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Hair cells cochlea

Randall J Harley, Joseph P Murdy, Zhirong Wang, Michael C Kelly, Tessa-Jonne F Ropp, SeHoon H Park, Patricia F Maness, Paul B Manis, Thomas M Coate
BACKGROUND: In the cochlea, auditory development depends on precise patterns of innervation by afferent and efferent nerve fibers, as well as a stereotyped arrangement of hair and supporting cells. NrCAM is a homophilic cell adhesion molecule that controls diverse aspects of nervous system development, but the function of NrCAM in cochlear development is not well understood. RESULTS: Throughout cochlear innervation, NrCAM is detectable on spiral ganglion neuron (SGN) afferent and olivocochlear efferent fibers, and on the membranes of developing hair and supporting cells...
March 14, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Hengchao Chen, Yazhi Xing, Li Xia, Zhengnong Chen, Shankai Yin, Jian Wang
The synapse between inner hair cells (IHCs) and type I spiral ganglion neurons (SGNs) has been identified as a sensitive structure to noise-induced damage in the mammalian cochlea. Since this synapse provides the major information pathway from the cochlea to the auditory brain, it is important to maintain its integrity. Neurotrophin-3 (NT-3) has been known to play an important role in the development and the functional maintenance of this synapse. Application of exogenous NT-3, or overexpression of this gene in a transgenic animal model, have shown the value to protect this synapse from noise-induced damage...
March 13, 2018: Gene Therapy
David S Sharlin, Lily Ng, François Verrey, Theo J Visser, Ye Liu, Rafal T Olszewski, Michael Hoa, Heike Heuer, Douglas Forrest
Transmembrane proteins that mediate the cellular uptake or efflux of thyroid hormone potentially provide a key level of control over neurodevelopment. In humans, defects in one such protein, solute carrier SLC16A2 (MCT8) are associated with psychomotor retardation. Other proteins that transport the active form of thyroid hormone triiodothyronine (T3) or its precursor thyroxine (T4) have been identified in vitro but the wider significance of such transporters in vivo is unclear. The development of the auditory system requires thyroid hormone and the cochlea is a primary target tissue...
March 13, 2018: Scientific Reports
Wen-Wei Luo, Xin-Wei Wang, Rui Ma, Fang-Lu Chi, Ping Chen, Ning Cong, Yu-Yan Gu, Dong-Dong Ren, Juan-Mei Yang
Notch inhibition is known to generate supernumerary hair cells (HCs) at the expense of supporting cells (SCs) in the mammalian inner ear. However, inhibition of Notch activity becomes progressively less effective at inducing SC-to-HC conversion in the postnatal cochlea and balance organs as the animal ages. It has been suggested that the SC-to-HC conversion capacity is inversely correlated with E-cadherin accumulation in postnatal mammalian utricles. However, whether E-cadherin localization is linked to the SC-to-HC conversion capacity in the mammalian inner ear is poorly understood...
2018: Frontiers in Molecular Neuroscience
Wei Liu, Matyas Molnar, Carolyn Garnham, Heval Benav, Helge Rask-Andersen
The human inner ear, which is segregated by a blood/labyrinth barrier, contains resident macrophages [CD163, ionized calcium-binding adaptor molecule 1 (IBA1)-, and CD68-positive cells] within the connective tissue, neurons, and supporting cells. In the lateral wall of the cochlea, these cells frequently lie close to blood vessels as perivascular macrophages. Macrophages are also shown to be recruited from blood-borne monocytes to damaged and dying hair cells induced by noise, ototoxic drugs, aging, and diphtheria toxin-induced hair cell degeneration...
2018: Frontiers in Immunology
Judith S Kempfle, Kim Nguyen, Christine Hamadani, Nicholas Koen, Albert S Edge, Boris A Kashemirov, David H Jung, Charles E McKenna
Hearing loss affects more than two-thirds of the elderly population, and more than 17% of all adults in the U.S. Sensorineural hearing loss related to noise exposure or aging is associated with loss of inner ear sensory hair cells (HCs), cochlear spiral ganglion neurons (SGNs), and ribbon synapses between HCs and SGNs, stimulating intense interest in therapies to regenerate synaptic function. 7,8-Dihydroxyflavone (DHF) is a selective and potent agonist of tropomyosin receptor kinase B (TrkB) and protects the neuron from apoptosis...
