• One hallmark embodying the auditory temporal precision is phase-locking, a phenomenon that the timing of spikes fired by single auditory neurons is synchronized and therefore phase-locked to acoustic cycles ( Heil and Peterson, 2017 ). (jneurosci.org)
  • The flow of information through the brain relies on the coordinated activity of thousands or millions of cells, and on ensembles of neurons that are active simultaneously. (amherst.edu)
  • The vibrations from the bones of the middle ear cause the fluid in the cochlea to move, which causes tiny hair cells inside to interact with neurons. (hearingresearch.org)
  • These neurons change the sound waves into electrical signals sent through the semicircular canals to the auditory nerve. (hearingresearch.org)
  • To date, Kirupa's research has broadly focused on neurons within the auditory efferent circuit, which allow the brain to modulate incoming sound signals at the ear. (aro.org)
  • A research team led by Eri Hashino, Ph.D., Ruth C. Holton Professor of Otolaryngology at Indiana University School of Medicine, reported that by using a three-dimensional cell culture method, they were able to coax stem cells to develop into inner-ear sensory epithelia - containing hair cells, supporting cells and neurons - that detect sound, head movements and gravity. (iu.edu)
  • Moreover, neurons like those that normally link the inner-ear cells to the brain had also developed in the cell culture and were connected to the hair cells. (iu.edu)
  • Loss of hair cells and their innervating neurons is the most frequent cause of hearing impairment. (elsevierpure.com)
  • Preliminary work shows that Clrn1 mRNA was expressed as early as embryonic day 16.5 in the auditory and vestibular hair cells and associated ganglionic neurons. (grantome.com)
  • Our laboratories use biophysical, electrophysiological, molecular biological and histological methods to determine fundamental molecular mechanisms by which neurotransmitters are released from primary sensory cells ('hair cells') to excite second order neurons carrying information to the brain. (hopkinsmedicine.org)
  • During week 4 of embryonic development, the human inner ear develops from the auditory placode, a thickening of the ectoderm that gives rise to the bipolar neurons of the cochlear and vestibular ganglions. (medscape.com)
  • gem homozygous receptor mutant HCs display normal cell viability, afferent synaptogenesis, and peripheral innervation, yet exhibit strongly reduced extracellular potentials (∼50% of wild-type potentials). (jneurosci.org)
  • Bending the stereocilia toward the kinocilium depolarizes the cell and results in increased afferent activity. (wikipedia.org)
  • Bending the stereocilia away from the kinocilium hyperpolarizes the cell and results in a decrease in afferent activity. (wikipedia.org)
  • In particular, sound-evoked spikes in auditory afferent fibers in vivo are phase-locked to sound frequencies up to 5 kHz, but it is not clear how hair cells can handle intracellular Ca 2+ changes with such high speed and efficiency. (jneurosci.org)
  • Given that phase-locking can be achieved in auditory afferent fibers for frequencies up to ∼5 kHz ( Taberner and Liberman, 2005 ), exocytosis from hair cells must be tightly controlled with sufficient temporal precision required. (jneurosci.org)
  • Displacement of stereocilia opens ion channels resulting in depolarization and release of transmitter from synapses at the basal end of the cell to terminals of innervating afferent nerves. (washington.edu)
  • And finally, route three gets us in the primary afferent auditory and/or vestibular nerve cells. (iflscience.com)
  • For many years an outstanding question in vestibular physiology was how the transduction current in the type I hair cell was sufficient, in the face of large conductances on at rest, to depolarize it to potentials necessary for conventional synaptic transmission with its unique afferent calyx. (aro.org)
  • In sum, we hypothesize that Clrn1 is necessary for hair cell function and hair cell to afferent communication, and that CLRN1 interacts with MYO7A to mediate its function. (grantome.com)
  • ESPN , a gene in the DFNB36 critical interval at 1p36.3, was a good positional candidate because a mutation of Espn is known to cause deafness and vestibular dysfunction in the jerker mouse. (bmj.com)
  • We invite primary papers, review papers or commentaries that provide insight into hair cell or supporting cell function and dysfunction - dynamic or homeostatic, and from a diversity of approaches and model organisms and hair cell organs. (frontiersin.org)
