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Auditory evoked potentials in Rett syndrome

B A Stach1, W R Stoner, S L Smith

  • 1Department of Otorhinolaryngology & Communicative Sciences, Baylor College of Medicine, Houston, Texas.

Journal of the American Academy of Audiology
|May 1, 1994
PubMed
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This study assessed auditory function in Rett syndrome, finding normal auditory brainstem responses but impaired middle latency and late vertex responses, indicating central auditory processing disorders despite intact peripheral hearing.

Area of Science:

  • Neuroscience
  • Audiology
  • Genetics

Background:

  • Rett syndrome is a rare neurodevelopmental disorder affecting girls, characterized by progressive neurological deficits.
  • Auditory function assessment is crucial for understanding communication and cognitive impairments in Rett syndrome.

Observation:

  • Auditory evoked potentials (AEPs), including ABR, MLR, and LVR, were recorded in 36 individuals with Rett syndrome.
  • A systematic decline in auditory function was observed from peripheral to central auditory pathways.

Findings:

  • All subjects demonstrated normal auditory brainstem responses (ABR), indicating intact peripheral auditory pathways and brainstem function.
  • Fifty percent of subjects had normal middle latency responses (MLR), and 36% had normal late vertex responses (LVR).

Related Experiment Videos

  • Abnormalities in MLR and LVR suggest the presence of central auditory processing disorders in individuals with Rett syndrome.

    • Implications:

    • Hearing sensitivity and the integrity of the eighth nerve and auditory brainstem are preserved in Rett syndrome.
    • The findings highlight the presence of central auditory disorders, impacting communication and cognitive function in Rett syndrome.
    • Further research into central auditory processing deficits is warranted to develop targeted interventions.