Autoimmune sensorineural hearing loss: clinical course and treatment outcome.
BACKGROUND
Autoimmune sensorineural hearing loss (ASHL) originally was defined both by the presence of progressive hearing loss (with or without vertigo) and a positive response to glucocorticoid therapy. Subsequently, antibodies to a 68-kd inner-ear antigen were identified in a high percentage of patients with ASHL.


OBJECTIVE
To analyze the disease progression and treatment outcome of ASHL.


METHODS
Retrospective chart review of 30 patients with a diagnosis of ASHL referred to Texas Tech Rheumatology Clinic. All subjects were tested for anti-68-kd antibody, had audiometric evidence of hearing loss, and were treated with glucocorticoids.


RESULTS
The median duration from onset of symptoms to audiometric testing was 25.5 weeks (mean, 144 weeks; range, 3-1,040 weeks), and to testing for anti-68-kd antibody was 40 weeks (mean, 157.3; range, 4-1,092 weeks). Ninety percent of subjects tested positive for anti-68-kd antibodies. By audiometric testing, 50% of subjects were steroid-responsive; minimal improvement or no change was recorded in 12%, and worsening occurred in 39% after steroid therapy. The 3 subjects who were anti-68-kd antibody-negative were steroid-unresponsive, and 1 progressed to complete deafness.


CONCLUSIONS
This series of patients with ASHL suggests a more variable and benign course with a better prognosis than previously reported. Immunosuppressive therapy other than steroids rarely was required, and rapid progression to complete hearing loss was very uncommon in this cohort.
Loveman
Donald M
DM
Department of Internal Medicine, Texas Tech University Health Sciences Center at Odessa, TX, USA. Donald.Loveman@ttuhsc.edu
de Comarmond
Charles
C
Cepero
Ralph
R
Baldwin
David M
DM
D000328:Adult; D000368:Aged; D001299:Audiometry; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy*; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D013256:Steroids