Typical and Atypical Cogan's Syndrome: 7 Cases and Review of the Literature

Abstract

Cogan's syndrome (CS) is a rare presumed autoimmune disorder characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms similar to those of Meniere's syndrome. Material and methods: We reviewed the records of a prospective study carried in CHU Mohammed VI of Marrakech, from September 2012 to June 2014, including 7 patients with a Cogan’s syndrome. Clinical data regarding age, sex, ethnic origin, presenting manifestations, ocular, audiovestibular and systemic manifestations, treatment and outcome were collected. Results: 7 patients were identified, 3 with typical Cogan’s syndrome and the remaining 4 with atypical Cogan’s syndrome. The age ranged from 12 to 65 years, with median age of 31 years, The presenting manifestations were ocular in four patients and audio vestibular in three. Examination revealed a bilateral interstitial keratitis (stromal scarring with ghost vessels) in six patients. Which was isolated in three cases and associated in 1 case with conjunctivitis, 1 with subconjunctival hemorrhage and one with corneal neovascularization.Audiovestibular manifestations in the two groups were typical, with vestibular symptoms, followed by progressive hearing loss of variable severity. Five of our patients presented systemic manifestations. All patients initially received local and systemic corticosteroids (CTC) given the occuular symptom but Clinical follow-up showed recurrence of audiovestibular symptoms after dose reduction of prednisone in 4 patients. In these cases, immunosuppressive therapy was able to improve hearing in 3 cases and visual function in 2 cases. Overall, 5 patients maintained good visual; 2 patients did not respond to medical treatment and thus were candidates for corneal transplant, 1 patient presented a total right deafness 3 years after the onset. The treatment strategies are not clearly defined; therefore, early assessment and treatment for systemic inflammation are needed to prevent life threatening complications.