活障礙的重要原因。當球囊壓力過大突然破裂時，壓力驟變，機械性地刺激了球囊的耳石與耳石膜，重力造成線性加速度的變化，患者產生被推向前或推向後的感覺，然後出現房間傾倒的感覺，接著突然跌倒，但不會感到天旋地轉。另外引起了前庭脊髓反射(VSR)，刺激大腿與小腿肌肉群收縮，接著姿態張力消失，造成立體感覺之跌倒發作。It usually occurs without warning in patients with late or end-stage endolymphatic hydrops. Drop attacks, known as Tumarkin’s Otolithic Crisis, are when a person falls to the ground with no warning. The person remains awake and does not lose consciousness. Drop attacks are sometimes experienced in the later stages of Ménière's disease. They do not affect everyone. A drop attack feels as if you are being pushed violently and suddenly, causing you to fall. Symptoms are usually gone as quickly as they appear, and you can get up straight away and carry on with whatever you were doing (unless you get a drop attack at the same time as an acute attack of vertigo). During these attacks, the hair cells on your otoliths are suddenly activated, causing your balance to be severely disrupted. Alex Tumarkin, a British otologist, in 1936 described a distinctive drop attack (sudden fall) in 3 subjects with Ménière's disease which he named otolith crisis. Typically, the drop occurred within seconds, without warning, and was not associated with nausea, diaphoresis pallor, or altered awareness; Drop attacks were reported in 7 subjects with a prior history of episodic vertigo, aural symptoms, and asymmetric audiovestibular function, all of whom reported a sensation of being pushed suddenly.

＜艾力克斯‧杜馬丁是英國耳科醫生. Alex Tumarkin (1900-1990) was a British otologist.＞

● Turner's syndrome 特納症 (= Ullrich-Turner syndronme 烏利區-特納症, Gonodal dysgenesis 性腺發育不良) (= 45,X, or 45,X0)：

會造成聽損，可能眩暈。是雌性個體因X染色體部分或完全缺失而引發的疾病。透納氏症患者的症狀各異，患者常常會有短而成蹼狀的頸部、低耳位、頸後髮際線較低、身材矮小，出生時即手腳水腫等特徵。一般來說，透納氏症患者沒有月經、乳房不發育而且不孕，且患有先天性心臟病、糖尿病以及甲狀腺功能低下症的機率較正常人高。大部分的透納氏症患者的智力水平正常，但許多患者的空間視覺能力低下，比如學習數學所需要的空間視覺能力。透納氏症患者有視覺和聽覺障礙的機率也較正常人高。A genetic condition in which a female is partly or completely missing an X chromosome. Signs and symptoms vary among those affected. Often, a short and webbed neck, low-set ears, low hairline at the back of the neck, short stature, and swollen hands and feet are seen at birth. Typically, they develop menstrual periods and breasts only with hormone treatment, and are unable to have children without reproductive technology. Heart defects, diabetes, and low thyroid hormone occur more frequently. Most people with TS have normal intelligence. Many have troubles with spatial visualization that may be needed for mathematics. Vision and hearing problems occur more often.

＜特納是美國內分泌醫生. Henry Hubert Turner (August 28, 1892 – August 4, 1970) was an American endocrinologist.＞

● Usher's syndrome 尤塞症 (= Hallgren syndrome 霍格倫症, Usher–Hallgren syndrome, Retinitis pigmentosa-dysacusis syndrome 色素性視網膜炎-聽聲不適症 or Dystrophia retinae dysacusis syndrome 視網膜營養不良-聽聲不適症)：

會造成聽損，可能眩暈。一種體染色體隱性遺傳性疾病，主要特徵為聽力受損及漸進性的視力喪失。尤塞綜合症有三種類型，患者會有 Clarin-1 基因突變的情形，會導致耳蝸內毛細胞表現缺失，進而使聽覺喪失。第一型尤塞症，在出生時就有重度失聰和嚴重的平衡問題。第二型尤塞症，在出生就有中度至重度聽力損失和正常的平衡。第三型尤塞症較罕見，在出生時有正常的聽力和視力，有些人可能在以後出現平衡問題。即使是在同一個家庭，聽力和視力的惡化也因人而異。夜盲往往在十幾歲才開始，逐漸慢慢地惡化。A rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment. It is a major cause of deafblindness and is at present incurable. Usher syndrome is classed into three subtypes (I, II and III) according to the genes responsible and the onset of deafness. All three subtypes are caused by mutations in genes involved in the function of the inner ear and retina. These mutations are inherited in an autosomal recessive pattern. The occurrence of Usher syndrome varies across the world and across the different syndrome types, with rates as high as 1 in 12,500 in Germany to as low as 1 in 28,000 in Norway. Type I is most common in Ashkenazi Jewish and Acadian populations, and type III is rarely found outside Ashkenazi Jewish and Finnish populations. Usher syndrome is named after Scottish ophthalmologist Charles Usher, who examined the pathology and transmission of the syndrome in 1914.

