%0 journal article
%@ 0001-6489
%A Schmutzhard, J., Schwentner, I., Glueckert, R., Sergi, C., Beckmann, F., Abraham, I., Riechelmann, H., Schrott-Fischer, A., Mueller, B.

%D 2009
%J Acta Oto-Laryngologica
%N 12
%P 1395-1399 
%R doi:10.1080/00016480802698866
%T Pelizaeus Merzbacher disease: morphological analysis of the vestibulo-cochlear system
%U https://doi.org/10.1080/00016480802698866
12 
%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. 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.