Auditory function in Pelizaeus-Merzbacher disease

TitleAuditory function in Pelizaeus-Merzbacher disease
Publication TypeJournal Article
Year of Publication2018
AuthorsMorlet, T., Nagao K., Bean S. C., Mora S. E., Hopkins S. E., & Hobson G. M.
JournalJournal of neurology
Date Published2018 Jul
KeywordsAcoustic Impedance Tests; Acoustic Stimulation; Adolescent; Adult; Auditory brainstem responses; Auditory Diseases, Central; Auditory Pathways; Child; Child, Preschool; Cortical auditory evoked potentials; Electroencephalography; Evoked Potentials, Auditory, Brain Stem; Female; Hearing; Humans; Infant; Male; Otoacoustic Emissions, Spontaneous; Otoscopy; Pelizaeus-Merzbacher Disease; Pelizaeus–Merzbacher disease; Young Adult

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.

Alternate JournalJ. Neurol
Refereed DesignationRefereed