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.

Share this

Related Posts

Implants Are Not Just for the Cochlea Anymore

Implants Are Not Just for the Cochlea Anymore

Severe bilateral vestibular loss (BVL), regardless of etiology, often is accompanied by significant, chronic symptoms of general imbalance, instability, and blurred vision (oscillopsia) with head movement. These symptoms necessitate lifestyle changes, and possible discontinuation of certain everyday activities. Furthermore, patients become reliant on visual and somatosensory cues to maintain balance. This creates significant functional impairment…

Read More

Government Accountability Office Releases Assessment of COVID-19 Telehealth Flexibilities in Medicare and Medicaid

The United States Government Accountability Office (GAO) released testimony on May 19 regarding their ongoing assessment of COVID-19 flexibilities within the Medicare and Medicaid programs, as required under federal pandemic response oversight provisions included the Coronavirus Aid, Relief, and Economic Security (CARES) Act. While the GAO reported telehealth flexibilities as critical to reducing obstacles of care, they also stressed considering its potential…

Read More

Sounds of Summer: Brood X

By Bre Myers “I feel like I have cicadas in my head” is a regional description of tinnitus I have heard more than a few times in my career. Now, with the emergence of Brood X in the Northeast/Mid-Atlantic section of the United States, that drone is stimulating most everyone’s auditory cortex. Once quiet walks in…

Read More