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Hearing loss in children with sickle cell disease: A prospective French cohort study

Emilie Bois1, Martine Francois1, Malika Benkerrou2

  • 1Otolaryngology-Head and Neck Surgery Department, Robert Debré Hospital, Paris, France.

Pediatric Blood & Cancer
|September 26, 2018
PubMed

Insights

Hearing loss affects over 11% of French children with sickle cell disease (SCD), necessitating regular audiological follow-up to manage potential complications.

Area of Science:

  • Pediatrics
  • Genetics
  • Otolaryngology

Background:

  • Sickle cell disease (SCD) is the most prevalent genetic disorder in France.
  • SCD is linked to a high incidence of hearing loss in developing nations.
  • The study investigates hearing loss prevalence in French children with SCD.

Purpose of the Study:

  • To determine the prevalence of hearing loss in French children diagnosed with sickle cell disease.
  • To assess the need for close audiological monitoring in pediatric SCD patients.
  • To identify factors associated with hearing impairment in this population.

Main Methods:

  • A single-center prospective cross-sectional study was conducted.
  • Eighty-nine children with SCD (ages 5-19) underwent ENT examinations and hearing assessments.
  • Data collected included SCD genotype, audiological thresholds, and clinical characteristics.

Main Results:

  • 11.2% of children exhibited hearing thresholds above 20 dB.
  • Causes included subnormal hearing, otitis media with effusion (OME), and sensorineural hearing loss.
  • OME was associated with later SCD diagnosis, high platelet count, and low hematocrit; 12.4% reported tinnitus.

Conclusions:

  • Multiple factors contribute to hearing loss in children with SCD.
  • Regular audiological follow-up is crucial for early detection and management of hearing complications.
  • Proactive monitoring can prevent complications from curable conditions and manage progressive hearing loss.
Abstract

Keywords:
hearing losssickle cell diseasevaso-occlusive crisis

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