Hearing and vestibular disturbances in Behçet's syndrome

In order to evaluate the prevalence of audiovestibular disturbances in Behçet's syndrome, we submitted 20 consecutive patients and 20 control subjects to detailed audiologic and vestibular examination in the last 3 years. A sensorineural hearing loss was found in 12 patients, 2 of whom revealed sudden deafness. Two other patients with neuro-Behçet's syndrome showed a vestibular function deficit, and 3 others exhibited altered caloric stimulation test results. Two of these last patients also revealed a simultaneous bilateral auditory deficit. HLA typing showed the presence of the B51 antigen in 10 of the 14 patients with ear involvement, while only 3 of the 6 patients without ear involvement were HLA-B51-positive. Results suggest that audiovestibular involvement is common in Behçet's syndrome: sudden deafness may be the first sign of ear disturbance; vestibular lesions may represent an early sign of neuro-Behçet's syndrome; and the HLA-B51 antigen is associated with ear involvement. Otoneurologic study can reveal hidden brain stem lesions in Behçet's patients during flare-ups of the disease, even without obvious signs of neurologic deficits.