[The Cogan syndrome]

Abstract

Exactly 30 years ago D. C. Cogan reported 4 cases of "nonsyphilitic interstitial keratitis associated with vestibulo-auditory symptoms". Report of 57 of these cases have come to our attention. They mostly concern relatively young people, although the age range is now 4-1/2 to 63 years. The etiology of Cogan's syndrome is unknown. It very frequently has been associated with some generalized vascular diseases. In our first case, a 13 year old boy, we found the symptoms of a serous meningitis with pathological signs in the EEG. No vascular changes were encountered. During 4 weeks the rapidly progressive hearing loss was accompanied by tinnitus and vertigo and an interstitial keratitis. The recession of vestibular symptoms is followed by the loss of function of the endorgans. The voice seemed high-pitched and monotonous. In our patient the eye condition improved, but the cochleo-vestibular disturbances did not. The second case, a 27 year old woman, could be observed over a period of 19 years. The ocular symptoms disappeared nearly completely after 1 year, but the cochleo-vestibular disturbances remained. The audition shows even after 19 years some fluctuations.