A case of Neuro-Behcet syndrome with hearing impairment.

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A 52-year-old woman with Neuro-Behçet syndrome had fluctuating sensory hear-ing loss and gait disturbance. The patient had had recurrent painful ulcerations in the oral and genital regions over the past 34 years and frequent headaches and high fever due to encephalomeningitis for the past two years.In addition, she had had hearing impairment and tinnitus for two years on the right and for one year on the left. These symptoms had become progressively worse. The pure tone audiogram revealed a sensorineural hearing loss which averaged 50 dB bilaterally. Speech audiometory and Békésy's audiometory suggested some type of inner ear deafness. The results of ABR recording, eye tracking test and optokinetic nystagmus test suggested the possibility of a central lesion in the right brain-stem. The patient was considered to have lesions in both inner ears and the right brain-stem.
耳鼻咽喉科臨床学会の論文