Prevalence and nature of hearing loss in a cohort of children with sickle cell disease

Abstract

Background: Sickle cell disease (SCD) may cause injury to any organ, including the auditory system. Although the association of SCD and hearing loss has been described, the nature of this complication is unknown. We sought to establish the prevalence and nature of hearing loss in a referred cohort of children with SCD and to identify correlating disease-or treatment-associated factors. Procedure:We conducted a retrospective review of patients with SCD < 22 years of age who had hearing evaluations between August 1990 and December 2014. Demographics, audiograms, and disease and treatment variables were analyzed.Results: Two hundred and ten audiograms among 81 patients were reviewed, and 189 were evaluable. Seventy-two children constituted the referred cohort. Fourteen (19.4%) had hearing loss documented on at least one audiogram. Seven (9.7%) patients had only conductive hearing loss, and the loss persisted for up to 10.3 years. The median age of first identification was eight years.Six (8.3%) patients had hearing loss that was at least partially sensorineural. One patient's hearing loss was ambiguous. All sensorineural hearing losses were unilateral and 4/6 patients had prior documented normal hearing, indicating acquired loss. No correlations were identified. Conclusions:Both conductive and sensorineural hearing losses are more prevalent in our study population than those observed in the general pediatric population. In children with SCD, sensorineural hearing loss appears to be acquired and unilateral. Conductive hearing loss was identified in older children and can persist. Serial screening is needed for early detection and more prompt intervention in this population.

Dysfunction of the auditory system in sickle cell anaemia: a systematic review with meta‐analysis

“…Through this systematic review and meta‐analysis of population‐based prevalence studies, we observed an SNHL prevalence of 20.5% among in SCA patients, indicating a significantly higher prevalence in SCA patients than in healthy controls (HC) (0–7.5%) despite the variability between the studies.…”

“…Of these 27 studies, six were excluded because they did not differentiate between homozygotes (SCA) and heterozygotes in the methods and/or in the results. Thus, 21 articles were included in this systematic review (Figure ).…”

“…All the studies were observational cross‐sectional studies. Among the 21 articles included, 14 primarily aimed to determine the prevalence of SNHL in SCA using PTA. Six studies used the OAE test for identifying sensorial dysfunctions.…”

“…The studies that analysed the prevalence of SNHL using PTA assessed a total of 884 participants with SCA. The samples ranged from 23 to 167 participants of both sexes; the age range was 4–56 years.…”

“…With reference to the characteristics of SNHL in SCA patients, to the severity of hearing loss, we observed a predominance in the mild range [13,33,[35][36][37][38] although different degrees were noted in five studies [12,21,24,32,33]. Apparently, the initial effect was observed at the higher frequencies (4-8 kHz) [11,13,[35][36][37] followed by the low frequencies (0.25-0.5 kHz) [11,13,35].…”