Article
Vascular defects and sensorineural deafness in a mouse model of Norrie disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 1 Jun 2002
Rehm Heidi L, Zhang Duan-Sun, Brown M Christian, Burgess Barbara, Halpin Chris, Berger Wolfgang, Morton Cynthia C, Corey David P, Chen Zheng-Yi
Abstract excerpt
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...
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