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[Inner ear in C.H.A.R.G.E. association].

L Amanou1, M Morisseau-Durand, S Wiener-Vacher

  • 1Service ORL, Hôpital Boucicaut, Paris.

Annales D'Oto-Laryngologie Et De Chirurgie Cervico Faciale : Bulletin De La Societe D'Oto-Laryngologie Des Hopitaux De Paris
|June 23, 2000
PubMed
Summary

Children with CHARGE association often have inner ear anomalies affecting hearing and balance. Early intervention with hearing aids and therapy can aid language development despite these cochleovestibular dysfunctions.

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Area of Science:

  • Otolaryngology
  • Pediatric Neurology
  • Genetics

Context:

  • CHARGE association is a rare genetic disorder with complex congenital anomalies.
  • Cochleovestibular dysfunction is a significant feature impacting development.
  • Inner ear anomalies are a key diagnostic characteristic.

Purpose:

  • To investigate the specific inner ear (cochleovestibular) anomalies in children with CHARGE association.
  • To correlate temporal bone findings with audiological and vestibular function.
  • To understand the impact of these dysfunctions on development and inform therapeutic strategies.

Summary:

  • Temporal bone CT scans revealed specific anomalies in 17 children with CHARGE, including posterior labyrinth aplasia and variable anterior labyrinth abnormalities.

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  • Audiological evaluations showed variable hearing loss, often exacerbated by middle ear effusion and neurological immaturity.
  • Vestibular testing indicated constant canal areflexia but preserved otolith function, suggesting a role in posturo-motor delays.

    • Impact:

    • Findings highlight the unique temporal bone abnormalities in CHARGE association.
    • Early and regular audiological monitoring is crucial due to variable hearing loss.
    • Understanding vestibular deficits aids in tailoring physical therapy for improved motor development and adaptation to sensory impairments.