  • A recessive mutation of ESPN is known to cause hearing loss and vestibular dysfunction in the jerker mouse. (bmj.com)
  • however, a substantial number of treated patients were found to develop irreversible cochlear and vestibular dysfunction. (medscape.com)
  • Atoh1-based gene therapy to ameliorate auditory and vestibular dysfunction has been proposed. (elsevierpure.com)
  • The hair cells turn the fluid pressure and other mechanical stimuli into electric stimuli via the many microvilli that make up stereocilia rods. (wikipedia.org)
  • Hair cells of the inner ear act as mechanosensors, converting mechanical stimuli to electrical signals transmitted to the brain. (washington.edu)
  • Sensory hair cells in the mammalian cochlea convert mechanical stimuli into electrical impulses that subserve audition. (elsevierpure.com)
  • The inner ear contains the cochlea, the semicircular canals, and the vestibular and auditory nerves. (hearingresearch.org)
  • In most cases, the tiny hair cells inside the cochlea experience trauma or injury. (hearingresearch.org)
  • Hair cells (HCs) in the cochlea mainly function in converting the sound mechanical waves into the electric neural signals [ 2 - 4 ] which make it extremely critical for the hearing ability. (hindawi.com)
  • The inner ear contains the semi-circular canals, which are involved in balance and movement (the vestibular sense), and the cochlea. (lumenlearning.com)
  • The cochlea is a fluid-filled, snail-shaped structure that contains the sensory receptor cells (hair cells) of the auditory system ( [link] ). (lumenlearning.com)
  • As the stapes presses into the oval window, the fluid inside the cochlea begins to move, which in turn stimulates hair cells , which are auditory receptor cells of the inner ear embedded in the basilar membrane. (lumenlearning.com)
  • The stereocila of the cochlea started to degenerate on post-natal day 10 and subsequently the hair bundles continued to degenerate. (iiarjournals.org)
  • Here we show that in utero gene transfer of Atoh1 produces functional supernumerary hair cells in the mouse cochlea. (elsevierpure.com)
  • Our results demonstrate that manipulation of cell fate by transcription factor misexpression produces functional sensory cells in the postnatal mammalian cochlea. (elsevierpure.com)
  • The cochlea is filled with a liquid that carries the vibrations to thousands of tiny hair cells. (aussiedeafkids.org.au)
  • The cochlea contains fluid and about 15,000 tiny hair cells that move when the fluid moves with a sound. (aussiedeafkids.org.au)
  • NGS of RNA from inner ear sensory epithelial cells led to the identification of 455 miRNAs in both cochlear and vestibular sensory epithelium, with 30 and 44 miRNAs found in only cochlea or vestibule, respectively. (biomedcentral.com)
  • Within the cochlea is the organ of Corti, which contains about 20,000 specialized cells called hair cells. (msdmanuals.com)
  • In mammals, the anatomy of the inner ear consists of the bony labyrinth, a system of passages making up the following 2 main functional parts: (1) the cochlea, which is dedicated to hearing, and (2) the vestibular system, which is dedicated to balance. (medscape.com)
  • The vestibular wall separates the cochlear duct from the perilymphatic scala vestibuli, a cavity inside the cochlea. (medscape.com)
  • In hearing, stereocilia transform the mechanical energy of sound waves into electrical signals for the hair cells, which ultimately leads to an excitation of the auditory nerve. (wikipedia.org)
  • Proper perception of sounds in the environment requires auditory signals to be encoded with extraordinary temporal precision up to tens of microseconds, but how it originates from the hearing organs in the periphery is poorly understood. (jneurosci.org)
  • SIGNIFICANCE STATEMENT Hair cell synapses can transmit sound-driven signals precisely in the kHz range. (jneurosci.org)
  • Previous attempts to "grow" inner-ear hair cells in standard cell culture systems have worked poorly in part because necessary cues to develop hair bundles - a hallmark of sensory hair cells and a structure critically important for detecting auditory or vestibular signals - are lacking in the flat cell-culture dish. (iu.edu)
  • Plasticity in the auditory and olfactory systems has been well-documented, however, fewer studies have tested for plasticity in the visual system, a surprising detail since courtship and mate choice are largely dependent on visual signals across taxa. (nsf.gov)
