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Auditory function in Pelizaeus-Merzbacher disease

Thierry Morlet1,2,3, Kyoko Nagao4,5, S Charles Bean6

  • 1Auditory Physiology and Psychoacoustics Research Laboratory, Center for Pediatric Auditory Speech Sciences, Nemours/Alfred I. duPont Hospital for Children, 1701 Rockland Rd., Wilmington, DE, 19803, USA. thierry.morlet@nemours.org.

Journal of Neurology
|May 5, 2018
PubMed

Insights

Pelizaeus-Merzbacher disease (PMD) impacts hearing by causing auditory dyssynchrony in the brainstem. While central auditory pathways show abnormalities, most patients retain some auditory function, similar to auditory neuropathy spectrum disorder.

Area of Science:

  • Neuroscience
  • Genetics
  • Audiology

Background:

  • Pelizaeus-Merzbacher disease (PMD) is an inherited disorder affecting central nervous system myelin formation.
  • PMD can impact auditory and language functions due to its neurological effects.

Purpose of the Study:

  • To evaluate auditory pathway function in 18 PMD patients using auditory brainstem responses (ABR) and cortical auditory evoked potentials (CAEP).
  • To investigate the impact of PMD on central auditory processing of speech sounds.

Main Methods:

  • Utilized auditory brainstem responses (ABR) to assess brainstem auditory pathway function.
  • Employed cortical auditory evoked potentials (CAEP) to evaluate higher-level auditory processing.
  • Administered speech sound stimuli to assess responses along the central auditory pathways.

Main Results:

  • Significant ABR anomalies confirmed brainstem dyssynchrony in PMD patients.
  • CAEPs were present in most patients, indicating some preserved auditory function.
  • CAEPs exhibited abnormal morphology and latency, suggesting a resemblance to auditory neuropathy spectrum disorder.

Conclusions:

  • Auditory brainstem response anomalies highlight significant dyssynchrony in the central auditory pathways of PMD patients.
  • Cortical auditory evoked potentials, despite abnormalities, suggest preserved, albeit altered, auditory processing in the central auditory system.
  • Findings indicate a potential link between PMD's neurological deficits and auditory neuropathy spectrum disorder characteristics.
Keywords:
Auditory brainstem responsesCortical auditory evoked potentialsHearingPelizaeus–Merzbacher disease

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