Audio-vestibular Findings in a Patient with Pelizaeus- Merzbacher Disease.

Autor: Yuvaraj P; Department of Speech Pathology and Audiology, NIMHANS, Bengaluru, Karnataka, India., Swamy SN; Department of Speech Pathology and Audiology, NIMHANS, Bengaluru, Karnataka, India., Chethan K; Department of Speech Pathology and Audiology, NIMHANS, Bengaluru, Karnataka, India., Kenchaiah R; Department of Neurology, NIMHANS, Bengaluru, Karnataka, India., Udupi GA; Department of Human Genetics, NIMHANS, Bengaluru, Karnataka, India., Rajasekaran AK; Department of Speech Pathology and Audiology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Karnataka, India.
Jazyk: angličtina
Zdroj: The journal of international advanced otology [J Int Adv Otol] 2024 Jul 29; Vol. 20 (4), pp. 375-378.
DOI: 10.5152/iao.2024.231261
Abstrakt: Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.
Databáze: MEDLINE