COGAN-I-SYNDROME - TOO OFTEN TOO LATE REC OGNIZED - EARLY DIAGNOSIS IN COGAN-I-SYNDROME

Background Cogan's syndrome is an uncommon disease characterized by oc ular inflammation, vestibuloauditory dysfunction and symptoms of syste mic disease. The etiology is unknown, however there is evidence for an autoimmune pathogenesis. The ''typical'' Cogan's syndrome presents as bilateral interstitial keratitis and progressing vestibuloauditory dy sfunction. The presence of other inflammatory manifestations in additi on of keratitis has been termed as ''atypical'' Cogan's syndrome. Pati ents We report on six patients presenting with typical as well as atyp ical ocular manifestations between 1982 and 1994. Typically, the illne ss was accompanied by systemic symptoms. Each patient had audiovestibu lar involvement, that was the initial presentation in 4 cases. Vestibu lar dysfunction often preceeded hearing loss. Five of our patients not only presented with keratitis but also demonstrated signs of ocular i nflammation diagnosed as scleritis or episcleritis. In two patients th ese ocular symptoms were the first signs of Cogan's syndrome, recurred periodically and did not respond to corticosteroids. Results In 3 pat ients that were diagnosed early and treatment with corticosteroids was initiated early, hearing could be stabilized, in the remaining patien ts total bilateral deafness could not be prevented. Conclusion The imp ortance of being aware that vestibuloauditory dysfunction may occur in patients with ocular inflammation, and the fact that early immunother apy may prevent the risk of deafness, has to be emphasized.