Cogan's syndrome is a systemic vasculitis of autoimmunologic origin. The main disturbances involve the eye, the ear and the heart, but many other structures can also be affected. Nonspecific abnormal laboratory findings are also present. The disease usually involves a cranial nerve, followed by aortitis which can affect the aortic valve and, more rarely, the cusps of the valve. Aortic lesions strongly influence the course of the disease and therefore the prognosis. When choosing the type of valve replacement, long-term corticosteroid therapy and the age and sex of the patient must be taken into account.