Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model
Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy ove...
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| creator | Giraudet, Fabrice Blavignac, Christelle Macedo de Resende, Luciana Alves da Silva Carvalho, Sirley Labanca, Ludimila Souchal, Marion Avan, Paul |
| description | Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18–30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This “discordant profile” came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of “hidden” neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation. |
| doi_str_mv | 10.1155/2018/6280969 |
| format | Article |
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However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18–30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This “discordant profile” came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of “hidden” neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation.</description><identifier>ISSN: 2314-6133</identifier><identifier>EISSN: 2314-6141</identifier><identifier>DOI: 10.1155/2018/6280969</identifier><identifier>PMID: 29662891</identifier><language>eng</language><publisher>Cairo, Egypt: Hindawi Publishing Corporation</publisher><subject>Abnormalities ; Acoustics ; Animals ; Auditory system ; Auditory Threshold ; Cochlea ; Degeneration ; Disease Models, Animal ; Evoked Potentials, Auditory, Brain Stem ; Frequency dependence ; Frequency shift ; Hair ; Hair cells ; Hair Cells, Auditory, Outer - pathology ; Hair Cells, Auditory, Outer - ultrastructure ; Hearing loss ; Hearing Loss - pathology ; Hearing Loss - physiopathology ; Hearing protection ; Histology ; Life Sciences ; Male ; Masking ; Mice ; Mutation ; Neurobiology ; Neuropathy ; Neurosciences ; Noise ; Otoacoustic emissions ; Otoacoustic Emissions, Spontaneous ; Outer hair cells ; Perceptual Masking ; Problem solving ; Rehabilitation ; Sound ; Stereocilia - pathology ; Stereocilia - ultrastructure ; Studies ; Thresholds ; Transposition ; Tuning</subject><ispartof>BioMed research international, 2018-01, Vol.2018 (2018), p.1-12</ispartof><rights>Copyright © 2018 Marion Souchal et al.</rights><rights>COPYRIGHT 2018 John Wiley & Sons, Inc.</rights><rights>Copyright © 2018 Marion Souchal et al.; This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><rights>Copyright © 2018 Marion Souchal et al. 2018</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c533t-bfe8ff64bf2a4cb03fdac9ca9aef913665a715de0a70b6d5674da05eedd2bb7f3</citedby><cites>FETCH-LOGICAL-c533t-bfe8ff64bf2a4cb03fdac9ca9aef913665a715de0a70b6d5674da05eedd2bb7f3</cites><orcidid>0000-0002-2004-2692 ; 0000-0001-7660-7253 ; 0000-0001-7816-7503 ; 0000-0003-2999-7644 ; 0000-0003-3296-4800 ; 0000-0003-3705-9471 ; 0000-0003-1054-1479</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832037/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832037/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,728,781,785,886,27929,27930,53796,53798</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29662891$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-01709750$$DView record in HAL$$Hfree_for_read</backlink></links><search><contributor>Lee, Jeong-Han</contributor><contributor>Jeong-Han Lee</contributor><creatorcontrib>Giraudet, Fabrice</creatorcontrib><creatorcontrib>Blavignac, Christelle</creatorcontrib><creatorcontrib>Macedo de Resende, Luciana</creatorcontrib><creatorcontrib>Alves da Silva Carvalho, Sirley</creatorcontrib><creatorcontrib>Labanca, Ludimila</creatorcontrib><creatorcontrib>Souchal, Marion</creatorcontrib><creatorcontrib>Avan, Paul</creatorcontrib><title>Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model</title><title>BioMed research international</title><addtitle>Biomed Res Int</addtitle><description>Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18–30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This “discordant profile” came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of “hidden” neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation.