There is considerable evidence to suggest that hearing and vestibular function can be influenced by autoimmune processes. A number of systemic autoimmune disorders include hearing loss and vertigo as part of their constellation of symptoms. Although classic evidence for a specific autoimmune etiology mediated by immune response directed solely at the inner ear is elusive, it appears that autoimmune damage can also exist as an entity confined to the labyrinth. Antigenic targets of autoimmunity within the labyrinth seem to be diverse. Partly because of this, the condition is difficult to diagnose. However, autoimmune disorders of the inner ear are of special interest since they are among the few forms of hearing loss that are amenable to medical treatment. Recent progress in understanding the etiology, diagnosis and treatment of autoimmune damage to the inner ear is reviewed.