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https://www.readbyqxmd.com/read/28432353/severe-hearing-loss-and-outer-hair-cell-death-in-homozygous-foxo3-knockout-mice-after-moderate-noise-exposure
#1
Felicia Gilels, Stephen T Paquette, Holly J Beaulac, Anwen Bullen, Patricia M White
Noise induced hearing loss (NIHL) is a disease that affects millions of Americans. Identifying genetic pathways that influence recovery from noise exposure is an important step forward in understanding NIHL. The transcription factor Foxo3 integrates the cellular response to oxidative stress and plays a role in extending lifespan in many organisms, including humans. Here we show that Foxo3 is required for auditory function after noise exposure in a mouse model system, measured by ABR. Absent Foxo3, outer hair cells are lost throughout the middle and higher frequencies...
April 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28424585/efr3a-insufficiency-attenuates-the-degeneration-of-spiral-ganglion-neurons-after-hair-cell-loss
#2
Haixia Hu, Bin Ye, Le Zhang, Quan Wang, Zhiwei Liu, Suying Ji, Qiuju Liu, Jingrong Lv, Yan Ma, Ying Xu, Hao Wu, Fude Huang, Mingliang Xiang
Sensorineural hearing loss (SNHL) is caused by an irreversible impairment of cochlear hair cells and subsequent progressive degeneration of spiral ganglion neurons (SGNs). Eighty-five requiring 3 (Efr3) is a plasma membrane protein conserved from yeast to human, and knockout of Efr3a was reported to facilitate the survival of hippocampal newborn neurons in adult mice. Previously, we found Efr3a expression in the auditory neural pathway is upregulated soon after the destruction of hair cells. Here we conducted a time-course analysis of drug-caused damage to hearing ability, hair cells and SGNs in Efr3a knocking down mice (Efr3a(-/+), Efr3a KD) and their wild type littermates...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28367981/cochlear-gene-therapy-with-ancestral-aav-in-adult-mice-complete-transduction-of-inner-hair-cells-without-cochlear-dysfunction
#3
Jun Suzuki, Ken Hashimoto, Ru Xiao, Luk H Vandenberghe, M Charles Liberman
The use of viral vectors for inner ear gene therapy is receiving increased attention for treatment of genetic hearing disorders. Most animal studies to date have injected viral suspensions into neonatal ears, via the round window membrane. Achieving transduction of hair cells, or sensory neurons, throughout the cochlea has proven difficult, and no studies have been able to efficiently transduce sensory cells in adult ears while maintaining normal cochlear function. Here, we show, for the first time, successful transduction of all inner hair cells and the majority of outer hair cells in an adult cochlea via virus injection into the posterior semicircular canal...
April 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28345280/otoprotective-effects-of-mouse-nerve-growth-factor-in-dba-2j-mice-with-early-onset-progressive-hearing-loss
#4
Qingzhu Wang, Hongchun Zhao, Tihua Zheng, Wenjun Wang, Xiaolin Zhang, Andi Wang, Bo Li, Yanfei Wang, Qingyin Zheng
As it displays progressive hair-cell loss and degeneration of spiral ganglion neurons (SGNs) characterized by early-onset progressive hearing loss (ePHL), DBA/2J is an inbred mouse strain widely used in hearing research. Mouse nerve growth factor (mNGF), as a common exogenous nerve growth factor (NGF), has been studied extensively for its ability to promote neuronal survival and growth. To determine whether mNGF can ameliorate progressive hearing loss (PHL) in DBA/2J mice, saline or mNGF was given to DBA/2J mice of either sex by daily intramuscular injection from the 1st to the 9th week after birth...
March 27, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28344071/exposure-to-diphtheria-toxin-during-the-juvenile-period-impairs-both-inner-and-outer-hair-cells-in-c57bl-6-mice
#5
Hiroyuki Konishi, Nobutaka Ohgami, Aika Matsushita, Yuki Kondo, Yuki Aoyama, Masaaki Kobayashi, Taku Nagai, Shinya Ugawa, Kiyofumi Yamada, Masashi Kato, Hiroshi Kiyama
Diphtheria toxin (DT) administration into transgenic mice that express the DT receptor (DTR) under control of specific promoters is often used for cell ablation studies in vivo. Because DTR is not expressed in mice, DT injection has been assumed to be nontoxic to cells in vivo. In this study, we demonstrated that DT application during the juvenile stage leads to hearing loss in wild-type mice. Auditory brainstem response measurement showed severe hearing loss in C57BL/6 mice administered DT during the juvenile period, and the hearing loss persisted into adulthood...
