Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study

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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

Published on: January 25, 2016

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L De Schrijver1, V Topsakal2, M Wojciechowski3

  • 1Faculty of Medicine, University of Antwerp, Belgium.

International Journal of Pediatric Otorhinolaryngology
|December 18, 2018
PubMed

Insights

Sensorineural hearing loss (SNHL) is uncommon in children with Down syndrome (DS), affecting 4.5%. Etiological work-ups can identify causes like cochlear nerve deficiency, which was found in four children with single-sided deafness.

Area of Science:

  • Pediatrics
  • Genetics
  • Audiology

Background:

  • Sensorineural hearing loss (SNHL) prevalence and causes in children with Down syndrome (DS) are not well understood.
  • Early identification and management of hearing loss are crucial for development in children with DS.

Purpose of the Study:

  • To determine the prevalence, severity, laterality, and causes of SNHL in children with DS.
  • To investigate specific etiologies contributing to hearing loss in this population.

Main Methods:

  • A cross-sectional study was conducted on children with DS followed at the Antwerp University Hospital's multidisciplinary Downteam.
  • Data collected included patient characteristics, hearing loss risk factors, audiometric results, and etiological work-up findings.

Main Results:

  • Out of 291 children with DS, 138 (47.4%) had hearing loss, primarily due to middle ear effusion.
  • SNHL was present in 13 patients (4.5%), with bilateral involvement in 8 cases.
  • Identified causes included cochlear nerve deficiency (4 patients), genetic factors (1), and noise exposure (2); etiology was unknown in 6.

Conclusions:

  • SNHL is relatively uncommon in children with DS, with a prevalence of 4.5%.
  • Etiological investigation is valuable for identifying specific causes of hearing loss.
  • Cochlear nerve deficiency was a notable finding in children with single-sided deafness.
Abstract

Keywords:
Down syndromeSensorineural hearing lossSingle sided deafnessTrisomy 21

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