  • One branch of this nerve, the auditory nerve, carries sound signals to the brain and another carries balance signals. (msdmanuals.com)
  • Vibrations of the cilia make the hair cells send signals through nerves to the brain. (msdmanuals.com)
  • Such influx of ions causes a depolarization of the cell, resulting in an electrical potential that ultimately leads to a signal for the auditory nerve and the brain. (wikipedia.org)
  • This, in turn, causes receptor depolarization and leads to the excitement of the cochlear nerve afferents that are located at the base of the hair cell. (wikipedia.org)
  • In this situation, the hair cells become hyperpolarized and the nerve afferents are not excited. (wikipedia.org)
  • The auditory nerve sends the electrical signal to the brain to be interpreted into the sounds we hear. (hearingresearch.org)
  • As hair cells become activated, they generate neural impulses that travel along the auditory nerve to the brain. (lumenlearning.com)
  • as these little hair cells move in the fluid, they carry a message to the nerve which is connected to your brain, which turns this signal into what you hear. (aussiedeafkids.org.au)
  • Each hair cell connects to the hearing (auditory) nerve. (aussiedeafkids.org.au)
  • The hair cells initiate nerve impulses that tell the brain which way the head is moving so that appropriate action can be taken to maintain balance. (msdmanuals.com)
  • We conclude that, in addition to fast diffusion mediated by mobile Ca 2+ buffer, multiple Ca 2+ extrusion pumps are required for phase-locking at the auditory hair cell ribbon synapse. (jneurosci.org)
  • Our research studies leverage the strengths of the zebrafish model system by using genetics and in vivo imaging to dissect the molecular and functional requirements underlying hair-cell synapse function and assembly. (amherst.edu)
  • The induced hair cells display stereociliary bundles, attract neuronal processes and express the ribbon synapse marker carboxy-terminal binding protein 2 (refs 12,13). (elsevierpure.com)
  • The purposes of these aims are: (1) To dissect the cell type-autonomous functions of Clrn1, (2) To assess the development of ribbon synapse in Clrn1-/-hair cells, (3) To determine whether CLRN1 is expressed in the bundle and synaptic region of hair cells, and (4) To demonstrate biological interaction between CLRN1 and MYO7A. (grantome.com)
  • Our lab is using dendritic patch clamp recordings to examine mechanisms of synaptic transmission at this first, critical synapse in the auditory pathway. (hopkinsmedicine.org)
  • In the vestibular system, the stereocilia are located in the otolithic organs and the semicircular canals. (wikipedia.org)
  • Although it is not yet known, it will be exciting to see if our data are generalizable to other hair-cell organs and model systems. (amherst.edu)
  • Hair cells and supporting cells of inner ear and lateral line sensory organs evolved from a common cell type to accommodate diverse forms of mechanical input. (frontiersin.org)
  • We seek to highlight contemporary research into the function of sensory hair cells and supporting cells in diverse hair cell organs, with consideration of the major questions and impediments to full understanding of sensory processing in the inner ear. (frontiersin.org)
  • those in the vestibular organs respond to gravity and head movements for perception of balance. (washington.edu)
  • The abnormal vestibular phenotype associated with ESPN mutations will be a useful clinical marker for refining the differential diagnosis of non-syndromic deafness. (bmj.com)
  • It is one of the most common causes of ADNSHL in Mideast countries due to prevalent consanguineous marriage [ 22 , 23 ], with majority associated with prelingual severe-to-profound hearing loss and mutations in exon 2 leading to a milder auditory phenotype [ 23 ]. (hindawi.com)
  • Vestibular function in Clrn1-/- mice mirrored the cochlear phenotype, although it deteriorated more gradually than cochlear function. (grantome.com)
  • Consistently, phase-locking of synaptic vesicle releases from hair cells was also significantly reduced by blocking PMCA, MCU, or SERCA, but not NCX. (jneurosci.org)
  • Here we examine Ca 2+ clearance from hair cell synaptic ribbons in a fully mature preparation at physiological concentration of external Ca 2+ and at physiological temperature. (jneurosci.org)
  • My research focuses on characterizing the biophysics of synaptic transmission between hair cells and primary afferents in the vestibular system. (aro.org)
