This study investigated the incidence of peripheral hearing loss in sickle cell anemia and the possibility of central auditory nervous system involvement. Nine Black subjects with sickle cell disease and nine with normal hemoglobin were administered an auditory test battery. There appeared to be no correlation between number of crisis episodes, duration of symptoms, severity of symptoms, and audiologic manifestations. Acoustic reflex testing suggested the possibility of "aired neural function in the sickle cell group. Whether impaired function was due to peripheral VIIIth nerve or to central brain stem involvement could not be determined. Results of the central auditory test battery suggested the possibility of impaired or reduced central auditory function in subjects with sickle cell anemia.