March 24, 2017: Neuroscience
https://www.readbyqxmd.com/read/28316840/hearing-loss-reestablish-the-neural-plasticity-in-regenerated-spiral-ganglion-neurons-and-sensory-hair-cells
#6
EDITORIAL
Renjie Chai, Geng-Lin Li, Jian Wang, Jing Zou
No abstract text is available yet for this article.
2017: Neural Plasticity
https://www.readbyqxmd.com/read/28283883/a-three-dimensional-culture-system-with-matrigel-promotes-purified-spiral-ganglion-neuron-survival-and-function-in-vitro
#7
Wenqing Yan, Wenwen Liu, Jieyu Qi, Qiaojun Fang, Zhaomin Fan, Gaoying Sun, Yuechen Han, Daogong Zhang, Lei Xu, Mingming Wang, Jianfeng Li, Fangyi Chen, Dong Liu, Renjie Chai, Haibo Wang
In vitro culture of spiral ganglion neurons (SGNs) is a useful approach to investigate numerous aspects of neuronal behavior and to identify potential therapeutic targets for SGN protection and regeneration. However, the isolation of SGNs and the long-term maintenance of their structure and function in vitro remain challenging. In this study, we isolated SGNs from Bhlhb5-cre and Rosa26-tdTomato mice with fluorescence-activated cell sorting and determined the cell purity. We then encapsulated the pure SGNs in matrigel and cultured the SGNs in vitro...
March 10, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28280753/mouse-auditory-brainstem-response-testing
#8
Omar Akil, A E Oursler, Kevin Fan, Lawrence R Lustig
The auditory brainstem response (ABR) test provides information about the inner ear (cochlea) and the central pathways for hearing. The ABR reflects the electrical responses of both the cochlear ganglion neurons and the nuclei of the central auditory pathway to sound stimulation (Zhou et al., 2006; Burkard et al., 2007). The ABR contains 5 identifiable wave forms, labeled as I-V. Wave I represents the summated response from the spiral ganglion and auditory nerve while waves II-V represent responses from the ascending auditory pathway...
March 20, 2016: Bio-protocol
https://www.readbyqxmd.com/read/28264554/hearing-improvement-in-a-j-mice-via-the-mouse-nerve-growth-factor
#9
Lixiang Gao, Ruli Ge, Gang Xie, Dandan Yao, Ping Li, Oumei Wang, Xiufang Ma, Fengchan Han
Objectives: To investigate the otoprotective effects of mouse nerve growth factor (mNGF) in A/J mice. Methods: The mice at postnatal day 7 (P7) were randomly separated into a mNGF treated group (mNGF group) and a distilled water (for injection) treated group (control group). The mNGF dissolved in distilled water or distilled water alone was given to the mice once every other day from P7 by intramuscular injection in the hips. The otoprotective effects of mNGF in A/J mice were observed in a time course manner...
March 8, 2017: Clinical and Experimental Otorhinolaryngology
https://www.readbyqxmd.com/read/28239347/hearing-dysfunction-in-xpa-deficient-mice
#10
Hitomi Shinomiya, Daisuke Yamashita, Takeshi Fujita, Eiji Nakano, Go Inokuchi, Shingo Hasegawa, Naoki Otsuki, Chikako Nishigori, Ken-Ichi Nibu
Xeroderma pigmentosum (XP) is a rare recessive heredity disease caused by DNA repair impairment characterized by photosensitivity and neurologic symptoms in half of the cases. There are eight subtypes of XP: XP-A-XP-G and XP variant. Among eight subtypes, XP complementation group A (XP-A) display the lowest DNA repair ability and the severest cutaneous and neurologic symptoms. While its pathogenesis of skin symptoms have been well-studied, that of neurological symptoms, including sensorineural hearing loss (SNHL) remains unknown...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28216123/ablation-of-the-auditory-cortex-results-in-changes-in-the-expression-of-neurotransmission-related-mrnas-in-the-cochlea
#11
Verónica Lamas, José M Juiz, Miguel A Merchán
The auditory cortex (AC) dynamically regulates responses of the Organ of Corti to sound through descending connections to both the medial (MOC) and lateral (LOC) olivocochlear efferent systems. We have recently provided evidence that AC has a reinforcement role in the responses to sound of the auditory brainstem nuclei. In a molecular level, we have shown that descending inputs from AC are needed to regulate the expression of molecules involved in outer hair cell (OHC) electromotility control, such as prestin and the α10 nicotinic acetylcholine receptor (nAchR)...
