The present investigation examines the effects of sickle cell crisis on peripheral auditory function and auditory brainstem response (ABR). The study includes 10 homozygous sickle cell anemia patients with documented sickle cell (SS) hemoglobin. Testing was performed during the crisis and 1 month after it subsided. Unilateral high frequency hearing loss was found in three patients but persisted in only one of them. Abnormal ABR tracings occurred in four patients but reversed to normal in one of them. The study suggests a trend toward increased incidence of sensorineural hearing loss (SNHL) and ABR changes as frequency and severity of crisis episodes increases. The relationship between SNHL and ABR is analyzed and discussed.