PROGRESSIVE SENSORINEURAL HEARING IMPAIRMENT IN SYSTEMIC VASCULITIDES

Objectives: A large series of patients with various forms of systemic vasculitis were evaluated to analyze the prevalence of progressive sen sorineural hearing loss (PSNHL), its characteristics and evolution, an d the effects of different therapies. Methods: A total of 673 patients were questioned about the presence of subjective audiovestibular dist urbances. Of those, 80 subjects complained of subjective audiological disturbances and underwent oto-rhino-laryngological and audiovestibula r evaluation. Those patients with progressive hearing impairment were selected and studied carefully. Results: A PSNHL was observed in 14 pa tients. The hearing loss was bilateral and asymmetrical in most subjec ts. It was usually sensorineural, with a cochlear lesion. Unsteadiness was the most frequent vestibular symptom and canal paresis or palsy w as noted in most patients. Systemic corticosteroids and cyclophosphami de were useful treatments; in unresponsive patients, satisfactory resu lts were obtained with methotrexate and plasma exchange. Conclusions: PSNHL is a rare complication of systemic vasculitis, but occasionally is one of the presenting symptoms. Its clinical evolution is variable, but timely clinical assessment and treatment can positively affect pr ognosis. Copyright (C) 1998 by W.B. Saunders Company.