%0 Journal Article
%A Schmutzhard, J.
%A Schwentner, I.
%A Glueckert, Robert
%A Sergi, C.
%A Beckmann, F.
%A Abraham, I.
%A Riechelmann, H.
%A Schrott-Fischer, A.
%A Müller, B.
%A DESY
%T Pelizaeus Merzbacher Disease: Morphological Analysis of the Vestibulo-cochlear System
%J Acta oto-laryngologica
%V 129
%@ 0001-6489
%C Stockholm
%I Informa Healthcare
%M PHPPUBDB-10174
%P 1395 - 1399
%D 2009
%Z (c) Informa UK Limited. Post referee full text in progress (embargo 1 year from 18 Nov 2009).
%X 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.
%K Aborted Fetus: pathology
%K Aborted Fetus: radiography
%K Adult
%K Ear: pathology
%K Ear: radiography
%K Female
%K Humans
%K Pelizaeus-Merzbacher Disease: pathology
%K Pelizaeus-Merzbacher Disease: radiography
%K Pregnancy
%K Temporal Bone: pathology
%K Temporal Bone: radiography
%K X-Ray Microtomography
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:19922087
%U <Go to ISI:>//WOS:000272981000008
%R 10.3109/00016480802698866
%U https://bib-pubdb1.desy.de/record/87375