CONCLUSION: 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. OBJECTIVE: 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. MATERIALS AND METHODS: The temporal bone morphology of an affected fetus was examined with light microscopy and synchrotron radiation-based micro computed tomography. RESULTS: The regular structure of the vestibulo-cochlear system was shown in this multi-modular analysis.
CONCLUSION: 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. OBJECTIVE: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. MATERIALS AND METHODS: The temporal bone morphology of an affected fetus was examined with light microscopy and synchrotron radiation-based micro computed tomography. RESULTS: The regular structure of the vestibulo-cochlear system was shown in this multi-modular analysis.
Authors: Thierry Morlet; Kyoko Nagao; S Charles Bean; Sara E Mora; Sarah E Hopkins; Grace M Hobson Journal: J Neurol Date: 2018-05-03 Impact factor: 4.849
Authors: Rudolf Glueckert; Lejo Johnson Chacko; Dominik Schmidbauer; Thomas Potrusil; Elisabeth J Pechriggl; Romed Hoermann; Erich Brenner; Alen Reka; Anneliese Schrott-Fischer; Stephan Handschuh Journal: Front Neurosci Date: 2018-07-31 Impact factor: 4.677