To study the prevalence and pattern of sensorineural hearing loss (SNHL) in Nigerian children with sickle cell disease (SCD).Fifty-two children with SCD were assessed. Otologic examination and audiometric tests wee performed. There were 36 males and 16 females. Their age ranged from 6 to 19 years.Seven children had hearing impairment. After hemoglobin categorization, all the SCD patients were found to belong to the homozygous (HbSS) group. There were no HbSC nor other variants detected. Prevalence of SNHL was 13.4% in these patients but the general population prevalence rate found in a control group was 6.2%.SCD has many organ manifestations and complication among which is sensorineural hearing loss. Regular audiologic assessment, counselling and rehabilitation of these patients with hearing aids are recommended.