Assessment of auditory function in sickle cell anaemia patients in Nigeria.

The auditory function of 30 patients with homozygous sickle cell disease (sicklers) was compared to a control group of 20 patients with a normal haemoglobin AA (non-sicklers). The sicklers exhibited a relatively higher mean pure tone hearing threshold at most frequencies than the control group, the difference at 4000 cps being statistically significant. The sicklers and non-sicklers (control group) did not show any significant differences in the pattern of tympanograms, static middle ear compliance and mean acoustic reflex thresholds, suggesting essentially normal middle ear mechanisms in both groups. The higher mean pure tone hearing threshold in sicklers may be due to inner ear sensori-neural deafness due to lifelong vaso-occlusion of some of the micro-vasculature supplying the inner ear apparatus in sicklers. Clinical implications are discussed and the need for periodic assessment of auditory function in Nigeria sicklers for preventive management of auditory problems is emphasized.