2023
Cx26 heterozygous mutations cause hyperacusis-like hearing oversensitivity and increase susceptibility to noise
Liu L, Liang C, Chen J, Fang S, Zhao H. Cx26 heterozygous mutations cause hyperacusis-like hearing oversensitivity and increase susceptibility to noise. Science Advances 2023, 9: eadf4144. PMID: 36753545, PMCID: PMC9908021, DOI: 10.1126/sciadv.adf4144.Peer-Reviewed Original ResearchConceptsActive cochlear amplificationCochlear amplificationHeterozygous mutationsPermanent hearing threshold shiftHearing threshold shiftCochlear lateral wallNonsyndromic hearing lossHearing lossMouse modelGeneral populationNoise exposureThreshold shiftHeterozygote carriersHearing sensitivityLateral wallJunction genesGap junction genesPotential generationUnexpected findingExposureMutationsOversensitivityProtein prestinDeafness
2021
Efferent neurons control hearing sensitivity and protect hearing from noise through the regulation of gap junctions between cochlear supporting cells
Zhao H, Liu L, Yu N, Zhu Y, Mei L, Chen J, Liang C. Efferent neurons control hearing sensitivity and protect hearing from noise through the regulation of gap junctions between cochlear supporting cells. Journal Of Neurophysiology 2021, 127: 313-327. PMID: 34907797, PMCID: PMC8759971, DOI: 10.1152/jn.00468.2021.Peer-Reviewed Original ResearchConceptsOuter hair cellsActive cochlear amplificationCochlear efferent systemDistortion product otoacoustic emissionsEfferent systemEfferent pathwaysHearing sensitivityMedial olivocochlear efferent fibersPresynaptic vesicular acetylcholine transportersGap junctionsOlivocochlear efferent fibersHair cellsApplication of acetylcholineCochlear supporting cellsProtection of hearingVesicular acetylcholine transporterActive cochlear amplifierCochlear amplificationPostsynaptic ACh receptorsProduct otoacoustic emissionsMOC efferent systemHair cell activityEfferent nervesEfferent inhibitionEfferent fibers
2018
Knockout of Pannexin-1 Induces Hearing Loss
Chen J, Liang C, Zong L, Zhu Y, Zhao H. Knockout of Pannexin-1 Induces Hearing Loss. International Journal Of Molecular Sciences 2018, 19: 1332. PMID: 29710868, PMCID: PMC5983795, DOI: 10.3390/ijms19051332.Peer-Reviewed Original ResearchConceptsDistortion product otoacoustic emissionsHearing lossKO miceKO mouse lineMouse linesCochlear microphonicsAuditory brainstem response thresholdActive cochlear amplificationPanx1 KO miceAuditory function testsProduct otoacoustic emissionsKnockout mouse lineFunction testsNonsyndromic hearing lossABR thresholdHearing functionHigh incidenceRecent studiesGap junctional proteinReceptor currentsOtoacoustic emissionsMiceCochlear amplificationConsistent phenotypeResponse threshold
2017
Progressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing
Zong L, Chen J, Zhu Y, Zhao H. Progressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing. Biochemical And Biophysical Research Communications 2017, 489: 223-227. PMID: 28552523, PMCID: PMC5555358, DOI: 10.1016/j.bbrc.2017.05.137.Peer-Reviewed Original ResearchConceptsActive cochlear amplificationHearing lossCochlear amplificationMice ageGap junctionsAge-related hearing lossSignificant hearing lossPostnatal day 25Cochlear gap junctionsAuditory sensory hair cellsSensory hair cellsNonsyndromic hearing lossHigh incidenceOuter pillar cellsDay 25Deiters' cellsConnexin expressionHair cellsConnexin 26Outer hair cell electromotilityHair cell electromotilityPillar cellsPrevious reportsCochleaAge
2016
Expression and function of pannexins in the inner ear and hearing
Zhao H. Expression and function of pannexins in the inner ear and hearing. BMC Molecular And Cell Biology 2016, 17: 16. PMID: 27229462, PMCID: PMC4896268, DOI: 10.1186/s12860-016-0095-7.Peer-Reviewed Original ResearchConceptsFunction of pannexinsAuditory sensory hair cellsDistinct expression patternsCell apoptotic pathwaysGap junction genesSensory hair cellsGap junction proteinGene familyATP releaseExpression patternsApoptotic pathwayEndocochlear potentialJunction genesPannexinsActive cochlear amplificationPannexin expressionLateral wallCochlear lateral wallJunction proteinsOrgan of CortiHair cellsCritical roleCochlear amplificationIsoformsStria vascularis
