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Auditory brainstem response findings in Rett syndrome.

R O Pelson1, S S Budden

  • 1Crippled Children's Division, Oregon Health Sciences University, Portland 97207.

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This study compared auditory brainstem response (ABR) findings in Rett syndrome subjects to controls. Results indicate auditory brainstem pathway involvement in Rett syndrome, supporting diffuse central nervous system pathology.

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Area of Science:

  • Neuroscience
  • Audiology
  • Genetics

Background:

  • Rett syndrome is a rare neurodevelopmental disorder.
  • Auditory brainstem pathway function is crucial for hearing.
  • Previous research suggests central nervous system (CNS) involvement in Rett syndrome.

Purpose of the Study:

  • To investigate auditory brainstem response (ABR) characteristics in individuals with Rett syndrome.
  • To compare ABR findings in Rett syndrome with normal female and male control groups.
  • To explore potential auditory brainstem pathway involvement in Rett syndrome.

Main Methods:

  • Auditory brainstem response (ABR) testing was performed on 11 subjects with Rett syndrome.
  • ABR data were compared to two control groups (normal females and normal males).
  • Ipsilateral and contralateral responses were recorded and analyzed for specific parameters like Wave V latency and III-V interval.

Main Results:

  • Significant differences were observed in Wave V latency and the III-V interval between Rett subjects and normal female controls (ipsilateral recording).
  • The difference between ipsilateral and contralateral III-V intervals showed significant variations between the Rett group and both control groups.
  • These findings suggest some degree of auditory brainstem pathway involvement in Rett syndrome.

Conclusions:

  • The study provides evidence for auditory brainstem pathway involvement in Rett syndrome.
  • Findings support the hypothesis of diffuse central nervous system (CNS) pathology in Rett syndrome.
  • Further ABR studies are recommended to better understand this clinical entity.