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inner ear hair cell

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https://www.readbyqxmd.com/read/28109437/analysis-of-the-proteome-of-hair-cell-stereocilia-by-mass-spectrometry
#1
J F Krey, P A Wilmarth, L L David, P G Barr-Gillespie
Characterization of proteins that mediate mechanotransduction by hair cells, the sensory cells of the inner ear, is hampered by the scarcity of these cells and their sensory organelle, the hair bundle. Mass spectrometry, with its high sensitivity and identification precision, is the ideal method for determining which proteins are present in bundles and what proteins they interact with. We describe here the isolation of mouse hair bundles, as well as preparation of bundle protein samples for mass spectrometry...
2017: Methods in Enzymology
https://www.readbyqxmd.com/read/28105691/the-alcohol-sensitive-period-during-early-octavolateral-organ-development-in-zebrafish-danio-rerio
#2
Lilliann Y Zamora, Kayla C Miguel, Zhongmin Lu
Fetal alcohol exposure can cause Fetal Alcohol Spectrum Disorders (FASD), completely preventable developmental disabilities characterized by permanent birth defects. However, specific gestational timing when developing organs are most sensitive to alcohol exposure is unclear. In this study, we examined the temporal effects of embryonic alcohol exposure on octavolateral organs in zebrafish (Danio rerio), including inner ears and lateral line neuromasts that function in hearing, balance, and hydrodynamic detection, respectively...
January 20, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28087419/cochlear-synaptopathy-in-acquired-sensorineural-hearing-loss-manifestations-and-mechanisms
#3
REVIEW
M Charles Liberman, Sharon G Kujawa
Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis...
January 10, 2017: Hearing Research
https://www.readbyqxmd.com/read/28039372/global-analysis-of-protein-expression-of-inner-ear-hair-cells
#4
Ann E Hickox, Ann C Y Wong, Kwang Pak, Chelsee Strojny, Miguel Ramirez, John R Yates, Allen F Ryan, Jeffrey N Savas
: The mammalian inner ear subserves auditory and vestibular sensations via highly specialized cells and proteins. Sensory receptor hair cells (HCs) are necessary for transducing mechanical inputs and stimulating sensory neurons by employing a host of known and yet unknown protein machinery. To understand the protein composition of these unique post-mitotic cells, in which irreversible protein degradation or damage can lead to impaired hearing and balance, we analyzed inner ear samples by tandem mass spectrometry to generate an unbiased, shotgun-proteomics view of protein identities and abundances...
December 30, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28034618/adenosine-receptors-regulate-susceptibility-to-noise-induced-neural-injury-in-the-mouse-cochlea-and-hearing-loss
#5
Srdjan M Vlajkovic, Kaushi Ambepitiya, Meagan Barclay, Detlev Boison, Gary D Housley, Peter R Thorne
Our previous studies have shown that the stimulation of A1 adenosine receptors in the inner ear can mitigate the loss of sensory hair cells and hearing loss caused by exposure to traumatic noise. Here, we focus on the role of adenosine receptors (AR) in the development of noise-induced neural injury in the cochlea using A1AR and A2AAR null mice (A1AR(-/-) and A2AAR(-/-)). Wildtype (WT) and AR deficient mice were exposed to octave band noise (8-16 kHz, 100 dB SPL) for 2 hours to induce cochlear injury and hearing loss...
December 26, 2016: Hearing Research
https://www.readbyqxmd.com/read/28034617/cochlear-hair-cell-regeneration-after-noise-induced-hearing-loss-does-regeneration-follow-development
#6
REVIEW
Fei Zheng, Jian Zuo
Noise-induced hearing loss (NIHL) affects a large number of military personnel and civilians. Regenerating inner-ear cochlear hair cells (HCs) is a promising strategy to restore hearing after NIHL. In this review, we first summarize recent transcriptome profile analysis of zebrafish lateral lines and chick utricles where spontaneous HC regeneration occurs after HC damage. We then discuss recent studies in other mammalian regenerative systems such as pancreas, heart and central nervous system. Both spontaneous and forced HC regeneration occurs in mammalian cochleae in vivo involving proliferation and direct lineage conversion...
