The present case report constitutes the fourth autopsy study of the entity of nonsyphilitic interstitial keratitis with vestibuloauditory symptoms. Although approximately 25 clinical cases have been reported in the past 20 years the cause of the disease is still unknown.1Most of the cases have subsided leaving permanent deafness and variable, but usually mild, corneal scarring. Histologic studies including the three previous autopsies2-4have shown systemic vascular disease sometimes interpreted as polyarteritis. The clinical course of the disease in the present patient was typical of that of others. Death was caused by a rapidly progressive cardiovascular process that proved to be an unusual form of aortitis. Report of Case A 14-year-old boy who had always been well began to vomit and became unsteady at school one month before our examination. Three days after this initial episode, the loss of balance worsened and he began to lose his hearing.