2022
Outer hair cell function is normal in βV spectrin knockout mice
Stankewich MC, Bai JP, Stabach PR, Khan S, Tan WJT, Surguchev A, Song L, Morrow JS, Santos-Sacchi J, Navaratnam DS. Outer hair cell function is normal in βV spectrin knockout mice. Hearing Research 2022, 423: 108564. PMID: 35864018, DOI: 10.1016/j.heares.2022.108564.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCochleaEvoked Potentials, Auditory, Brain StemHair Cells, Auditory, OuterMiceMice, KnockoutOtoacoustic Emissions, SpontaneousSpectrinConceptsOuter hair cellsAuditory brainstem response wavesAuditory thresholdOuter hair cell functionSpiral ganglion neuronsEfferent nerve fibersHair cell functionNumber of afferentsGanglion neuronsNerve fibersKnockout miceNeuronal structuresMiceHair cellsCell functionElectromechanical activityPutative roleType IOngoing investigationExon deletionsSynaptopathyAfferentsData supportResponse wavesNeurons
2019
Functional alteration of ribbon synapses in inner hair cells by noise exposure causing hidden hearing loss
Liu H, Lu J, Wang Z, Song L, Wang X, Li G, Wu H. Functional alteration of ribbon synapses in inner hair cells by noise exposure causing hidden hearing loss. Neuroscience Letters 2019, 707: 134268. PMID: 31103727, DOI: 10.1016/j.neulet.2019.05.022.Peer-Reviewed Original ResearchMeSH KeywordsAcoustic StimulationAnimalsAuditory ThresholdEvoked Potentials, Auditory, Brain StemExocytosisHair Cells, Auditory, InnerHearing Loss, Noise-InducedMaleMice, Inbred CBASynapsesSynaptic VesiclesConceptsInner hair cellsModerate noise exposureHearing lossRibbon synapsesNoise exposureHair cellsPersistent reductionWhole-cell patch-clamp recordingsNoise-induced hearing lossSynapse functionAuditory afferent fibersHidden hearing lossABR wave ISynaptic vesiclesAuditory brainstem response (ABR) amplitudesPatch-clamp recordingsSevere noise exposureWhole mount immunofluorescence stainingPatch-clamp electrophysiologySensory hair cellsAfferent fibersABR thresholdPersistent alterationsExposure altersFunctional alterationsModulators of Kv3 Potassium Channels Rescue the Auditory Function of Fragile X Mice
El-Hassar L, Song L, Tan WJT, Large CH, Alvaro G, Santos-Sacchi J, Kaczmarek LK. Modulators of Kv3 Potassium Channels Rescue the Auditory Function of Fragile X Mice. Journal Of Neuroscience 2019, 39: 4797-4813. PMID: 30936239, PMCID: PMC6561694, DOI: 10.1523/jneurosci.0839-18.2019.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAuditory PathwaysAuditory PerceptionBrain StemCochlear NucleusElectrophysiological PhenomenaEvoked Potentials, Auditory, Brain StemFemaleFragile X Mental Retardation ProteinFragile X SyndromeHydantoinsIn Vitro TechniquesMaleMiceMice, KnockoutPatch-Clamp TechniquesPyridinesShaw Potassium ChannelsConceptsAuditory brainstem responseWild-type animalsRepetitive firingABR wave ICurrent-clamp recordingsAuditory brainstem nucleiVoltage-clamp recordingsHigh-frequency firingSingle action potentialFragile X syndromeTrapezoid bodyBrainstem nucleiBrainstem responseMedial nucleusAuditory brainstemAuditory nerveWave IWave IVAction potentialsSensory stimuliKv3.1 channelsCentral processingMental retardation proteinHigh sound levelsMice
2018
Characterizing a novel vGlut3-P2A-iCreER knockin mouse strain in cochlea
