Journal article
Auditory function in Pelizaeus-Merzbacher disease
Journal of neurology, v 265(7), pp 1580-1589
01 Jul 2018
PMID: 29725841
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Pelizaeus-Merzbacher disease (PMD; MIM 312080), an inherited defect of central nervous system myelin formation, affects individuals in many ways, including their hearing and language abilities. The aim of this study was to assess the auditory abilities in 18 patients with PMD by examining the functional processes along the central auditory pathways using auditory brainstem responses (ABR) and cortical auditory evoked potentials (CAEP) in response to speech sounds. The significant ABR anomalies confirm the existence of dyssynchrony previously described at the level of the brainstem in patients with PMD. Despite the significant auditory dyssynchrony observed at the level of the brainstem, CAEPs were present in most patients, albeit somehow abnormal in terms of morphology and latency, resembling a type of auditory neuropathy spectrum disorder.
Metrics
1 Record Views
Details
- Title
- Auditory function in Pelizaeus-Merzbacher disease
- Creators
- Thierry Morlet - University of DelawareKyoko Nagao - Alfred I. duPont Hospital for ChildrenS. Charles Bean - Alfred I. duPont Hospital for ChildrenSara E. Mora - Alfred I. duPont Hospital for ChildrenSarah E. Hopkins - Children's Hospital of PhiladelphiaGrace M. Hobson - Alfred I. duPont Hospital for Children
- Publication Details
- Journal of neurology, v 265(7), pp 1580-1589
- Publisher
- Springer Nature
- Number of pages
- 10
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Audiology - Distance
- Web of Science ID
- WOS:000436812900010
- Scopus ID
- 2-s2.0-85046403855
- Other Identifier
- 991022168371904721
UN Sustainable Development Goals (SDGs)
This publication has contributed to the advancement of the following goals:
InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Clinical Neurology