Loading…
Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome
Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be us...
Saved in:
| Published in: | Neuroscience 2015-12, Vol.310, p.188-197 |
|---|---|
| Main Authors: | , , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993 |
| container_end_page | 197 |
| container_issue | |
| container_start_page | 188 |
| container_title | Neuroscience |
| container_volume | 310 |
| creator | Ito, T Nishio, A Wangemann, P Griffith, A.J |
| description | Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be used to study primary strial presbycusis. |
| doi_str_mv | 10.1016/j.neuroscience.2015.09.016 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4633361</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>1_s2_0_S030645221500826X</els_id><sourcerecordid>1730681244</sourcerecordid><originalsourceid>FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993</originalsourceid><addsrcrecordid>eNqNkk2LFDEQhhtR3HH1L0jw5KXbfPWXhwVZP2FBYRW8hXRSPZsxk6yp7ob5M_5W08y4rJ7MoXKot96qypOieMFoxShrXu2qAHOKaBwEAxWnrK5oX-XUg2LDulaUbS3lw2JDBW1KWXN-VjxB3NF8aikeF2e8EY1gNd8Uv76kuE2A6BYgLiVYIKEbPJAb0MmFLfERkTgkRs8IlgwHglNymiwazeyzBomFLQRIenIxEBeIDuTaG95oSUoXcB5Ht846kX3MHjla8CSOJFdvgSyAkxtWK6J_zmBnMxE8BJviHp4Wj0btEZ6d7vPi2_t3Xy8_llefP3y6fHNVmlryqdTjCNAxYTXTNXRDRxlw1ox2GGgv-5bKjslaj6Id2l7DCIy2bb1GYbnpe3FeXBx9b-dhD9bkYZP26ja5vU4HFbVTf2eCu1HbuCjZCCEalg1engxSzEvgpPYODXivA-SlFWszi45xKbP09VFqMkNMMN61YVStgNVO3QesVsCK9iqncvHz-4Pelf4hmgVvjwLIz7U4SOpkY10CMykb3f_1ufjHxngXnNH-BxwAd3FOIQNRTCFXVF2vX239aaymtOPNd_Eb-ajYxQ</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1730681244</pqid></control><display><type>article</type><title>Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome</title><source>ScienceDirect Freedom Collection</source><creator>Ito, T ; Nishio, A ; Wangemann, P ; Griffith, A.J</creator><creatorcontrib>Ito, T ; Nishio, A ; Wangemann, P ; Griffith, A.J</creatorcontrib><description>Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be used to study primary strial presbycusis.</description><identifier>ISSN: 0306-4522</identifier><identifier>EISSN: 1873-7544</identifier><identifier>DOI: 10.1016/j.neuroscience.2015.09.016</identifier><identifier>PMID: 26363152</identifier><language>eng</language><publisher>United States: Elsevier Ltd</publisher><subject>Acoustic Stimulation ; Animals ; Anion Transport Proteins - deficiency ; Anion Transport Proteins - genetics ; Auditory Threshold - physiology ; Cell Death - genetics ; deafness ; Disease Models, Animal ; doxycycline ; Evoked Potentials, Auditory, Brain Stem - genetics ; Evoked Potentials, Auditory, Brain Stem - physiology ; fluctuation ; Gene Expression Regulation - genetics ; Genotype ; Hair Cells, Auditory - pathology ; Hearing Loss - etiology ; Hearing Loss - genetics ; Hearing Loss - physiopathology ; hypomorphic ; KCNQ1 Potassium Channel - genetics ; KCNQ1 Potassium Channel - metabolism ; Mice ; Mice, Transgenic ; Mutation - genetics ; Neurology ; Potassium Channels, Inwardly Rectifying - genetics ; Potassium Channels, Inwardly Rectifying - metabolism ; SLC26A4 ; stria vascularis ; Stria Vascularis - pathology ; Sulfate Transporters ; Vestibular Aqueduct - pathology ; Vestibular Diseases - complications ; Vestibular Diseases - genetics</subject><ispartof>Neuroscience, 2015-12, Vol.310, p.188-197</ispartof><rights>2015</rights><rights>Published by Elsevier Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993</citedby><cites>FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,780,784,885,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26363152$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ito, T</creatorcontrib><creatorcontrib>Nishio, A</creatorcontrib><creatorcontrib>Wangemann, P</creatorcontrib><creatorcontrib>Griffith, A.J</creatorcontrib><title>Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome</title><title>Neuroscience</title><addtitle>Neuroscience</addtitle><description>Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be used to study primary strial presbycusis.