March 2017: Hearing Research
https://www.readbyqxmd.com/read/28213135/genetic-variants-in-the-peripheral-auditory-system-significantly-affect-adult-cochlear-implant-performance
#12
A Eliot Shearer, Robert W Eppsteiner, Kathy Frees, Viral Tejani, Christina M Sloan-Heggen, Carolyn Brown, Paul Abbas, Camille Dunn, Marlan R Hansen, Bruce J Gantz, Richard J H Smith
BACKGROUND: Cochlear implantation is an effective habilitation modality for adults with significant hearing loss. However, post-implant performance is variable. A portion of this variance in outcome can be attributed to clinical factors. Recent physiological studies suggest that the health of the spiral ganglion also impacts post-operative cochlear implant outcomes. The goal of this study was to determine whether genetic factors affecting spiral ganglion neurons may be associated with cochlear implant performance...
May 2017: Hearing Research
https://www.readbyqxmd.com/read/28188805/sphingosine-1-phosphate-signaling-axis-mediates-fibroblast-growth-factor-2-induced-proliferation-and-survival-of-murine-auditory-neuroblasts
#13
Marina Bruno, Ilaria Maria Rizzo, Ricardo Romero-Guevara, Caterina Bernacchioni, Francesca Cencetti, Chiara Donati, Paola Bruni
Hearing loss affects millions of people in the world. In mammals the auditory system comprises diverse cell types which are terminally differentiated and with no regenerative potential. There is a tremendous research interest aimed at identifying cell therapy based solutions or pharmacological approaches that could be applied therapeutically alongside auditory devices to prevent hair cell and neuron loss. Sphingosine 1-phosphate (S1P) is a pleiotropic bioactive sphingolipid that plays key role in the regulation of many physiological and pathological functions...
May 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28186679/directed-differentiation-of-human-embryonic-stem-cells-toward-placode-derived-spiral-ganglion-like-sensory-neurons
#14
Akihiro J Matsuoka, Zachery D Morrissey, Chaoying Zhang, Kazuaki Homma, Abdelhak Belmadani, Charles A Miller, Duncan M Chadly, Shun Kobayashi, Alexandra N Edelbrock, Miho Tanaka-Matakatsu, Donna S Whitlon, Ljuba Lyass, Tammy L McGuire, Samuel I Stupp, John A Kessler
The ability to generate spiral ganglion neurons (SGNs) from stem cells is a necessary prerequisite for development of cell-replacement therapies for sensorineural hearing loss. We present a protocol that directs human embryonic stem cells (hESCs) toward a purified population of otic neuronal progenitors (ONPs) and SGN-like cells. Between 82% and 95% of these cells express SGN molecular markers, they preferentially extend neurites to the cochlear nucleus rather than nonauditory nuclei, and they generate action potentials...
March 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28183797/ca-2-binding-protein-2-inhibits-ca-2-channel-inactivation-in-mouse-inner-hair-cells
#15
Maria Magdalena Picher, Anna Gehrt, Sandra Meese, Aleksandra Ivanovic, Friederike Predoehl, SangYong Jung, Isabelle Schrauwen, Alberto Giulio Dragonetti, Roberto Colombo, Guy Van Camp, Nicola Strenzke, Tobias Moser
Ca(2+)-binding protein 2 (CaBP2) inhibits the inactivation of heterologously expressed voltage-gated Ca(2+) channels of type 1.3 (CaV1.3) and is defective in human autosomal-recessive deafness 93 (DFNB93). Here, we report a newly identified mutation in CABP2 that causes a moderate hearing impairment likely via nonsense-mediated decay of CABP2-mRNA. To study the mechanism of hearing impairment resulting from CABP2 loss of function, we disrupted Cabp2 in mice (Cabp2(LacZ/LacZ) ). CaBP2 was expressed by cochlear hair cells, preferentially in inner hair cells (IHCs), and was lacking from the postsynaptic spiral ganglion neurons (SGNs)...
