13,235 results match your criteria cochlear hair

Transcription Factor Reprogramming in the Inner Ear: Turning on Cell Fate Switches to Regenerate Sensory Hair Cells.

Front Cell Neurosci 2021 29;15:660748. Epub 2021 Mar 29.

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, United States.

Non-mammalian vertebrates can restore their auditory and vestibular hair cells naturally by triggering the regeneration of adjacent supporting cells. The transcription factor ATOH1 is a key regulator of hair cell development and regeneration in the inner ear. Following the death of hair cells, supporting cells upregulate ATOH1 and give rise to new hair cells. Read More

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Ultrastructural maturation of the endbulb of Held active zones comparing wild-type and otoferlin-deficient mice.

iScience 2021 Apr 8;24(4):102282. Epub 2021 Mar 8.

Molecular Architecture of Synapses Group, Institute for Auditory Neuroscience, InnerEarLab and Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, 37075 Göttingen, Germany.

Endbulbs of Held are located in the anteroventral cochlear nucleus and present the first central synapses of the auditory pathway. During development, endbulbs mature functionally to enable rapid and powerful synaptic transmission with high temporal precision. This process is accompanied by morphological changes of endbulb terminals. Read More

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Nox3-derived superoxide in cochleae induces sensorineural hearing loss Mechanisms of Nox3-dependent hearing loss.

J Neurosci 2021 Apr 13. Epub 2021 Apr 13.

Laboratory of Molecular Pharmacology, Biosignal Research Center, Kobe University, Kobe 657-8501, Japan

Reactive oxygen species (ROS) produced by NADPH oxidases (Nox) contribute to the development of different types of sensorineural hearing loss (SNHL), a common impairment in humans with no established treatment. Although the essential role of Nox3 in otoconia biosynthesis and its possible involvement in hearing have been reported in rodents, immunohistological methods targeted at detecting Nox3 expression in inner ear cells reveal ambiguous results. Therefore, the mechanism underlying -dependent SNHL remains unclear and warrants further investigation. Read More

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Neuroplastin expression is essential for hearing and hair cell PMCA expression.

Brain Struct Funct 2021 Apr 12. Epub 2021 Apr 12.

Neurogenetics Laboratory, Leibniz Institute for Neurobiology, Brenneckestr. 6, 39118, Magdeburg, Germany.

Hearing deficits impact on the communication with the external world and severely compromise perception of the surrounding. Deafness can be caused by particular mutations in the neuroplastin (Nptn) gene, which encodes a transmembrane recognition molecule of the immunoglobulin (Ig) superfamily and plasma membrane Calcium ATPase (PMCA) accessory subunit. This study investigates whether the complete absence of neuroplastin or the loss of neuroplastin in the adult after normal development lead to hearing impairment in mice analyzed by behavioral, electrophysiological, and in vivo imaging measurements. Read More

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Epac1 signaling pathway mediates the damage and apoptosis of inner ear hair cells after noise exposure in a rat model.

Neuroscience 2021 Apr 7. Epub 2021 Apr 7.

Department of Otolaryngology-Head and Neck Surgery, The First Affiliated Hospital of USTC, Hefei, 230001, China. Electronic address:

To investigate the role of the exchange protein directly activated by cAMP (Epac) signaling pathway in inner ear hair cell damage and apoptosis after noise exposure, we analyzed the expression level of Epac1 in a rat model of noise-induced hearing loss (NIHL), based on rat exposure to a 4-kHz and 106-dB sound pressure level (SPL) for 8 h. Loss of outer hair cells (OHCs), mitochondrial lesions, and hearing loss were examined after treatment with the Epac agonist, 8-CPT, or the Epac inhibitor, ESI-09. The effects of 8-CPT and ESI-09 on cell proliferation and apoptosis were examined by CCK-8 assays, holographic microscopy imaging, and Annexin-V FITC/PI staining in HEI-OC1 cells. Read More

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Mapping the regulatory landscape of auditory hair cells from single-cell multi-omics data.