</description><subject>Acoustic Stimulation</subject><subject>Animals</subject><subject>Anion Transport Proteins - deficiency</subject><subject>Anion Transport Proteins - genetics</subject><subject>Auditory Threshold - physiology</subject><subject>Cell Death - genetics</subject><subject>deafness</subject><subject>Disease Models, Animal</subject><subject>doxycycline</subject><subject>Evoked Potentials, Auditory, Brain Stem - genetics</subject><subject>Evoked Potentials, Auditory, Brain Stem - physiology</subject><subject>fluctuation</subject><subject>Gene Expression Regulation - genetics</subject><subject>Genotype</subject><subject>Hair Cells, Auditory - pathology</subject><subject>Hearing Loss - etiology</subject><subject>Hearing Loss - genetics</subject><subject>Hearing Loss - physiopathology</subject><subject>hypomorphic</subject><subject>KCNQ1 Potassium Channel - genetics</subject><subject>KCNQ1 Potassium Channel - metabolism</subject><subject>Mice</subject><subject>Mice, Transgenic</subject><subject>Mutation - genetics</subject><subject>Neurology</subject><subject>Potassium Channels, Inwardly Rectifying - genetics</subject><subject>Potassium Channels, Inwardly Rectifying - metabolism</subject><subject>SLC26A4</subject><subject>stria vascularis</subject><subject>Stria Vascularis - pathology</subject><subject>Sulfate Transporters</subject><subject>Vestibular Aqueduct - pathology</subject><subject>Vestibular Diseases - complications</subject><subject>Vestibular Diseases - genetics</subject><issn>0306-4522</issn><issn>1873-7544</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><recordid>eNqNkk2LFDEQhhtR3HH1L0jw5KXbfPWXhwVZP2FBYRW8hXRSPZsxk6yp7ob5M_5W08y4rJ7MoXKot96qypOieMFoxShrXu2qAHOKaBwEAxWnrK5oX-XUg2LDulaUbS3lw2JDBW1KWXN-VjxB3NF8aikeF2e8EY1gNd8Uv76kuE2A6BYgLiVYIKEbPJAb0MmFLfERkTgkRs8IlgwHglNymiwazeyzBomFLQRIenIxEBeIDuTaG95oSUoXcB5Ht846kX3MHjla8CSOJFdvgSyAkxtWK6J_zmBnMxE8BJviHp4Wj0btEZ6d7vPi2_t3Xy8_llefP3y6fHNVmlryqdTjCNAxYTXTNXRDRxlw1ox2GGgv-5bKjslaj6Id2l7DCIy2bb1GYbnpe3FeXBx9b-dhD9bkYZP26ja5vU4HFbVTf2eCu1HbuCjZCCEalg1engxSzEvgpPYODXivA-SlFWszi45xKbP09VFqMkNMMN61YVStgNVO3QesVsCK9iqncvHz-4Pelf4hmgVvjwLIz7U4SOpkY10CMykb3f_1ufjHxngXnNH-BxwAd3FOIQNRTCFXVF2vX239aaymtOPNd_Eb-ajYxQ</recordid><startdate>20151203</startdate><enddate>20151203</enddate><creator>Ito, T</creator><creator>Nishio, A</creator><creator>Wangemann, P</creator><creator>Griffith, A.J</creator><general>Elsevier Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20151203</creationdate><title>Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome</title><author>Ito, T ; Nishio, A ; Wangemann, P ; Griffith, A.J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Acoustic Stimulation</topic><topic>Animals</topic><topic>Anion Transport Proteins - deficiency</topic><topic>Anion Transport Proteins - genetics</topic><topic>Auditory Threshold - physiology</topic><topic>Cell Death - genetics</topic><topic>deafness</topic><topic>Disease Models, Animal</topic><topic>doxycycline</topic><topic>Evoked Potentials, Auditory, Brain Stem - genetics</topic><topic>Evoked Potentials, Auditory, Brain Stem - physiology</topic><topic>fluctuation</topic><topic>Gene Expression Regulation - genetics</topic><topic>Genotype</topic><topic>Hair Cells, Auditory - pathology</topic><topic>Hearing Loss - etiology</topic><topic>Hearing Loss - genetics</topic><topic>Hearing Loss - physiopathology</topic><topic>hypomorphic</topic><topic>KCNQ1 Potassium Channel - genetics</topic><topic>KCNQ1 Potassium