Autoimmune inner ear disease was first described by McCabe in 1979. The diagnosis is supported by cell mediated immune responses to inner ear membrane antigen stimulation. Meniere's disease consists of episodic vertigo, fluctuating sensorineural hearing loss, tinnitus and often aural pressure. Although most clinicians feel that Meniere's disease has no known cause, recently several researchers have suggested that some cases of Meniere's disease may be autoimmune in origin. In the present study, 10 patients with bilateral Meniere's disease were studied prospectively for autoimmune inner ear disease using both cellular and humoral immune tests. Results were compared with normal control subjects. One additional patient with Meniere's disease was identified retrospectively to have positive autoimmune test results. Preliminary conclusions suggest that at least some cases of presumed idiopathic Meniere's disease are autoimmune in origin. Therapeutic implications are significant: the addition of steroids, cytotoxic drugs and/or apheresis to conventional treatment may help prevent total deafness which otherwise might be inevitable.