Prevalence, Patterns, and Factors Associated with Hearing Impairment Among Children and Adolescents with Sickle Cell Disease in Eastern Uganda: A Cross-Sectional Study

Abstract

Background: Sickle cell disease (SCD) imposes a substantial burden in sub-Saharan Africa, and the cochlea is vulnerable to the vasoocclusion and chronic anaemia that characterise the condition. Hearing impairment in children with SCD remains poorly described in Uganda, including the eastern districts around Mbale that carry one of the highest SCD burdens in the country. This study determined the prevalence, patterns, and factors associated with hearing impairment among children and adolescents with SCD attending Mbale Regional Referral Hospital. Methods: A hospital-based cross-sectional study enrolled 225 children and adolescents aged 5 to 18 years attending the sickle cell clinic in a steady clinical state. An audiologist performed pure-tone audiometry, and hearing impairment was defined as a worse-ear average air-conduction threshold above 20 dB at 500, 1000, and 2000 Hz, following the World Health Organization definition. Factors associated with hearing impairment were examined using Firth penalised logistic regression, with crude and adjusted odds ratios reported with 95% confidence intervals. Results: Hearing impairment was present in 52 of 225 participants, a prevalence of 23.1% (95% CI 18.1 to 29.0). Impairment was unilateral in 29 and bilateral in 23, and most losses were mild. A central nervous system event (adjusted odds ratio [aOR] 3.54; 95% CI 1.01 to 12.43; p = 0.049) and a history of ear infection (aOR 2.42; 95% CI 1.01 to 5.82; p = 0.048) were independently associated with hearing impairment. Three or more transfusions showed a comparable association that just missed significance (aOR 2.16; 95% CI 0.99 to 4.73; p = 0.054). Conclusion: One in four children and adolescents with SCD at this referral hospital had hearing impairment, and the affected children carried markers of severe disease and middle-ear pathology. Audiometry should be integrated into routine sickle cell care to detect impairment before it harms language and learning.