Hughes G B, Kinney S E, Barna B P, Tomsak R L, Calabrese L H
Otolaryngol Head Neck Surg. 1983 Feb;91(1):24-32. doi: 10.1177/019459988309100106.
Autoimmune inner ear disease was first described by McCabe in 1979. The diagnosis is supported by cell-mediated immune responses to inner ear membrane antigen stimulation. Cogan's syndrome consists of vestibuloauditory dysfunction, ocular inflammation, and nonreactive serologic tests for syphilis. The ocular disease can be controlled by steroids; unfortunately, some patients ultimately become totally deaf. An autoimmune etiology has long been suspected but only recently confirmed by McCabe. In our study two patients with Cogan's syndrome were studied for autoimmune inner ear disease with the use of both cellular and humoral immune tests. Results were compared to normal control subjects. On the basis of test results, preliminary conclusions were that (1) the vestibuloauditory symptoms of Cogan's syndrome are autoimmune in origin; (2) the autoimmune process is mediated through cellular rather than humoral (antibody) pathways; (3) systemic steroids may suppress positive test results; and (4) test results are more likely to be positive when symptoms are acute. Therapeutic implications are significant: the addition of cytotoxic drugs to steroids in selected cases may help prevent total deafness that otherwise might be inevitable.
自身免疫性内耳疾病于1979年由麦凯布首次描述。对内耳膜抗原刺激的细胞介导免疫反应支持这一诊断。科根综合征包括前庭听觉功能障碍、眼部炎症以及梅毒血清学检查无反应。眼部疾病可用类固醇控制；不幸的是，一些患者最终会完全失聪。长期以来一直怀疑其病因是自身免疫性的，但直到最近才被麦凯布证实。在我们的研究中，对两名科根综合征患者进行了自身免疫性内耳疾病的研究，采用了细胞免疫和体液免疫检测方法。并将结果与正常对照受试者进行了比较。根据检测结果，初步结论是：（1）科根综合征的前庭听觉症状起源于自身免疫；（2）自身免疫过程是通过细胞途径而非体液（抗体）途径介导的；（3）全身性类固醇可能会抑制阳性检测结果；（4）症状急性发作时检测结果更可能为阳性。治疗意义重大：在某些病例中，在类固醇治疗基础上加用细胞毒性药物可能有助于预防原本可能不可避免的全聋。
