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Vestibular function in children with the CHARGE association.

S R Wiener-Vacher1, L Amanou, P Denise

  • 1Department of Otorhinolaryngology, Hôpital Robert Debré, University of Paris VII, France. sylvette.wiener@rdb.ap-hop-paris.fr

Archives of Otolaryngology--Head & Neck Surgery
|April 6, 1999
PubMed
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Children with CHARGE association lack semicircular canals but retain functional otolith organs. This residual vestibular function may aid posturomotor development despite developmental delays.

Area of Science:

  • Otolaryngology
  • Neuroscience
  • Developmental Pediatrics

Background:

  • CHARGE association is a complex malformative syndrome characterized by multiple anomalies, including ear malformations and hearing loss.
  • Previous studies identified absence of semicircular canals and cochlear dysplasia in CHARGE association patients.
  • Vestibular function, particularly otolith function, has not been extensively studied in this population.

Purpose of the Study:

  • To investigate residual vestibular function in patients diagnosed with CHARGE association.
  • To evaluate the otolith-vestibular system's response in individuals with CHARGE association.

Main Methods:

  • Electro-oculography was used to record vestibulo-ocular responses in 7 patients with CHARGE association.
  • Rotational chair testing involved earth-vertical axis and off-vertical axis rotations.

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  • Assessment focused on differentiating canal and otolith-vestibular system function.

    • Main Results:

    • Computed tomographic scans confirmed the absence of semicircular canals in all 7 patients.
    • No canal vestibulo-ocular responses were detected during earth-vertical axis rotation.
    • All patients demonstrated normal otolith vestibulo-ocular responses during off-vertical axis rotation.

    Conclusions:

    • The findings support the presence of a functional otolith organ in the hypoplastic posterior labyrinth of children with CHARGE association.
    • Severe psychomotor delays are likely multifactorial, including canal vestibular deficits, visual impairment, and environmental factors.
    • Harnessing the remaining otolith system sensitivity could inform early educational interventions for improved posturomotor development.