Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice.

PloS One
Konrad Noben-TrauthBeth Bennett

Abstract

Progressive sensorineural hearing loss is the most common form of acquired hearing impairment in the human population. It is also highly prevalent in inbred strains of mice, providing an experimental avenue to systematically map genetic risk factors and to dissect the molecular pathways that orchestrate hearing in peripheral sensory hair cells. Therefore, we ascertained hearing function in the inbred long sleep (ILS) and inbred short sleep (ISS) strains. Using auditory-evoked brain stem response (ABR) and distortion product otoacoustic emission (DPOAE) measurements, we found that ISS mice developed a high-frequency hearing loss at twelve weeks of age that progressed to lower frequencies by 26 weeks of age in the presence of normal endocochlear potentials and unremarkable inner ear histology. ILS mice exhibited milder hearing loss, showing elevated thresholds and reduced DPOAEs at the higher frequencies by 26 weeks of age. To map the genetic variants that underlie this hearing loss we computed ABR thresholds of 63 recombinant inbred stains derived from the ISS and ILS founder strains. A single locus was linked to markers associated with ISS alleles on chromosome 10 with a highly significant logarithm of odds (LOD) score of 15.8....Continue Reading

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Citations

Oct 4, 2011·The Journal of Clinical Investigation·Byung Yoon ChoiAndrew J Griffith
May 20, 2011·Journal of the Association for Research in Otolaryngology : JARO·James M KellerKonrad Noben-Trauth
Mar 17, 2016·Journal of the Association for Research in Otolaryngology : JARO·Kevin K OhlemillerPatricia M Gagnon
Jun 21, 2015·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Kenneth R JohnsonLeona H Gagnon
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Oct 19, 2016·Journal of the Association for Research in Otolaryngology : JARO·Kevin K OhlemillerKenneth R Johnson
Jan 3, 2018·Human Molecular Genetics·Ayesha ImtiazThomas B Friedman
Jul 29, 2021·Human Genetics·Ely Cheikh BoussatyRoyce E Clifford

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Methods Mentioned

BETA
ISS
genotyping

Software Mentioned

WebQTL
GraphPad Prism
Intelligent Hearing System , IHS
LabView
AxoScope
EP
IHS
Smart

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