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[ENT changes in Crouzon's syndrome].

M A Campos Del Alamo1, J Pérez Obón, P Gil Paraíso

  • 1Hospital Miguel Servet de Zaragoza.

Anales Otorrinolaringologicos Ibero-Americanos
|May 7, 1999
PubMed
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Crouzon's syndrome, a craniosynostosis, can cause hearing loss. This case highlights severe otic and sinus malformations in a patient with this genetic disorder.

Area of Science:

  • Genetics
  • Otolaryngology
  • Developmental Biology

Background:

  • Crouzon's syndrome is an autosomal dominant craniosynostosis characterized by premature cranial suture closure.
  • Genetic mutations, specifically in FGFR2 on chromosome 10, are linked to Crouzon's syndrome.
  • While cranial deformities are common, otic and ENT-sphere malformations are less frequently reported.

Observation:

  • A 16-year-old female with Crouzon's syndrome presented with bilateral hearing impairment (hypacusis).
  • Clinical examination revealed bilateral auditory canal atresia and underdeveloped mastoid air cells.
  • Additional findings included anomalous facial nerve pathways, significant bilateral conductive hearing loss, deviated nasal septum, and hypoplastic paranasal sinuses.

Findings:

  • The patient exhibited severe bilateral conductive hearing loss due to auditory canal atresia.

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  • Associated findings included mastoid hypopneumatization and nasal/sinus abnormalities.
  • These otic and craniofacial findings are consistent with complex presentations of Crouzon's syndrome.

    • Implications:

    • This case underscores the importance of thorough otolaryngological evaluation in patients with Crouzon's syndrome.
    • Early identification of hearing impairment and associated malformations is crucial for timely intervention.
    • Understanding the spectrum of Crouzon's syndrome aids in comprehensive patient management and genetic counseling.