Genome Res 2021 Apr 9. Epub 2021 Apr 9.

University of Michigan, Kresge Hearing Research Institute;

Auditory hair cells transduce sound to the brain and in mammals these cells reside together with supporting cells in the sensory epithelium of the cochlea, called the organ of Corti. To establish the organ's delicate function during development and differentiation, spatiotemporal gene expression is strictly controlled by chromatin accessibility and cell type-specific transcription factors, jointly representing the regulatory landscape. Bulk-sequencing technology and cellular heterogeneity obscured investigations on the interplay between transcription factors and chromatin accessibility in inner ear development. Read More

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Apelin-13 prevents apoptosis in the cochlear tissue of noise-exposed rat via Sirt-1 regulation.

J Chem Neuroanat 2021 Apr 5;114:101956. Epub 2021 Apr 5.

Hearing Disorders Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Electronic address:

Noise-induced hearing loss (NIHL) is the second most common cause of acquired hearing loss. Acoustic trauma can cause oxidative damage in the cochlear hair cells (HCs) through apoptotic pathways. Apelin is a newly discovered neuropeptide with neuroprotective effects against the oxidative stress in neurodegenerative disorder. Read More

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Comparative assessment of Fgf's diverse roles in inner ear development: A zebrafish perspective.

Bruce B Riley

Dev Dyn 2021 Apr 8. Epub 2021 Apr 8.

Biology Department, Texas A&M University, College Station, TX.

Progress in understanding mechanisms of inner ear development has been remarkably rapid in recent years. The research community has benefited from the availability of several diverse model organisms, including zebrafish, chick and mouse. The complexity of the inner ear has proven to be a challenge, and the complexity of the mammalian cochlea in particular has been the subject of intense scrutiny. Read More

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Change in gene expression levels of GABA, glutamate and neurosteroid pathways due to acoustic trauma in the cochlea.

J Neurogenet 2021 Apr 7:1-13. Epub 2021 Apr 7.

Department of Medical Genetics, School of Medicine, Erciyes University, Kayseri, Turkey.

The characteristic feature of noise-induced hearing loss (NIHL) is the loss or malfunction of the outer hair cells (OHC) and the inner hair cells (IHC) of the cochlea. 90-95% of the spiral ganglion neurons, forming the cell bodies of cochlear nerve, synapse with the IHCs. Glutamate is the most potent excitatory neurotransmitter for IHC-auditory nerve synapses. Read More

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New Tmc1 deafness mutations impact mechanotransduction in auditory hair cells.

J Neurosci 2021 Apr 2. Epub 2021 Apr 2.

Department of Neuroscience, University of Wisconsin School of Medicine and Public Health, Madison, WI 53706.

Transmembrane channel-like protein isoform 1 (TMC1) is a major component of the mechano-electrical transducer (MET) channel in cochlear hair cells and is subject to numerous mutations causing deafness. We report a new dominant human deafness mutation, p.T422K, and have characterized the homologous mouse mutant, p. Read More

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Combination photobiomodulation/N-acetyl-L-cysteine treatment appears to mitigate hair cell loss associated with noise-induced hearing loss in rats.

Lasers Med Sci 2021 Apr 6. Epub 2021 Apr 6.

Beckman Laser Institute Korea, Dankook University, 119 Dandae-ro, Cheonan, 31116, Republic of Korea.

Sensorineural hearing loss is an intractable disease. Acoustic overstimulation creates hearing loss; many patients exhibit social and emotional dysfunctions. In a model of noise-induced hearing loss (NIHL), low-level laser photobiomodulation (PBM) at a near-infrared wavelength significantly improved auditory brainstem response (ABR) thresholds. Read More

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N-Acetylcysteine Combined With Dexamethasone Treatment Improves Sudden Sensorineural Hearing Loss and Attenuates Hair Cell Death Caused by ROS Stress.

Front Cell Dev Biol 2021 18;9:659486. Epub 2021 Mar 18.

Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Sudden sensorineural hearing loss (SSNHL) is a common emergency in the world. Increasing evidence of imbalance of oxidant-antioxidant were found in SSNHL patients. Steroids combined with antioxidants may be a potential strategy for the treatment of SSNHL. Read More

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Cochlear protection against noise exposure requires serotonin type 3A receptor via the medial olivocochlear system.

FASEB J 2021 May;35(5):e21486

Department of Neuroscience and Cell Biology, Graduate School of Medicine, Osaka University, Suita, Japan.

The cochlear efferent feedback system plays important roles in auditory processing, including regulation of the dynamic range of hearing, and provides protection against acoustic trauma. These functions are performed through medial olivocochlear (MOC) neurons. However, the underlying cellular and molecular mechanisms are not fully understood. Read More

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Spike Generators and Cell Signaling in the Human Auditory Nerve: An Ultrastructural, Super-Resolution, and Gene Hybridization Study.

Front Cell Neurosci 2021 16;15:642211. Epub 2021 Mar 16.

Section of Otolaryngology, Department of Surgical Sciences, Head and Neck Surgery, Uppsala University Hospital, Uppsala, Sweden.

The human auditory nerve contains 30,000 nerve fibers (NFs) that relay complex speech information to the brain with spectacular acuity. How speech is coded and influenced by various conditions is not known. It is also uncertain whether human nerve signaling involves exclusive proteins and gene manifestations compared with that of other species. Read More

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Regulation of otocyst patterning by and is required for inner ear morphogenesis in the mouse.

Development 2021 Apr 1. Epub 2021 Apr 1.

Institut für Molekularbiologie, Medizinische Hochschule Hannover, 30625 Hannover, Germany

All epithelial components of the inner ear, including sensory hair cells and innervating afferent neurons, arise by patterning and differentiation of epithelial progenitors residing in a simple sphere, the otocyst. Here, we identify the transcriptional repressors TBX2 and TBX3 as novel regulators of these processes in the mouse. Ablation of from the otocyst led to cochlear hypoplasia whereas loss of was associated with vestibular malformations. Read More

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[Development of novel drug carrier via round window membrane].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2021 Apr;35(4):380-384

As isolated anatomical position, limited labyrinthine artery supply, and blood-labyrinth barrier hampers systemic drug delivery to the inner ear. The efficient concentration of drug treatment is unsatisfactory and there's possible side effects after systemic administration. Intratympanic injection of drug can bypass the blood-labyrinth and permeated to the hair cells or synaptic area via the round-or oval window of the cochlea. Read More

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The protective effect of astaxanthin on cisplatin-induced ototoxicity.

Adv Clin Exp Med 2021 Mar;30(3):315-321

Department of Otorhinolaryngology, Faculty of Medicine, Recep Tayyip Erdogan University, Rize, Turkey.

Background: Promising studies have been conducted with many substances to reduce the ototoxic effects of cisplatin, but there is no treatment that completely eliminates the ototoxic effect.

Objectives: To determine the effectiveness of astaxanthin (ASX) as a protective agent against cisplatin-induced ototoxicity.

Material And Methods: Thirty-six rats were randomly divided into 6 groups. Read More

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Association of Conductive Hearing Loss with the Structural Changes in the Organ of Corti.

ORL J Otorhinolaryngol Relat Spec 2021 Mar 30:1-8. Epub 2021 Mar 30.

Department of Otolaryngology, Faculty of Medicine, Medipol University, Istanbul, Turkey.

Objective: The aim of the study was to evaluate the association of conductive hearing loss (CHL) with the structural changes in the organ of Corti.

Methods: Twenty ears of 10 healthy adult Wistar albino rats were included in the study. The right ears (n = 10) of the animals served as controls (group 1), and no surgical intervention was performed in these ears. Read More

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Assessing evidence for adaptive evolution in two hearing-related genes important for high-frequency hearing in echolocating mammals.

G3 (Bethesda) 2021 Apr;11(4)

Jilin Provincial Key Laboratory of Animal Resource Conservation and Utilization, Northeast Normal University, Changchun 130117, China.