2015
Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss – A Common Hereditary Deafness
Wingard J, Zhao H. Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss – A Common Hereditary Deafness. Frontiers In Cellular Neuroscience 2015, 9: 202. PMID: 26074771, PMCID: PMC4448512, DOI: 10.3389/fncel.2015.00202.Peer-Reviewed Original ResearchHearing lossPathological changesDeafness mechanismCongenital deafnessActive cochlear amplificationProgressive hearing lossDetailed cellular mechanismsCochlear hair cellsHair cell degenerationHereditary deafnessConnexin 26 mutationsDistinct pathological changesNon-syndromic hearing lossProfound congenital deafnessAuditory phenotypeHigh incidenceTherapeutic strategiesGap junctional proteinCell degenerationConnexin expressionHair cellsDeafnessCellular mechanismsLate childhoodCx26 mutations
2014
Connexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss
Zhu Y, Chen J, Liang C, Zong L, Chen J, Jones R, Zhao H. Connexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss. Neuroscience 2014, 284: 719-729. PMID: 25451287, PMCID: PMC4268423, DOI: 10.1016/j.neuroscience.2014.10.061.Peer-Reviewed Original ResearchConceptsLate-onset hearing lossActive cochlear amplificationDistortion product otoacoustic emissionsHearing lossNonsyndromic hearing lossTherapeutic interventionsProgressive hearing lossHair cell lossPostnatal day 5Cochlear amplificationProduct otoacoustic emissionsConditional knockout miceKnockout miceClinical observationsDay 5Cell lossEndocochlear potentialOtoacoustic emissionsNormal hearingCx26 expressionDeafness mechanismMiceCx26 deficiencyCochleaInterventionDeafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders
Chen J, Chen J, Zhu Y, Liang C, Zhao H. Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders. Biochemical And Biophysical Research Communications 2014, 448: 28-32. PMID: 24732355, PMCID: PMC4105360, DOI: 10.1016/j.bbrc.2014.04.016.Peer-Reviewed Original ResearchConceptsAuditory brainstem responseHair cell degenerationKO miceCongenital deafnessEP reductionEndocochlear potentialHearing lossCell degenerationDevelopmental disordersActive cochlear amplificationCx26 knockout miceComplete hearing lossCx26 deficiencyPostnatal day 5Connexin 26 mutationsNonsyndromic hearing lossBrainstem responseMouse modelKnockout miceDay 5Deafness mechanismMajor causeMiceDeafnessDisorders
2013
Active cochlear amplification is dependent on supporting cell gap junctions
Zhu Y, Liang C, Chen J, Zong L, Chen G, Zhao H. Active cochlear amplification is dependent on supporting cell gap junctions. Nature Communications 2013, 4: 1786. PMID: 23653198, PMCID: PMC3675877, DOI: 10.1038/ncomms2806.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAuditory ThresholdCochlear Microphonic PotentialsConnexin 26ConnexinsEvoked Potentials, Auditory, Brain StemGap JunctionsGene DeletionGene TargetingHair Cells, Auditory, OuterHearing LossLabyrinth Supporting CellsMiceMice, KnockoutMolecular Motor ProteinsNonlinear DynamicsOtoacoustic Emissions, SpontaneousSpiral GanglionConceptsActive cochlear amplificationOuter hair cellsCell gap junctionsHearing lossCochlear amplificationHair cellsGap junctionsDistortion product otoacoustic emissionsOuter hair cell electromotilityHair cell electromotilitySevere hearing lossProduct otoacoustic emissionsShorter outer hair cellsHair-bundle movementsOuter pillar cellsLeftward shiftOtoacoustic emissionsAcoustic stimulationDeiters' cellsHearing sensitivityConnexin 26Active cochlear mechanicsNovel findingsPillar cellsBundle movement