Auditory function in Pelizaeus-Merzbacher disease.
J Neurol
; 265(7): 1580-1589, 2018 Jul.
Article
en En
| MEDLINE
| ID: mdl-29725841
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Enfermedades Auditivas Centrales
/
Potenciales Evocados Auditivos del Tronco Encefálico
/
Enfermedad de Pelizaeus-Merzbacher
Tipo de estudio:
Diagnostic_studies
Límite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
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Humans
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Infant
/
Male
Idioma:
En
Revista:
J Neurol
Año:
2018
Tipo del documento:
Article
País de afiliación:
Estados Unidos