The cochlea in fetuses with neural tube defects.
In this study different malformations of the cochlea could be demonstrated. Nevertheless, we could not delineate a distinct malformation of the inner ear, that can be linked to a neural tube defect. Neural tube defects are a frequent and heterogeneous group of malformations, ranging from the survivable spina bifida to fatal anencephaly. In multiple animal models an involvement of the vestibulocochlear system has been demonstrated. In this article human fetal temporal bones of neural tube defects were analysed in a multimodular work-up. The morphologic study was performed with light microscopy, transmission electron microscopy and synchrotron radiation-based microcomputed tomography. Immunohistochemistry for different neuronal markers such as peripherin, beta-III-tubulin and vimentin helped to evaluate ontogenetic tissue development. Eight fetal temporal bones with neural tube defects and five control temporal bones were included into the morphologic study. The morphologic results of the neural tube defect temporal bones showed six regularly developed cochleas and two with only a single cochlear turn. Three of the neural tube defect temporal bones were further examined with immunohistochemical analysis. No differences in the staining pattern for peripherin, beta-III-tubulin and vimentin were detected.
Schmutzhard
Joachim
J
Department of Otorhinolaryngology, Innsbruck Medical University, A 6020 Innsbruck, Austria. Joachim.Schmutzhard@i-med.ac.at
Glueckert
Rudolf
R
Bitsche
Mario
M
Abraham
Irene
I
Falkeis
Christina
C
Schwentner
Ilona
I
Riechelmann
Herbert
H
Müller
Bert
B
Beckmann
Felix
F
Sergi
Consolato
C
Schrott-Fischer
Annelies
A
D000818:Animals; D003051:Cochlea / Q000002:abnormalities* / Q000378:metabolism / Q000648:ultrastructure; D005260:Female; D005333:Fetus / Q000002:abnormalities*; D005865:Gestational Age; D006801:Humans; D007381:Intermediate Filament Proteins / Q000378:metabolism; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D051379:Mice; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009436:Neural Tube Defects / Q000473:pathology*; D064531:Peripherins; D011247:Pregnancy; D013701:Temporal Bone / Q000002:abnormalities; D014054:Tomography / Q000379:methods; D014404:Tubulin / Q000378:metabolism; D014746:Vimentin / Q000378:metabolism
D007381:Intermediate Filament Proteins; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; C578994:PRPH protein, human; D064531:Peripherins; C498585:TUBB3 protein, human; D014404:Tubulin; D014746:Vimentin