Hearing thresholds in sickle cell anemia patients: emerging new trends?

Advances in medicine resulting in better understanding of sickle cell disease and general improvement of the well-being of the sufferers even in the developing countries have positively affected the dreadful outlook of this disease with resultant increase in the population of sickle cell disease patients reaching adulthood, and less severe complications. We therefore set out to evaluate the presence and severity of sensorineural hearing loss in sickle cell anemia (SCA) patients in the light of the overall improvement in the morbidity and mortality.

A prospective case control study of SCA patients attending our adult SCA clinic and control subjects from homozygous hemoglobin AA patients attending the staff clinic of the hospital for routine medical tests. Tympanometry and diagnostic audiometry were performed on each patient.

Forty-six SCA patients (21 males, 45.7%) aged 16-48 years with a mean age of 22.9 years +/- 6.45 and 42 controls (24 males, 57.1%) aged 15-39 years with a mean age of 23.7 years +/- 5.69 were included in this study. The average hearing thresholds of SCA patients were consistently higher than controls in all frequencies tested in both right and left ears. Of the 92 ears of SCA patients tested, 95.7% exhibited hearing thresholds within normal limits, and 4.3% had mild hearing loss. The controls had thresholds within normal limits.

The incidence of significant sensorineural hearing loss in SCA seems to have reduced in line with the general improvement and survival of SCA patients. The hearing loss is worse in the right ear and has a female preponderance. We hope that more aggressive primary and secondary prevention and adequate treatment of sickle cell crisis would reduce if not eliminate the hearing loss found in SCA.