808 THE EVALUATION OF AUDITORY FUNCTION IN HOMOZYGOUS SICKLE CELL DISEASE (SCD)

Abstract

Vaso-occlusive crises in SCD may compromise the blood flow of the inner ear. For this reason the incidence of peripheral hearing loss in patients with SCD may be greater than in the normal population. Forty-five patients with SCD, aged from four to nineteen years, were evaluated for peripheral and central auditory function. Results were compared with an age and race matched control group.Routine audiometry and electroacoustic measurements of middle ear impedance were performed on all patients. Katzs' Staggered Spondaic Word test was administered to patients ten years of age and older to assess central auditory function. Mean hearing and acoustic reflex levels were well within normal limits for both groups. Eight of 33 patients (24 percent) on whom central auditory function was assessed showed various degrees of dysfunction. Two of these 8 patients previously had a cerebro-vascular accident.This study is the first to suggest a significant degree of central auditory impairment in patients with SCD. It also reveals, contrary to previous studies, no greater incidence of either conductive or sensorineural hearing loss in these patients. Frequent CNS involvement in SCD is the possible cause of central auditory dysfunction.