Background: Cogan I syndrome is a rare, inflammatory, systemic disease that is typically characterized by severe audiovestibular dysfunction and various inflammatory eye changes.
Patients and methods: Between 1978 and 1996 ten patients with Cogan I syndrome were treated at the eye hospital, ENT clinic and medical clinic of the University of Tübingen. Retrospectively, the clinical picture, course of the disease and outcome were investigated by reviewing the charts of these patients.
Results: Patient ages ranged from 18 to 78 years (mean 37 years) at the time 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-threatening course of the disease despite immunosuppressive therapy. Severe hearing loss was seen in all patients. Further manifestations of Cogan's syndrome 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 disease process. Various ocular inflammatory changes may occur including uveitis, scleritis, keratitis and episcleritis. The visual prognosis is mostly good, although severe sight-threatening scleritis may occur.