Channel - metabolism</topic><topic>Mice</topic><topic>Mice, Transgenic</topic><topic>Mutation - genetics</topic><topic>Neurology</topic><topic>Potassium Channels, Inwardly Rectifying - genetics</topic><topic>Potassium Channels, Inwardly Rectifying - metabolism</topic><topic>SLC26A4</topic><topic>stria vascularis</topic><topic>Stria Vascularis - pathology</topic><topic>Sulfate Transporters</topic><topic>Vestibular Aqueduct - pathology</topic><topic>Vestibular Diseases - complications</topic><topic>Vestibular Diseases - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ito, T</creatorcontrib><creatorcontrib>Nishio, A</creatorcontrib><creatorcontrib>Wangemann, P</creatorcontrib><creatorcontrib>Griffith, A.J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Neuroscience</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ito, T</au><au>Nishio, A</au><au>Wangemann, P</au><au>Griffith, A.J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome</atitle><jtitle>Neuroscience</jtitle><addtitle>Neuroscience</addtitle><date>2015-12-03</date><risdate>2015</risdate><volume>310</volume><spage>188</spage><epage>197</epage><pages>188-197</pages><issn>0306-4522</issn><eissn>1873-7544</eissn><abstract>Highlights • SLC26A4 mutations can cause fluctuating and progressive hearing loss in humans. • Slc26a4 -insufficient mice have irreversible hearing loss after 6 months of age. • Stria vascularis degeneration underlies hearing loss in Slc26a4 -insufficient ears. • Slc26a4 -insufficient mice may be used to study primary strial presbycusis.</abstract><cop>United States</cop><pub>Elsevier Ltd</pub><pmid>26363152</pmid><doi>10.1016/j.neuroscience.2015.09.016</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0306-4522 |
| ispartof | Neuroscience, 2015-12, Vol.310, p.188-197 |
| issn | 0306-4522 1873-7544 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4633361 |
| source | ScienceDirect Freedom Collection |
| subjects | Acoustic Stimulation Animals Anion Transport Proteins - deficiency Anion Transport Proteins - genetics Auditory Threshold - physiology Cell Death - genetics deafness Disease Models, Animal doxycycline Evoked Potentials, Auditory, Brain Stem - genetics Evoked Potentials, Auditory, Brain Stem - physiology fluctuation Gene Expression Regulation - genetics Genotype Hair Cells, Auditory - pathology Hearing Loss - etiology Hearing Loss - genetics Hearing Loss - physiopathology hypomorphic KCNQ1 Potassium Channel - genetics KCNQ1 Potassium Channel - metabolism Mice Mice, Transgenic Mutation - genetics Neurology Potassium Channels, Inwardly Rectifying - genetics Potassium Channels, Inwardly Rectifying - metabolism SLC26A4 stria vascularis Stria Vascularis - pathology Sulfate Transporters Vestibular Aqueduct - pathology Vestibular Diseases - complications Vestibular Diseases - genetics |
| title | Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4 -insufficient mouse model of large vestibular aqueduct syndrome |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-08T05%3A15%3A21IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Progressive%20irreversible%20hearing%20loss%20is%20caused%20by%20stria%20vascularis%20degeneration%20in%20an%20Slc26a4%20-insufficient%20mouse%20model%20of%20large%20vestibular%20aqueduct%20syndrome&rft.jtitle=Neuroscience&rft.au=Ito,%20T&rft.date=2015-12-03&rft.volume=310&rft.spage=188&rft.epage=197&rft.pages=188-197&rft.issn=0306-4522&rft.eissn=1873-7544&rft_id=info:doi/10.1016/j.neuroscience.2015.09.016&rft_dat=%3Cproquest_pubme%3E1730681244%3C/proquest_pubme%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c542t-affee813da1a5e8b801e216fdbb09497048145af37b79aefe10775e1073d2c993%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=1730681244&rft_id=info:pmid/26363152&rfr_iscdi=true |