原田氏病之神經耳科學所見

Neurotological Findings in Harada Disease

原理氏病是一種罕見的自體免疫性疾病，病患會產生抗體侵犯全身含黑素細胞的組織，包括眼睛、內耳、皮膚、毛髮及腦膜等，而表現出各種病狀，諸如視力喪失、內耳症狀（眩暈、耳鳴、重聽）、白斑、禿髮、毛髮變白及頭痛。自1994年4月至1998年3月，計四年間，經本院眼科診斷為原田氏痛者共43名；其中19名（44%）伴有內耳症狀，包括重聽、耳鳴、或眩暈。患者接受詳細的歷史詢問、耳鼻喉局部鏡檢、及一系列的聽力平衡功能檢查。結果19名具內耳症狀的原田氏病患者，計男性10名、女性9名，年齡分佈在16至72歲之間，平均為41歲。在臨床症狀方面，視力模糊19例，聽力喪失12例、頭痛11例、眩暈或頭暈10例。而耳鳴5例。在聽力圖型的分佈，14耳正常、18耳呈高音漸傾型、4耳呈水平型，其他型則有2耳。經給予高劑量類固醇治療後，重聽及眩暈的情況均獲改善。臨床上，當耳鼻喉科醫師面對同時伴有紅眼睛及內耳症狀的病人前來求診時，需考慮原田氏病的可能性，進而照會眼科醫師以確立診斷。

Background；Harada disease, or Vogt-Koyanagi-Harada syndrome, is a rare autoimmune disease characterized by autoantibodies that attack melanocyte-containing tissues or organs such as skin, hair, meninges, uvea, and the inner ear. The clinical manifestations include neurological symptoms(headache and meningismus), dermatological symptoms(alopecia, vitiligo, and poliocytosis), ophthalmological symptoms (vision loss), and inner ear symptoms (vertigo, tinnitus, and hearing loss). A total of 43 patients with Harada disease were treated at our hospital from April 1994 through March 1998. Nineteen of these patients had inner ear symptoms and were referred to the otolaryngological department for further evaluation. Sults: The patients comprised 10 men and 9 women, ranging in age from 16 to 72 years. Clinically, all developed blurred vision, 12 had hearing loss, 11 had headache, and 5 had tinnitus. Audiogram types included sloping type (18 ears), normal hearing (14 ears), flat type (4 ears), and others (2 ears). High-dose steroid treatment greatly improved vertigo in 4 patients. Harada disease should be taken into consideration in the differential diagnosis in patients with red eyes and inner ear symptoms.