Severe deafness in systemic lupus erythematosus: Its immediate relief by plasma exchange

Abstract

Although neurological features occur in 60% of patients with systemic lupus erythematosus, deafness is not an established characteristic of the disease. This paper describes a patient in whom deafness started abruptly even though she had had other features of the disease for several weeks. Audiological tests and clinical features suggested that the deafness was of the cochlea type. Plasma exchange is thought to work in systemic lupus erythematosus by clearing circulating immune complexes and thus removing blockade of the reticuloendothelial system. The rapid and dramatic response to plasma exchange in this patient implies a vascular mechanism and suggests that circulating immune complexes cause sludging in the microcirculation of the stria vascularis in the scala media of the cochlea. The presence of immune complexes in the microcirculation may cause swelling of the endothelial cells and diminish the flow through the small vessels by reducing the size of the lumen. This leads to anoxia and a temporary change in the ionic composition of the endolymph. A cochlea-type deafness results which clears as the anoxia is reversed by the removal of the immune complexes. Vascular mechanisms causing anoxia in the stria vascularis but not involving immune complexes possibly account for the reversible deafness which is a feature of Meniere's disease.