Corticosteroid response and supporting cell antibody in autoimmune hearing loss.

OBJECTIVE

To determine whether antibodies to supporting cells are associated with response to corticosteroids in patients with autoimmune sensorineural hearing loss.

DESIGN

Prospective analysis of antibody to inner-ear antigens.

SETTING

Collaborating otology practices in Pennsylvania, Michigan, and Indiana.

PATIENTS

Sixty-three patients with rapidly progressive unilateral or bilateral sensorineural hearing loss of unknown cause suggestive of autoimmune sensorineural hearing loss.

INTERVENTIONS

Pretreatment audiometry, serum analysis by Western blot (WB) and immunofluorescence (IF) tests, corticosteroid therapy, and follow-up audiometry.

MAIN OUTCOME MEASURES

Antibody reactivity and audiogram changes were analyzed for association with response to treatment.

RESULTS

More than half of the patients (37/63) had antibodies to both a 68- to 72-kDa protein and to inner-ear supporting cells, 16 patients had positive results on one assay only, and 10 had negative results on both. Twenty-eight patients improved and 35 did not. The WB findings did not correlate with response. Of the WB-positive patients, 49% (21/43) improved, as did 35% (7/20) of the WB-negative patients (P = .30). In contrast, 53% (25/47) of IF-positive patients improved, compared with only 19% (3/16) in the IF-negative group (P = .02). Of those who improved, 89% (25/28) were IF positive.

CONCLUSIONS

Antibody to an inner-ear supporting cell antigen was significantly associated with hearing improvement after corticosteroid therapy (relative rate, 2.8). Patients with IF-positive serum are nearly 3 times more likely to experience improved hearing with corticosteroid treatment than those who are IF negative. Antibodies to inner-ear supporting cell antigen may have value in diagnosis and treatment of patients with autoimmune sensorineural hearing loss.