Oldenski R
Bayfront Medical Center, St. Petersburg, FL 33701.
J Am Board Fam Pract. 1993 Nov-Dec;6(6):577-81.
Cogan syndrome is an uncommon disorder that presents with symptoms involving the eyes and ears. At its onset, it can mimic many common entities. Family physicians should be aware of Cogan syndrome because it can be successfully treated if it is recognized early in its course. It is one of the few treatable causes of deafness.
MEDLINE files were searched from 1982 to the present for "Cogan's syndrome." Additional references were obtained by cross-referencing bibliographies from available articles.
As first defined in 1945, Cogan syndrome includes nonsyphilitic interstitial keratitis and attacks of vertigo, tinnitus, and hearing loss. Although it usually begins with only one symptom, most patients have both auditory and ocular findings within 1 year of the onset. If untreated, most patients become deaf within 36 months. Blindness occurs in about 5 percent of patients, but ocular symptoms relapse during a period of years. The disease eventually involves other organs with clinical and pathologic findings that suggest vasculitis. Aortic insufficiency, the most serious complication, develops in 15 percent of patients. The cause of Cogan syndrome remains unknown, but several studies suggest an autoimmune-mediated process. Many reports document an improvement in symptoms with immunosuppressive therapy, particularly if started early in the course of the illness. Family physicians should include Cogan syndrome in their differential diagnosis when a young adult seeks care with audiovestibular symptoms or ocular inflammation.
科根综合征是一种罕见疾病，表现为涉及眼睛和耳朵的症状。发病时，它可能类似许多常见病症。家庭医生应了解科根综合征，因为如果在病程早期得到识别，它是可以成功治疗的。它是少数可治疗的致聋原因之一。
检索了1982年至今的医学文献数据库（MEDLINE）中关于“科根综合征”的资料。通过交叉引用现有文章的参考文献获取了其他资料。
科根综合征于1945年首次定义，包括非梅毒性间质性角膜炎以及眩晕、耳鸣和听力丧失发作。虽然通常仅以一种症状起病，但大多数患者在发病1年内会出现听觉和眼部表现。如果不治疗，大多数患者会在36个月内失聪。约5%的患者会失明，但眼部症状会在数年内复发。该疾病最终会累及其他器官，出现提示血管炎的临床和病理表现。最严重的并发症主动脉瓣关闭不全在15%的患者中出现。科根综合征的病因尚不清楚，但多项研究提示是自身免疫介导的过程。许多报告记录了免疫抑制治疗可改善症状，尤其是在疾病早期开始治疗时。当年轻成人因视听前庭症状或眼部炎症寻求治疗时，家庭医生应将科根综合征纳入鉴别诊断。
