Impact of Severity of Sickle Cell Anemia on Auditory Discrimination Ability and Speech Perception in Noise

Introduction  Sickle cell anemia (SCA) is a genetic disorder with clinical manifestations due to circulatory changes, leading to adverse effects on the auditory system that might impact auditory processing, such as auditory discrimination and speech perception ability. This condition is associated with the severity level of anemia. Objective  The purpose of the present study was to investigate the influence of anemia severity on auditory discrimination ability and speech perception in noise among SCA patients with normal hearing sensitivity. Methods  A total of 52 normal-hearing adults diagnosed with SCA in the age range of 15 to 40 were grouped into mild, moderate, and severe, based on anemia severity. Auditory discrimination tests for frequency, intensity, and duration were evaluated at 500 and 4,000 Hz along with speech perception in noise (SPIN) at 0 dB SNR using the mlp toolbox in the MATLAB software, version 2014a (MathWorks, Natick, MA, USA). The IBM Statistical Package for Social Sciences (SPSS) version 26.0 (IBM Corp., Armonk, NY, USA) was used for statistical analysis. Results  The results revealed an increase in median and interquartile range among anemia groups with increasing severity. Additionally, the median scores were found to be poorer for the higher frequency in all auditory discrimination tests than for the lower one. A regression in performance with an increase in severity for the SPIN test was observed. Conclusion  The severity of anemia plays an important role in functional auditory processing deterioration. Circulatory changes secondary to SCA affected auditory discrimination processing and speech perception in noise. However, all auditory discrimination abilities are not necessarily affected equally.