Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice
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 orchest...
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| description | 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. The 2-LOD confidence interval spans approximately 4 Megabases located at position 54-60 Mb. This locus, termed sensorineural hearing loss 1 (Snhl1), accounts for approximately 82% of the phenotypic variation. In summary, this study identifies a novel hearing loss locus on chromosome 10 and attests to the prevalence and genetic heterogeneity of progressive hearing loss in common mouse strains. |
| doi_str_mv | 10.1371/journal.pone.0011459 |
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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. The 2-LOD confidence interval spans approximately 4 Megabases located at position 54-60 Mb. This locus, termed sensorineural hearing loss 1 (Snhl1), accounts for approximately 82% of the phenotypic variation. In summary, this study identifies a novel hearing loss locus on chromosome 10 and attests to the prevalence and genetic heterogeneity of progressive hearing loss in common mouse strains.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0011459</identifier><identifier>PMID: 20628639</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Age ; Animals ; Brain stem ; Chromosome 10 ; Chromosomes ; Chromosomes, Human, Pair 10 - genetics ; Cochlea - metabolism ; Communication ; Confidence intervals ; Ear ; Ear, Inner - metabolism ; Emission measurements ; Evoked Potentials, Auditory - genetics ; Genetic aspects ; Genetic diversity ; Genetic variance ; Genetics ; Genetics and Genomics/Complex Traits ; Genetics and Genomics/Disease Models ; Genetics and Genomics/Genetics of Disease ; Genomes ; Genotype ; Hair cells ; Hearing impairment ; Hearing loss ; Hearing Loss, Sensorineural - genetics ; Hearing protection ; Heterogeneity ; Histology ; Human populations ; Humans ; Inbreeding ; Inner ear ; Laboratories ; Language disorders ; Loci ; Mice ; Mitochondrial DNA ; Molecular biology ; Phenotype ; Phenotypic variations ; Position sensing ; Quantitative Trait Loci ; Regression Analysis ; Risk analysis ; Risk factors ; Rodents ; Sleep ; Stains ; Thresholds</subject><ispartof>PloS one, 2010-07, Vol.5 (7), p.e11459-e11459</ispartof><rights>COPYRIGHT 2010 Public Library of Science</rights><rights>2010 Noben-Trauth et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. 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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. The 2-LOD confidence interval spans approximately 4 Megabases located at position 54-60 Mb. This locus, termed sensorineural hearing loss 1 (Snhl1), accounts for approximately 82% of the phenotypic variation. In summary, this study identifies a novel hearing loss locus on chromosome 10 and attests to the prevalence and genetic heterogeneity of progressive hearing loss in common mouse strains.</description><subject>Age</subject><subject>Animals</subject><subject>Brain stem</subject><subject>Chromosome 10</subject><subject>Chromosomes</subject><subject>Chromosomes, Human, Pair 10 - genetics</subject><subject>Cochlea - metabolism</subject><subject>Communication</subject><subject>Confidence intervals</subject><subject>Ear</subject><subject>Ear, Inner - metabolism</subject><subject>Emission measurements</subject><subject>Evoked Potentials, Auditory - genetics</subject><subject>Genetic aspects</subject><subject>Genetic diversity</subject><subject>Genetic variance</subject><subject>Genetics</subject><subject>Genetics and Genomics/Complex Traits</subject><subject>Genetics and Genomics/Disease Models</subject><subject>Genetics and Genomics/Genetics of Disease</subject><subject>Genomes</subject><subject>Genotype</subject><subject>Hair cells</subject><subject>Hearing impairment</subject><subject>Hearing loss</subject><subject>Hearing Loss, Sensorineural - 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genetics</topic><topic>Cochlea - metabolism</topic><topic>Communication</topic><topic>Confidence intervals</topic><topic>Ear</topic><topic>Ear, Inner - metabolism</topic><topic>Emission measurements</topic><topic>Evoked Potentials, Auditory - genetics</topic><topic>Genetic aspects</topic><topic>Genetic diversity</topic><topic>Genetic variance</topic><topic>Genetics</topic><topic>Genetics and Genomics/Complex Traits</topic><topic>Genetics and Genomics/Disease Models</topic><topic>Genetics and Genomics/Genetics of Disease</topic><topic>Genomes</topic><topic>Genotype</topic><topic>Hair cells</topic><topic>Hearing impairment</topic><topic>Hearing loss</topic><topic>Hearing Loss, Sensorineural - genetics</topic><topic>Hearing protection</topic><topic>Heterogeneity</topic><topic>Histology</topic><topic>Human populations</topic><topic>Humans</topic><topic>Inbreeding</topic><topic>Inner ear</topic><topic>Laboratories</topic><topic>Language disorders</topic><topic>Loci</topic><topic>Mice</topic><topic>Mitochondrial DNA</topic><topic>Molecular biology</topic><topic>Phenotype</topic><topic>Phenotypic variations</topic><topic>Position sensing</topic><topic>Quantitative Trait Loci</topic><topic>Regression Analysis</topic><topic>Risk analysis</topic><topic>Risk factors</topic><topic>Rodents</topic><topic>Sleep</topic><topic>Stains</topic><topic>Thresholds</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Noben-Trauth, Konrad</creatorcontrib><creatorcontrib>Latoche, Joseph R</creatorcontrib><creatorcontrib>Neely, Harold R</creatorcontrib><creatorcontrib>Bennett, Beth</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Animal Behavior Abstracts</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Biotechnology Research Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Ecology Abstracts</collection><collection>Entomology Abstracts (Full archive)</collection><collection>Immunology Abstracts</collection><collection>Meteorological & Geoastrophysical Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Agricultural Science Collection</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Materials Science & Engineering Collection</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>Agricultural & Environmental Science Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Materials Science Collection</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Materials Science Database</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Meteorological & Geoastrophysical Abstracts - 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Academic</collection><collection>Neurosciences Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Noben-Trauth, Konrad</au><au>Latoche, Joseph R</au><au>Neely, Harold R</au><au>Bennett, Beth</au><au>Schrijver, Iris</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2010-07-07</date><risdate>2010</risdate><volume>5</volume><issue>7</issue><spage>e11459</spage><epage>e11459</epage><pages>e11459-e11459</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><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. The 2-LOD confidence interval spans approximately 4 Megabases located at position 54-60 Mb. This locus, termed sensorineural hearing loss 1 (Snhl1), accounts for approximately 82% of the phenotypic variation. In summary, this study identifies a novel hearing loss locus on chromosome 10 and attests to the prevalence and genetic heterogeneity of progressive hearing loss in common mouse strains.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>20628639</pmid><doi>10.1371/journal.pone.0011459</doi><oa>free_for_read</oa></addata></record> |
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| subjects | Age Animals Brain stem Chromosome 10 Chromosomes Chromosomes, Human, Pair 10 - genetics Cochlea - metabolism Communication Confidence intervals Ear Ear, Inner - metabolism Emission measurements Evoked Potentials, Auditory - genetics Genetic aspects Genetic diversity Genetic variance Genetics Genetics and Genomics/Complex Traits Genetics and Genomics/Disease Models Genetics and Genomics/Genetics of Disease Genomes Genotype Hair cells Hearing impairment Hearing loss Hearing Loss, Sensorineural - genetics Hearing protection Heterogeneity Histology Human populations Humans Inbreeding Inner ear Laboratories Language disorders Loci Mice Mitochondrial DNA Molecular biology Phenotype Phenotypic variations Position sensing Quantitative Trait Loci Regression Analysis Risk analysis Risk factors Rodents Sleep Stains Thresholds |
| title | Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice |
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