There is considerable evidence to suggest that hearing and vestibular funct
ion can be influenced by autoimmune processes. A number of systemic autoimm
une disorders include hearing loss and vertigo as part of their constellati
on of symptoms. Although classic evidence for a specific autoimmune etiolog
y 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 t
he labyrinth, Antigenic targets of autoimmunity within the labyrinth seem t
o be diverse. Partly because of this, the condition is difficult to diagnos
e. However, autoimmune disorders of the inner ear are of special interest s
ince they are among the few forms of hearing loss that are amenable to medi
cal treatment. Recent progress in understanding the etiology, diagnosis and
treatment of autoimmune damage to the inner ear is reviewed. Curr Opin Neu
rol 14:35-40. (C) 2001 Lippincott Williams & Wilkins.