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The management of otolaryngological problems in the mucopolysaccharidoses: a retrospective review

M J Ruckenstein1, R E Macdonald, J T Clarke

  • 1Department of Otolaryngology, Hospital for Sick Children, University of Toronto, Ontario, Canada.

The Journal of Otolaryngology
|June 1, 1991
PubMed

Insights

Mucopolysaccharidoses (MPS) are rare genetic disorders impacting children, frequently affecting hearing and breathing. Early otolaryngologic intervention is crucial for managing auditory and respiratory issues in MPS patients.

Area of Science:

  • Genetics
  • Pediatrics
  • Otolaryngology

Background:

  • Mucopolysaccharidoses (MPS) are rare, inherited metabolic disorders affecting children.
  • These conditions often involve the auditory system and upper airways, necessitating otolaryngologic care.

Observation:

  • A 10-year retrospective review analyzed the management of MPS patients.
  • Auditory and upper respiratory pathologies were specifically examined.

Findings:

  • Persistent serous otitis media and sensorineural hearing loss are common in MPS.
  • Upper respiratory obstruction, leading to sleep apnea, is a frequent complication.

Implications:

  • Otolaryngologists play a vital role in managing MPS patients.
  • Specific interventions are recommended for otolaryngologic care in children with MPS.

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