Sensorineural hearing loss (SNHL) has been a well-documented complication of sickle cell disease in the literature from West Africa, West Indies, United States of America and the Middle East. We present a study of 52 patients with homozygous sickle cell disease and 36 control patients with haemoglobin genotype AA, matched for age and sex. Seven patients with sickle cell disease (13.5 per cent) were found to have sensorineural hearing loss i.e.>20 dB at two or more frequencies, while all the patients in the control group had normal hearing (p<0.05).Our study shows the incidence of SNHL in the UK to be similar to that reported in the US A and much lower than that found in malaria endemic areas of the tropics.We highlight the factors which we consider responsible for these differences and suggest that the crucial period in the development of SNHL in sickle cell disease may be intra-uterine or during the first few years of life. All sickle cell patients should be encouraged to have regular hearing assessment.