December 26, 2016: Hearing Research
https://www.readbyqxmd.com/read/28024462/predicting-the-perceptual-consequences-of-hidden-hearing-loss
#7
Andrew J Oxenham
Recent physiological studies in several rodent species have revealed that permanent damage can occur to the auditory system after exposure to a noise that produces only a temporary shift in absolute thresholds. The damage has been found to occur in the synapses between the cochlea's inner hair cells and the auditory nerve, effectively severing part of the connection between the ear and the brain. This synaptopathy has been termed hidden hearing loss because its effects are not thought to be revealed in standard clinical, behavioral, or physiological measures of absolute threshold...
January 2016: Trends in Hearing
https://www.readbyqxmd.com/read/28013291/slc44a4-mutation-causes-autosomal-dominant-hereditary-postlingual-non-syndromic-mid-frequency-hearing-loss
#8
Zhaoxin Ma, Wenjun Xia, Fei Liu, Jing Ma, Shaoyang Sun, Jin Zhang, Nan Jiang, Xu Wang, Jiongjiong Hu, Duan Ma
Clinical, genetic, and functional investigations were performed to identify the causative mutation in a distinctive Chinese family with postlingual non-syndromic mid-frequency sensorineural hearing loss. Whole-exome sequencing revealed SLC44A4, which encodes the choline transport protein, as the pathogenic gene in this family. In the zebrafish model, downregulation of slc44a4 using morpholinos led to significant abnormalities in zebrafish inner ear and lateral line neuromasts and contributed, to some extent, to disabilities in hearing and balance...
December 23, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28013217/strain-and-sex-differences-in-the-vestibular-and-systemic-toxicity-of-3-3-iminodipropionitrile-in-mice
#9
Pere Boadas-Vaello, Lara Sedó-Cabezón, Enrique Verdú, Jordi Llorens
The nitrile 3,3'-iminodipropionitrile (IDPN) causes a loss of hair cells in the vestibular epithelium of the inner ear in several species of both mammals and nonmammals. It is of interest as a model compound in ototoxicity and vestibular regeneration research, but its effects on the mouse, including the potential relevance of strain and sex differences for susceptibility, have not yet been thoroughly characterized. In this study, we compared the vestibular toxicity of IDPN in dose-response studies (0, 8, 12, 16, and 24 mmol/kg IDPN p...
December 24, 2016: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28012509/changes-in-the-inner-ear-structures-in-cystic-fibrosis-patients
#10
Henrique F Pauna, Rafael C Monsanto, Natsuko Kurata, Michael M Paparella, Sebahattin Cureoglu
OBJECTIVE: Although prolonged use of antibiotics is very common in cystic fibrosis (CF) patients, no studies have assessed the changes in both cochlear and peripheral vestibular systems in this population. METHODS: We used human temporal bones to analyze the density of vestibular dark, transitional, and hair cells in specimens from CF patients who were exposed to several types of antibiotics, as compared with specimens from an age-matched control group with no history of ear disease or antibiotic use...
January 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28004290/quantitative-analysis-of-aquaporin-expression-levels-during-the-development-and-maturation-of-the-inner-ear
#11
Takushi Miyoshi, Taro Yamaguchi, Kiyokazu Ogita, Yasuko Tanaka, Ken-Ichi Ishibashi, Hiroaki Ito, Taisuke Kobayashi, Takayuki Nakagawa, Juichi Ito, Koichi Omori, Norio Yamamoto
Aquaporins (AQPs) are a family of small membrane proteins that transport water molecules across the plasma membrane along the osmotic gradient. Mammals express 13 subtypes of AQPs, including the recently reported "subcellular AQPs", AQP11 and 12. Each organ expresses specific subsets of AQP subtypes, and in the inner ear, AQPs are essential for the establishment and maintenance of two distinct fluids, endolymph and perilymph. To evaluate the contribution of AQPs during the establishment of inner ear function, we used quantitative reverse transcription polymerase chain reaction to quantify the expression levels of all known AQPs during the entire development and maturation of the inner ear...
