Solomon Amos, Shofoluwe Nurudeen Adebola, Ahmad Hafsat, Isa Abdulkadir, Yunusa Shuiabu Iliyasu, Manir Hamza Anka
Department of Pediatrics, Faculty of Clinical Scinces, College of Medical Sciences, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria.
Division of Otorhinolaryngology, Department of Surgery, Faculty of Clinical Scinces, College of Medical Sciences, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria.
Iran J Otorhinolaryngol. 2025;37(3):123-133. doi: 10.22038/ijorl.2025.74314.3500.
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.
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).
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; = < 0.001), non-compliance with SCD medications (OR 9.634; 95% CI 1.830-50.718; = 0.008), and frequent severe sickle cell crises requiring hospitalization (OR 2.106,; 95% CI 0.019-0.598; = 0.001).
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.
镰状细胞病（SCD）是一项重大的全球健康负担，具有重大的临床、社会和经济影响。感音神经性听力损失（SNHL）是SCD一种未被充分报告的并发症，主要归因于血管闭塞性危机和局部缺血。这种情况对生活质量、教育和社会融合产生不利影响，在资源有限环境中的儿童中尤为如此。了解SNHL的危险因素对于预防、早期发现和及时干预至关重要。本研究评估了SCD患儿中SNHL的患病率，并确定了相关危险因素。
这项前瞻性比较研究在尼日利亚西北部的一家三级医疗机构进行。共纳入250名5至16岁的儿童，包括125名病情稳定的确诊镰状细胞病（SCD）患者和125名年龄及性别匹配、血红蛋白基因型正常（HbAA）的对照者。
在25.6%的SCD病例中发现双侧SNHL，而对照组未观察到SNHL。受影响儿童的男女比例为1.2:1。多因素逻辑回归显示，SNHL与白细胞计数升高（比值比{OR}1.035；95%置信区间{CI}1.020 - 1.050）、血小板计数升高（OR 1.209；95% CI 1.070 - 1.365）、门诊就诊情况差（OR 28.668；95% CI 4.879 - 168.458；P < 0.001）、不遵守SCD药物治疗（OR 9.634；95% CI 1.830 - 50.718；P = 0.008）以及频繁发生需要住院治疗的严重镰状细胞危机（OR 2.106；95% CI 0.019 - 0.598；P = 0.001）之间存在显著关联。
本研究突出了SCD患儿中SNHL的高患病率及其与可改变危险因素的关联。常规听力筛查、持续门诊就诊、药物依从性以及血液学参数的定期监测对于SNHL的早期识别和管理至关重要。有针对性的干预措施可以显著改善结局并减轻这种使人衰弱的并发症的负担。