February 27, 2018: Bioconjugate Chemistry
Michael D Weston, Shikha Tarang, Marsha L Pierce, Umesh Pyakurel, Sonia M Rocha-Sanchez, JoAnn McGee, Edward J Walsh, Garrett A Soukup
Germline mutations in Mir96, one of three co-expressed polycistronic miRNA genes (Mir96, Mir182, Mir183), cause hereditary hearing loss in humans and mice. Transgenic FVB/NCrl- Tg(GFAP-Mir183,Mir96,Mir182)MDW1 mice (Tg1MDW ), which overexpress this neurosensory-specific miRNA cluster in the inner ear, were developed as a model system to identify, in the aggregate, target genes and biologic processes regulated by the miR-183 cluster. Histological assessments demonstrate Tg1MDW/1MDW homozygotes have a modest increase in cochlear inner hair cells (IHCs)...
February 23, 2018: Scientific Reports
Sarah Verhulst, Alessandro Altoè, Viacheslav Vasilkov
Models of the human auditory periphery range from very basic functional descriptions of auditory filtering to detailed computational models of cochlear mechanics, inner-hair cell (IHC), auditory-nerve (AN) and brainstem signal processing. It is challenging to include detailed physiological descriptions of cellular components into human auditory models because single-cell data stems from invasive animal recordings while human reference data only exists in the form of population responses (e.g., otoacoustic emissions, auditory evoked potentials)...
March 2018: Hearing Research
Arianna Di Stadio, Valentina Pegoraro, Laura Giaretta, Laura Dipietro, Roberta Marozzo, Corrado Angelini
AIM: To evaluate the feasibility of microRNAs (miR) in clinical use to fill in the gap of current methodology commonly used to test hearing impairment in MELAS patients. MATERIAL AND METHOD: A literature review was performed using the following keywords, i.e., MELAS, Hearing Loss, Hearing Impairment, Temporal Bone, Otoacustic Emission (OTOAE), Auditory Brain Response (ABR), and microRNA. We reviewed the literature and focused on the aspect of the temporal bone, the results of electrophysiological tests in human clinical studies, and the use of miR for detecting lesions in the cochlea in patients with MELAS...
February 21, 2018: Orphanet Journal of Rare Diseases
Wei Liu, Hubert Löwenheim, Peter A Santi, Rudolf Glueckert, Annelies Schrott-Fischer, Helge Rask-Andersen
TMPRSS3 (Trans-membrane Serine Protease 3) is a type II trans-membrane serine protease that has proteolytic activity essential for hearing. Mutations in the gene cause non-syndromic autosomal recessive deafness (DFNB8/10) in humans. Knowledge about its cellular distribution in the human inner ear may increase our understanding of its physiological role and involvement in deafness, ultimately leading to therapeutic interventions. In this study, we used super-resolution structured illumination microscopy for the first time together with transmission electron microscopy to localize the TMPRSS3 protein in the human organ of Corti...
February 19, 2018: Cell and Tissue Research
Qianqian Yang, Gaoying Sun, Haiyan Yin, Hongrui Li, Zhixin Cao, Jinghan Wang, Meijuan Zhou, Haibo Wang, Jianfeng Li
Phosphatase and tensin homologue (PTEN)-induced putative kinase 1 (PINK1) gene encodes a serine/threonine kinase, which acts as a molecular sensor of mitochondrial health necessary for mitochondrial quality control. The present study was designed to examine whether PINK1 expressed in C57BL/6 murine cochlea and HEI-OC1 cells and, if so, to investigate the possible mechanisms underlying the action of PINK1 in cisplatin-induced death of sensory hair cells (HCs) and spiral ganglion neurons (SGNs) in vitro. The expression pattern of PINK1, formation of parkin particles, and autophagy were determined by immunofluorescent staining...
February 16, 2018: Free Radical Biology & Medicine
Hidekane Yoshimura, Seiji B Shibata, Paul T Ranum, Richard J H Smith
Cochlear gene therapy holds promise for the treatment of genetic deafness. Assessing its impact in adult murine models of hearing loss, however, has been hampered by technical challenges that have made it difficult to establish a robust method to deliver transgenes to the mature murine inner ear. Here in we demonstrate the feasibility of a combined round window membrane injection and semi-circular canal fenestration technique in the adult cochlea. Injection of both AAV2/9 and AAV2/Anc80L65 via this approach in P15-16 and P56-60 mice permits robust eGFP transduction of virtually all inner hair cells throughout the cochlea with variable transduction of vestibular hair cells...
February 14, 2018: Scientific Reports
Claus-Peter Richter, Hunter Young, Sonja V Richter, Virginia Smith-Bronstein, Stuart R Stock, Xianghui Xiao, Carmen Soriano, Donna S Whitlon
Exposure to noise and ototoxic drugs are responsible for much of the debilitating hearing loss experienced by about 350 million people worldwide. Beyond hearing aids and cochlear implants, there have been no other FDA approved drug interventions established in the clinic that would either protect or reverse the effects of hearing loss. Using Auditory Brainstem Responses (ABR) in a guinea pig model, we demonstrate that fluvastatin, an inhibitor of HMG-CoA reductase, the rate-limiting enzyme of the mevalonate pathway, protects against loss of cochlear function initiated by high intensity noise...
