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TEMPORAL BONE FINDINGS IN 13-15 TRISOMY SYNDROME Ryusuke Saito 1 pp.953-960
Published Date 1977/11/20
DOI https://doi.org/10.11477/mf.1492208584
  • Abstract
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 Histopathological study was made of two temporal bones from a 3【1/2】 month-old female infant with 13-15 trisomy syndrome.

 The temporal bones showed multiple inner ear abnormalities without apparent abnormalities in both external and middle ear structures. The right temporal bone presented Mondini type inner ear dysplasia, while the left temporal bone could be classed as the Alexander type dysplasia. The right cochlea was incompletely developed with scala communis, (21.4mm long) missing interscalar septum.

 In the membranous labyrinth, the morphological abnormalities were mainly limited in the pars inferior and pars endolymphaticus. Extentive irregular dilatation of the cochlear duct and the saccule, hypoplastic striae vascularis and the deformed and encapsulated tectoral membrane were widely found throughout the cochlea.

 The organ of Corti was present throughout the cochlea.

 The endolymphatic duct and sac were dilated and lined by flattened epithelium with a fewrugosities provided with a poor connective tissues and vessels.

 In the left ear no apparent abnormalities were found in the osseous labyrinth, except for slightly shortened cochlea measuring 27. 6mm in length. The organ of corti was fully developed, but was replaced by a low mound of undifferentiated cells in its basal turn.

 No significant changes were noted in the vestibular labyrinth.


Copyright © 1977, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 印刷版ISSN 0386-9679 医学書院

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