Immune-mediated sensorineural hearing loss

Abstract

The immune-mediated sensorineural hearing loss (ISHL) is characterized as an asymmetric and progressive sensorineural hearing loss. Three patients with ISHL were studied, regarding clinical aspects, diagnostic tests, treatment options and disease evolution. They presented positive response to immunosuppressive therapy or to Western Blot test for hsp 70-68kD. Two patients presented rapidly progressive sensorineural hearing loss, associated with vestibular symptoms and the other presented unilateral sudden deafness. No patient reacted to rheumatological tests, and one presented increased erythrocyte sedimentation rate. None responded satisfactorily to corticoid therapy, but two presented clinical improvement with other immunosuppressive therapies. The ISHL diagnosis is based on clinical aspects and on the positive response to therapeutic tests with immunosuppressant drugs. The Western Blot test for hsp 70-68kD, with 42% sensitivity and 90% specificity, is the only specific laboratory exam for ISHL. One patient presented positive response to this exam and did not respond to immunosuppressive therapy. Two patients with negative tests satisfactorily responded to immunosuppressive therapy. The low sensitivity and high costs of Western Blot test hinder its spread use. The early introduction of treatment has a major importance in the diagnosis of ISHL and the auditory prognosis.