Report Of A Case Of Sudden Unilateral Audiovestibular Disturbance In A Patient With Adamantiades Behçet’s Disease And Review Of The Literature.

Abstract

Introduction: Adamantiades-Behcet’s disease is generally known as an autoimmune disease characterised by multisystem generalized vasculitis with perivascular infiltration. Typical loci of manifestations are the mucous membranes, skin and eyes, as well as the joints. The central nervous system is often involved in Adamantiades-Behcet’s disease and diverse neurological manifestations have been described. Disequilibrium or isolated hearing impairment is also reported in the literature, especially in the Middle East. Case Report: We present a case of a patient, with a 20 year history of mucocutaneous and occular manifestations of Adamantiades-Behcet’s disease. The patient presented with a rarely described episode of sudden unilateral, low and middle frequency hearing deterioration, tinnitus and peripheral vestibular disturbance. He received steroid therapy, which lead to significant remission of all his symptoms. Discussion: Inner ear involvement in patients with AdamantiadesBehcet’s disease in Middle East populations is documented, and there has been an effort the past few years to determine its characteristics. Predominant patterns of the auditory and vestibular impairment and potential predictive markers for their appearance have been suggested, but there exist cases with distinct and uncommon characteristics. The administration of corticoides improves the symptoms of the disease especially those involving the inner ear.