Hearing profile and MRI myelination of auditory pathway in Pelizaeus-Merzbacher disease

Acta Oto-laryngologica
Chen-Chieh KuanKazuo Kodama

Abstract

This study showed that delayed auditory pathway myelination is common in Pelizaeus-Merzbacher disease (PMD), but this delay does not necessarily indicate poor hearing function. PMD is a rare recessively inherited X-linked leukodystrophy characterized by defective central nervous system myelination owing to a mutation in the proteolipid protein gene (PLP). The aims of this study were to evaluate the hearing function and auditory brain response (ABR) findings of patients with PMD and relate these findings to MRI-assessed myelination in the central auditory pathway. We retrospectively studied eight male pediatric patients with PMD. Serial auditory examinations included audiometry, behavior audiometry, distortion product otoacoustic emission (DPOAE), and ABR. MRI-assessed myelination in the auditory pathway was evaluated in the PMD patients and in 23 normal young children as a control group. Audiometry showed normal to moderate hearing impairment and the hearing threshold improved with age and became almost normal over time. DPOAEs positivity and only ABR wave I or waves I and II were found in all the patients. MRI showed delayed myelination in all the patients and the auditory pathway was myelinated up to the inferior colliculus i...Continue Reading

References

Feb 1, 1987·Annals of Neurology·A H KoeppenM B Hans
May 1, 1995·Brain & Development·A Nezu
Sep 1, 1993·Early Human Development·Y KonishiY Ishii
Jul 1, 1995·Brain Pathology·F Seitelberger
May 20, 1998·Pediatric Neurology·A NezuK Inoue
Sep 17, 2002·Journal of Neuropathology and Experimental Neurology·Arnulf H Koeppen, Yves Robitaille

❮ Previous
Next ❯

Citations

May 16, 2014·PloS One·Georg von JonquieresMatthias Klugmann
Jan 7, 2015·Neurobiology of Disease·Joshua A MayerIan D Duncan
Jul 19, 2020·The Journal of Comparative Neurology·Alejandra Lopez-JuarezVictor H Hernandez
May 5, 2018·Journal of Neurology·Thierry MorletGrace M Hobson
Feb 10, 2021·Developmental Neurobiology·David C KohrmanGabriel Corfas

❮ Previous
Next ❯

Related Concepts

Related Feeds

Brain developing: Influences & Outcomes

This feed focuses on influences that affect the developing brain including genetics, fetal development, prenatal care, and gene-environment interactions. Here is the latest research in this field.