NO EFFECT OF FETAL HEMOGLOBIN LEVEL ON AUDITORY DISCRIMINATION IN INDIVIDUALS WITH SICKLE CELL ANEMIA

Introduction Sickle cell anemia (SCA) is an inherited disorder characterized by hemolysis and vaso-occlusive crises. Hydroxyurea (HDU) is the primary drug available for SCA which increases the concentration of fetal hemoglobin (HbF) and has shown clinically beneficial effects ranging from 15 to 20% on sickle cells. The present study aimed to investigate the impact of HbF level on auditory discrimination ability and speech perception in noise in normal hearing individuals with SCA under HDU treatment. Material and methods A between-subject design was used for group comparison. Non-random purposive sampling was used to select participants from a local Hindi-speaking community. A total of 66 normal-hearing adults diagnosed with SCA and under medication with HDU in the age range of 18–40 years were divided into three groups based on HbF%. Auditory discrimination for frequency (DLF), intensity (DLI), and duration (DDT) were evaluated at 500 Hz and 4000 Hz along with SPIN at 0 dB SNR using the MLP toolbox in Matlab. Results Comparing the three HbF groups, Kruskal–Wallis tests revealed no significant difference in auditory discrimination based on either DLF, DLI, DDT, or SPIN scores. Conclusions The present study indicated no impact on the auditory system of raised HbF level in SCA individuals. The findings suggest that in such individuals not every system in the body is affected equally by raised HbF levels.