High-frequency hearing is particularly important for echolocating bats and toothed whales. Previously, studies of the hearing-related genes Prestin, KCNQ4, and TMC1 documented that adaptive evolution of high-frequency hearing has taken place in echolocating bats and toothed whales. In this study, we present two additional candidate hearing-related genes, Shh and SK2, that may also have contributed to the evolution of echolocation in mammals. Read More

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Matrigel is required for efficient differentiation of isolated, stem cell-derived otic vesicles into inner ear organoids.

Stem Cell Res 2021 Mar 18;53:102295. Epub 2021 Mar 18.

Kresge Hearing Research Institute, Department of Otolaryngology-Head & Neck Surgery, University of Michigan, Ann Arbor, USA. Electronic address:

Inner ear organoids derived from pluripotent stem cells could be a useful model system to study development, disease, and regeneration. However, there is considerable heterogeneity in the size, morphology, and efficiency of organoid production using standard protocols. Greater control of the culture microenvironment could decrease heterogeneity and increase the yield of organoids. Read More

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HIC1 Represses Atoh1 Transcription and Hair Cell Differentiation in the Cochlea.

Stem Cell Reports 2021 Apr 25;16(4):797-809. Epub 2021 Mar 25.

Department of Otolaryngology, Harvard Medical School, Boston, MA, USA; Eaton Peabody Laboratory, Massachusetts Eye and Ear, Boston, MA, USA; Harvard Stem Cell Institute, Cambridge, MA, USA. Electronic address:

Across species, expression of the basic helix-loop-helix transcription factor ATOH1 promotes differentiation of cochlear supporting cells to sensory hair cells required for hearing. In mammals, this process is limited to development, whereas nonmammalian vertebrates can also regenerate hair cells after injury. The mechanistic basis for this difference is not fully understood. Read More

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Inhibition of connexin 43 induces hearing loss in postnatal mice.

J Wang Q Song

Physiol Int 2021 Mar 26. Epub 2021 Mar 26.

Department of Otolaryngology, Weifang City Hanting District People's Hospital, Weifang 261100, Shandong, China.

Background: Connexin 43 (Cx43) is the most ubiquitously expressed member of the family of connexins, constituting gap junctions and mediating cell communication, still its role in hearing loss has been little studied.

Methods: Immunohistochemistry was used to detect the expression pattern of Cx43. Spiral ganglia neurons (SGNs) and Corti co-culture were utilized to assay the re-innervation of hair cells by newborn SGNs. Read More

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Transcriptomic characterization of dying hair cells in the avian cochlea.

Cell Rep 2021 Mar;34(12):108902

Department of Otolaryngology-Head and Neck Surgery, Stanford University School of Medicine, Stanford, CA 94305, USA; Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address:

Sensory hair cells are prone to apoptosis caused by various drugs including aminoglycoside antibiotics. In mammals, this vulnerability results in permanent hearing loss because lost hair cells are not regenerated. Conversely, hair cells regenerate in birds, making the avian inner ear an exquisite model for studying ototoxicity and regeneration. Read More

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Cell-type identity of the avian cochlea.

Cell Rep 2021 Mar;34(12):108900

Department of Otolaryngology - Head & Neck Surgery, Institute for Stem Cell Biology & Regenerative Medicine, Stanford University School of Medicine, Palo Alto, CA 94305, USA. Electronic address:

In contrast to mammals, birds recover naturally from acquired hearing loss, which makes them an ideal model for inner ear regeneration research. Here, we present a validated single-cell RNA sequencing resource of the avian cochlea. We describe specific markers for three distinct types of sensory hair cells, including a previously unknown subgroup, which we call superior tall hair cells. Read More

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Projections from the ventral nucleus of the lateral lemniscus to the cochlea in the mouse.

J Comp Neurol 2021 Mar 23. Epub 2021 Mar 23.

Hearing Research, Garvan Institute of Medical Research, Darlinghurst, New South Wales, Australia.

