TY  - JOUR
AU  - Schmutzhard, J.
AU  - Schwentner, I.
AU  - Glueckert, Robert
AU  - Sergi, C.
AU  - Beckmann, F.
AU  - Abraham, I.
AU  - Riechelmann, H.
AU  - Schrott-Fischer, A.
AU  - Müller, B.
AU  - DESY
TI  - Pelizaeus Merzbacher Disease: Morphological Analysis of the Vestibulo-cochlear System
JO  - Acta oto-laryngologica
VL  - 129
SN  - 0001-6489
CY  - Stockholm
PB  - Informa Healthcare
M1  - PHPPUBDB-10174
SP  - 1395 - 1399
PY  - 2009
N1  - (c) Informa UK Limited. Post referee full text in progress (embargo 1 year from 18 Nov 2009).
AB  - 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.
KW  - Aborted Fetus: pathology
KW  - Aborted Fetus: radiography
KW  - Adult
KW  - Ear: pathology
KW  - Ear: radiography
KW  - Female
KW  - Humans
KW  - Pelizaeus-Merzbacher Disease: pathology
KW  - Pelizaeus-Merzbacher Disease: radiography
KW  - Pregnancy
KW  - Temporal Bone: pathology
KW  - Temporal Bone: radiography
KW  - X-Ray Microtomography
LB  - PUB:(DE-HGF)16
C6  - pmid:19922087
UR  - <Go to ISI:>//WOS:000272981000008
DO  - DOI:10.3109/00016480802698866
UR  - https://bib-pubdb1.desy.de/record/87375
ER  -