The pathophysiology of sensorineural hearing loss (SNHL) in children with sickle cell disease (SCD) is thought to be due to microvascular occlusion incidents that compromise oxygenation through the labyrinthine artery to the cochlea. Ischemia of the highly metabolic cochlea and Organ of Corti results in decreased oxygenation of the stria vascularis and a failure to maintain endolymph.
Strum and colleagues (2021) performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of SCD and hearing loss in populations less than 18 years of age. They excluded studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis.
They identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33.
This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research, specifically into the predictors of SNHL severity. Audiologists will need to routinely monitor patients with SCD to ensure early identification an intervention for those found with hearing loss at a young age.
Strum, et al. (2021) Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-Analysis. The Laryngoscope 131(5):1147-1156. doi:10.1002/lary.29199.
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