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https://www.readbyqxmd.com/read/27867060/the-role-of-efr3a-in-age-related-hearing-loss
#1
Haixia Hu, Yan Ma, Bin Ye, Quan Wang, Tao Yang, Jingrong Lv, Jun Shi, Hao Wu, Mingliang Xiang
Efr3a has been found to be involved in the functional maintenance and structural degeneration of sensory and motor nervous tissues. Our previous data have suggested that Efr3a may be associated with the initiation of the degeneration of spiral ganglion neurons (SGNs). In this study, we used Efr3a knockdown (Efr3a KD) and Efr3a overexpression (Efr3a OE) mice to determine the role of Efr3a in age-related hearing loss. Measurements of hearing thresholds showed that Efr3a had little or no influence on the hearing threshold at all frequencies in adult mice, whereas in an early stage of senescence, Efr3a reduction resulted in better hearing function, especially at 10 and 12months of age...
November 17, 2016: Neuroscience
https://www.readbyqxmd.com/read/27863646/regenerative-efficacy-of-mesenchymal-stromal-cells-from-human-placenta-in-sensorineural-hearing-loss
#2
Kicheol Kil, Mi Young Choi, Ji Sun Kong, Woo Jin Kim, Kyoung Ho Park
INTRODUCTION: Hearing loss is a common chronic disorder characterized by decline of auditory function. The global population have suffered from deafness and the transplantation of stem cells is regarded as a therapeutic strategy for this disease. METHODS: We collected placenta from a total of 13 samples of full term pregnant women and isolated MSCs derived from human placenta and transplanted MSCs on deaf animal model. The normal group and the sensorineural hearing loss (SNHL) group and the experimental (transplanted MSCs) group were compared and estimated hearing level using auditory brainstem response (ABR) recordings and the otoacoustic emission (OAE) test...
December 2016: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27858145/role-of-antioxidants-in-prevention-of-age-related-hearing-loss-a-review-of-literature
#3
REVIEW
Elham Tavanai, Ghassem Mohammadkhani
Age-related hearing loss (ARHL), also known as presbycusis, is one of the most prevalent chronic degenerative conditions. It is characterized by a decline in auditory function. ARHL is caused by the interaction of multiple factors, including cochlear aging, environment, genetic predisposition, and health comorbidities. The primary pathology of ARHL includes the hair cells loss, stria vascularis atrophy, and loss of spiral ganglion neurons as well as the changes in central auditory pathways. The research to date suggests that oxidative stress and mitochondrial DNA deletion (mtDNA) play a major role in pathophysiology of ARHL...
November 17, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27829169/slc44a2-single-nucleotide-polymorphisms-isoforms-and-expression-association-with-severity-of-meniere-s-disease
#4
Thankam S Nair, Pavan K Kommareddi, Maria M Galano, Danielle M Miller, Bala Naveen Kakaraparthi, Steven A Telian, H Alex Arts, Hussam El-Kashlan, Alyse Kilijanczyk, Amy Anne D Lassig, Martin P Graham, Susan G Fisher, Stefan W Stoll, Rajan P Nair, James T Elder, Thomas E Carey
SLC44A2 was discovered as the target of an antibody that causes hearing loss. Knockout mice develop age related hearing loss, loss of sensory cells and spiral ganglion neurons. SLC44A2 has polymorphic sites implicated in human disease. Transfusion related acute lung injury (TRALI) is linked to rs2288904 and genome wide association studies link rs2288904 and rs9797861 to venous thromboembolism (VTE), coronary artery disease and stroke. Here we report linkage disequilibrium of rs2288904 with rs3087969 and the association of these SLC44A2 SNPs with Meniere's disease severity...
November 6, 2016: Genomics
https://www.readbyqxmd.com/read/27824154/manganese-mediated-acceleration-of-age-related-hearing-loss-in-mice
#5
Nobutaka Ohgami, Ichiro Yajima, Machiko Iida, Xiang Li, Reina Oshino, Mayuko Y Kumasaka, Masashi Kato
Despite the fact that manganese (Mn) is known to be a neurotoxic element relevant to age-related disorders, the risk of oral exposure to Mn for age-related hearing loss remains unclear. In this study, we orally exposed wild-type young adult mice to Mn (Mn-exposed WT-mice) at 1.65 and 16.50 mg/L for 4 weeks. Mn-exposed WT-mice showed acceleration of age-related hearing loss. Mn-exposed WT-mice had neurodegeneration of spiral ganglion neurons (SGNs) with increased number of lipofuscin granules. Mn-exposed WT-mice also had increased hypoxia-inducible factor-1 alpha (Hif-1α) protein with less hydroxylation at proline 564 and decreased c-Ret protein in SGNs...
