Hair cell

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For hair cells on the external skin, see Hair follicle. For algal 'hair cells', see Trichocyte (disambiguation).
Neuron: PswiandfSW
Gray931.png
Section through the spiral organ of Corti. Magnified. ("Outer hair cells" labeled near top; "inner hair cells" labeled near center).
Location Cochlea
Function Amplify sound waves and transduce auditory information to the Brain Stem
Morphology Unique (see text)
Presynaptic connections None
Postsynaptic connections Via auditory nerve to vestibulocochlear nerve to inferior colliculus
Gray's p.1057
NeuroLex ID nifext_61

Hair cells are the sensory receptors of both the auditory system and the vestibular system in all vertebrates. In mammals, the auditory hair cells are located within the organ of Corti on a thin basilar membrane in the cochlea of the inner ear. They derive their name from the tufts of stereocilia that protrude from the apical surface of the cell, a structure known as the hair bundle, into the scala media, a fluid-filled tube within the cochlea. Mammalian cochlear hair cells come in two anatomically and functionally distinct types: the outer and inner hair cells. Damage to these hair cells results in decreased hearing sensitivity, i.e. sensorineural hearing loss.

Hair bundles as sound detectors and amplifiers[edit]

Research of the past decades[who?] has shown that outer hair cells do not send neural signals to the brain, but that they mechanically amplify low-level sound that enters the cochlea. The amplification may be powered by movement of their hair bundles, or by an electrically driven motility of their cell bodies. The inner hair cells transform the sound vibrations in the fluids of the cochlea into electrical signals that are then relayed via the auditory nerve to the auditory brainstem and to the auditory cortex.

Results in recent years[by whom?] further indicate that mammals apparently have conserved an evolutionarily earlier type of hair-cell motility. This so-called hair-bundle motility amplifies sound in all non-mammalian land vertebrates. It is affected by the closing mechanism of the mechanical sensory ion channels at the tips of the hair bundles. Thus, the same hair-bundle mechanism that detects sound vibrations also actively "vibrates back" and thereby mechanically amplifies weak incoming sound.

Inner hair cells – from sound to nerve signal[edit]

Section through the organ of corti, showing inner and outer hair cells

The deflection of the hair-cell stereocilia opens mechanically gated ion channels that allow any small, positively charged ions (primarily potassium and calcium) to enter the cell.[1] Unlike many other electrically active cells, the hair cell itself does not fire an action potential. Instead, the influx of positive ions from the endolymph in the scala media depolarizes the cell, resulting in a receptor potential. This receptor potential opens voltage gated calcium channels; calcium ions then enter the cell and trigger the release of neurotransmitters at the basal end of the cell. The neurotransmitters diffuse across the narrow space between the hair cell and a nerve terminal, where they then bind to receptors and thus trigger action potentials in the nerve. In this way, the mechanical sound signal is converted into an electrical nerve signal. Repolarization of hair cells is done in a special manner. The perilymph in the scala tympani has a very low concentration of positive ions. The electrochemical gradient makes the positive ions flow through channels to the perilymph.

Hair cells chronically leak Ca2+. This leakage causes a tonic release of neurotransmitter to the synapses. It is thought that this tonic release is what allows the hair cells to respond so quickly in response to mechanical stimuli. The quickness of the hair cell response may also be due to that fact that it can increase the amount of neurotransmitter release in response to a change as little as 100 μV in membrane potential.[2]

Outer hair cells – acoustical pre-amplifiers[edit]

In mammalian outer hair cells, the receptor potential triggers active vibrations of the cell body. This mechanical response to electrical signals is termed somatic electromotility[3] and drives oscillations in the cell’s length, which occur at the frequency of the incoming sound and provide mechanical feedback amplification. A movie clip showing an isolated outer hair cell moving in response to electrical stimulation can be seen here. Outer hair cells are found only in mammals. While hearing sensitivity of mammals is similar to that of other classes of vertebrates, without functioning outer hair cells, the sensitivity decreases by approximately 50 dB[who?]. Outer hair cells extend the hearing range to about 200 kHz in some marine mammals.[4] They have also improved frequency selectivity (frequency discrimination), which is of particular benefit for humans, because it enabled sophisticated speech and music.