＜查爾斯·霍華德·尤塞是蘇格蘭眼科醫生. Charles Howard Usher (2 March 1865 – 3 March 1942) was a Scottish ophthalmologist from Edinburgh.＞

● Vannucchi maneuver 范努奇手法：

水平半規管BPPV的治療手法。Vannucchi maneuver就是FPP(forced prolonged positioning)強制長時間側躺法。Vannucchi maneuver, also called "forced prolonged positioning" : incomplete. Simply sleeping with the "affected" ear up has been reported to cure about 75% of patients (see Vannucchi et al, 1997). This is the second to last step of the log-roll (position 3 above). Considering the mechanics of the situation, one would expect that bad-ear up would work only for case where the debris is close to coming out already -- i.e. the geotrophic variant of BPPV. It would not be expected to work for the ageotropic variant of lateral canal BPPV. The biophysical rationale for this maneuver is puzzling. Debris probably does not take all night to sediment. 10 minutes, in theory, is plenty.

● Vannucchi-Asprella maneuver 范努奇-阿斯普雷拉手法：

水平半規管BPPV的治療手法。Vannucchi-Asprella" maneuver則是反作用力緩慢躺下的慣性流動力去將耳石復位，先平躺，頭快速轉向患側，快速坐起來，頭轉正，最後緩慢躺下。Another Vannucchi maneuver is the "Vannucchi-Asprella" maneuver (2005). In this maneuver, while supine, the head is rapidly rotated away from the "bad" ear, then moved into sitting, then slowly aligned with the body, and then returned to supine. This maneuver attempts to use inertial force to displace otoconia. In our opinion, inertial force is not likely to move debris as intertial force is less than gravitational force, but perhaps it "breaks loose" otoconia adherent to the canal wall. We advise against this one due to lack of logic and also we think it best to avoid rapid head movements for safety.

● Vogt-Koyanagi-Harada (VKH) syndrome 原田氏症：

可能眩暈。一種罕見的自體免疫多系統疾病。自體免疫反應攻擊含黑色素的細胞，影響到眼睛脈絡膜、皮膚、腦膜、內耳膜。主要表現為侵犯眼睛造成顆粒性葡蔔膜炎，內耳方面可能出現聽損、耳鳴、眩暈。A multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes.[2][3] VKH may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. Vogt-Koyanagi-Harada (VKH) disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting young adults. Originally, VKH disease was classified as two separate entities: (1) Vogt-Koyanagi syndrome , characterized by chronic severe anterior uveitis, alopecia, poliosis, cutaneous as well as perilimbal vitiligo (also known as Sugiura’s sign), and dysacusia. (2) Harada’s disease, characterized by bilateral exudative uveitis accompanied by pleocytosis of cerebrospinal fluid. Since there is much overlapping of signs and symptoms between the two entities, in 1932 Babel suggested to call the entity Vogt-Koyanagi-Harada disease. The incidence of VKH will vary depending on the geographic location and the ethnicity encountered. The disease primarily affects darkly pigmented races. In Japan it accounts for 6.8% to 9.2% of uveitis cases, meanwhile in the United States it hovers around 1%-4%. The majority of the cases found are around the second and fifth decades of life. Women have been reported as being more affected than men; but this will vary depending on the population studied.

＜瑞士眼科醫生. Alfred Vogt (1879-1943) was a Swiss ophthalmologist. ＞

＜日本眼科醫生. Yoshizo Koyanagi (1880-1954) and Einosuke Harada (1892-1946) were Japan ophthalmologists.＞

● von Recklinghausen's disease 馮銳克林豪森病 (= Neurofibromatosis type I 第一型神經纖維瘤)：

NF-1可能聽損，NF-2造成聽損、眩暈機率更高。神經纖維瘤(NF)是一種最常見的神經科單一基因疾病，依照臨床表現和致病基因的不同，分為兩種類型：NF1和NF2。NF1的發生率全世界皆在1/4000，而NF2則較為罕見，發生率為1/40000，依此估計，台灣約有5500人為NF1的患者。NF1的病徵主要出現於周邊神經系統，最常見的為表皮有大小不一的神經纖維瘤，和牛奶咖啡斑，眼睛的虹膜上有特殊的結節，少數個案其神經纖維瘤亦可出現於中樞神經系統，另外患者亦有可能出現智障、脊椎側彎，和其他骨骼病變等。NF2則主要侵犯中樞神經系統，其神經纖維瘤最常出現於聽覺神經，皮膚的病變則很少出現。NF1的基因位於第17號染色體，而NF2的基因則位於第22號染色體。A complex multi-system human disorder caused by the mutation of a gene on chromosome 17 that is responsible for production of a protein called neurofibromin which is needed for normal function in many human cell types. NF-1 causes tumors along the nervous system which can grow anywhere on the body. NF-1 is one of the most common genetic disorders and is not limited to any person's race or sex. NF-1 is an autosomal dominant disorder which means that mutation or deletion of one copy (or allele) of the NF-1 gene is sufficient for the development of NF-1, although presentation varies widely.

