[Autoimmune deafness]

Abstract

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.

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

Diagnosis: 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.

Treatment: 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.