The effect of this system is to non-linearly amplify quiet sounds more than large ones, so that a wide range of sound pressures can be reduced to a much smaller range of hair displacements.[5] This property of amplification is called the cochlear amplifier.

The molecular biology of hair cells has seen considerable progress in recent years, with the identification of the motor protein (prestin) that underlies somatic electromotility in the outer hair cells. Santos-Sacchi et al. have shown that prestin's function is dependent on chloride channel signalling and that it is compromised by the common marine pesticide tributyltin (TBT). Because this class of pollutant bioconcentrates up the food chain, the effect is pronounced in top marine predators such as orcas and toothed whales.[6]

Neural connection[edit]

Neurons of the auditory or vestibulocochlear nerve (the VIIIth cranial nerve) innervate cochlear and vestibular hair cells.[7] The neurotransmitter released by hair cells that stimulates the terminal neurites of peripheral axons of the afferent neurons is thought to be glutamate. At the presynaptic juncture, there is a distinct presynaptic dense body or ribbon. This dense body is surrounded by synaptic vesicles and is thought to aid in the fast release of neurotransmitter.

Nerve fiber innervation is much denser for inner hair cells than for outer hair cells. A single inner hair cell is innervated by numerous nerve fibers, whereas a single nerve fiber innervates many outer hair cells. Inner hair cell nerve fibers are also very heavily myelinated, which is in contrast to the unmyelinated outer hair cell nerve fibers. The region of the basilar membrane supplying the inputs to a particular afferent nerve fibre can be considered to be its receptive field.

Efferent projections from the brain to the cochlea also play a role in the perception of sound. Efferent synapses occur on outer hair cells and on afferent (towards the brain) axons under inner hair cells. The presynaptic terminal bouton is filled with vesicles containing acetylcholine and a neuropeptide called Calcitonin gene-related peptide (CGRP). The effects of these compounds varies, in some hair cells the acetylcholine hyperpolarized the cell, which reduces the sensitivity of the cochlea locally.

Regrowth[edit]

Research on the regrowth of cochlea cells may lead to medical treatments that restore hearing. Unlike fish, birds, and reptiles, post-birth humans and other mammals are normally unable to regrow the cells of the inner ear that convert sound into neural signals when those cells are damaged by loud sounds, age or disease.[8] There is some contradictory information regarding the possibility of hair cell regeneration in mature mammals.[9][10][11]

Researchers are making progress toward gene and stem-cell therapies that may allow the damaged cells to be regenerated.[12] Researchers have identified a mammalian gene that normally acts as a molecular switch to block the regrowth of cochlear hair cells in adults.[13] The Rb1 gene encodes the retinoblastoma protein that performs several physiological functions.[14] Not only do hair cells in a culture dish regenerate when the Rb1 gene is deleted, but mice bred to be missing the gene grow more hair cells than control mice that have the gene. The cell cycle inhibitor p27kip1 has also been shown to allow regrowth of cochlear hair cells in mice following genetic deletion or knock down with siRNA targeting p27.[15][16][17]

Additional images[edit]

Notes[edit]