＜馮銳克林豪森是德國病理科醫生. Friedrich Daniel von Recklinghausen (2 December 1833 – 26 August 1910) was a German pathologist.＞

● Waardenburg's syndrome 瓦登伯格症：

可能眩暈。一種罕見的遺傳性疾病，又叫藍眼珠(Blue iris)。藍眼珠只是瓦登伯格症候群諸多症狀當中的一個，患者的眼珠雖呈藍色，視力卻完全不受影響，需要注意的反倒是容易合併聽障及長期便秘的問題，必須早期發現及矯治，以使他們得到良好的療效，健康正常的成長。一般而言，瓦登伯格症有以下幾項特徵：1.藍眼珠或兩眼一藍一正常，稱為虹膜異色症，不過也有部分患者眼珠顏色正常；2.單耳或雙耳聽力障礙，發生率為9~38％；3.額前一撮白髮或易有少年白；4.兩眼眼距較寬，但瞳孔間距離正常，又稱為內眥外移；5.鼻根寬闊且鼻翼發育不良；6.併眉；7.下巴較大、較寬；8.有的人會長期便秘，一小部分病人甚至同時罹患了先天性巨結腸症；9.少數的病人會有皮膚脫色斑、唇顎裂、先天性心臟病或肌肉、骨骼異常。並不是每位瓦登伯格症的患者都會有以上的症狀，深入去研究發現可以將此症細分為四型：第一型為典型的瓦症，具有上述1.至6.的特徵；第二型則為剔除了第一型的內眥外移一項特徵；第三型為第一型的特徵另外再合併有顏面、肌肉或骨骼的異常；第四型則為第一型或第二型合併先天性巨結腸症。A rare genetic condition characterised by at least some degree of congenital hearing loss and pigmentation deficiencies, which can include bright blue eyes (or one blue eye and one brown eye), a white forelock or patches of light skin. These basic features constitute type 2 of the condition; in type 1, there is also a wider gap between the inner corners of the eyes called telecanthus, or dystopia canthorum. In type 3, which is rare, the arms and hands are also malformed, with permanent finger contractures or fused fingers, while in type 4 the person also has Hirschsprung's disease, which is a congenital lack of nerves in the intestines leading to bowel dysfunction. There also exist at least two types (2E and PCWH) that can result in central nervous system symptoms such as developmental delay and muscle tone abnormalities. The syndrome is caused by mutations in any of several genes that affect the division and migration of neural crest cells during embryonic development (though some of the genes involved also affect the neural tube). Neural crest cells are stem cells left over after the closing of the neural tube that go on to form diverse non-central nervous system cells in different parts of the body, including melanocytes, various bones and cartilage of the face and inner ear and the peripheral nerves of the intestines. Type 1 is caused by a mutation in the PAX3 gene, while the gene that most often causes type 2 when mutated is MITF. Type 3 is a more severe presentation of type 1 and is caused by a mutation in the same gene, while type 4 is most often caused by a mutation in SOX10. Other genes can also cause the different types, and some of these have been given their own lettered subtypes. Most types are autosomal dominant. Descriptions of the syndrome date back to at least the first half of the 20th century, however it is named after Dutch ophthalmologist and geneticist Petrus Johannes Waardenburg, who described it in 1951. Its subtypes were progressively discovered in the following decades and had genes attributed to them mostly in the 1990s and 2000s.

＜瓦登伯格是荷蘭眼科醫生，遺傳學家. Petrus Johannes Waardenburg (3 June 1886, Nijeveen, Drenthe – 23 September 1979) was a Dutch ophthalmologist, geneticist,＞

