Autoimmune sensorineural hearing loss: clinical course and treatment outcome

Abstract

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 and relevance: 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.