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Hearing loss in children with primary ciliary dyskinesia

Kathryn L Kreicher1, Heather K Schopper1, Akash N Naik1

  • 1Department of Otolaryngology-Head & Neck Surgery, Medical University of South Carolina, 135 Rutledge Ave, MSC 550, Charleston, SC 29425, USA.

International Journal of Pediatric Otorhinolaryngology
|December 31, 2017
PubMed

Insights

Pediatric patients with primary ciliary dyskinesia (PCD) often experience hearing loss, primarily conductive. Early otolaryngologist evaluation is recommended for all children with situs inversus at birth.

Area of Science:

  • Otolaryngology
  • Pediatric Audiology
  • Genetics

Background:

  • Primary ciliary dyskinesia (PCD) is a genetic disorder affecting cilia function.
  • Hearing impairment is a common but understudied complication in pediatric PCD.
  • Otologic manifestations in PCD require further investigation to guide management.

Purpose of the Study:

  • To assess the characteristics of hearing impairment in children with PCD.
  • To correlate hearing loss with patient demographics and otologic factors.
  • To evaluate the impact of treatments on hearing outcomes in PCD patients.

Main Methods:

  • Retrospective analysis of pediatric patients with PCD, Kartagener's syndrome, or situs inversus.
  • Audiometric data reviewed for type, severity, and progression of hearing loss.
  • Medical records analyzed for associated otologic conditions and treatments.

Main Results:

  • 42 out of 56 pediatric PCD patients had hearing loss (HL).
  • Conductive hearing loss (CHL) was most frequent; 30% had sensorineural hearing loss (SNHL) component.
  • Otitis media was highly prevalent (92.9%), but HL often persisted despite treatment.

Conclusions:

  • Slight to mild CHL and otitis media are common in pediatric PCD.
  • SNHL can also occur in this population.
  • Mandatory otolaryngologist evaluation for all newborns with situs inversus is advised.
Abstract

Keywords:
Pediatric hearing lossPrimary ciliary dyskinesia

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