● Waldenstrom's macroglobulinemia 華登斯強巨球蛋白血症：

可能眩暈。血液高黏滯性。是一種淋巴癌，也就是淋巴系統的癌症。這種病症會發生在一種稱為 B 淋巴球或 B 細胞的白血球中，這種白血球成熟時通常會成為漿細胞(plasma cell)，其功能是生產免疫球蛋白 (抗體)，協助身體對抗感染。而在 WM 中，B 細胞的成熟晚期出現惡性變化，並且持續增殖出一群完全相同的細胞，這種情況主要發生在骨髓中，但是也會發生於淋巴結，以及淋巴系統的其他組織和器官。這些株落細胞會過量生產一種稱為 IgM 的特定類別抗體。在顯微鏡下，WM 細胞同時擁有 B 淋巴球和漿細胞的特徵，被稱為淋巴漿細胞。因此，WM 被歸類為一種非何杰金淋巴瘤 (non-Hodgkin’s lymphoma)，稱為淋巴漿細胞淋巴瘤 (LPL)。大約 95% 的 LPL 病例均為 WM，但 WM 是非常罕見的疾病，在美國每年只有 1,500 名左右的患者，被診斷為罹患 WM。WM 通常是惰性 (成長緩慢) 疾病，可以當作慢性疾病管理數年。不過，這種疾病目前還無法治癒。WM 的淋巴漿細胞在骨髓和其他部位增殖後，可能會干擾該處的正常功能。在製造血球細胞的骨髓中，WM 細胞會「排擠」正常血球細胞，並可能導致正常血球細胞數減少；在淋巴結和其他器官中，WM 細胞可能導致這些組織器官的結構增大，或引發其他併發症。A type of cancer affecting two types of B cells: lymphoplasmacytoid cells and plasma cells. Both cell types are white blood cells. WM is characterized by having high levels of a circulating antibody, immunoglobulin M (IgM), which is made and secreted by the cells involved in the disease. WM is an "indolent lymphoma" (i.e., one that tends to grow and spread slowly) and a type of lymphoproliferative disease which shares clinical characteristics with the indolent non-Hodgkin lymphomas. WM is commonly classified as a form of plasma cell dyscrasia. Similar to other plasma cell dyscrasias that, for example, lead to multiple myeloma, WM is commonly preceded by two clinically asymptomatic but progressively more pre-malignant phases, IgM monoclonal gammopathy of undetermined significance (i.e. IgM MGUS) and smoldering Waldenström macroglobulinemia. The WM spectrum of dysplasias differs from other spectrums of plasma cell dyscrasias in that it involves not only aberrant plasma cells but also aberrant lymphoplasmacytoid cells and that it involves IgM while other plasma dyscrasias involve other antibody isoforms.

＜華登斯強是瑞典內科醫師. Jan Gösta Waldenström (17 April 1906 – 1 December 1996) was a Swedish doctor of internal medicine.＞

● Wallenberg's syndrome 瓦倫堡症 (= PICA syndrome 後下小腦症, Lateral medullary syndrome 外側延髓症候群)：

會造成眩暈。由延髓病變所致壹種疾病,臨床上以腦神經如舌咽神經,迷走神經,舌下神經為主要表現。是腦幹梗死最常見類型。導致眩暈、嘔吐、眼球震顫(前庭神經核)；交叉性感覺障礙(三叉神經背束核及對側交叉的脊髓丘腦束受損)；同側Horner征(下行交感神經纖維受損)；飲水嗆咳、吞咽困難和聲音嘶啞(疑核受損)；同側小腦性共濟失調(繩狀體或小腦受損)。小腦後下動脈解剖變異較多，常見不典型臨床表現。A neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery. Lateral medullary syndrome is also called Wallenberg's syndrome, posterior inferior cerebellar artery (PICA) syndrome and vertebral artery syndrome. This syndrome is characterized by sensory deficits that affect the trunk and extremities contralaterally (opposite to the lesion), and sensory deficits of the face and cranial nerves ipsilaterally (same side as the lesion). Specifically a loss of pain and temperature sensation if the lateral spinothalamic tract is involved. The cross body finding is the chief symptom from which a diagnosis can be made. Patients often have difficulty walking or maintaining balance (ataxia), or difference in temperature of an object based on which side of the body the object of varying temperature is touching. Some patients may walk with a slant or suffer from skew deviation and illusions of room tilt. The nystagmus is commonly associated with vertigo spells. These vertigo spells can result in falling, caused from the involvement of the region of Deiters’ nucleus. Common symptoms with lateral medullary syndrome may include difficulty swallowing, or dysphagia. This can be caused by the involvement of the nucleus ambiguus, as it supplies the vagus and glossopharyngeal nerves. Slurred speech (dysarthria), and disordered vocal quality (dysphonia) are also common. The damage to the cerebellum or the inferior cerebellar peduncle can cause ataxia. Damage to the hypothalamospinal fibers disrupts sympathetic nervous system relay and gives symptoms that are similar to the symptoms caused by Horner syndrome – such as miosis, anhidrosis and partial ptosis. Palatal myoclonus, the twitching of the muscles of the mouth, may be observed due to disruption of the central tegmental tract. Other symptoms include: hoarseness, nausea, vomiting, a decrease in sweating, problems with body temperature sensation, dizziness, difficulty walking, and difficulty maintaining balance. Lateral medullary syndrome can also cause bradycardia, a slow heart rate, and increases or decreases in the patients average blood pressure.

