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Sensori-neural hearing loss in Jamaicans with SS disease.

The pure-tone hearing levels in 83 Jamaican patients aged from 10-39 with homozygous sickle cell disease were compared with appropriate controls. A sensori-neural hearing loss, defined as a deficit of at least 25 dB in one or more frequencies, was found in 18/83 (22%) patients and in 3/83 (4%) controls. Both sexes and ears were equally affected, but the extremes of the tested range, especially the high tones, were involved most frequently. The hearing loss was of slow onset. The relative contributions of thrombosis, anaemia, atherosclerosis and Jamaican neuropathy are discussed. It is felt that the most likely aetiology is a thrombotic process.