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[Hearing loss and airway problems in children with mucopolysaccharidoses]

Saturnino Santos1, Laura López, Luis González

  • 1Servicio de ORL, Hospital Infantil Universitario Niño Jesús, Madrid, España. satursantos@gmail.com

Acta Otorrinolaringologica Espanola
|July 16, 2011
PubMed

Insights

Children with mucopolysaccharidoses (MPS) have higher risks of hearing loss and obstructive sleep apnea/hypopnea syndrome (OSAHS). OSAHS may recur more frequently after surgery in these patients.

Area of Science:

  • Otorhinolaryngology
  • Genetics
  • Pediatrics

Context:

  • Mucopolysaccharidoses (MPS) are rare genetic disorders affecting multiple organ systems.
  • Early childhood onset leads to ENT issues like otitis media, hearing loss, and airway obstruction.

Purpose:

  • To describe audiological findings and airway pathology in 9 children diagnosed with MPS.
  • To evaluate ENT-related health issues in pediatric MPS patients.

Summary:

  • A retrospective review of 9 MPS patients (types I, II, IV, VI) revealed chronic middle ear effusions in all.
  • Moderate bilateral conductive hearing loss was common; sensorineural and mixed hearing loss occurred in some.
  • Four patients had obstructive sleep apnea/hypopnea syndrome (OSAHS) due to Waldeyer ring hyperplasia, with one case complicated by spinal cord compression.

Impact:

  • Children with MPS face increased risks of sensorineural hearing loss.
  • OSAHS is more prevalent in MPS patients, with higher recurrence rates post-surgery.
  • These children require careful airway management due to potential complications.
Abstract

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