December 21, 2016: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/27992391/auditory-brainstem-response-altered-in-humans-with-noise-exposure-despite-normal-outer-hair-cell-function
#12
Naomi F Bramhall, Dawn Konrad-Martin, Garnett P McMillan, Susan E Griest
OBJECTIVES: Recent animal studies demonstrated that cochlear synaptopathy, a partial loss of inner hair cell-auditory nerve fiber synapses, can occur in response to noise exposure without any permanent auditory threshold shift. In animal models, this synaptopathy is associated with a reduction in the amplitude of wave I of the auditory brainstem response (ABR). The goal of this study was to determine whether higher lifetime noise exposure histories in young people with clinically normal pure-tone thresholds are associated with lower ABR wave I amplitudes...
January 2017: Ear and Hearing
https://www.readbyqxmd.com/read/27966334/study-of-mouse-induced-pluripotent-stem-cell-transplantation-intowistar-albino-rat-cochleae-after-hair-cell-damage
#13
Mustafa Kürşat Gökcan, Selçuk Mülazimoğlu, Emre Ocak, Pınar Can, Murat Çalışkan, Ömer Beşaltı, Serpil Dizbay Sak, Gülşah Kaygusuz
BACKGROUND/AIM: As the regeneration capacity of hair cells is limited, inner ear stem cell therapies hold promise. Effects of mouse induced pluripotent stem cells (IPSCs) on Wistar albino rats (WARs) with hearing impairment were investigated. MATERIALS AND METHODS: Thirty-five adult WARs with normal hearing were divided into 4 groups. Excluding the study group (n = 15), the other three groups served as control groups for ototoxicity and IPSC injection models. IPSC injections were performed via cochleostomy after a retroauricular approach...
November 17, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/27964936/musicianship-enhances-ipsilateral-and-contralateral-efferent-gain-control-to-the-cochlea
#14
Gavin M Bidelman, Amy D Schneider, Victoria R Heitzmann, Shaum P Bhagat
Human hearing sensitivity is easily compromised with overexposure to excessively loud sounds, leading to permanent hearing damage. Consequently, finding activities and/or experiential factors that distinguish "tender" from "tough" ears (i.e., acoustic vulnerability) would be important for identifying people at higher risk for hearing damage. To regulate sound transmission and protect the inner ear against acoustic trauma, the auditory system modulates gain control to the cochlea via biological feedback of the medial olivocochlear (MOC) efferents, a neuronal pathway linking the lower brainstem and cochlear outer hair cells...
December 11, 2016: Hearing Research
https://www.readbyqxmd.com/read/27942598/microrna-183-family-in-inner-ear-hair-cell-development-and-deafness
#15
REVIEW
Mohammad Reza Mahmoodian Sani, Morteza Hashemzadeh-Chaleshtori, Massoud Saidijam, Mohammad-Saeid Jami, Payam Ghasemi-Dehkordi
miRNAs are essential factors of an extensively conserved post-transcriptional process controlling gene expression at mRNA level. Varoius biological processes such as growth and differentiation are regulated by miRNAs. Web of Science and PubMed databases were searched using the Endnote software for the publications about the role miRNA-183 family in inner ear: hair cell development and deafness published from 2000 to 2016. A triplet of these miRNAs particularly the miR-183 family is highly expressed in vertebrate hair cells, as with some of the peripheral neurosensory cells...
December 2016: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/27926851/energy-output-from-a-single-outer-hair-cell
#16
Kuni H Iwasa
Electromotility of outer hair cells (OHCs) has been extensively studied with in vitro experiments because of its physiological significance to the cochlear amplifier, which provides the exquisite sensitivity and frequency selectivity of the mammalian ear. However, these studies have been performed largely under load-free conditions or with static load, while these cells function in vivo in a dynamic environment, receiving electrical energy to enhance mechanical oscillation in the inner ear. This gap leaves uncertainties in addressing a key issue, how much mechanical energy an OHC provides...
