A Case of Cochlear Implantation in a Postlingual Deaf Patient with Behcet Disease / 대한이비인후과학회지

ABSTRACT

Recurrent iritis and oral/genital ulcers have been described for the first time as a separate pathological entity by the Turkish dermatologist Hulusi Behcet. Behcet disease is a chronic, multi-systemic disorder, which affects many organs as a result of vasculitis. The clinical manifestations are divided into two groups major (oral ulcers, eye lesions, genital ulcerations, and skin lesions) and minor (arthritis, gastrointestinal lesions, epididymitis, thrombophlebitis, and central nervous system involvement) criteria. The incidence of hearing loss in Behcet disease has been reported as 12% to 80% in several studies. A 37-year-old man diagnosed as Behcet disease was admitted to our department with complaints of profound right hearing loss and dizziness. He was treated with steroid without improvement. After one year, he suffered from complete hearing loss on the left side. A temporal bone CT scan revealed right cochlear ossification. Cochlear implantation was performed within 4 weeks after the completion of steroid therapy in the left cochlea, which was partially obstructed by fibrotic tissue. However, the electrode was fully inserted without resistance. Wound healing complications were not encountered in the postoperative period. The hearing threshold was restored to 30 dBHL, while speech discrimination did not improve as much as expected. We propose that the routine hearing examinations be employed in the evaluation and management of Behcet disease. According to our experience, we find that cochlear implantation should be carried out at an early stage, before the development of labyrinthine ossification.