[Cogan I syndrome. Audio-vestibular, ophthalmologic findings and therapy in 6 patients]

We report clinical experiences with six patients treated at the University of Tübingen between 1982 and 1994 for symptoms of typical and atypical ocular manifestations of Cogan's syndrome. All cases had systemic symptoms in addition to involvement of the cochleovestibular system. Otologic symptoms were the initial presentation in four cases. Vestibular dysfunction often preceded hearing loss. Five patients had keratitis and also demonstrated signs of ocular inflammation diagnosed as scleritis or episcleritis. In two patients these ocular symptoms were the first signs of Cogan's syndrome, recurred periodically and did not respond to corticosteroids. Three patients were diagnosed early, allowing corticosteroid treatment to begin early with stabilization of hearing. Total bilateral deafness could not be prevented in the remaining patients. The importance of being aware of auditory-vestibular dysfunction occurring in patients with ocular inflammation and the role of early immunotherapy in preventing deafness has to be emphasized.