A Case of Behçet’s Disease with Otologic Symptoms

Abstract

Behçet’s disease is generally known as an autoimmune disease. We report a case of Behçet’s disease who had fluctuating hearing loss, tinnitus and dizziness. A 37-year-old woman had bilateral hearing loss in low audiometric frequencies. Audiological and vestibular examinations revealed an inner ear lesion. The patient had an enlarged negative summation potential in the electrocochleogram and a positive glycerol test, which suggested endolymphatic hydrops. The pathological characteristic of Behçet’s disease is thought to be vasculitis. So this case suggests an association of autoimmune vasculitis with endolymphatic hydrops.

Distortion Product Otoacoustic Emission Findings in Behçet’s Disease and Rheumatoid Arthritis

“…Sudden hearing loss and fluctuating hearing loss were also reported in Behçet's disease [7,8] . Hearing loss in Behçet's disease can be attributed to vasculitis or inflammatory-autoimmune factors [8][9][10] .…”

Rinossinusite em paciente com síndrome de Behçet

“…Other authors 1,11 found the involvement of the inner ear as a late complication of Behçet's Syndrome (approximately one decade after the onset of the condition), vasculitis (primary pathological lesion of this disease) was considered the only responsible factor for labyrinth disorder. Detailed otology and vestibular examination revealed cochlea and vestibular apparatus abnormalities, in addition to results indicating that 62% of Behçet's Syndrome patients complain of auditory disorders and 37% of them present vertigo symptoms.…”

“…Behçet's syndrome is an inflammatory disorder of unknown etiology that affects blood vessels, primarily venules, and is characterized by recurrent oral and genital ulcer lesions, uveitis, aphtous-like skin lesions and acne like eruptions 1,2 . There is no isolated infectious agent.…”

Prevention of autoimmune hearing loss in MRL/lpr mice by bone marrow transplantation

“…1 Inner ear dysfunction causes various audiovestibular symptoms such as SNHL, tinnitus and vertigo. The presence of SNHL as a part of or in combination with systemic autimmune diseases such as systemic lupus erythematosus (SLE), 6-8 rheumatoid arthritis (RA), 9-12 Behçet's disease, 13,14 Cogan's syndrome, 15,16 and ulcerative colitis 17,18 has been reported.MRL/lpr mice were found to spontaneously develop SNHL as well as SLE by autoimmune mechanisms. The mice carrying the lymphoproliferation (lpr) mutation have defects in the Fas gene, and a defect in negative selection of self-reactive T cells in the thymus.…”

“…1 Inner ear dysfunction causes various audiovestibular symptoms such as SNHL, tinnitus and vertigo. The presence of SNHL as a part of or in combination with systemic autimmune diseases such as systemic lupus erythematosus (SLE), [6][7][8] rheumatoid arthritis (RA), [9][10][11][12] Behçet's disease, 13,14 Cogan's syndrome, 15,16 and ulcerative colitis 17,18 has been reported.…”