Prevalence of Hearing Loss in Young Adults with Sickle Cell Disease in Lagos, Nigeria

ABSTRACT Background: Sickle cell disease (SCD) is an autosomal recessive inherited blood disorder characterized by an abnormality in haemoglobin in red blood cells, which leads to vaso-occlusive crises. The microvasculature of the inner ear is at risk of cochlear damage in SCD patients. The degree of cochlear damage may be related to the prevalence and pattern of hearing loss. Objective: To determine the prevalence and pattern of hearing loss in young adult patients with SCD attending the Lagos University Teaching Hospital (LUTH). Methodology: This was a prospective and comparative study of young adult patients with SCD and a control group of medical students with the HbAA genotype. The inclusion criteria were age 18 to 30 years and genotype HbSS, HbSC, or HbS β-thalassemia for the study group and HBAA for the control group. Demographic and clinical data of the study participants were obtained with a structured questionnaire. Pure Tone Audiograms (PTA) and Tympanometry were performed on all participants at the audiology section of the Otorhinolaryngology Clinic, LUTH. Results: A total of 99 SCD patients aged between 18 and 29 years (Study group) and 99 medical students aged between 22 and 28 years with HBAA genotype (control group) were studied. The prevalence of hearing loss in the study and control groups was 21.2% and 9.1%, respectively. The difference in the prevalence was statistically significant, P=0.014. Bilateral SNHL was noted in all those who had hearing loss. Hearing loss was mild in 78.2% and 100% of the study and control groups, respectively, moderate and moderate-severe in 14.3 % each of the study group. Conclusion: The prevalence of hearing loss was significantly higher in young adult patients with SCD than in those with the HbAA genotype.  The hearing loss was bilateral and mostly mild. However, a quarter of the patients with SCD who had hearing loss had moderate to moderate-severe hearing loss.