＜阿道夫·瓦倫堡是德國的內科醫生和神經科醫生. Adolf Wallenberg (10 November 1862 – 10 April 1949) was a German internist and neurologist.＞

● Weber's test 韋伯測驗：

又稱骨導偏向試驗，系比較兩耳骨導聽力的強弱。取C256或C512振動的音叉柄底置於前額或頭頂正中，讓患者比較哪一側耳聽到的聲音較響。A screening test for hearing performed with a tuning fork. It can detect unilateral (one-sided) conductive hearing loss (middle ear hearing loss) and unilateral sensorineural hearing loss (inner ear hearing loss). The test is named after Ernst Heinrich Weber (1795–1878). Conductive hearing ability is mediated by the middle ear composed of the ossicles: the malleus, the incus, and the stapes. Sensorineural hearing ability is mediated by the inner ear composed of the cochlea with its internal basilar membrane and attached cochlear nerve (cranial nerve VIII). The outer ear consisting of the pinna, ear canal, and ear drum or tympanic membrane transmits sounds to the middle ear but does not contribute to the conduction or sensorineural hearing ability save for hearing transmissions limited by cerumen impaction (wax collection in the ear canal). The Weber test has had its value as a screening test questioned in the literature.

＜恩斯特·海因里希·韋伯是德國醫生，被認為是實驗心理學的奠基人之一. Ernst Heinrich Weber (24 June 1795 – 26 January 1878) was a German physician who is considered one of the founders of experimental psychology.＞

● Wegener's granulomatosus 韋格納肉芽腫 (= Granulomatosis with polyangiitis 血管性肉芽腫)：

可能眩暈。一種壞死性肉芽腫性血管炎，屬自體免疫性疾病，病程反覆，其病理以血管壁的炎症為特徵，主要侵犯上呼吸道(鼻竇)、下呼吸道(肺部)和腎臟(腎絲球炎)，韋格納肉芽腫通常以鼻黏膜和肺組織的局灶性肉芽腫性炎症為開始，繼而進展為血管的彌漫性壞死性肉芽腫性炎症。臨床常表現為鼻和副鼻竇炎、肺病變和進行性腎功能衰竭。還可累及關節、眼、皮膚，亦可侵及眼、心臟、神經系統及耳等。An extremely rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs and kidneys can be fatal. The cause of GPA is unknown. Genetics have been found to play a role in GPA though the risk of inheritance appears to be low. GPA treatment depends on the severity of the disease. Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control. Plasma exchange is also used in severe cases with damage to the lungs, kidneys, or intestines. The number of new cases of GPA each year is estimated to be 2.1-14.4 new cases per million people in Europe. GPA is rare in Japanese and African-American populations but occurs more often in people of Northern European descent. GPA is estimated to affect 3 cases per 100,000 people in the United States and equally affects men and women.

＜弗里希·韋格納博士是德國病理學家. Friedrich Wegener (April 7, 1907, Varel – July 9, 1990, Lübeck) was a German pathologist.＞

● Wernicke encephalopathy 魏尼克腦病：

可能眩暈。一種因營養不良(Acquired thiamine deficiency 後天性維生素B1缺乏)造成的腦病變表現。臨床上會有神智不清、眼動障礙、眼振及厲害的搖晃，造成此疾病致病機轉就是維生素B1缺乏，最常見在酒精濫用的病人當中，此外洗腎透析的病人、AIDS、化療、胃整形手術者也容易產生，大量攝取碳水化合物、身體厲害的壓力如外傷、感染等均有可能會加重該病症，但並非大量攝取碳水化合物即會造成魏尼克腦病。The presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of B-vitamin reserves, in particular thiamine (vitamin B1). The condition is part of a larger group of thiamine deficiency disorders, that includes beriberi in all its forms, and alcoholic Korsakoff syndrome. When it occurs simultaneously with alcoholic Korsakoff syndrome it is known as Wernicke–Korsakoff syndrome. Classically, Wernicke encephalopathy is characterised by the triad – ophthalmoplegia, ataxia, and confusion. Around 10% of patients exhibit all three features, and other symptoms may also be present. While it is commonly regarded as a condition peculiar to malnourished people with alcohol misuse, it can be caused by a variety of diseases. It is treated with thiamine supplementation, which can lead to improvement of the symptoms and often complete resolution, particularly in those where alcohol misuse is not the underlying cause.[citation needed] Often other nutrients also need to be replaced, depending on the cause. Wernicke encephalopathy may be present in the general population with a prevalence of around 2%, and is considered underdiagnosed; probably, many cases are in patients who do not have commonly-associated symptoms.

