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Two infants of Pelizaeus-Merzbacher disease with abnormal ABR configurations and fairly preserved hearing ability Yuuki Saito 1 1Department of Otolaryngology,Tokyo University School of Medicine pp.833-838
Published Date 2005/10/20
DOI https://doi.org/10.11477/mf.1411100206
  • Abstract
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We report the audiological and radiological findings including auditory brainstem responses(ABR)in two isolated Japanese boys with Pelizaeus-Merzbacher disease. Although both patients show wave Ⅰ only or wavesⅠ and Ⅱ in ABR,their hearing ability is preserved by conditioned orientation response audiometry(COR). T2 weighted MRI revealed high intensity of white matter and low intensity of gray matter in the cerebrum and spinal cord;These findings indicate the demyelinated area of the brain. The reason why discrepancy between the ABR and audiological findings is not clear.


Copyright © 2005, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1316 印刷版ISSN 0914-3491 医学書院

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