  1. ^ Müller, U (October 2008). "Cadherins and mechanotransduction by hair cells". Current opinion in cell biology 20 (5): 557–566. doi:10.1016/j.ceb.2008.06.004. PMC 2692626. PMID 18619539. 
  2. ^ Chan DK, Hudspeth AJ (February 2005). "Ca2+ current-driven nonlinear amplification by the mammalian cochlea in vitro". Nature Neuroscience 8 (2): 149–155. doi:10.1038/nn1385. PMC 2151387. PMID 15643426. 
  3. ^ Brownell WE, Bader CR, Bertrand D, de Ribaupierre Y (1985-01-11). "Evoked mechanical responses of isolated cochlear outer hair cells". Science 227 (4683): 194–196. Bibcode:1985Sci...227..194B. doi:10.1126/science.3966153. PMID 3966153. 
  4. ^ Wartzog D, Ketten DR (1999). "Marine Mammal Sensory Systems". In J. Reynolds, S. Rommel. Biology of Marine Mammals. Smithsonian Institution Press. p. 132. 
  5. ^ Hudspeth AJ (2008-08-28). "Making an effort to listen: mechanical amplification in the ear". Neuron 59 (4): 530–45. doi:10.1016/j.neuron.2008.07.012. PMC 2724262. PMID 18760690. 
  6. ^ Santos-Sacchi Joseph, Song Lei, Zheng Jiefu, Nuttall Alfred L (2006-04-12). "Control of mammalian cochlear amplification by chloride anions". Journal of Neuroscience 26 (15): 3992–8. doi:10.1523/JNEUROSCI.4548-05.2006. PMID 16611815. 
  7. ^ "Cranial Nerve VIII. Vestibulocochlear Nerve". Meddean. Retrieved 2008-06-04. 
  8. ^ Edge AS, Chen ZY (2008). "Hair cell regeneration". Current Opinion in Neurobiology 18 (4): 377–82. doi:10.1016/j.conb.2008.10.001. PMID 18929656. 
  9. ^ last1=Forge, A; Li, L; Corwin, JT; Nevill, G (1993). "Ultrastructural evidence for hair cell regeneration in the mammalian inner ear". Science 259 (5101): 1616–9. Bibcode:1993Sci...259.1616F. doi:10.1126/science.8456284. PMID 8456284. 
  10. ^ Lefebvre first1=PP; Malgrange, B; Staecker, H; Moonen, G; Van De Water, TR (1993). "Retinoic acid stimulates regeneration of mammalian auditory hair cells". Science 260 (5108): 692–5. Bibcode:1993Sci...260..692L. doi:10.1126/science.8480180. PMID 8480180. 
  11. ^ Edge AS, Chen ZY (2008). "Hair cell regeneration". Curr Opin Neurobiol 18 (4): 377–82. doi:10.1016/j.conb.2008.10.001. PMID 18929656. 
  12. ^ Lefèbvre P, Malgrange MB, Moonen MG (2008). "Regeneration of hair cells and auditory neurons in the ear". Bull Mem Acad R Med Belg 163 (7–9): 391–6. PMID 19445109. 
  13. ^ Henderson M (2005-01-15). "Gene that may no longer turn a deaf ear to old age". Times Online. 
  14. ^ Raphael Y, Martin DM (2005). "Deafness: Lack of regulation encourages hair cell growth". Gene Therapy 12 (13): 1021–22. doi:10.1038/sj.gt.3302523. 
  15. ^ Löwenheim H, Furness DN, Kil J, Zinn C, Gültig K, Fero ML, Frost D, Gummer AW, Roberts JM, Rubel EW, Hackney CM, Zenner HP (1999-03-30). "Gene disruption of p27(Kip1) allows cell proliferation in the postnatal and adult organ of corti". Proc Natl Acad Sci U S A 96 (7): 4084–8. Bibcode:1999PNAS...96.4084L. doi:10.1073/pnas.96.7.4084. PMC 22424. PMID 10097167.  (primary source)
  16. ^ Ono K, Nakagawa T, Kojima K, Matsumoto M, Kawauchi T, Hoshino M, Ito J (December 2009). "Silencing p27 reverses post-mitotic state of supporting cells in neonatal mouse cochleae". Mol Cell Neurosci 42 (4): 391–8. doi:10.1016/j.mcn.2009.08.011. PMID 19733668.  (primary source)
  17. ^ Mizutari K, Fujioka M, Hosoya M, Bramhall N, et al. (2013). "Notch inhibition induces cochlear hair cell regeneration and recovery of hearing after acoustic trauma". Neuron 77 (1): 58–69. doi:10.1016/j.neuron.2012.10.032. PMC 3573859. PMID 23312516. 

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