Behçet's disease is generally known as an autoimmune disease. We report a case of Behçet's disease who had fluctuating hearing loss, tinnitus and dizziness. A 37-year-old woman had bilateral hearing loss in low audiometric frequencies. Audiological and vestibular examinations revealed an inner ear lesion. The patient had an enlarged negative summation potential in the electrocochleogram and a positive glycerol test, which suggested endolymphatic hydrops. The pathological characteristic of Behçet's disease is thought to be vasculitis. So this case suggests an association of autoimmune vasculitis with endolymphatic hydrops.