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Large vestibular aqueduct syndrome: a case study.

Jackie L Clark1, Ross J Roeser

  • 1UT Dallas/Callier Center for Communication Disorders, 1966 Inwood Road, Dallas, TX 75235, USA. jclark@utdallas.edu

Journal of the American Academy of Audiology
|March 7, 2006
PubMed
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A child with moderate-to-severe hearing loss showed improved audibility with hearing aids. A subsequent cochlear implant for large vestibular aqueduct syndrome led to rapid speech-language gains.

Area of Science:

  • Audiology
  • Pediatric Otolaryngology
  • Genetics

Background:

  • A 23-month-old female presented with moderate-to-severe bilateral mixed hearing loss.
  • Previous tympanostomy tube insertion did not significantly improve hearing sensitivity.

Observation:

  • Initial hearing aid fitting confirmed hearing loss but revealed abnormal middle ear findings.
  • A six-month follow-up showed decreased hearing sensitivity.
  • Radiological studies identified large vestibular aqueduct syndrome (LVAS).

Findings:

  • Binaural digital hearing aids improved audibility based on behavioral and probe microphone measures.
  • Cochlear implantation in the right ear, following LVAS diagnosis, resulted in immediate speech-language progress.

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Implications:

  • Early intervention with hearing aids and cochlear implants can significantly benefit children with complex hearing loss.
  • LVAS requires careful audiological and otological management.
  • Prompt diagnosis and intervention are crucial for optimal speech and language development in pediatric hearing loss.