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000087375 1001_ $$0P:(DE-HGF)0$$aSchmutzhard, J.$$b0
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000087375 245__ $$aPelizaeus Merzbacher Disease: Morphological Analysis of the Vestibulo-cochlear System
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000087375 500__ $$3Converted on 2013-05-30 12:57$$a(c) Informa UK Limited. Post referee full text in progress (embargo 1 year from 18 Nov 2009).
000087375 500__ $$3Converted on 2013-06-21 19:20
000087375 520__ $$aIn 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.
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000087375 650_2 $$2MeSH$$aAborted Fetus: pathology
000087375 650_2 $$2MeSH$$aAborted Fetus: radiography
000087375 650_2 $$2MeSH$$aAdult
000087375 650_2 $$2MeSH$$aEar: pathology
000087375 650_2 $$2MeSH$$aEar: radiography
000087375 650_2 $$2MeSH$$aFemale
000087375 650_2 $$2MeSH$$aHumans
000087375 650_2 $$2MeSH$$aPelizaeus-Merzbacher Disease: pathology
000087375 650_2 $$2MeSH$$aPelizaeus-Merzbacher Disease: radiography
000087375 650_2 $$2MeSH$$aPregnancy
000087375 650_2 $$2MeSH$$aTemporal Bone: pathology
000087375 650_2 $$2MeSH$$aTemporal Bone: radiography
000087375 650_2 $$2MeSH$$aX-Ray Microtomography
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000087375 7001_ $$0P:(DE-HGF)0$$aSchwentner, I.$$b1
000087375 7001_ $$0P:(DE-HGF)0$$aGlueckert, Robert$$b2$$eCorresponding author
000087375 7001_ $$0P:(DE-HGF)0$$aSergi, C.$$b3
000087375 7001_ $$0P:(DE-H253)PIP1002967$$aBeckmann, F.$$b4
000087375 7001_ $$0P:(DE-HGF)0$$aAbraham, I.$$b5
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000087375 7001_ $$aMüller, B.$$b8
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