＜卡爾·魏尼克是德國醫生、解剖學家、精神病學家與神經病理學家Carl Wernicke (15 May 1848 – 15 June 1905) was a German physician, anatomist, psychiatrist and neuropathologist.＞

● Wilde's incision 魏爾德切開法：

一種乳突切除術的耳後切開法。Post-aural incision is used for a variant mastoiditis drainage, and was named after Sir William Wilde, an ENT surgeon in Dublin who first described it at the end of the nineteenth century.

＜魏爾德是愛爾蘭眼耳鼻喉科醫生. Sir William Robert Wills Wilde FRCSI (March 1815 – 19 April 1876) was an Irish otolaryngologist and ophthalmologist.＞

● Wildervanck's syndrome 魏爾德凡克症 (= Cervico-oculo-acoustic syndrome 頸椎眼耳症)：

會造成聽損。Wildervanck syndrome comprises a triad of:Duane syndrome, Klippel-Feil anomaly (fused cervical vertebrae), congenital hearing loss. Wildervanck syndrome is a developmental disorder that may be characterized by accessory tragi.

● Willis' paracusis 威利斯聽覺倒錯：

耳硬化症的一個症狀，在吵雜的環境下，耳硬化症患者的語言聽力更好。The symptom of paracusis Willisii, where the patient perceives speech better in a noisy background, is said to be frequently present in otosclerosis as well as other causes of conductive hearing loss. It is said to be due to the tendency of people to speak louder in noisy environments. This being said, it is still difficult to see the logic and we ourselves have never seen a patient who has volunteered this observation. Another possible explanation is that persons with sensorineural hearing loss have great problems hearing in noise, perhaps because of loss of outer hair cells, but this is simply not the case in conductive hearing loss.

＜湯瑪士‧威利斯是英國英國醫生，在解剖學，神經病學和精神病學史上發揮了重要作用. Thomas Willis (27 January 1621 – 11 November 1675) was an English doctor who played an important part in the history of anatomy, neurology and psychiatry.＞

● Willis' circle 威利斯環 (= circle of Willis, loop of Willis, cerebral arterial circle, and Willis polygon)：

連通腦部前循環、後循環血流的血管。威利斯環可以對供應腦組織的動脈進行血液調配，防止腦血液循環的過剩或不足。當組成威利斯環的某一動脈或某一部分出現阻塞或者狹窄，可以通過調節其他血管的血流量彌補缺少的部分，保證腦的血流灌注，避免出現缺血的症狀，維持腦的營養和機能活動。A circulatory anastomosis that supplies blood to the brain and surrounding structures. The circle of Willis is a part of the cerebral circulation and is composed of the following arteries: Anterior cerebral artery (left and right), Anterior communicating artery, Internal carotid artery (left and right), Posterior cerebral artery (left and right), Posterior communicating artery (left and right). The middle cerebral arteries, supplying the brain, are not considered part of the circle.

