title: Pelizaeus Merzbacher disease: morphological analysis of the vestibulo-cochlear system.
abstract: CONCLUSIONS
  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.
  
  
  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.affiliation: Department of Otorhinolaryngology, Innsbruck Medical University, Austria.
authors.forename: Joachim
authors.initials: J
authors.lastname: Schmutzhard
authors.forename: Ilona
authors.initials: I
authors.lastname: Schwentner
authors.forename: Rudolf
authors.initials: R
authors.lastname: Glueckert
authors.forename: Consolato
authors.initials: C
authors.lastname: Sergi
authors.forename: Felix
authors.initials: F
authors.lastname: Beckmann
authors.forename: Irene
authors.initials: I
authors.lastname: Abraham
authors.forename: Herbert
authors.initials: H
authors.lastname: Riechelmann
authors.forename: Annelies
authors.initials: A
authors.lastname: Schrott-Fischer
authors.forename: Bert
authors.initials: B
authors.lastname: Müller
mesh_terms: D037881:Aborted Fetus / Q000000981:diagnostic imaging / Q000473:pathology; D000328:Adult; D004423:Ear / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D020371:Pelizaeus-Merzbacher Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D011247:Pregnancy; D013701:Temporal Bone / Q000000981:diagnostic imaging / Q000473:pathology*; D055114:X-Ray Microtomography