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  Vascular Defects and Sensorineural Deafness in a Mouse Model of Norrie Disease

Rehm, H. L., Zhang, D.-S., Brown, M. C., Burgess, B., Halpin, C., Berger, W., et al. (2002). Vascular Defects and Sensorineural Deafness in a Mouse Model of Norrie Disease. Journal of Neuroscience, 22(11), 4286-4292.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0010-8BF2-8 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-0010-8BF3-6
Genre: Journal Article

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 Creators:
Rehm, Heidi L., Author
Zhang, Duan-Sun, Author
Brown, M. Christian, Author
Burgess, Barbara, Author
Halpin, Chris, Author
Berger, Wolfgang1, Author              
Morton, Cynthia C., Author
Corey, David P., Author
Chen, Zheng-Yi, Author
Affiliations:
1Dept. of Human Molecular Genetics (Head: Hans-Hilger Ropers), Max Planck Institute for Molecular Genetics, Max Planck Society, ou_1433549              

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Free keywords: Norrie disease; mouse model; deafness; blindness; retina; cochlea; stria vascularis; marginal cells; vascular; angiogenesis; norrin
 Abstract: Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. A knock-out mouse model with an Ndp gene disruption was studied. We examined the hearing phenotype, including audiological, histological, and vascular evaluations. As is seen in humans, the mice had progressive hearing loss leading to profound deafness. The primary lesion was localized to the stria vascularis, which houses the main vasculature of the cochlea. Fluorescent dyes showed an abnormal vasculature in this region and eventual loss of two-thirds of the vessels. We propose that one of the principal functions of norrin in the ear is to regulate the interaction of the cochlea with its vasculature.

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Language(s): eng - English
 Dates: 2002-06
 Publication Status: Published in print
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Type: -
 Identifiers: eDoc: 24249
 Degree: -

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Title: Journal of Neuroscience
Source Genre: Journal
 Creator(s):
Affiliations:
Publ. Info: -
Pages: - Volume / Issue: 22 (11) Sequence Number: - Start / End Page: 4286 - 4292 Identifier: -