  • Research in the Glowatzki Lab focuses on the auditory system, with a particular focus on synaptic transmission in the inner ear. (hopkinsmedicine.org)
  • In this study, we combined patch-clamp recording and two-photon Ca 2+ imaging to examine Ca 2+ dynamics in hair cell ribbon synapses in the bullfrog amphibian papilla of both sexes. (jneurosci.org)
  • This allowed us to reveal the Ca 2+ extrusion mechanisms that are required for phase-locking at auditory hair cell ribbon synapses. (jneurosci.org)
  • While the majority of hearing loss is due to damage of sensory hair cells, there is accumulating evidence that in cases of noise-induced hearing loss, the pathology may be due to damage of hair-cell synapses rather than hair cells. (amherst.edu)
  • Recent advances feature the identification of proteins constituting the mechanosensing machinery of hair bundles, how electromechanical amplification arises from prestin, novel mechanisms of signal transmission across specialized synapses and how supporting cells contribute to the homeostasis , maturation and regeneration of hair cell epithelia. (frontiersin.org)
  • Here we show that dopami-nergic efferent fibers innervate the acousticolateralis epithelium of the zebrafish during development but do not directly form synapses with hair cells. (wustl.edu)
  • However, a member of the D1-like receptor family, D1b, tightly localizes to ribbon synapses in inner ear and lateral-line hair cells. (wustl.edu)
  • INDIANAPOLIS - Indiana University scientists have transformed mouse embryonic stem cells into key structures of the inner ear. (iu.edu)
  • The three-dimensional culture allows the cells to self-organize into complex tissues using mechanical cues that are found during embryonic development," Koehler said. (iu.edu)
  • As the auditory placode invaginates towards the embryonic mesoderm, it forms the auditory vesicle or otocysts. (medscape.com)
  • As an example, hair cells express different sets of ion channels that shape the frequency, gain and time course of receptor potentials to optimize sensitivity to sounds, head or body motions. (frontiersin.org)
  • Taste buds are formed by groupings of taste receptor cells with hair-like extensions that protrude into the central pore of the taste bud (Figure 5.21). (vesios.com)
  • Taste molecules bind to receptors on this extension and cause chemical changes within the sensory cell that result in neural impulses being transmitted to the brain via different nerves, depending on where the receptor is located. (vesios.com)
  • Olfactory receptor cells are located in a mucous membrane at the top of the nose. (vesios.com)
  • The vestibular system consists of two fluid-filled sacs called the saccule and the utricle and three fluid-filled tubes called the semicircular canals. (msdmanuals.com)
  • The saccule and utricle are located in the vestibule and contain cells that sense movement of the head from side to side (horizontally), sensing acceleration, or up and down (vertically), sensing gravity. (msdmanuals.com)
  • They contain the sensory hair cells and otoliths of the macula of utricle and of the saccule, respectively, which respond to linear acceleration and the force of gravity. (medscape.com)
  • The utricular division of the auditory vesicle also responds to angular acceleration, as well as the endolymphatic sac and duct that connect the saccule and utricle. (medscape.com)
  • We encourage functional and molecular-genetic studies in the development, mature function, and aging of hair cell epithelia, as well as dysfunctions arising through genetic mutations or ototoxic drug treatment. (frontiersin.org)
  • A comprehensive identification of miRNAs in the sensory epithelia and their gene targets will enable pathways of auditory and vestibular function to be defined. (biomedcentral.com)
  • The results provide further support of the essential regulatory role of miRNAs in inner ear sensory epithelia and in regulating pathways that define development and growth of these cells. (biomedcentral.com)
  • In order to further identify and characterize miRNAs in the mammalian inner ear, we used NGS for the first time to identify miRNAs in cochlear and vestibular sensory epithelia. (biomedcentral.com)
  • In the semicircular canals, the hair cells are found in the crista ampullaris, and the stereocilia protrude into the ampullary cupula. (wikipedia.org)
  • Sensory hair-cells are required to reliably transmit auditory and vestibular information to the brain. (amherst.edu)
  • Figure 5.21 (a) Taste buds are composed of a number of individual taste receptors cells that transmit information to nerves. (vesios.com)
