title: Inheritance patterns of progressive hearing loss in laboratory strains of mice.
abstract: 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.
authors.affiliation: Section on Neurogenetics, NIDCD, National Institutes of Health, 5 Research Court, Rockville, MD 20850-3227, USA. nobentk@nidcd.nih.gov
authors.forename: Konrad
authors.initials: K
authors.lastname: Noben-Trauth
authors.forename: Kenneth R
authors.initials: KR
authors.lastname: Johnson
mesh_terms: D000367:Age Factors; D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D034381:Hearing Loss / Q000235:genetics*; D006801:Humans; D040582:Inheritance Patterns / Q000235:genetics*; D051379:Mice; D008815:Mice, Inbred Strains; D009154:Mutation / Q000235:genetics; D010641:Phenotype; D013045:Species Specificity