November 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27808407/extension-of-the-clinical-and-molecular-phenotype-of-diaph1-associated-autosomal-dominant-hearing-loss-dfna1
#6
Christine Neuhaus, Ruth Lang-Roth, Ulrike Zimmermann, Raoul Heller, Tobias Eisenberger, Matthias Weikert, Susanne Markus, Marlies Knipper, Hanno J Bolz
In about 20% of non-syndromic hearing loss (NSHL) cases, inheritance is autosomal dominant (ADNSHL). DIAPH1 mutations define the ADNSHL locus DFNA1. We identified two new families with heterozygous truncating DIAPH1 mutations (p.Ala1210Serfs*31 and p.Arg1213*). In contrast to the extensively studied original DFNA1 family, hearing loss was not confined to low frequencies, but congenital manifestation and rapid progression were confirmed. In line with a recent unrelated study, we identified an association with thrombocytopenia, reclassifying DFNA1 as a syndrome...
November 3, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27807171/effects-of-long-term-exercise-on-age-related-hearing-loss-in-mice
#7
Chul Han, Dalian Ding, Maria-Cecilia Lopez, Senthilvelan Manohar, Yanping Zhang, Mi-Jung Kim, Hyo-Jin Park, Karessa White, Yong Hwan Kim, Paul Linser, Masaru Tanokura, Christiaan Leeuwenburgh, Henry V Baker, Richard J Salvi, Shinichi Someya
: Regular physical exercise reduces the risk for obesity, cardiovascular diseases, and disability and is associated with longer lifespan expectancy (Taylor et al., 2004; Pahor et al., 2014; Anton et al., 2015; Arem et al., 2015). In contrast, decreased physical function is associated with hearing loss among older adults (Li et al., 2013; Chen et al., 2015). Here, we investigated the effects of long-term voluntary wheel running (WR) on age-related hearing loss (AHL) in CBA/CaJ mice, a well established model of AHL (Zheng et al...
November 2, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27805613/spiral-ganglion-neuron-explant-culture-and-electrophysiology-on-multi-electrode-arrays
#8
Stefan Hahnewald, Marta Roccio, Anne Tscherter, Jürg Streit, Ranjeeta Ambett, Pascal Senn
Spiral ganglion neurons (SGNs) participate in the physiological process of hearing by relaying signals from sensory hair cells to the cochlear nucleus in the brain stem. Loss of hair cells is a major cause of sensory hearing loss. Prosthetic devices such as cochlear implants function by bypassing lost hair cells and directly stimulating SGNs electrically, allowing for restoration of hearing in deaf patients. The performance of these devices depends on the functionality of SGNs, the implantation procedure and on the distance between the electrodes and the auditory neurons...
October 19, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27789624/distinct-capacity-for-differentiation-to-inner-ear-cell-types-by-progenitor-cells-of-the-cochlea-and-vestibular-organs
#9
Will J McLean, Dalton T McLean, Ruth Anne Eatock, Albert S B Edge
Disorders of hearing and balance are most commonly associated with damage to cochlear and vestibular hair cells or neurons. Although these cells are not capable of spontaneous regeneration, progenitor cells in the hearing and balance organs of the neonatal mammalian inner ear have the capacity to generate new hair cells after damage. To investigate whether these cells are restricted in their differentiation capacity, we assessed the phenotypes of differentiated progenitor cells isolated from three compartments of the mouse inner ear - the vestibular and cochlear sensory epithelia and the spiral ganglion - by measuring electrophysiological properties and gene expression...
December 1, 2016: Development
https://www.readbyqxmd.com/read/27788219/improved-auditory-nerve-survival-with-nanoengineered-supraparticles-for-neurotrophin-delivery-into-the-deafened-cochlea
#10
Andrew K Wise, Justin Tan, Yajun Wang, Frank Caruso, Robert K Shepherd
Cochlear implants electrically stimulate spiral ganglion neurons (SGNs) in order to provide speech cues to severe-profoundly deaf patients. In normal hearing cochleae the SGNs depend on endogenous neurotrophins secreted by sensory cells in the organ of Corti for survival. SGNs gradually degenerate following deafness and consequently there is considerable interest in developing clinically relevant strategies to provide exogenous neurotrophins to preserve SGN survival. The present study investigated the safety and efficacy of a drug delivery system for the cochlea using nanoengineered silica supraparticles...