February 28, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28118374/dynamic-expression-of-sox2-gata3-and-prox1-during-primary-auditory-neuron-development-in-the-mammalian-cochlea
#16
Koji Nishimura, Teppei Noda, Alain Dabdoub
Primary auditory neurons (PANs) connect cochlear sensory hair cells in the mammalian inner ear to cochlear nucleus neurons in the brainstem. PANs develop from neuroblasts delaminated from the proneurosensory domain of the otocyst and keep maturing until the onset of hearing after birth. There are two types of PANs: type I, which innervate the inner hair cells (IHCs), and type II, which innervate the outer hair cells (OHCs). Glial cells surrounding these neurons originate from neural crest cells and migrate to the spiral ganglion...
2017: PloS One
https://www.readbyqxmd.com/read/28113296/pressure-in-the-cochlea-during-infrared-irradiation
#17
Nan Xia, Xiaodong Tan, Yingyue Xu, Wensheng Hou, Teresa Mao, Claus-Peter Richter
OBJECTIVE: The purpose of the study is to demonstrate laser evoked pressure waves in small confined volumes such as the cochlea. METHODS: Custom fabricated pressure probes were used to determine the pressure in front of the optical fiber in a small dish and patch pipettes to measure temperature changes. Pressure probes were inserted into scala tympani or vestibuli during laser stimulation. With a sensitive microphone the pressure was measured in the outer ear canal...
December 7, 2016: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/28101341/influence-of-high-altitude-hypoxic-environments-on-the-survival-of-cochlear-hair-cells-and-spiral-ganglion-neurons-in-rats
#18
Dongyan Fan, Hailong Ren, Dunzhu Danzeng, Haonan Li, Ping Wang
The aim of the present study was to observe the histological changes in the peripheral auditory system in rats at different time-points after relocating from low altitude to high altitude (3,600 m). The general physical condition of the rats was observed and cochlear tissue samples were obtained every month. The morphology and survival of the cochlear hair cells (HCs) were observed using cochlear surface preparation at 1, 30, 90, 120, 150 and 180 days after moving to the plateau area. Changes in spiral ganglion neurons (SGNs) were detected at different time-points using immunofluorescence technology on frozen sections...
December 2016: Biomedical Reports
https://www.readbyqxmd.com/read/28087338/auditory-hindbrain-atrophy-and-anomalous-calcium-binding-protein-expression-after-neonatal-exposure-to-monosodium-glutamate
#19
Lindsey Foran, Kaitlyn Blackburn, Randy J Kulesza
Glutamate is the most abundant excitatory neurotransmitter in the central nervous system, and is stored and released by both neurons and astrocytes. Despite the important role of glutamate as a neurotransmitter, elevated extracellular glutamate can result in excitotoxicity and apoptosis. Monosodium glutamate (MSG) is a naturally occurring sodium salt of glutamic acid that is used as a flavor enhancer in many processed foods. Previous studies have shown that MSG administration during the early postnatal period results in neurodegenerative changes in several forebrain regions, characterized by neuronal loss and neuroendocrine abnormalities...
March 6, 2017: Neuroscience
https://www.readbyqxmd.com/read/28062185/allicin-protects-auditory-hair-cells-and-spiral-ganglion-neurons-from-cisplatin-induced-apoptosis
#20
Xianmin Wu, Xiaofei Li, Yongdong Song, He Li, Xiaohui Bai, Wenwen Liu, Yuechen Han, Lei Xu, Jianfeng Li, Daogong Zhang, Haibo Wang, Zhaomin Fan
Cisplatin is a broad-spectrum anticancer drug that is commonly used in the clinic. Ototoxicity is one of the major side effects of this drug, which caused irreversible sensorineural hearing loss. Allicin, the main biologically active compound derived from garlic, has been shown to exert various anti-apoptotic and anti-oxidative activities in vitro and in vivo studies. We took advantage of C57 mice intraperitoneally injected with cisplatin alone or with cisplatin and allicin combined, to investigate whether allicin plays a protective role in vivo against cisplatin ototoxicity...
January 3, 2017: Neuropharmacology
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