  • Moreover, the hair cells are capable of mechanoelectrical transduction and show basolateral conductances with age-appropriate specializations. (elsevierpure.com)
  • Hair cells of the auditory and vestibular systems transform mechanical input into electrical potentials through the mechanoelectrical transduction process (MET). (bvsalud.org)
  • Via positional cloning, we show that mutations in a zebrafish LTCC encoding gene, cav1.3a , underlie the auditory-vestibular defects of gemini ( gem ) circler mutants. (jneurosci.org)
  • Recently we examined how ensembles of sensory hair cells function in vivo using optical indicators in intact zebrafish. (amherst.edu)
  • Now, genetic analyses, especially in zebrafish and mice, are accelerating our understanding of both canonical hair cell functions and the impact of evolved diversity. (frontiersin.org)
  • Zebrafish lateral line hair cells share many properties with those of the inner ear, and are sensitive to the same types of damaging agents as human cells. (washington.edu)
  • We use the zebrafish system to understand why hair cells die, how they can be protected from damage, and how they can regenerate. (washington.edu)
  • Identification of genetic and chemical modulators of zebrafish mechanosensory hair cell death. (washington.edu)
  • Information from these travel down the vestibular 1 and cochlear 3 nerves to the brain. (aussiedeafkids.org.au)
  • When the hair cells move in response to a sound, they send messages along a complex pathway of nerves to the brain. (aussiedeafkids.org.au)
  • Cations from the endolymph flow into the cell, depolarizing the hair cell and triggering the release of neurotransmitters to nearby nerves, which send an electrical signal to the central nervous system. (wikipedia.org)
  • Hair cells in the vestibular system are slightly different from those in the auditory system, in that vestibular hair cells have one tallest cilium, termed the kinocilium. (wikipedia.org)
  • Notably, the visual system is tightly couples to the auditory system. (aro.org)
  • I began studying the vestibular system during my dissertation research at the Università di Pavia with Professors Ivo Prigioni and GianCarlo Russo. (aro.org)
  • Our auditory system converts pressure waves into meaningful sounds. (lumenlearning.com)
  • This section will provide an overview of the basic anatomy and function of the auditory system. (lumenlearning.com)
  • How does the auditory system differentiate among various pitches? (lumenlearning.com)
  • Like the monocular and binocular cues that provided information about depth, the auditory system uses both monaural (one-eared) and binaural (two-eared) cues to localize sound. (lumenlearning.com)
  • Drug-induced damage to these structures of the auditory and balance system can result in hearing loss, tinnitus , and dysequilibrium or dizziness . (medscape.com)
  • Auditory disturbances such as hearing loss and tinnitus (ringing in the ears) have been reported by people infected with SARS-CoV-2, the pathogen that causes the disease COVID-19. (iflscience.com)
  • We are particularly interested in plastic changes in the brain that compensate for some aspects of altered auditory input, and how those changes relate to central auditory processing deficits, tinnitus, and hyperacusis. (hopkinsmedicine.org)
  • Figure 5.22 Olfactory receptors are the hair-like parts that extend from the olfactory bulb into the mucous membrane of the nasal cavity. (vesios.com)
  • Diverse techniques in molecular biology, immunology, and physiology are utilized to study epithelial cell innate immunity, olfactory loss, and response to viral infection. (hopkinsmedicine.org)
  • Ongoing work explores how epithelial cells of the sinuses and olfactory mucosa participate in the immune response and contribute to chronic inflammation. (hopkinsmedicine.org)
  • Reproductive females also show differences in catecholaminergic innervation of auditory circuitry compared with winter, non-reproductive females as measured by tyrosine hydroxylase (TH), the rate-limiting enzyme in catecholaminergic synthesis. (nsf.gov)
  • Importantly, catecholaminergic input to the inner ear from a dopaminergic-specific forebrain nucleus is decreased in the summer and dopamine inhibits the sensitivity of the inner ear, suggesting that gonadal steroids may alter auditory sensitivity by regulating dopamine innervation. (nsf.gov)
