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Related Experiment Videos

Hyperacusis in Williams syndrome.

L B Johnson1, M Comeau, K D Clarke

  • 1Department of Otolaryngology, Dalhousie University, Halifax, Nova Scotia.

The Journal of Otolaryngology
|January 5, 2002
PubMed
Summary

Children with Williams syndrome experience hyperacusis, potentially linked to cochlear issues. Audiologic tests revealed sensorineural hearing loss in some, suggesting cochlear disease may affect outer hair cell function.

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Area of Science:

  • Audiology
  • Neuroscience
  • Genetics

Background:

  • Hyperacusis theories are debated.
  • Williams syndrome is associated with universal hyperacusis in children, impacting social interactions.
  • Reduced inhibitory modulation of efferent sensory input to the cochlea is a potential mechanism.

Purpose of the Study:

  • Define hyperacusis using audiologic parameters.
  • Investigate central and peripheral auditory pathways in Williams syndrome.
  • Explore the link between cochlear function and hyperacusis.

Main Methods:

  • Nine patients with Williams syndrome underwent comprehensive audiologic evaluations.
  • Tests included audiogram, speech reception thresholds, acoustic reflexes, impedance, and transient evoked otoacoustic emissions (TEOAEs).
  • Efferent auditory system function was assessed by measuring TEOAE changes with contralateral ear stimulation.

Main Results:

  • Three patients had high-frequency sensorineural hearing loss (SNHL) with absent TEOAEs.
  • Two patients had normal hearing and normal TEOAEs.
  • Four patients exhibited normal hearing but absent TEOAEs, suggesting cochlear pathology.

Conclusions:

  • Findings suggest cochlear disease and support the hypothesis of outer hair cell modulation by the medial olivocochlear system.
  • Behavioral challenges in Williams syndrome complicate audiologic diagnosis.
  • Increased SNHL in older patients blurs the line between loudness recruitment and hyperacusis.

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