Li C, Shu Y, Wang G, Zhang H, Lu Y, Li X, Li G, Song L, Liu Z. Characterizing a novel vGlut3-P2A-iCreER knockin mouse strain in cochlea. Hearing Research 2018, 364: 12-24. PMID: 29706463, DOI: 10.1016/j.heares.2018.04.006.Peer-Reviewed Original ResearchMeSH KeywordsAcoustic StimulationAmino Acid Transport Systems, AcidicAnimalsCochleaEvoked Potentials, Auditory, Brain StemFemaleGene Knock-In TechniquesGenes, ReporterGenotypeHair Cells, Auditory, OuterIntegrasesLuminescent ProteinsMaleMice, Inbred C57BLMice, TransgenicNeurogliaPhenotypeReaction TimeReceptors, EstrogenSelective Estrogen Receptor ModulatorsSpiral GanglionTamoxifenTime FactorsConceptsInner hair cellsOuter hair cellsSpiral ganglion neuronsKnockin mouse strainGlia cellsVesicular glutamate transporter 3Mouse strainsHair cellsCochlear outer hair cellsRosa26-LSLVGLUT3 expressionGanglion neuronsVGLUT3Mouse cochleaTransporter 3Negative cellsMouse genetic studiesAntibody stainingTamoxifenUnique expression patternP2/P3Specific cell typesCell typesSitu hybridizationCochlea
2017
Novel Role of the Mitochondrial Protein Fus1 in Protection from Premature Hearing Loss via Regulation of Oxidative Stress and Nutrient and Energy Sensing Pathways in the Inner Ear
Tan WJ, Song L, Graham M, Schettino A, Navaratnam D, Yarbrough WG, Santos-Sacchi J, Ivanova AV. Novel Role of the Mitochondrial Protein Fus1 in Protection from Premature Hearing Loss via Regulation of Oxidative Stress and Nutrient and Energy Sensing Pathways in the Inner Ear. Antioxidants & Redox Signaling 2017, 27: 489-509. PMID: 28135838, PMCID: PMC5564041, DOI: 10.1089/ars.2016.6851.Peer-Reviewed Original ResearchMeSH KeywordsAcetylcysteineAnimalsAntioxidantsDisease Models, AnimalEar, InnerEvoked Potentials, Auditory, Brain StemGene Knockout TechniquesHearing LossHumansMiceMicroscopy, Electron, TransmissionMitochondriaOxidative StressProto-Oncogene Proteins c-aktPTEN PhosphohydrolaseSignal TransductionSpiral GanglionTOR Serine-Threonine KinasesTumor Suppressor ProteinsConceptsAge-related hearing lossAuditory brainstem responseHearing lossKO miceEndocochlear potentialOxidative stressMitochondrial dysfunctionMitochondrial dysfunction/oxidative stressEnergy sensing pathwaysNovel therapeutic strategiesMolecular mechanismsPremature hearing lossCochlear stria vascularisMajor hearing lossChronic mitochondrial dysfunctionMetabolic etiologyWorldwide epidemicBrainstem responseClinical trialsVascular pathologyTherapeutic strategiesPathological alterationsABR parametersAO treatmentStria vascularis
2015
Auditory Pathology in a Transgenic mtTFB1 Mouse Model of Mitochondrial Deafness
McKay SE, Yan W, Nouws J, Thormann MJ, Raimundo N, Khan A, Santos-Sacchi J, Song L, Shadel GS. Auditory Pathology in a Transgenic mtTFB1 Mouse Model of Mitochondrial Deafness. American Journal Of Pathology 2015, 185: 3132-3140. PMID: 26552864, PMCID: PMC5801480, DOI: 10.1016/j.ajpath.2015.08.014.Peer-Reviewed Original ResearchMeSH KeywordsAMP-Activated Protein KinasesAnimalsApoptosisDeafnessDisease Models, AnimalDNA, MitochondrialEvoked Potentials, Auditory, Brain StemHair Cells, Auditory, InnerMice, Inbred C57BLMice, KnockoutMice, TransgenicMitochondrial DiseasesMutationOrgan of CortiReaction TimeSignal TransductionSpiral GanglionStria VascularisTranscription FactorsConceptsAMP kinaseReactive oxygen species-mediated activationTranscription factor E2F1A1555G mutationAuditory pathologyHair cellsTFB1MHearing loss phenotypeRRNA geneAMPK-α1AMPK activityProlonged wave I latencyLoss phenotypeMitochondrial pathologyNonsyndromic deafnessTransgenic mouse strainWave I latencySpiral ganglion neuronsProgressive hearing lossMitochondrial deafnessPotential therapeutic valueDNA causeG mutationOuter hair cellsI latency