There is considerable evidence to suggest that hearing and vestibular function can be influenced by immunity in the inner ear. While immunity can protect against infections of the labyrinth, immune response also has the capacity to damage the delicate tissues of the inner ear. Antigenic challenge of the inner ear of sensitized animals leads to rapid accumulation of leukocytes, antibody production, hearing loss and tissue damage. Moreover, a number of systemic autoimmune disorders include hearing loss and vertigo as part of their constellation of symptoms. It also appears that autoimmune damage can exist as an entity confined to the labyrinth. Immune disorders of the inner ear are of special interest since they are among the few forms of hearing loss that are currently amenable to medical treatment. In addition, recent developments in understanding the intracellular pathways that participate in damage to the inner ear provide new opportunities for pharmacotherapy of immune-mediated disorders of hearing and balance.