2016: PloS One
https://www.readbyqxmd.com/read/27761977/concomitant-differentiation-of-a-population-of-mouse-embryonic-stem-cells-into-neuron-like-cells-and-schwann-cell-like-cells-in-a-slow-flow-microfluidic-device
#11
Poornapriya Ramamurthy, Joshua B White, Joong Yull Park, Richard I Hume, Fumi Ebisu, Flor Mendez, Shuichi Takayama, Kate F Barald
BACKGROUND: To send meaningful information to the brain, an inner ear cochlear implant (CI) must become closely coupled to as large and healthy a population of remaining Spiral Ganglion Neurons (SGN) as possible. Inner ear gangliogenesis depends on macrophage migration inhibitory factor (MIF), a directionally attractant neurotrophic cytokine made by both Schwann and supporting cells (Bank et al., 2012). MIF-induced mouse embryonic stem cell (mESC)-derived "neurons" could potentially substitute for lost or damaged SGN...
October 19, 2016: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/27760385/recent-advances-in-the-development-and-function-of-type-ii-spiral-ganglion-neurons-in-the-mammalian-inner-ear
#12
Kaidi D Zhang, Thomas M Coate
In hearing, mechanically sensitive hair cells (HCs) in the cochlea release glutamate onto spiral ganglion neurons (SGNs) to relay auditory information to the central nervous system (CNS). There are two main SGN subtypes, which differ in morphology, number, synaptic targets, innervation patterns and firing properties. About 90-95% of SGNs are the type I SGNs, which make a single bouton connection with inner hair cells (IHCs) and have been well described in the canonical auditory pathway for sound detection. However, less attention has been given to the type II SGNs, which exclusively innervate outer hair cells (OHCs)...
October 16, 2016: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/27738526/cochlear-synaptopathy-and-noise-induced-hidden-hearing-loss
#13
Lijuan Shi, Ying Chang, Xiaowei Li, Steve Aiken, Lijie Liu, Jian Wang
Recent studies on animal models have shown that noise exposure that does not lead to permanent threshold shift (PTS) can cause considerable damage around the synapses between inner hair cells (IHCs) and type-I afferent auditory nerve fibers (ANFs). Disruption of these synapses not only disables the innervated ANFs but also results in the slow degeneration of spiral ganglion neurons if the synapses are not reestablished. Such a loss of ANFs should result in signal coding deficits, which are exacerbated by the bias of the damage toward synapses connecting low-spontaneous-rate (SR) ANFs, which are known to be vital for signal coding in noisy background...
2016: Neural Plasticity
https://www.readbyqxmd.com/read/27736723/biofunctionalized-peptide-based-hydrogels-provide-permissive-scaffolds-to-attract-neurite-outgrowth-from-spiral-ganglion-neurons
#14
Claudia Frick, Marcus Müller, Ute Wank, Anke Tropitzsch, Benedikt Kramer, Pascal Senn, Helge Rask-Andersen, Karl-Heinz Wiesmüller, Hubert Löwenheim
Cochlear implants (CI) allow for hearing rehabilitation in patients with sensorineural hearing loss or deafness. Restricted CI performance results from the spatial gap between spiral ganglion neurons and the CI, causing current spread that limits spatially restricted stimulation and impairs frequency resolution. This may be substantially improved by guiding peripheral processes of spiral ganglion neurons towards and onto the CI electrode contacts. An injectable, peptide-based hydrogel was developed which may provide a permissive scaffold to facilitate neurite growth towards the CI...
October 4, 2016: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/27717379/biohybrid-cochlear-implants-in-human-neurosensory-restoration
#15
Ariane Roemer, Ulrike Köhl, Omid Majdani, Stephan Klöß, Christine Falk, Sabine Haumann, Thomas Lenarz, Andrej Kral, Athanasia Warnecke
BACKGROUND: The success of cochlear implantation may be further improved by minimizing implantation trauma. The physical trauma of implantation and subsequent immunological sequelae can affect residual hearing and the viability of the spiral ganglion. An ideal electrode should therefore decrease post-implantation trauma and provide support to the residual spiral ganglion population. Combining a flexible electrode with cells producing and releasing protective factors could present a potential means to achieve this...
