Risk Factors for Sensorineural Hearing Loss in Children and Adolescents with Sickle Cell Disease.

Abstract

Introduction: Sickle cell disease (SCD) is a major global health burden with significant clinical, social, and economic impacts. Sensorineural hearing loss (SNHL) is an underreported complication of SCD that is, primarily attributed to vaso-occlusive crises and ischemia. This condition adversely affects the quality of life, education, and social integration, particularly among children in resource-limited settings. Understanding the risk factors for SNHL is crucial for prevention, early detection, and timely intervention. This study evaluated the prevalence of SNHL in children with SCD and identified associated risk factors.

Materials and methods: This prospective comparative study was conducted at a tertiary healthcare facility in Northwest Nigeria. A total of 250 children aged 5-16 years were enrolled, comprising 125 patients with confirmed sickle cell disease (SCD) in a steady state and 125 age- and sex-matched controls with a normal haemoglobin genotype (HbAA).

Results: Bilateral SNHL was identified in 25.6% of SCD cases, whereas no SNHL was observed in the control group. The male-to-female ratio among the affected children was 1.2:1. Multivariate logistic regression revealed significant associations between SNHL and elevated white blood cell count (Odds Ratio {OR} 1.035; 95% Confidence Interval {CI} 1.020-1.050), elevated platelet count (OR 1.209; 95% CI 1.070-1.365), poor clinic attendance (OR 28.668; 95% CI 4.879-168.458; P= < 0.001), non-compliance with SCD medications (OR 9.634; 95% CI 1.830-50.718; P = 0.008), and frequent severe sickle cell crises requiring hospitalization (OR 2.106,; 95% CI 0.019-0.598; P = 0.001).

Conclusion: This study highlights the high prevalence of SNHL in children with SCD and its association with modifiable risk factors. Routine audiological screening, consistent clinic attendance, medication adherence, and regular monitoring of haematological parameters are essential for early identification and management of SNHL. Targeted interventions can significantly improve the outcomes and reduce the burden of this debilitating complication.