ORIGINAL ARTICLE Corticosteroid Response and Supporting Cell Antibody in Autoimmune Hearing Loss

Objective: To determine whether antibodies to sup-porting cells are associated with response to corticoste-roids in patients with autoimmune sensorineural hear-ing loss. Design: Prospective analysis of antibody to inner-ear antigens. Setting: Collaborating otology practices in Pennsylva-nia, Michigan, and Indiana. Patients: Sixty-three patients with rapidly progressive unilateral or bilateral sensorineural hearing loss of un-known cause suggestive of autoimmune sensorineural hearing loss. Interventions: Pretreatment audiometry, serum analy-sis by Western blot (WB) and immunofluorescence (IF) tests, corticosteroid therapy, and follow-up audiometry. MainOutcomeMeasures:Antibody reactivity and au-diogram changes were analyzed for association with re-sponse to treatment. Results: More than half of the patients (37/63) had an-tibodies 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. TheWB findings did not correlate with response. Of the WB-positive patients, 49 % (21/43) improved, as did 35 % (7/ 20) of theWB-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 im-provement after corticosteroid therapy (relative rate, 2.8). Patients with IF-positive serum are nearly 3 times more likely to experience improved hearing with corticoste-roid treatment than those who are IF negative. Antibod-ies to inner-ear supporting cell antigen may have value in diagnosis and treatment of patients with autoim-mune sensorineural hearing loss