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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
Summary
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.
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.
