Welling D B
Ohio State University, Columbus 43210, USA.
Ear Nose Throat J. 1996 May;75(5):301-5.
Immune-mediated inner ear disease, first described by McCabe in 1979, typically presents with an idiopathic, rapidly progressive bilateral sensorineural hearing loss. The course of the hearing loss occurs over weeks to months. It may occur in both sexes and at a variety of ages, but is most common in middle-aged females. It may be accompanied by tinnitus, Meniere's-like vertigo, or more commonly, ataxia or unsteadiness. Approximately 30% of patients will have associated systemic immune-mediated disease. Although refinements in laboratory tests for specific inner ear antigens are being made, and non-specific laboratory indicators of inflammatory or systemic immune disease may be useful in confirming the diagnosis, the most important diagnostic finding is the improvement in hearing seen with a trial of immunosuppressants. This report includes a typical evaluation of the patient with suspected immune-mediated inner ear disease and an illustrative case. Sensorineural hearing loss due to immune-mediated inner ear disease, although unusual as a cause of hearing loss, is important to recognize because early diagnosis and treatment can have a marked effect on the clinical outcome.
免疫介导性内耳疾病于1979年由麦凯布首次描述，通常表现为特发性、快速进展的双侧感音神经性听力损失。听力损失过程持续数周数月。男女均可发病，各年龄段都有，但多见于中年女性。可能伴有耳鸣、梅尼埃样眩晕，或更常见的共济失调或不稳。约30%的患者会伴有全身性免疫介导性疾病。尽管针对特定内耳抗原的实验室检测在不断完善，炎症或全身性免疫疾病的非特异性实验室指标可能有助于确诊，但最重要的诊断依据是试用免疫抑制剂后听力改善。本报告包括对疑似免疫介导性内耳疾病患者的典型评估及一个病例说明。免疫介导性内耳疾病导致的感音神经性听力损失虽不常见，但因其早期诊断和治疗对临床结果有显著影响，所以识别该疾病很重要。
