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Brain Res. 2009 Jun 24;1277:42-51. doi: 10.1016/j.brainres.2009.02.012. Epub 2009 Feb 21.

Inheritance patterns of progressive hearing loss in laboratory strains of mice.

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  • 1Section on Neurogenetics, NIDCD, National Institutes of Health, 5 Research Court, Rockville, MD 20850-3227, USA.


Positional cloning of mouse deafness mutations uncovered a plethora of proteins that have important functions in the peripheral auditory system in particular in the cochlear organ of Corti and stria vascularis. Most of these mutant variants follow a monogenic form of inheritance and are rare, highly penetrant, and deleterious alleles. Inbred and heterogenous strains of mice, in contrast, present with non-syndromic hearing impairment due to the effects of multiple genes and hypomorphic and less penetrant alleles that are often transmitted in a non-Mendelian manner. Here we review hearing loss inheritance patterns as they were discovered in different strains of mice and discuss the relevance of candidate genes to late-onset progressive hearing impairment in mouse and human.

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