醫院, hospital, 醫學中心, medical center, 院長, dean, 主任, director, 教授, professor, 博士, PhD, 醫師, doctor, 權威, expert, 推薦, recommended, 效果, effect, 一次見效, effective, 健保, 保險, insurance, 診斷證明書, certificate, 自費, 費用, charge, 價格, price, 萬, 千, 百, 元, dollar, 檢查, examination, 門診, clinic, 急診, emergence, 住院, admission, 手術, surgery, 藥, medication, 飲食, diet, 生活, life, 運動, exercise, 上班, go to work, 注意, careful, 術後, post-operation, 恢復期, convalescence, 正常現象, normal, 不正常abnormal, 瘀青, bruise, 消腫, swelling, 發炎, inflammation, 感染, infection, 副作用, side effect, 後遺症, 按摩, massage, 疤痕, scar, 動暈症, Motion sickness, 良性陣發性姿勢性暈眩, BPPV, benign paroxysmal positional vertigo, 美尼爾病, Meniere's disease, 拉莫葉滋症, Lermoyez syndrome, 遲發性內淋巴水腫, Delayed endolymphatic hydrops, 小兒良性陣發性暈眩症, BPVC, benign paroxysmal vertigo of childhood, 內耳迷路震盪, Labyrinthine concussion, 外淋巴瘻管, Perilymphatic fistula, 上半規管裂損症, SSCD, semicircular canal dehiscence syndrome, 前庭神經炎, Vestibular neuronitis, 侖謝亨特症, Ramsay Hunt syndrome, 梅毒性內耳迷路炎, Syphilic labyrinthitis, 脊椎腦底動脈循環不全, VBI, vertebrobasilar insufficiency, 內耳迷路中風, Labyrinthine infarction, 聽神經瘤, 小腦橋腦角瘤, Acoustic neuroma, CP (cerebellopontine) angle tumor, 自體免疫內耳疾病, AIED, autoimmune inner ear disease, 寇甘症, Cogan's syndrome, 偏頭痛, Migraine, 暈眩型癲癇, Epileptic vertigo, 新陳代謝型暈眩, Metabolic vertigo, 藥致耳毒性, Drug-induced ototoxicity 多發性硬化症, Multiple sclerosis, 神經退化性疾患, Neurodegenerative disorder, 陣發性不協調第二型, Episodic ataxia type 2, 阿諾德-奇阿里畸形, Arnold-Chiari malformation, 暈眩, vertigo, 美尼爾病, Meniere’s disease, 耳鳴, tinnitus, 喉咽逆流, laryngopharyngeal reflux, 鼻骨折, nasal bone fracture, 鼻扭曲症, twisted nose, 各種耳鼻喉頭頸疾病, various diseases of otorhinolaryngology head and neck surgery, 耳前瘻管, pre-auricular fistula, 耳廓外傷, auricular trauma, 耳廓血腫, othematoma, 耳痛, otalgia, 耳癢, auricular itching, 耳流膿, purulent ear discharge, 耳垢, cerumen, 閉塞性角化症, keratosis obturans, 外耳炎, otitis externa, 耳鰴菌症, otomycosis, 異物, FB in ear, 耳帶狀疱疹, herpes zoster oticus, Ramsay Hunt症, 顏面神經麻痺, facial palsy, Bell麻痺, 顳顎關節症, TM joint disorder, 中耳炎, otitis media, 耳膜破洞, perforation of tympanic membrane, 耳膜硬化症, tympanosclerosis, 乳突炎, mastoiditis, Bezold膿腫, abscess, 珍珠瘤, cholesteatoma, 膽脂瘤, 聽小骨疾病, ossicular chain disorder, 耳硬化症, otosclerosis, 耳膜內陷, retracted tympanic membrane, 耳壓不平衡, tympanic pressure imbalance, 耳咽管開放症, e-tube patulus, 突發性耳聾, sudden sensorineural hearing loss, 重聽, 聽力障礙, 聽力損失, 聲創傷, acoustic trauma, 助聽器, hearing aid, 聽力語言訓練, 電子耳, cochlear implant, 頭暈, dizziness, 動暈症, motion sickness, 姿勢性暈眩, benign paroxysmal positional vertigo, 耳石脫落症, Lermoyez症, 前庭神經炎, vestibular neuronitis, 內耳迷路炎, labyrinthitis, 內耳迷路梅毒, syphilis of labyrinth, 多發性硬化症, multiple sclerosis, 偏頭痛, migraine, 失眠, insomnia, 脊椎顱底動脈循環不良症, vertebrobasilar insufficiency, 神經纖維瘤, neurofibromatosis, 聽神經瘤, acoustic neuroma, 小腦橋腦角腫瘤, cerebellopontine angle tumor, 鼻痛, nasal pain, 鼻癤, nasal vestibular furuncle, 蜂窩性組織炎, cellulitis, 流鼻涕, purulent rhinorrhea, 流鼻血, epistaxis, 異物, FB in nose, 鼻癢, nasal itching, 打噴嚏, sneezing, 過敏性鼻炎, allergic rhinitis, 慢性肥厚性鼻炎, chronic hypertrophic rhinitis, 鼻甲肥大, hypertrophy of nasal turbinate, 血管動力性鼻炎, vasomotor rhinitis, 藥物性鼻炎, rhinitis medicamentosa, 萎縮性鼻炎, atrophic rhinitis, 鼻中膈彎曲deviated nasal septum, 鼻中膈穿孔, perforation of nasal septum, 鼻因性頭痛, rhinogenic headache, 空鼻症, empty nose syndrome, 鼻竇炎, rhinosinusitis, 