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https://www.readbyqxmd.com/read/27926851/energy-output-from-a-single-outer-hair-cell
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/27895556/neurosensory-differentiation-and-innervation-patterning-in-the-human-fetal-vestibular-end-organs-between-the-gestational-weeks-8-12
#6
Lejo Johnson Chacko, Elisabeth J Pechriggl, Helga Fritsch, Helge Rask-Andersen, Michael J F Blumer, Anneliese Schrott-Fischer, Rudolf Glueckert
Balance orientation depends on the precise operation of the vestibular end organs and the vestibular ganglion neurons. Previous research on the assemblage of the neuronal network in the developing fetal vestibular organ has been limited to data from animal models. Insights into the molecular expression profiles and signaling moieties involved in embryological development of the human fetal inner ear have been limited. We present an investigation of the cells of the vestibular end organs with specific focus on the hair cell differentiation and innervation pattern using an uninterrupted series of unique specimens from gestational weeks 8-12...
2016: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/27895287/sudden-sensorineural-hearing-loss-is-there-a-connection-with-inner-ear-electrolytic-disorders-a-literature-review
#7
EDITORIAL
Andrea Ciorba, Virginia Corazzi, Chiara Bianchini, Claudia Aimoni, Henryk Skarzynski, Piotr Henryk Skarzynski, Stavros Hatzopoulos
Electrolytic disorders of the inner ear represent a model that could be implicated in partially explaining the pathogenesis of sudden sensorineural hearing loss (SSNHL). Different types of electrolytes and different inner-ear loci are involved in cochlear homeostasis physiologically, to ensure the maintenance of an ion-balanced cochlear environment allowing a normal hair cell function. It has been hypothesized that a sudden loss of endocochlear potential, due to a rapid disruption of the inner ear fluid osmolality, could be responsible for a deterioration of the hearing function caused by damaged hair cells...
December 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/27890677/time-course-of-organ-of-corti-degeneration-after-noise-exposure
#8
Barbara A Bohne, Melissa Kimlinger, Gary W Harding
From our permanent collection of plastic-embedded flat preparations of chinchilla cochleae, 22 controls and 199 ears from noise-exposed animals were used to determine when, postexposure, hair cell (HC) and supporting cell (SC) degeneration were completed. The exposed ears were divided into four groups based on exposure parameters: 0.5- or 4-kHz octave band of noise at moderate (M) or high (H) intensities. Postexposure survival ranged from <1 h to 2.5 y. Ears fixed ≤ 0-12 h postexposure were called 'acute'...
November 24, 2016: Hearing Research
https://www.readbyqxmd.com/read/27890673/functional-validation-of-abhd12-mutations-in-the-neurodegenerative-disease-pharc
#9
Angèle Tingaud-Sequeira, Demetrio Raldúa, Julie Lavie, Guilaine Mathieu, Magali Bordier, Anja Knoll-Gellida, Pierre Rambeau, Isabelle Coupry, Michèle André, Eva Malm, Claes Möller, Sten Andreasson, Nanna D Rendtorff, Lisbeth Tranebjærg, Michel Koenig, Didier Lacombe, Cyril Goizet, Patrick J Babin
ABHD12 mutations have been linked to neurodegenerative PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract), a rare, progressive, autosomal, recessive disease. Although ABHD12 is suspected to play a role in the lysophosphatidylserine and/or endocannabinoid pathways, its precise functional role(s) leading to PHARC disease had not previously been characterized. Cell and zebrafish models were designed to demonstrate the causal link between an identified new missense mutation p...
November 23, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27881722/maintenance-of-neuronal-size-gradient-in-mntb-requires-sound-evoked-activit
#10
Jessica H Weatherstone, Conny Kopp-Scheinpflug, Nadia Pilati, Yuan Wang, Ian D Forsythe, Edwin W Rubel, Bruce L Tempel
The medial nucleus of the trapezoid body (MNTB) is an important source of inhibition during the computation of sound location. It transmits fast and precisely timed action potentials at high frequencies; this requires an efficient calcium clearance mechanism, in which the plasma membrane calcium ATPase 2 (PMCA2) is a key component. Deafwaddler (dfw(2J)) mutant mice have a null mutation in PMCA2 causing deafness in homozygotes (dfw(2J)/dfw(2J)) and high frequency hearing loss in heterozygotes (+/dfw(2J)). Despite the deafness phenotype, no significant differences in MNTB volume or cell number were observed in dfw(2J) homozygous mutants, suggesting PMCA2 is not required for MNTB neuron survival...
