Autoantibodies against inner ear proteins in patients with delayed endolymphatic hydrops and unilateral juvenile deafness

Abstract

Conclusions: Patients with the contralateral type of delayed endolymphatic hydrops (DEH) may undergo an autoimmune attack against the other inner ear. As patients with unilateral juvenile deafness show no progression, despite lengthy observation, the autoantibody against the 68-kDa protein may be unrelated to the pathogenesis of DEH.

Objective: The contralateral type of DEH is believed to have an autoimmune etiology, and sometimes develops from unilateral juvenile deafness. The purpose of this study was to determine whether autoantibodies are pathogenetically important in DEH.

Material and methods: Sera from 9 patients with DEH, 18 patients with profound unilateral juvenile hearing loss and 15 control volunteer without inner ear diseases were investigated by means of Western blot assay against rat inner ear proteins.

Results: Among 8 patients with the contralateral type of DEH, 6 (75%) showed at least 1 reactive band on Western blotting. The protein that reacted most frequently had a molecular weight of 28 kDa, which was consistent with our previous results. Among 18 patients with unilateral juvenile deafness, 5 (28%) showed reactive bands, exclusively at 68 kDa.