October 7, 2016: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/27704212/high-mobility-group-box-1-hmgb1-dual-functions-in-the-cochlear-auditory-neurons-in-response-to-stress
#16
Sabine Ladrech, Jing Wang, Marc Mathieu, Jean-Luc Puel, Marc Lenoir
High mobility group box 1 (HMGB1) is a DNA-binding protein that facilitates gene transcription and may act extracellularly as a late mediator of inflammation. The roles of HMGB1 in the pathogenesis of the spiral ganglion neurons (SGNs) of the cochlea are currently unknown. In the present study, we tested the hypothesis that early phenotypical changes in the SGNs of the amikacin-poisoned rat cochlea are mediated by HMGB1. Our results showed that a marked downregulation of HMGB1 had occurred by completion of amikacin treatment, coinciding with acute damage at the dendrite extremities of the SGNs...
October 4, 2016: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/27696081/tyrosine-hydroxylase-expression-in-type-ii-cochlear-afferents-in-mice
#17
Pankhuri Vyas, Jingjing Sherry Wu, Amanda Zimmerman, Paul Fuchs, Elisabeth Glowatzki
Acoustic information propagates from the ear to the brain via spiral ganglion neurons that innervate hair cells in the cochlea. These afferents include unmyelinated type II fibers that constitute 5 % of the total, the majority being myelinated type I neurons. Lack of specific genetic markers of type II afferents in the cochlea has been a roadblock in studying their functional role. Unexpectedly, type II afferents were visualized by reporter proteins induced by tyrosine hydroxylase (TH)-driven Cre recombinase...
September 30, 2016: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/27683884/transmission-of-auditory-sensory-information-decreases-in-rate-and-temporal-precision-at-the-endbulb-of-held-synapse-during-age-related-hearing-loss
#18
Ruili Xie
Age-related hearing loss (ARHL) is largely attributed to structural changes and functional declines in the peripheral auditory system, which include synaptopathy at the inner hair cell/spiral ganglion cell (SGC) connection and the loss of SGCs. However, functional changes at the central terminals of SGCs, namely the auditory nerve synapses in the cochlear nucleus, are not yet fully understood during ARHL. With the use of young (1-3 mo) and old (25-30 mo) CBA/CaJ mice, this study evaluated the intrinsic properties of the bushy neurons postsynaptic to the endbulb of Held synapses, and the firing properties of these neurons to direct current injections as well as to synaptic inputs from the auditory nerve...
December 1, 2016: Journal of Neurophysiology
https://www.readbyqxmd.com/read/27669149/schwann-cells-genetically-modified-to-express-s100a4-increases-gap43-expression-in-spiral-ganglion-neurons-in-vitro
#19
Li Lei, Li Tang
Schwann cells (SCs) have been reported as a possible source of neurotrophic support for spiral ganglion neurons (SGNs). This study was aimed to investigate whether S100A4 was contributed in the functional effects of SCs on SGNs. SCs were transfected with S100A4 vector or small interfering RNA (siRNA) against S100A4, and the transfection efficiency was verified by quantitative PCR (qPCR) and Western blot. The migration of transfected SCs was determined by Transwell assay, and the expression levels of vascular endothelial growth factor precursor (VEGF) and matrix metallopeptidase 9 (MMP-9) were measured by Western blot...
September 26, 2016: Bioengineered
https://www.readbyqxmd.com/read/27667674/age-related-hearing-loss-prevention-of-threshold-declines-cell-loss-and-apoptosis-in-spiral-ganglion-neurons
#20
Robert D Frisina, Bo Ding, Xiaoxia Zhu, Joseph P Walton
Age-related hearing loss (ARHL) -presbycusis - is the most prevalent neurodegenerative disease and number one communication disorder of our aged population; and affects hundreds of millions of people worldwide. Its prevalence is close to that of cardiovascular disease and arthritis, and can be a precursor to dementia. The auditory perceptual dysfunction is well understood, but knowledge of the biological bases of ARHL is still somewhat lacking. Surprisingly, there are no FDA-approved drugs for treatment. Based on our previous studies of human subjects, where we discovered relations between serum aldosterone levels and the severity of ARHL, we treated middle age mice with aldosterone, which normally declines with age in all mammals...
September 23, 2016: Aging
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