Sensorineural hearing loss in children with sickle cell anemia.

Various degrees of hearing loss have been associated with sickle cell anemia. Fifty-six Nigerian children 6 to 15 years old were investigated for presence, type, and degree of hearing loss. Sensorineural hearing loss greater than or equal to 25 dB in two or more frequencies was found in 12 of 56 patients (21.4%). Tympanometry was normal in all patients, but acoustic reflex was elevated or absent in seven of 12 patients with hearing loss. Analysis of the data revealed a significant association between sensorineural hearing loss and the homozygous sickle cell disease, and a significant association between hearing loss and early occurrence of vaso-occlusive crisis. This latter finding suggest that the microvasculature of the cochlea in young infants may be more susceptible to occlusion during sickle cell crisis.