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Related Experiment Videos

Chronological changes of hearing in pediatric patients with large vestibular aqueduct syndrome.

Chien-Chung Lai1, An-Suey Shiao

  • 1Department of Otolaryngology, National Yang-Ming University, Taipei, Taiwan, Republic of China.

The Laryngoscope
|May 6, 2004
PubMed
Summary

Large vestibular aqueduct syndrome (LVAS) causes fluctuating hearing loss, often moderate to profound in childhood. Hearing stability is common in at least one ear, with standard deviation at 500 Hz predicting progression.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Large vestibular aqueduct syndrome (LVAS) is a congenital inner ear malformation associated with hearing loss.
  • Understanding the natural history and prognostic factors of hearing loss in LVAS is crucial for patient management.

Purpose of the Study:

  • To describe the chronologic hearing changes in patients with LVAS.
  • To identify prognostic factors influencing hearing loss progression in LVAS.

Main Methods:

  • Retrospective chart review of 12 consecutive LVAS patients.
  • Analysis of serial audiograms and high-resolution computed tomography (HRCT) scans.
  • Statistical analysis of variables including hearing loss type, vestibular aqueduct size, and chronologic hearing changes.

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Main Results:

  • Hearing loss in early childhood ranged from moderate to profound.
  • 16 out of 24 ears demonstrated stable hearing thresholds.
  • High-tone hearing loss was more common than low-tone, with smaller fluctuations.
  • Vestibular aqueduct size correlated with hearing fluctuation patterns.

Conclusions:

  • LVAS is characterized by fluctuating sensorineural hearing loss.
  • Hearing stability in at least one ear is common.
  • Standard deviation of hearing at 500 Hz is a significant prognostic factor for hearing progression.