</description><subject>Abnormalities</subject><subject>Acoustics</subject><subject>Animals</subject><subject>Auditory system</subject><subject>Auditory Threshold</subject><subject>Cochlea</subject><subject>Degeneration</subject><subject>Disease Models, Animal</subject><subject>Evoked Potentials, Auditory, Brain Stem</subject><subject>Frequency dependence</subject><subject>Frequency shift</subject><subject>Hair</subject><subject>Hair cells</subject><subject>Hair Cells, Auditory, Outer - pathology</subject><subject>Hair Cells, Auditory, Outer - ultrastructure</subject><subject>Hearing loss</subject><subject>Hearing Loss - pathology</subject><subject>Hearing Loss - physiopathology</subject><subject>Hearing protection</subject><subject>Histology</subject><subject>Life Sciences</subject><subject>Male</subject><subject>Masking</subject><subject>Mice</subject><subject>Mutation</subject><subject>Neurobiology</subject><subject>Neuropathy</subject><subject>Neurosciences</subject><subject>Noise</subject><subject>Otoacoustic emissions</subject><subject>Otoacoustic Emissions, Spontaneous</subject><subject>Outer hair cells</subject><subject>Perceptual Masking</subject><subject>Problem solving</subject><subject>Rehabilitation</subject><subject>Sound</subject><subject>Stereocilia - pathology</subject><subject>Stereocilia - ultrastructure</subject><subject>Studies</subject><subject>Thresholds</subject><subject>Transposition</subject><subject>Tuning</subject><issn>2314-6133</issn><issn>2314-6141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>RHX</sourceid><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqNkk1vEzEQhi0EolXpjTNaiQuohPpjba8vSFFUCFIqOIQjsmZ3x6nLxtva2VT993iVkNKe6oPHGj_zemY8hLxl9DNjUp5zyqpzxStqlHlBjrlg5USxkr08nIU4IqcpXdO8KqYy-JoccaNykGHH5PcyQkgew6aY1qGPa-j8xmMqfCguIf3xYVUshzCa2RC3OPovIHb3xc_YryKm5LNzjhBHZNGnVFz2Q8K8t9i9Ia8cdAlP9_aE_Pp6sZzNJ4sf377PpotJI4XYTGqHlXOqrB2HsqmpcC00pgED6AwTSknQTLZIQdNatVLpsgUqEduW17V24oR82eneDPUa2yaXE6GzN9GvId7bHrx9fBP8lV31WysrwanQWeDjTuDqSdh8urCjjzJNjZZ0yzL7Yf9Y7G8HTBu79qnBroOAuXTLKVclk1XJM_r-CXrdDzHkVmSKasM1U-UDtYIOrQ-uzzk2o6idKs6q_HPcZOrTjmpi7nJEd8iTUTsOgx2Hwe6HIePv_m_JAf739Rk425fsQwt3_plymBl08EAzritWir9yNMV6</recordid><startdate>20180101</startdate><enddate>20180101</enddate><creator>Giraudet, Fabrice</creator><creator>Blavignac, Christelle</creator><creator>Macedo de Resende, Luciana</creator><creator>Alves da Silva Carvalho, Sirley</creator><creator>Labanca, Ludimila</creator><creator>Souchal, Marion</creator><creator>Avan, Paul</creator><general>Hindawi Publishing Corporation</general><general>Hindawi</general><general>John Wiley & Sons, Inc</general><general>Hindawi Limited</general><scope>ADJCN</scope><scope>AHFXO</scope><scope>RHU</scope><scope>RHW</scope><scope>RHX</scope><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>3V.</scope><scope>7QL</scope><scope>7QO</scope><scope>7T7</scope><scope>7TK</scope><scope>7U7</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>CWDGH</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>1XC</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0002-2004-2692</orcidid><orcidid>https://orcid.org/0000-0001-7660-7253</orcidid><orcidid>https://orcid.org/0000-0001-7816-7503</orcidid><orcidid>https://orcid.org/0000-0003-2999-7644</orcidid><orcidid>https://orcid.org/0000-0003-3296-4800</orcidid><orcidid>https://orcid.org/0000-0003-3705-9471</orcidid><orcidid>https://orcid.org/0000-0003-1054-1479</orcidid></search><sort><creationdate>20180101</creationdate><title>Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model</title><author>Giraudet, Fabrice ; Blavignac, Christelle ; Macedo de Resende, Luciana ; Alves da Silva Carvalho, Sirley ; Labanca, Ludimila ; Souchal, Marion ; Avan, Paul</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c533t-bfe8ff64bf2a4cb03fdac9ca9aef913665a715de0a70b6d5674da05eedd2bb7f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Abnormalities</topic><topic>Acoustics</topic><topic>Animals</topic><topic>Auditory system</topic><topic>Auditory Threshold</topic><topic>Cochlea</topic><topic>Degeneration</topic><topic>Disease Models, Animal</topic><topic>Evoked