Cogan氏综合征少见,以一侧或双侧迅速进展的感觉神经性聋、平衡失调和反复发作的间质性角膜炎为特征。作者报告18例,指出症状多变,多迅速进展至全聋且可侵犯全身各器官而致死。及时采用类固醇治疗能挽救某些患者的听力。18例中男5女13,年龄12～54岁,平均32岁。13例有听觉前庭症状和体征、继之迅速发生眼部症状,一般先发生一侧高音调耳鸣和迅速进展的波动性感觉神经性聋,数日内另耳发生同样症状。17例有前庭症状,一些患者出现典型的内淋巴积水性眩晕,更多患者则诉不平衡或不稳感,行走或站立时欲倒向一侧。另5例与前述13例不同,其中2例眼部症状出现前2年双耳听觉前庭机能即出现严重障碍;1例在出现听觉前庭机能障碍前2 Cogan’s syndrome is rare and is characterized by sensory nerve deafness that progresses rapidly on one or both sides, balance disorders, and recurrent interstitial keratitis. The authors report 18 cases, indicating that the symptoms change rapidly and more rapidly to all deafness and can invade all organs and death. Timely use of steroid therapy can save some patients hearing. 18 males and 5 females 13, aged 12 to 54 years old, with an average of 32 years old. 13 cases of auditory vestibular symptoms and signs, followed by the rapid occurrence of ocular symptoms, the general side of the first high-pitched tinnitus and rapid progress in Vocal Nerve deafness, the other within a few days the same symptoms. There were 17 cases of vestibular symptoms, some patients showed typical endolymphatic hydroplane vertigo, more patients complained of imbalance or instability, walking or standing sideways to the side. The other 5 cases were different from the aforementioned 13 cases, of which 2 cases of binocular auditory vestibular function appeared severe disorder in the first 2 years of ocular symptoms; 1 case had auditory vestibular dysfunction 2