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.
Reference
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.
Recent Posts
Academy Recognizes First Distinguished Fellows of the American Academy of Audiology (DFAAA)
The American Academy of Audiology is proud to announce the inaugural cohort of Distinguished Fellows of the American Academy of Audiology (DFAAA), a prestigious honor…
The Government Is Shutdown, Now What?
Congress has failed to avert a government shutdown. The Department of Health and Human Services (HHS) is expected to face significant workforce reductions across its…
Meet the Academy Board of Directors
The American Academy of Audiology is proud to announce its Board of Directors, a group of dedicated leaders guiding the profession forward and ensuring the…