February 14, 2018: Scientific Reports
Yoshie Narui, Marcos Sotomayor
Human hearing relies upon the tip-to-tip interaction of two non-classical cadherins, protocadherin-15 (PCDH15) and cadherin-23 (CDH23). Together, these proteins form a filament called the tip link that connects neighboring stereocilia of mechanosensitive hair cells. As sound waves enter the cochlea, the stereocilia deflect and tension is applied to the tip link opening nearby transduction channels. Disruption of the tip link by loud sound or calcium chelators eliminates transduction currents and illustrates that tip-link integrity is critical for mechanosensing...
February 14, 2018: Biochemistry
Yong-Li Song, Ke-Yong Tian, Wen-Juan Mi, Zhong-Jia Ding, Yang Qiu, Fu-Quan Chen, Ding-Jun Zha, Jian-Hua Qiu
Cochlear progenitor cells are considered as one of the best candidates for hair cell regeneration, thus, the regulation of cochlear progenitor cell proliferation has become a focus in this field. Several genes expressed in the inner ear during postnatal development have been demonstrated to be involved in maintaining the proliferative potential of progenitor cells, but the mechanism for regulating the proliferation and differentiation of cochlear progenitor cells remains poorly understood. Telomerase reverse transcriptase (TERT) has rate limiting telomerase activity and the overexpression of TERT has been shown to promote cell proliferation in series of cell lines...
February 6, 2018: Molecular Medicine Reports
T Chen, W Zhang, Y Liang, Q Li, C Yang, Y X Yuan, M L Ban
Objective: To investigate the effect of melatonin on the expression of prestin protein in the inner ear of mice following a single dose radiation therapy, so as to provide the basis for the mechanism study of radiation induced inner ear injury and its prevention. Methods: Sixty 4-week-old male mice were randomly divided into six groups, including the control group (A group), 50 mg/kg MLT group (B group), 5 mg/kg MLT group (C group), 50 mg/kg MLT + radiotherapy group (D group), 5 mg/kg MLT+ radiotherapy group (E group), and 16 Gy radiotherapy group (F group)...
February 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
J Hu, Z C Chen, Y Z Zhang, P Han, W J Ma, Q Zhang, M Xu
Objective: To test the mechanism and upstream pathway of outer hair cell apoptosis in Cadherin 23 (Cdh23) gene mutant mice. Method: The mutant Cdh23(erl/erl)(erl) mice were collected as the study group, while the C57BL/6J (B6) mice were chosen as the control group. A total of 70 mice per group were used in this study. The study group and control group underwent auditory-evoked brainstem response (ABR) tests at the same age. The terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) assay was performed to detect outer hair cell(OHC) apoptosis...
February 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
Thomas Bowling, Julien Meaud
Distortion product otoacoustic emissions are sounds that are emitted by the cochlea due to the nonlinearity of the outer hair cells. These emissions play an important role both in clinical settings and research laboratories. However, how distortion products propagate from their generation location to the middle ear remains unclear; whether distortion products propagate as a slow reverse traveling wave, or as a fast compression wave, through the cochlear fluid has been debated. In this article, we evaluate the contributions of the slow reverse wave and fast compression wave to the propagation of intracochlear distortion products using a physiologically based nonlinear model of the gerbil cochlea...
February 6, 2018: Biophysical Journal
Shin Hye Kim, Gaon Jung, Sangjae Kim, Ja-Won Koo
The cell-penetrating peptide GV1001 has been investigated as an anticancer agent and recently demonstrated anti-oxidant and anti-inflammatory effects. It has shown a protective effect on a kanamycin (KM)-induced ototoxicity mouse model. In the present study, we administered GV1001 at different time points after inducing hair cell damage, and examined if it rescues hair cell loss and restores hearing. A deaf mouse model was created by intraperitoneal injection of KM and furosemide. First, to test the early temporal change of hearing and extent of hair cell damage after KM and furosemide injection, hearing and outer hair cells (OHCs) morphology were evaluated on day 1, day 2 and day 3 after injection...
2018: Frontiers in Cellular Neuroscience
Aritra Sasmal, Karl Grosh
Acoustical excitation of the organ of Corti induces radial fluid flow in the subtectorial space (STS) that excites the hair bundles (HBs) of the sensory inner hair cell of the mammalian cochlea. The inner hair cell HBs are bathed in endolymphatic fluid filling a thin gap in the STS between the tectorial membrane and the reticular lamina. According to the fluctuation dissipation theorem, the fluid viscosity gives rise to mechanical fluctuations that are transduced into current noise. Conversely, the stochastic fluctuations of the mechanically gated channels of the HBs also induce dissipation...
January 23, 2018: Biophysical Journal
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