December 6, 2016: Biophysical Journal
https://www.readbyqxmd.com/read/27918591/transcriptomic-analysis-of-mouse-cochlear-supporting-cell-maturation-reveals-large-scale-changes-in-notch-responsiveness-prior-to-the-onset-of-hearing
#17
Juan C Maass, Rende Gu, Tiantian Cai, Ying-Wooi Wan, Silvia C Cantellano, Joanna S T Asprer, Hongyuan Zhang, Hsin-I Jen, Renée K Edlund, Zhandong Liu, Andrew K Groves
Neonatal mouse cochlear supporting cells have a limited ability to divide and trans-differentiate into hair cells, but this ability declines rapidly in the two weeks after birth. This decline is concomitant with the morphological and functional maturation of the organ of Corti prior to the onset of hearing. However, despite this association between maturation and loss of regenerative potential, little is known of the molecular changes that underlie these events. To identify these changes, we used RNA-seq to generate transcriptional profiles of purified cochlear supporting cells from 1- and 6-day-old mice...
2016: PloS One
https://www.readbyqxmd.com/read/27917898/incomplete-and-delayed-sox2-deletion-defines-residual-ear-neurosensory-development-and-maintenance
#18
Martina Dvorakova, Israt Jahan, Iva Macova, Tetyana Chumak, Romana Bohuslavova, Josef Syka, Bernd Fritzsch, Gabriela Pavlinkova
The role of Sox2 in neurosensory development is not yet fully understood. Using mice with conditional Islet1-cre mediated deletion of Sox2, we explored the function of Sox2 in neurosensory development in a model with limited cell type diversification, the inner ear. In Sox2 conditional mutants, neurons initially appear to form normally, whereas late- differentiating neurons of the cochlear apex never form. Variable numbers of hair cells differentiate in the utricle, saccule, and cochlear base but sensory epithelium formation is completely absent in the apex and all three cristae of the semicircular canal ampullae...
December 5, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27898607/effects-of-intratympanic-dexamethasone-on-high-dose-radiation-ototoxicity-in-vivo
#19
Christine T Dinh, Si Chen, John Dinh, Stefania Goncalves, Esperanza Bas, Kyle Padgett, Perry Johnson, Nagy Elsayyad, Fred Telischi, Thomas Van De Water
BACKGROUND: Stereotactic radiosurgery for lateral skull base tumors can cause hearing loss when the cochleae are exposed to high doses of single-fraction radiation. Currently, there are no known nondosimetric preventative treatments for radiation-induced ototoxicity. HYPOTHESIS: Intratympanic (IT) dexamethasone (DXM), a synthetic steroid, protects against radiation-induced auditory hair cell (HC) and hearing losses in rats in vivo. METHODS: Seven rats received radiation (12 Gy) to both cochleae...
November 24, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27896618/loss-of-liver-kinase-b1-causes-planar-polarity-defects-in-cochlear-hair-cells-in-mice
#20
Yuqin Men, Aizhen Zhang, Liwen Zhang, Yecheng Jin, Zhishuo Wang, Jing Zhao, Xiaolin Yu, Jian Zhang, Jiangang Gao
The tumor suppressor gene liver kinase B1 (LKB1), also called STK11, encodes a serine/threonine kinase. LKB1 plays crucial roles in cell differentiation, proliferation, and polarity. In this study, LKB1 conditional knockout mice (LKB1(Pax2) CKO mice) were generated using Pax2-Cre mice to investigate the function of LKB1 in inner ear hair cells during early embryonic period. LKB1(Pax2) CKO mice died perinatally. Immunofluorescence and scanning electron microscopy revealed that stereociliary bundles in LKB1(Pax2) CKO mice were clustered and misoriented, respectively...
November 28, 2016: Frontiers of Medicine
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