Hughes G B, Barna B P, Kinney S E, Calabrese L H, Nalepa N J
Department of Otolaryngology and Communicative Disorders, Cleveland Clinic Foundation, OH 44195.
Laryngoscope. 1988 Mar;98(3):251-3. doi: 10.1288/00005537-198803000-00001.
The purpose of this study was to develop a high-risk clinical profile of patients with autoimmune (immune-mediated) inner-ear disease. The records of 52 patients diagnosed over the past 5 years were reviewed. Age, sex, bilateral versus unilateral involvement, otologic symptoms, concomitant systemic immune disease, and presenting clinical diagnoses were recorded. The presenting diagnoses were Cogan's syndrome, Meniere's syndrome, Dandy's syndrome without hearing loss, or progressive sensorineural hearing loss without dizziness. Because Cogan's and Dandy's syndromes were relatively uncommon, the typical high-risk clinical profile was a middle-aged patient (often female) with bilateral, asymmetric, progressive sensorineural hearing loss, with or without dizziness, and occasional systemic immune disease such as rheumatoid arthritis. When a more common clinical diagnosis cannot be reached in suspicious patients, immune laboratory tests should be obtained and a trial of immunotherapy offered. Positive test results and beneficial response to therapy support a presumptive diagnosis of immune inner-ear disease.
本研究的目的是建立自身免疫性（免疫介导性）内耳疾病患者的高风险临床特征。回顾了过去5年中确诊的52例患者的病历。记录了年龄、性别、双侧与单侧受累情况、耳科症状、伴随的全身性免疫疾病以及初诊临床诊断。初诊诊断为科根综合征、梅尼埃综合征、无听力损失的丹迪综合征或无头晕的进行性感音神经性听力损失。由于科根综合征和丹迪综合征相对不常见，典型的高风险临床特征是中年患者（通常为女性），伴有双侧、不对称、进行性感音神经性听力损失，有或无头晕，偶尔伴有类风湿关节炎等全身性免疫疾病。当可疑患者无法得出更常见的临床诊断时，应进行免疫实验室检查并提供免疫治疗试验。检测结果阳性及对治疗的有益反应支持免疫性内耳疾病的推定诊断。
