A prospective controlled clinical study was carried out at the Department of Ophthalmology and ENT, Inönü University Medical Faculty, Turgut Ozal Medical Center, Research Hospital, to evaluate the audio-vestibular involvement in patients with Behçet's syndrome compared with controls.

Twenty-five consecutive patients with Behçet's syndrome (mean age ± SD, 34.96 ± 8.50) and 20 age- and sex-matched healthy volunteers (hospital staff) as control subjects (mean age ± SD, 34.45 ± 9.16) were included in this study. Behçet's patients were divided into two groups according to the number of criteria, complete (all four major criteria) and incomplete (three major criteria without ocular involvement). The groups were compared with each other or controls regarding inner ear involvement. Audiometric pure-tone thresholds at 125 to 8000 Hz were obtained in all subjects in both groups, and pure tone average (PTA) hearing thresholds were calculated for the middle, high and low frequencies. In addition, short increment sensitivity index (SISI), tone decay and BERA examinations were performed in all Behçet's patients.

Sensorineural hearing loss (SNHL) was present in six of 25 patients with Behçet's syndrome. Two Behçet's patients had unilateral total SNHL, two had bilateral moderate level SNHL, one had bilateral low-frequency SNHL and one bilateral high frequency SNHL. In two, BERA, and in five SISI, examination disclosed inner ear involvement. In control subjects, the past medical history was normal and there was no consistent audio-vestibular complaint. Their PTA thresholds were all in the normal range. Otoscopic examination findings were normal, with intact, mobile tympanic membranes in both groups.

The present study showed that audio-vestibular involvement is not infrequent in Behçet's syndrome compared with age- and sex-matched healthy controls, and it is under-estimated. All Behçet's patients should regularly be followed by an otolaryngologists and be given information about the possibility of inner ear involvement. According to our results, hearing loss occurs more often in older patients and also in the complete form of Behçet's syndrome.

We report the neuro-otological findings in 26 consecutive patients with definite and probable Behçet's syndrome unselected for audiovestibular complaints. Auditory and/or vestibular abnormalities were found in 19 (73 per cent) patients, with auditory involvement in 14 (54 per cent) and vestibular in 10 (38.5 per cent) of patients. Peripheral involvement was more common than central involvement for both auditory and vestibular lesions. Bilateral cochlear hearing impairment was the most common audiological finding, whereas unilateral peripheral dysfunction was the prevailing vestibular abnormality. No correlation has been found between audiovestibular lesions and other organ lesions, disease duration or age or sex of the patients. Moreover, there was a lack of interdependence between cochlear and vestibular labyrinthine lesions. We conclude that a full neuro-otological assessment in patients under investigation for Behçet's syndrome may reveal labyrinth involvement in a substantial proportion of patients. In view of the absence of a specific diagnostic test for Behçet's syndrome, audiovestibular lesions may provide further diagnostic support for this disorder.

A case is reported in which a Nucleus 22 channel intracochlear device was implanted a deaf/blind Hungarian adult with discharging ears suffering from Behçet's disease. Preconditioning surgery was employed three months prior to the implantation procedure to ensure a sterile, dry protected environment for the electrodes. One month after implantation, the patient exhibited excellent auditory discrimination capability at the time of the first switch on. We suggest that some deaf/blind individuals may serve as very good candidates for intracochlear implantation.