November 23, 2016: Journal of Neurophysiology
https://www.readbyqxmd.com/read/27878601/reduction-of-permanent-hearing%C3%A2-loss-by-local-glucocorticoid-application-guinea-pigs-with-acute-acoustic-trauma
#11
M Müller, M Tisch, H Maier, H Löwenheim
BACKGROUND: High-intensity noise exposure from impulse and blast noise events often leads to acute hearing loss and may cause irreversible permanent hearing loss as a long-term consequence. Here, a treatment regime was developed to limit permanent damage based on a preclinical animal model of acute noise trauma. AIM: To develop clinical trials for the treatment of acute noise traumas using approved drugs. The otoprotective potential of glucocorticoids applied locally to the inner ear was examined...
November 23, 2016: HNO
https://www.readbyqxmd.com/read/27869308/signaling-in-the-auditory-system-implications-in-hair-cell-regeneration-and-hearing-function
#12
REVIEW
Rahul Mittal, Luca H Debs, Desiree Nguyen, Amit P Patel, M'hamed Grati, Jeenu Mittal, Denise Yan, Adrien A Eshraghi, Xue Zhong Liu
Ear is a sensitive organ involved in hearing and balance function. The complex signaling network in the auditory system plays a crucial role in maintaining normal physiological function of the ear. The inner ear is comprised of a variety of host signaling pathways working in synergy to deliver clear sensory messages. Any disruption, as minor as it can be, has the potential to affect this finely tuned system with temporary or permanent sequelae including vestibular deficits and hearing loss. Mutations linked to auditory symptoms, whether inherited or acquired, are being actively researched for ways to reverse, silence, or suppress them...
November 21, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27867666/plasma-membrane-targeting-of-protocadherin-15-is-regulated-by-the-golgi-associated-chaperone-protein-pist
#13
Hongyun Nie, Yueyue Liu, Xiaolei Yin, Huiren Cao, Yanfei Wang, Wei Xiong, Yushuang Lin, Zhigang Xu
Protocadherin 15 (PCDH15) is a core component of hair cell tip-links and crucial for proper function of inner ear hair cells. Mutations of PCDH15 gene cause syndromic and nonsyndromic hearing loss. At present, the regulatory mechanisms responsible for the intracellular transportation of PCDH15 largely remain unknown. Here we show that PIST, a Golgi-associated, PDZ domain-containing protein, interacts with PCDH15. The interaction is mediated by the PDZ domain of PIST and the C-terminal PDZ domain-binding interface (PBI) of PCDH15...
2016: Neural Plasticity
https://www.readbyqxmd.com/read/27864084/development-and-regeneration-of-vestibular-hair-cells-in-mammals
#14
REVIEW
Joseph Burns, Jennifer Stone
Vestibular sensation is essential for gaze stabilization, balance, and perception of gravity. The vestibular receptors in mammals, Type I and Type II hair cells, are located in five small organs in the inner ear. Damage to hair cells and their innervating neurons can cause crippling symptoms such as vertigo, visual field oscillation, and imbalance. In adult rodents, some Type II hair cells are regenerated and become re-innervated after damage, presenting opportunities for restoring vestibular function after hair cell damage...
November 15, 2016: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/27861854/lectins-selectively-label-cartilage-condensations-and-the-otic-neuroepithelium-within-the-embryonic-chicken-head
#15
Poulomi Ray, Ami J Hughes, Misha Sharif, Susan C Chapman
Cartilage morphogenesis during endochondral ossification follows a progression of conserved developmental events. Cells are specified towards a prechondrogenic fate and subsequently undergo condensation followed by overt differentiation. Currently available molecular markers of prechondrogenic and condensing mesenchyme rely on common regulators of the chondrogenic program that are not specific to the tissue type or location. Therefore tissue-specific condensations cannot be distinguished based on known molecular markers...
