Otolaryngologists have long sought to identify causes of sensorineural hearing loss that might be reversed by medical treatment. One such entity has become known as autoimmune inner ear disease. The potential improvement in auditory function in these patients subsequent to immunosuppressive therapy has created a desire in clinicians to better understand this disease. This paper begins by reviewing the basic concepts of autoimmunity. The experimental and clinical data concerning autoimmune inner ear disease are then described and analyzed. Finally, conclusions are drawn concerning our current state of understanding of this disease process.