Pelizaeus Merzbacher Disease: Morphological Analysis of the Vestibulo-cochlear System

Schmutzhard, J.; Schwentner, I.; Glueckert, Robert; Sergi, C.; Abraham, I.; Beckmann, F.; Schrott-Fischer, A.; Riechelmann, H.; Müller, B.

doi:10.3109/00016480802698866

Journal Article

Pelizaeus Merzbacher Disease: Morphological Analysis of the Vestibulo-cochlear System

Schmutzhard, J. ; Schwentner, I. ; Glueckert, R. (Corresponding author) ; Sergi, C. ; Beckmann, F.Extern* ; Abraham, I. ; Riechelmann, H. ; Schrott-Fischer, A. ; Müller, B. ; DESY

2009
Informa Healthcare Stockholm

Acta oto-laryngologica 129, 1395 - 1399 (2009) [10.3109/00016480802698866]

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Abstract: In agreement with previously published findings, our results demonstrate that Pelizaeus Merzbacher disease (PMD) does not affect the development and morphology of the peripheral vestibulo-cochlear system.PMD is a consequence of X-linked mutation of the main central nervous system (CNS) myelin protein resulting in a complex neurological syndrome. Otorhinolaryngological symptoms include nystagmus and alterations of auditory-evoked brainstem responses. To date no histopathological analysis of the inner ear has been performed.The temporal bone morphology of an affected fetus was examined with light microscopy and synchrotron radiation-based micro computed tomography.The regular structure of the vestibulo-cochlear system was shown in this multi-modular analysis.

Keyword(s): Aborted Fetus: pathology (MeSH) ; Aborted Fetus: radiography (MeSH) ; Adult (MeSH) ; Ear: pathology (MeSH) ; Ear: radiography (MeSH) ; Female (MeSH) ; Humans (MeSH) ; Pelizaeus-Merzbacher Disease: pathology (MeSH) ; Pelizaeus-Merzbacher Disease: radiography (MeSH) ; Pregnancy (MeSH) ; Temporal Bone: pathology (MeSH) ; Temporal Bone: radiography (MeSH) ; X-Ray Microtomography (MeSH)