Assessment of Cochlear Function in Children with Sickle Cell Anemia

Background: Sickle cell disease is characterized by vaso-occlusion which leads to impaired blood supply to several organs. The cochlear in particular has been known to be highly sensitive to anoxia which is, in turn, a proven cause of sensorineural hearing loss (SNHL). Early detection of hearing loss is paramount in these patients, as the cochlear ischemic damage is repetitive and progressive. Once the damage is sufficiently enough to cause noticeable hearing loss, the loss is mostly substantial and irreversible. Otoacoustic emission is useful in detecting early cochlear damage. Materials and Methods: Children with sickle cell anemia (SCA) (HbSS) aged between 1 and 12 years diagnosed by hemoglobin electrophoresis and equal number of controls matched (HbAA) matched for age and sex were recruited for the study. All participants underwent a detailed ear, nose, and throat examination and tympanometry. Those with normal middle ear function were further subjected to transient-evoked otoacoustic emissions (TEOAEs) testing. Results: There were 60 patients with SCA (cases) and 60 controls. The age of the participants ranged between 1-12 years with a mean of 6.5 +/- 3.74 among the cases and 7.02 +/- 3.54 among the controls. The male-female ratio was 0.9:1. Among participants with normal middle ear function, TEOAE was abnormal in 43 (48.8%) ears of the cases and 70 (73.7%) ears of the controls. There was a statistically significant difference in abnormal TEOAEs outcome between cases and the control group (P = 0.002). Conclusion: Children with SCA had a high rate of abnormal cochlear function. Therefore, there is a need for periodic monitoring of cochlear function in these patients so as to detect and treat SNHL early.