Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis

Abstract

Objectives and Main Outcome Measures: To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity. Study Design: Systematic Review and Meta-analysis. Methods: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD. Results: We identified 138 studies from the initial search, 12 of which met inclusion criteria and were utilized for data analysis. A total of 636 SCD patients and 360 controls were included in the Cochrane Review Manager 5.3 meta-analysis. There was a statistically significant increase in the prevalence of SNHL in adults with SCD compared with the general population with a cumulative risk ratio (RR) of 6.03. Conclusions: This is the first systematic investigation of the relationship between SCD and SNHL in adult patients across the globe. SNHL is more prevalent in patients with SCD, specifically those of the HbSS genotype, than the general population likely due to the pathophysiology of the disease and its effects on labyrinthine microvasculature. The increased prevalence of SNHL in the adult SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of adult SCD patients.

Sex and Race Representation in Temporal Bone Histopathology Studies in the United States: A Systematic Review

“…Such scientific inquiries have the potential to reveal basic mechanisms of sensorineural hearing loss such as recovery from sensorineural hearing loss in patients with sickle cell crisis (Urban 1973; Alkindi et al 2011; Kim et al 2018). The pathophysiology of functional hearing loss in sickle cell disease may be due to expanded bone marrow in the petrous temporal bone, ischemia of hair cells secondary to micro-occlusive crisis, or hypoxic injury secondary to decreased total hemoglobin (Kapoor et al 2021). Investigations into the reversibility of hearing loss in this context has the potential to reveal basic mechanisms of cochlear physiology.…”

Consideration of health inequity in systematic reviews and primary studies on risk factors for hearing loss

“…Of the 64 SRs included in Briscoe et al [35], 17 met the inclusion criteria for this secondary analysis (see Table 2) [38][39][40][41][42][43][44][45][46][47][48][49][50][51][52]. Of these, seven were published in 2021, and 10 were published in 2022.…”

“…Of the 17 included SRs, 10 considered physiological risk factors for hearing loss [38][39][40][41][42][43][44][45][46][47], four considered environmental risk factors [52][53][54], two considered demographic risk factors [49,50], and one considered behavioral risk factors [48]. Of the 251 primary studies, 248 were observational studies, and three were RCTs.…”

“…The occupation was the next most frequently reported data, including at baseline in 81 studies (32.66%) and in the results of 69 studies (27.82%) (see Table 3). All 17 SRs included studies which reported gender and personal characteristics at baseline, and 15 SRs included studies which reported gender and personal characteristics in the analysis of results, although in some reviews the latter was in a small proportion of included studies [38,[41][42][43][44][45][46][47][48][49][50][51][52][53][54]. Studies reporting data on occupation were included in a total of eight SRs [38,42,45,48,49,51,53,54].…”

“…All 17 SRs included studies which reported gender and personal characteristics at baseline, and 15 SRs included studies which reported gender and personal characteristics in the analysis of results, although in some reviews the latter was in a small proportion of included studies [38,[41][42][43][44][45][46][47][48][49][50][51][52][53][54]. Studies reporting data on occupation were included in a total of eight SRs [38,42,45,48,49,51,53,54]. Place of residence, race/ethnicity, education, socioeconomic status, social capital and features of relationships were also reported across the included studies, albeit in fewer than 10% of studies at both baseline and in results for all criteria.…”