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 sensorineural hearing loss (PSNHL), its characteristics and evolution, and the effects of different therapies. METHODS: A total of 673 patients were questioned about the presence of subjective audiovestibular disturbances. Of those, 80 subjects complained of subjective audiological disturbances and underwent oto-rhino-laryngological and audiovestibular evaluation. Those patients with progressive hearing impairment were selected and studied carefully. RESULTS: A PSNHL was observed in 14 patients. The hearing loss was bilateral and asymmetrical in most subjects. It was usually sensorineural, with a cochlear lesion. Unsteadiness was the most frequent vestibular symptom and canal paresis or palsy was noted in most patients. Systemic corticosteroids and cyclophosphamide were useful treatments; in unresponsive patients, satisfactory results 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 prognosis.