Potentials, Auditory, Brain Stem</topic><topic>Frequency dependence</topic><topic>Frequency shift</topic><topic>Hair</topic><topic>Hair cells</topic><topic>Hair Cells, Auditory, Outer - pathology</topic><topic>Hair Cells, Auditory, Outer - ultrastructure</topic><topic>Hearing loss</topic><topic>Hearing Loss - pathology</topic><topic>Hearing Loss - physiopathology</topic><topic>Hearing protection</topic><topic>Histology</topic><topic>Life Sciences</topic><topic>Male</topic><topic>Masking</topic><topic>Mice</topic><topic>Mutation</topic><topic>Neurobiology</topic><topic>Neuropathy</topic><topic>Neurosciences</topic><topic>Noise</topic><topic>Otoacoustic emissions</topic><topic>Otoacoustic Emissions, Spontaneous</topic><topic>Outer hair cells</topic><topic>Perceptual Masking</topic><topic>Problem solving</topic><topic>Rehabilitation</topic><topic>Sound</topic><topic>Stereocilia - pathology</topic><topic>Stereocilia - ultrastructure</topic><topic>Studies</topic><topic>Thresholds</topic><topic>Transposition</topic><topic>Tuning</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Giraudet, Fabrice</creatorcontrib><creatorcontrib>Blavignac, Christelle</creatorcontrib><creatorcontrib>Macedo de Resende, Luciana</creatorcontrib><creatorcontrib>Alves da Silva Carvalho, Sirley</creatorcontrib><creatorcontrib>Labanca, Ludimila</creatorcontrib><creatorcontrib>Souchal, Marion</creatorcontrib><creatorcontrib>Avan, Paul</creatorcontrib><collection>الدوريات العلمية والإحصائية - 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However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or “hidden” synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18–30 stages when high-frequency auditory thresholds remained near-normal. Nonetheless, total loss of high-frequency distortion product otoacoustic emissions pointed to nonfunctioning basal OHCs. This “discordant profile” came with a huge low-frequency shift of masking tuning curves that plot the level of interfering sound necessary to mask the response to a probe tone, against interfering frequency. Histology revealed intense OHC hair bundle abnormalities in the basal cochlea uncharacteristically associated with OHC survival and preserved coupling with the tectorial membrane. This pattern dismisses the superficial diagnosis of “hidden” neuropathy while underpinning a disorganization of cochlear frequency mapping with optimistic high-frequency auditory thresholds perhaps because responses to high frequencies are apically shifted. The audiometric advantage of frequency transposition is offset by enhanced masking by low-frequency sounds, a finding essential for guiding rehabilitation.</abstract><cop>Cairo, Egypt</cop><pub>Hindawi Publishing Corporation</pub><pmid>29662891</pmid><doi>10.1155/2018/6280969</doi><tpages>12</tpages><orcidid>https://orcid.org/0000-0002-2004-2692</orcidid><orcidid>https://orcid.org/0000-0001-7660-7253</orcidid><orcidid>https://orcid.org/0000-0001-7816-7503</orcidid><orcidid>https://orcid.org/0000-0003-2999-7644</orcidid><orcidid>https://orcid.org/0000-0003-3296-4800</orcidid><orcidid>https://orcid.org/0000-0003-3705-9471</orcidid><orcidid>https://orcid.org/0000-0003-1054-1479</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Abnormalities Acoustics Animals Auditory system Auditory Threshold Cochlea Degeneration Disease Models, Animal Evoked Potentials, Auditory, Brain Stem Frequency dependence Frequency shift Hair Hair cells Hair Cells, Auditory, Outer - pathology Hair Cells, Auditory, Outer - ultrastructure Hearing loss Hearing Loss - pathology Hearing Loss - physiopathology Hearing protection Histology Life Sciences Male Masking Mice Mutation Neurobiology Neuropathy Neurosciences Noise Otoacoustic emissions Otoacoustic Emissions, Spontaneous Outer hair cells Perceptual Masking Problem solving Rehabilitation Sound Stereocilia - pathology Stereocilia - ultrastructure Studies Thresholds Transposition Tuning |
| title | Transient Abnormalities in Masking Tuning Curve in Early Progressive Hearing Loss Mouse Model |
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