[Cogan syndrome. Case report, review of the literature, therapy].

We present a case report of Cogan's syndrome and a review of the literature. Cogan's syndrome is most likely due to autoimmune disease primarily affecting the vestibulocochlear organ and cornea and secondarily resulting in systemic disease. 40% of patients develop bilateral complete deafness and 70% show signs of systemic disease mostly in the form of cardiovascular symptoms. The mortality rate of 10% is mainly due to vasculitis. It is important to consider Cogan's syndrome in the differential diagnosis of sudden hearing loss accompanied by ocular signs in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.