The molecular and structural basis of hearing impairment in mice with the cpk mutant gene.

Abstract:

:We studied hearing impairment and cochlear ultrastructure in C57BL/6J mice containing the cpk mutant gene. Heterozygous cpk/+ mutant mice, 6-8 months old, showed a marked hearing loss as demonstrated by auditory evoked potentials with some showing hearing loss at 90 db sound pressure. Ultrastructural studies of first cochlear turn disclosed the absence of organs of Corti, no tissues on the basilar membrane, scanty spiral ganglia, normal tectorial membrane, and vacuolation of the stria vascularis. Reissner's membrane is normal at the endolymphatic side, but transparent at the perilymphatic side. In the second turn the organ of Corti are normal but Nuel's space is full of debris. The cpk mutant mice have hearing impairment perhaps due to deficiencies of genes expressing vital basement membrane components.

journal_name

Ann N Y Acad Sci

authors

Cho H,Buchanan J,Strong D,Yamada Y,Yoo TJ

doi

10.1111/j.1749-6632.1991.tb19599.x

subject

Has Abstract

pub_date

1991-01-01 00:00:00

pages

262-4

eissn

0077-8923

issn

1749-6632

journal_volume

630

pub_type

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