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
2017
Hypothesis of K+-Recycling Defect Is Not a Primary Deafness Mechanism for Cx26 (GJB2) Deficiency
Zhao H. Hypothesis of K+-Recycling Defect Is Not a Primary Deafness Mechanism for Cx26 (GJB2) Deficiency. Frontiers In Molecular Neuroscience 2017, 10: 162. PMID: 28603488, PMCID: PMC5445178, DOI: 10.3389/fnmol.2017.00162.Peer-Reviewed Original ResearchHearing lossDeafness mechanismCx26 deficiencyInner ear gap junctionsHair cell degenerationNonsyndromic hearing lossDisruption of permeabilityCongenital deafnessCell degenerationHair cellsHair cell excitationHereditary deafnessCell excitationConnexin26 MutationsGap junctional channelsGap junctionsDevelopmental disordersDeficiencyDeafnessExtracellular spaceReview articleJunctional channelsDegeneration
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
Deafness 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