Cogan I syndrome: clinical picture, therapy and prognosis

Background: Cogan 1 syndrome is a rare, inflammatory, systemic disease that is typically characterized by severe audiovestibular dysfunction and vario us inflammatory eye changes. Patients and methods: Between 1978 and 1996 ten patients with Cogan I syndr ome were treated at the eye hospital, ENT clinic and medical clinic of the University of Tubingen. Retrospectively, the clinical picture, course of th e disease and outcome were investigated by reviewing the charts of these pa tients. Results: Patient ages ranged from 18 to 78 years (mean 37 years) at the tim e of disease manifestation. Eight patients were female. Inflammatory ocular changes were observed in nine patients in both eyes. Uveitis was found in six patients, keratitis in five, episcleritis in three and scleritis in two patients. Both patients with scleritis showed a severe, partially sight-th reatening course of the disease despite immunosuppressive therapy. Severe h earing loss was seen in all patients. Further manifestations of Cogan's syn drome included pericarditis associated with arthritis, and polyserositis in one patient, and fibromyalgia in two patients. Conclusion: In Cogan I syndrome,typically both eyes are affected by the dis ease process. Various ocular inflammatory changes may occur including uveit is, scleritis, keratitis and episcleritis. The visual prognosis is mostly g ood, although severe sight-threatening scleritis may occur.