Sensorineural Hearing Loss in Children with Sickle Cell Anemia

Abstract
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 ≥ 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 1) a significant association between sensorineural hearing loss and the homozygous sickle cell disease, and 2) a significant association between hearing loss and early occurrence of vaso-occlusive crisis. This latter finding suggests that the microvasculature of the cochlea in young infants may be more susceptible to occlusion during sickle cell crisis.

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