[Retrospective and prospective studies of patients with Cogan I syndrome]

Since August 1987 we documented the course of three patients with Cogan-I-Syndrome (CS) (interstitial keratitis and audio vestibular disorder). The patients (2 female, 1 male) received oral protease therapy in addition to the corticosteroids. Under oral treatment with proteases the corticosteroid therapy could be discontinued. Regression of inflammatory reaction and improvement of hearing was encountered in all patients. In two cases alpha-1-antitrypsin (AAT) serum levels were profoundly reduced, reaching normal range during therapy. Concomitant to the raise of this parameter improvement of hearing and general status could be encountered. In all 3 cases pathological leukocytosis normalized under treatment with proteases. All patients had increased IgA-levels against varicella zoster virus (VZV) indicating a reactivation.