Auditory efferents originate in the central auditory system and project to the cochlea. Although the specific anatomy of the olivocochlear (OC) efferents can vary between species, two types of auditory efferents have been identified based upon the general location of their cell bodies and their distinctly different axon terminations in the organ of Corti. In the mouse, the relatively small somata of the lateral (LOC) efferents reside in the lateral superior olive (LSO), have unmyelinated axons, and terminate around ipsilateral inner hair cells (IHCs), primarily against the afferent processes of type I auditory nerve fibers. Read More

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Prevention of acquired sensorineural hearing loss in mice by in vivo Htra2 gene editing.

Genome Biol 2021 Mar 22;22(1):86. Epub 2021 Mar 22.

ENT institute and Department of Otorhinolaryngology, Eye & ENT Hospital, State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Fudan University, Shanghai, 200031, China.

Background: Aging, noise, infection, and ototoxic drugs are the major causes of human acquired sensorineural hearing loss, but treatment options are limited. CRISPR/Cas9 technology has tremendous potential to become a new therapeutic modality for acquired non-inherited sensorineural hearing loss. Here, we develop CRISPR/Cas9 strategies to prevent aminoglycoside-induced deafness, a common type of acquired non-inherited sensorineural hearing loss, via disrupting the Htra2 gene in the inner ear which is involved in apoptosis but has not been investigated in cochlear hair cell protection. Read More

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SCN11A gene deletion causes sensorineural hearing loss by impairing the ribbon synapses and auditory nerves.

BMC Neurosci 2021 Mar 22;22(1):18. Epub 2021 Mar 22.

College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Beijing, China.

Background: The SCN11A gene, encoded Nav1.9 TTX resistant sodium channels, is a main effector in peripheral inflammation related pain in nociceptive neurons. The role of SCN11A gene in the auditory system has not been well characterized. Read More

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Biodistribution of poly clustered superparamagnetic iron oxide nanoparticle labeled mesenchymal stem cells in aminoglycoside induced ototoxic mouse model.

Biomed Eng Lett 2021 Feb 8;11(1):39-53. Epub 2021 Jan 8.

Research Institute of Hearing Enhancement, Yonsei University Wonju College of Medicine, Wonju, 26426 South Korea.

Recently, application of stem cell therapy in regenerative medicine has become an active field of study. Mesenchymal stem cells (MSCs) are known to have a strong ability for homing. MSCs labeled with superparamagnetic iron oxide nanoparticles (SPIONs) exhibit enhanced homing due to magnetic attraction. Read More

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February 2021

Expression of serum proteins in noise induced hearing loss workers of mining based industry.

J Proteomics 2021 Mar 15;240:104185. Epub 2021 Mar 15.

MDS Analytics, First Floor Sakar Enclave, Plot No. 127, Shankar Nagar, Nagpur, Maharashtra, India.

Noise Induced Hearing Loss (NIHL) is caused by excessive noise exposure due to occupational activities thus affects communication and quality of life. Prolonged occupational and environmental exposure to loud noise damages key molecules present in the micro-machinery of the ear which are required for the mechano-electrical transduction of sound waves in cochlea. Specific proteins are known to be associated with hearing loss and related structural and functional disabilities in the human inner, outer hair cells and cochlea. Read More

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Identification of a series of hair-cell MET channel blockers that protect against aminoglycoside-induced ototoxicity.

JCI Insight 2021 Apr 8;6(7). Epub 2021 Apr 8.

Sussex Neuroscience and.

To identify small molecules that shield mammalian sensory hair cells from the ototoxic side effects of aminoglycoside antibiotics, 10,240 compounds were initially screened in zebrafish larvae, selecting for those that protected lateral-line hair cells against neomycin and gentamicin. When the 64 hits from this screen were retested in mouse cochlear cultures, 8 protected outer hair cells (OHCs) from gentamicin in vitro without causing hair-bundle damage. These 8 hits shared structural features and blocked, to varying degrees, the OHC's mechano-electrical transducer (MET) channel, a route of aminoglycoside entry into hair cells. Read More

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