The evaluation of auditory function in homozygous sickle cell disease

The most significant clinical manifestations of sickle cell disease (SCD) are those associated with CNS involvement. This disease is systemic in character, with the tissues of the CNS being the most sensitive to even temporary anoxia. For this reason, the incidence of peripheral and central auditory dysfunction in patients with SCD may be greater than in the "normal" population. We examined the peripheral auditory function of 54 patients with homozygous sickle cell disease and the central auditory function of 28 of these patients. Results were compared with a control population. Peripheral hearing loss was found in 11% (6) of the SCD population. Central auditory testing indicated that 46% (13) of the sickle cell population had mild central auditory dysfunction not involving Heschl's gyrus. This study suggests a higher than usual incidence of peripheral and central auditory impairment in sickle cell disease.