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Temporal bone CT findings in the CHARGE association

R J Admiraal1, F B Joosten, P L Huygen

  • 1Department of Otorhinolaryngology, University Hospital Nijmegen, The Netherlands. R.Admiraal@kno.azn.nl

International Journal of Pediatric Otorhinolaryngology
|December 16, 1998
PubMed
Summary

CHARGE association and CHARGE-like cases often present with inner ear abnormalities. Temporal bone CT scans are crucial for diagnosing these conditions, revealing semicircular canal aplasia and oval window obliteration, even with normal hearing.

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Area of Science:

  • Otolaryngology
  • Medical imaging
  • Genetics

Background:

  • CHARGE association is a complex genetic disorder with diverse clinical manifestations.
  • Inner ear malformations are frequently observed in CHARGE syndrome, impacting hearing and balance.

Observation:

  • Temporal bone CT and/or MRI were performed on 10 CHARGE and 2 CHARGE-like cases.
  • All cases exhibited bilateral semicircular canal aplasia and oval window obliteration.
  • Vestibular examination revealed areflexia in all evaluated CHARGE and CHARGE-like cases.

Findings:

  • Among 16 evaluable CHARGE cases, 8 had bilateral mixed hearing impairment.
  • 8 cases had sensorineural hearing impairment (6 bilateral, 2 unilateral).
  • Vestibular areflexia was present in all assessed cases.

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Implications:

  • Temporal bone CT scanning is recommended for suspected CHARGE cases.
  • Imaging is indicated regardless of initial hearing test results.
  • Early detection of inner ear anomalies aids in management and intervention planning.