[Autoimmune deafness].
A REAL ENTITY: Deafness resulting from an autoimmune mechanism is suggested by a growing number of clinical and experimental arguments. The audio-vestibular system is known to be involved in a certain number of systemic diseases, particularly Cogan's disease. In other cases, the inner ear alone is involved; deafness may be the first manifestation of a systemic disease or result from a possible immunological mechanism.


METHODS
Autoimmune deafness is very invalidating. Bilateral perception deafness is observed in 80% of the cases and vestibular involvement is found in 70%


METHODS
No one simple reliable test is known which can establish the diagnosis of autoimmune deafness. Other causes must be ruled out by appropriate clinical and complementary explorations. For humoral immunity, the western blot method has given promising results suggesting a possible role of the heat shock protein in the underlying immunological mechanism.


METHODS
Immediate care is needed but no standard treatment has been defined. High-dose corticosteroids can provide symptom relief, particularly in case of abnormal immunological tests. The role of immunosuppressive therapy, sometimes proposed in case of corticosteroid resistance, remains to be defined.
Vinceneux
P
P
Service de Médecine Interne, Hôpital Louis Mourier, Colombes.
Couloigner
V
V
Pouchot
J
J
Bouccara
D
D
Sterkers
O
O
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy; D001528:Behcet Syndrome / Q000150:complications; D003638:Deafness / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D004305:Dose-Response Relationship, Drug; D004351:Drug Resistance; D007758:Ear, Inner / Q000276:immunology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D011081:Polychondritis, Relapsing / Q000150:complications; D012859:Sjogren's Syndrome / Q000150:complications
D000305:Adrenal Cortex Hormones
Les surdités auto-immunes.