  • In this study, we gonadectomized non-reproductive females, implanted them with estradiol (E2) or testosterone (T), and measured TH immunoreactive (TH-ir) fibers in auditory nuclei where catecholaminergic innervation was previously shown to be seasonally plastic. (nsf.gov)
  • We found that treatment with T, but not E2, reduced TH-ir innervation in the auditory hindbrain. (nsf.gov)
  • The development, cell fate, patterning, and innervation of both the sensory and nonsensory regions of the inner ear are governed by tight regulation involving, among others, transcription factors and microRNAs (miRNAs). (biomedcentral.com)
  • At 2-3 weeks postnatal (P14-21), Clrn1-/- mice showed elevated auditory brainstem response (ABR) thresholds and prolonged peak and interpeak latencies. (grantome.com)
  • The overall goal of the Auditory Brainstem Library is to understand how abnormal auditory input from the ear affects the brainstem, and how the brain in turn affects activity in the ear through efferent feedback loops. (hopkinsmedicine.org)
  • L-type Ca 2+ channels (LTCCs) drive the bulk of voltage-gated Ca 2+ entry in vertebrate inner ear hair cells (HCs) and are essential for mammalian auditory processing. (jneurosci.org)
  • Our analysis of SCA1 transgenic mice revealed that the mutant protein aggregates in the nuclei of cerebellar Purkinje cells and that these aggregates stain positively for ubiquitin, the proteasome, and some chaperones. (bcm.edu)
  • Female plainfin midshipman fish (Porichthys notatus) undergo increased peripheral auditory sensitivity in time for the summer breeding season, improving their ability to detect mates, which is regulated by steroid hormones. (nsf.gov)
  • These T-treatment induced changes in TH-ir fibers mimic the seasonal downregulation of dopamine in the midshipman inner ear and provide evidence that steroid hormone regulation of peripheral auditory sensitivity is mediated, in part, by dopamine. (nsf.gov)
  • Regulation of MET channel sensitivity contributes to the auditory system's precision, wide dynamic range and, potentially, protection from overexcitation. (bvsalud.org)
  • Here, we investigated the role of cyclic adenosine monophosphate (cAMP) in rat outer hair cell MET and found that cAMP up-regulation lowers the sensitivity of the channel in a manner consistent with decreasing gating spring stiffness. (bvsalud.org)
  • These results reveal that cAMP signaling modulates gating spring stiffness to affect auditory sensitivity. (bvsalud.org)
  • To assess modulation of hair-cell activity, we reversibly activated or inhibited D1-like receptors (D1Rs) in lateral-line hair cells. (wustl.edu)
  • In the inner ear, stereocilia are the mechanosensing organelles of hair cells, which respond to fluid motion in numerous types of animals for various functions, including hearing and balance. (wikipedia.org)
  • There are two different types of fluid that surround the hair cells of the inner ear. (wikipedia.org)
  • The endolymph is the fluid that surrounds the apical surfaces of hair cells. (wikipedia.org)
  • These cells have small hairlike projections (cilia) that extend into the fluid. (msdmanuals.com)
  • The canals contain hair cells that respond to this movement of fluid. (msdmanuals.com)
  • Because of properties related to sodium channels on the neuronal membrane that are involved in action potentials, there is a point at which a cell cannot fire any faster (Shamma, 2001). (lumenlearning.com)
  • Therefore, hair cells that are in the base portion would be labeled as high-pitch receptors, while those in the tip of basilar membrane would be labeled as low-pitch receptors (Shamma, 2001). (lumenlearning.com)
  • We further show that SARS-CoV-2 can infect specific human inner ear cell types. (iflscience.com)
  • Additional research is needed to determine how inner-ear cells involved in auditory sensing might be developed, as well as how these processes can be applied to develop human inner-ear cells, the researchers said. (iu.edu)
  • In this study, we used Next-Generation Sequencing (NGS) to identify the most prominent miRNAs in the inner ear and to define miRNA-target pairs that form pathways crucial for the function of the sensory epithelial cells. (biomedcentral.com)
  • This would mean that a given hair cell would fire action potentials related to the frequency of the sound wave. (lumenlearning.com)
  • While this is a very intuitive explanation, we detect such a broad range of frequencies (20-20,000 Hz) that the frequency of action potentials fired by hair cells cannot account for the entire range. (lumenlearning.com)