November 16, 2016: Journal of Anatomy
https://www.readbyqxmd.com/read/27857071/an-elastic-element-in-the-protocadherin-15-tip-link-of-the-inner-ear
#16
Raul Araya-Secchi, Brandon L Neel, Marcos Sotomayor
Tip link filaments convey force and gate inner-ear hair-cell transduction channels to mediate perception of sound and head movements. Cadherin-23 and protocadherin-15 form tip links through a calcium-dependent interaction of their extracellular domains made of multiple extracellular cadherin (EC) repeats. These repeats are structurally similar, but not identical in sequence, often featuring linkers with conserved calcium-binding sites that confer mechanical strength to them. Here we present the X-ray crystal structures of human protocadherin-15 EC8-EC10 and mouse EC9-EC10, which show an EC8-9 canonical-like calcium-binding linker, and an EC9-10 calcium-free linker that alters the linear arrangement of EC repeats...
November 18, 2016: Nature Communications
https://www.readbyqxmd.com/read/27855522/melatonin-mitigates-neomycin-induced-hair-cell-injury-in-zebrafish
#17
Kyoung Ho Oh, Yoon Chan Rah, Kyu Ho Hwang, Seung Hoon Lee, Soon Young Kwon, Jae Hyung Cha, June Choi
CONTEXT: Ototoxicity due to medications, such as aminoglycosides, is irreversible, and free radicals in the inner ear are assumed to play a major role. Because melatonin has an antioxidant property, we hypothesize that it might mitigate hair cell injury by aminoglycosides. OBJECTIVE: The objective of this study was to evaluate whether melatonin has an alleviative effect on neomycin-induced hair cell injury in zebrafish (Danio rerio). METHODS: Various concentrations of melatonin were administered to 5-day post-fertilization zebrafish treated with 125 μM neomycin for 1 h...
November 18, 2016: Drug and Chemical Toxicology
https://www.readbyqxmd.com/read/27836639/insights-into-inner-ear-specific-gene-regulation-epigenetics-and-non-coding-rnas-in-inner-ear-development-and-regeneration
#18
REVIEW
Angelika Doetzlhofer, Karen B Avraham
The vertebrate inner ear houses highly specialized sensory organs, tuned to detect and encode sound, head motion and gravity. Gene expression programs under the control of transcription factors orchestrate the formation and specialization of the non-sensory inner ear labyrinth and its sensory constituents. More recently, epigenetic factors and non-coding RNAs emerged as an additional layer of gene regulation, both in inner ear development and disease. In this review, we provide an overview on how epigenetic modifications and non-coding RNAs, in particular microRNAs (miRNAs), influence gene expression and summarize recent discoveries that highlight their critical role in the proper formation of the inner ear labyrinth and its sensory organs...
November 9, 2016: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/27829082/influence-of-hearing-risk-information-on-the-motivation-and-modification-of-personal-listening-device-use
#19
Yula C Serpanos, Abbey L Berg, Brittany Renne
Purpose: The purpose of this study was (a) to investigate the behaviors, knowledge, and motivators associated with personal listening device (PLD) use and (b) to determine the influence of different types of hearing health risk education information (text with or without visual images) on motivation to modify PLD listening use behaviors in young adults. Method: College-age students (N = 523) completed a paper-and-pencil survey tapping their behaviors, knowledge, and motivation regarding listening to music or media at high volume using PLDs...
November 8, 2016: American Journal of Audiology
https://www.readbyqxmd.com/read/27821621/dopamine-d1a-directly-interacts-with-otoferlin-synaptic-pathway-proteins-ca2-and-phosphorylation-underlie-an-nsf-to-ap2mu1-molecular-switch
#20
Dakshnamurthy Selvakumar, Marian J Drescher, Nathan A Deckard, Neeliyath A Ramakrishnan, Barbara J Morley, Dennis G Drescher
Dopamine receptors regulate exocytosis via protein-protein interactions (PPIs) as well as via adenylyl cyclase transduction pathways. Evidence has been obtained for PPIs in inner ear hair cells coupling D1A to SNARE-related proteins snapin, otoferlin, NSF, and AP2mu1, dependent on [Ca(2+)] and phosphorylation. Specifically, the carboxy terminus of dopamine D1A was found to directly bind t-SNARE-associated protein snapin in teleost and mammalian hair cell models by yeast two-hybrid and pull-down assays, and snapin directly interacts with  hair cell calcium-sensor otoferlin...
November 7, 2016: Biochemical Journal
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