Hearing loss in children with sickle cell disease

Hearing loss in children with sickle cell disease. Sickle cell anemia (S/S) has been associated with a high incidence of hearing loss mostly of the sensorineural type (SNHL). Twenty-four patients with sickle cell disease (13 female and 11 male; 22 patients belonging to the S/beta(+)-Thal and 2 to the S/S phenotype) with a mean age of 12.5 +/- 3.6 years and hemoglobin (Hb) levels range 6.5-11 g/dl underwent ENT examination, pure tone audiometry, speech audiometry, tympanometry, auditory reflex evaluation, tone decay test and brain auditory evoked potentials (BAEP) in order to evaluate the presence, type and degree of hearing loss. Only one patient (4.6%) who also sustained an infarct of the middle cerebral artery, demonstrated a unilateral SNHL in high tone frequencies exceeding 70 dB, as well as a prolonged III-V interpeak latency at the same side. No abnormalities were detected in the control group. These findings suggest a low incidence of SNHL in Greek SCD patients probably due to different hematological and clinical profile (S/beta(+)-Thal).