Abstract
To evaluate the sensitivity of high-frequency audiometry in detecting early audiometric changes in patients with sickle cell disease. A prospective case control study of 47 patients with homozygous sickle cell disease and 47 normal individuals matched for age and sex with hemoglobin genotype AA. High-frequency audiometry and conventional audiometry were used to assess hearing. A 2-way analysis of variance showed a statistically significant effect of signal frequency and type of patient hearing. Eighteen of 47patients with sickle cell disease had sensorineural hearing loss of more than 25dB at two or more frequencies. SNHL was unilateral in 13 and bilateral in 5. High frequency audiometry is sensitive in detecting early hearing loss in sickle cell patients. Early detection of hearing loss may allow effective measures to be taken to protect