  • In extracellular recordings from hair cells, we observed that D1R agonist SKF-38393 increased microphonic potentials, whereas D1R antagonist SCH-23390 decreased microphonic potentials. (wustl.edu)
  • Deflection of the mechanosensory hair bundle increases tension in the gating springs that open MET channels. (bvsalud.org)
  • Atonal homologue 1 (encoded by Atoh1, also known as Math1) is a basic helix-loop-helix transcription factor required for hair-cell development, and its misexpression in vitro and in vivo generates hair-cell-like cells. (elsevierpure.com)
  • The team mimicked the early development process with a precisely timed use of several small molecules that prompted the stem cells to differentiate, from one stage to the next, into precursors of the inner ear. (iu.edu)
  • At the core of these hair-like stereocilia are rigid cross-linked actin filaments, which can renew every 48 hours. (wikipedia.org)
  • The actin filaments anchor to the terminal web and the top of the cell membrane and are arranged in grade of height. (wikipedia.org)
  • Hair cells are called such because they have actin-rich protrusions, stereocilia, at their apical end. (washington.edu)
  • Gene targets were identified for each of these miRNAs, including Arhgap12, a GTPase activating protein, for miR-6715-3p, implicating this miRNA in sensory hair cell bundle development, actin reorganization, cell adhesion and inner ear morphogenesis. (biomedcentral.com)
  • The skin along the outer part of the canal has stiff hairs and produces wax (called cerumen). (aussiedeafkids.org.au)
  • Stereocilia exist in the auditory and vestibular systems. (wikipedia.org)
  • Of all ototoxic drugs, the aminoglycosides are the most vestibulotoxic, although they vary greatly in their differential effects on the vestibular and cochlear systems. (medscape.com)
  • How DA influences the activity of the auditory and vestibular systems and its site of action are not well understood. (wustl.edu)
  • Vision is known to impact auditory perception and neural mechanisms in vision and audition are tightly coupled, thus, in order to understand how we hear and how CIs affect auditory perception we must consider the integrative effects across these senses. (aro.org)
  • Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia. (nih.gov)
  • Our laboratory uses genetic, cell biological, and biochemical approaches to explore the pathogenesis of polyglutamine neurodegenerative diseases, the function of Math1 in neurodevelopment, and how MECP2 mutations cause postnatal neurodevelopmental disorders. (bcm.edu)
  • Auditory features of the affected individuals are consistent with that previously reported for recessive mutations in TMC1 and MYO15A . (hindawi.com)
  • Recognizing the importance of protein interactions for mediating SCA1 pathogenesis, and given that SCA1 shares clinical and pathological features with several other inherited ataxias, we proposed that understanding the interactions of Ataxin-1 as they relate to the interactions of proteins implicated in other ataxias might provide better insight about molecular mechanisms leading to Purkinje cell degeneration and ataxia. (bcm.edu)
  • Although congenital, profound hearing loss with vestibular areflexia is accompanied by retinitis pigmentosa (RP) in Usher syndrome type 1, 3 affected individuals from PKSN32 or PKSR5A denied nyctalopia or other symptoms of RP. (bmj.com)
  • Therefore synaptically silent hair cells may be an important reserve that acts to maintain sensory function. (amherst.edu)
  • The motor and tail regions of myosin XV are critical for normal structure and function of auditory and vestibular hair cells. (nih.gov)
  • Our results establish espin as an essential protein for hearing and vestibular function in humans. (bmj.com)
  • Using a tight-binding model and some well-known approaches and methods based on Green's function theory and Landauer formalism, APR-246 cell line we numerically investigate the conductance properties and I-V characteristics of (n,0) zigzag single-walled BCN alloy nanotube in the CNT/BCN/CNT structure, where nanocontacts are considered as (n,0) zigzag single-walled carbon nanotubes. (apoptosisblog.com)
  • Preliminary data from our labs show that Clrn1 is necessary for hair cell function and associated neural activation, and previous studies show that CLRN1 biologically interacts with MYO7A. (grantome.com)
  • The aim of this proposal is to test the hypothesize that Clrn1 is necessary for hair cell function and associated neural activation, and that CLRN1 interacts with MYO7A to mediate its function. (grantome.com)