鰴菌性鼻竇炎, fungal, 鼻息肉, nasal polyp, Aspirin三徵, triad, 上頷竇後鼻孔息肉, choncal troanal polyp, 囊狀纖維化, cystic fibrosis, Kartagener症, Samter三徵, triad, 黏液囊腫, mucocele, 鼻唇囊腫nasolabial cyst, 嗅覺障礙, olfactory disorder, 倒生性乳頭瘤, inverted papilloa, 鼻腫瘤, nasal tumor, Wegener肉芽腫, granuloma, 鼻涕倒流, postnasal drip, 鼻淚管阻塞, obstruction of nasolacrimal duct, Tornwaldt囊腫, cyst, 鼻咽脊索瘤, chordoma, 口腔潰瘍, oral ulcer, 口腔白斑, oral leukoprekia, 紅斑, red spot, 黏膜下纖維化, submucosal fibrosis, 口腔食道念珠菌症, oral thrush, Bowen病, 舌頭炎, glossitis, 牙齦炎, gingivitis, 毛狀白斑, hairy leukoplakia, Kaposi肉瘤, sarcoma, 蛤蟆腫, ranula, Eagle症, 扁桃腺炎, tonsillitis, 扁桃腺結石, tonsolith, 口臭, halitosis, 扁桃腺旁膿瘍, peritonsillar abscess, Vincent咽峽炎, angina, 疱疹性咽峽炎, herpangina, 手足口病, HMF disease, 異物, FB, 魚刺, fish bone, 喉嚨癢, itching throat, 顎裂, cleft palate, 咽炎, pharyngitis, 喉炎, laryngitis, 結核性喉炎, tuberculus, 會厭炎, epiglottitis, 喉乳頭狀瘤, laryngeal papilloma, 聲音沙啞, hoarseness, 聲帶炎, vocal corditis, Reinke聲帶水腫, edema, 聲帶溝, sulcus vocalis, 聲帶結節, nodule, 聲帶息肉, polyp, 聲帶囊腫, cyst, 聲帶麻痺, paralysis, 老年性聲帶萎縮, presbylaryngis, 語言障礙, speech, language, 口吃, stutter, 聲門下峽窄, subglottic stenosis, 咳血, hemoptysis, 吞嚥困難, dysphagia, 清喉嚨, throat cleaning, 喉嚨卡卡, throat globus sensation, 喉嚨腫, throat swelling, 火燒心, heart burn, 吐酸, acid regurgitation, 喘鳴, stridor, 打呼, snoring, 打鼾, 睡眠呼吸中止症, sleep apnea, 起床頭暈頭痛, headache, 白天疲憊, malaise, 嗜睡, drowsiness, 記憶力差, poor memory, 脾氣暴躁, short temper, 慢性咳嗽, chronic cough, 深頸部感染, deep neck infection, Ludwig咽峽炎, 頸部腫瘤, neck mass, 甲狀舌管囊腫, thyroglossal duct cyst, 甲狀腺腫瘤, thyroid tumor, 甲狀腺炎, thyroditis, 胸腺瘤, thymoma, 鰓裂囊腫, branchial cleft cyst, 唾液腺結石, sialolithiasis, 唾液腺腫瘤, salivary gland tumor, 混合瘤, mixed, Warthin瘤, Frey症, 腮腺炎, mump, 乾燥症, sjorgren, 頸部淋巴腺腫大, enlargement of neck lymph node, Kikuchi病, Kimura病, 淋巴癌, lymphoma, EB病毒, EB virus, 鼻咽癌, nasopharyngeal carcinoma, 口腔癌, oral cancer, 口咽腫瘤, oropharyngeal, 下咽腫瘤, hypopharyngeal, 喉癌, laryngeal, 食道癌, esophageal, 小兒耳鼻喉, pediatric, 中耳積水, middle ear effusion, 腺樣體肥大, adenoid hypertrophy, 扁桃腺肥大, tonsillar hypertrophy, 發育不良, 生長遲緩, growth retardation, 哮吼, croup, 感冒, common cold, 上呼吸道感染, upper respiratory infection, 腸胃炎, acute gastroenteritis, 喉嚨痛, sore throat, 鼻塞, nasal obstruction, 流鼻水, clear rhinorrhea, 咳嗽, cough, 痰, sputum, 發燒, fever, 頭痛, headache, 肚子痛, abdominal pain, 拉肚子, diarrhea, 流感, influenza, , 全身無力, debility, 全身痠痛, general soreness, 鼻咽血管纖維瘤, angiofibroma, 喉頭軟化症, laryngomalacia, Langerhans細胞組織球增生症, cell histiocytosis, 囊狀水瘤, cystic hygroma, 隆鼻, augmentation rhinoplasty, 鼻整形, 眉心, glabella, 鼻山根, nasion, 鼻樑, dorsum, 鼻頭, nasal tip, 鼻柱, columella, 鼻翼, alar, 鼻基底, subnasale, 鼻唇溝, nasolabial groove, 矮鼻, low dorsum, 塌鼻, 短鼻, short nose, 朝天鼻, nostril show, 寬鼻, wide

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【醫師專欄-耳科】神經耳科學的各種症候群 (含人名姓氏的體徵/現象/症/病/方法)　The syndromes of the neurotology (Last name sign/phenomenon/syndrome/disease/method)

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【醫師專欄-耳科】 神經耳科學的各種症候群 (含人名姓氏的體徵/現象/症/病/方法) The syndromes of the neurotology (Last name sign/phenomenon/syndrome/disease/method)

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【醫師專欄-耳科】神經耳科學的各種症候群 (含人名姓氏的體徵/現象/症/病/方法)　The syndromes of the neurotology (Last name sign/phenomenon/syndrome/disease/method)

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