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In the
inner ear The inner ear (internal ear, auris interna) is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the ...
, stereocilia are the mechanosensing organelles of
hair cells Hair cells are the sensory receptors of both the auditory system and the vestibular system in the ears of all vertebrates, and in the lateral line organ of fishes. Through mechanotransduction, hair cells detect movement in their environment. ...
, which respond to fluid motion in numerous types of animals for various functions, including hearing and balance. They are about 10–50 micrometers in length and share some similar features of
microvilli Microvilli (singular: microvillus) are microscopic cellular membrane protrusions that increase the surface area for diffusion and minimize any increase in volume, and are involved in a wide variety of functions, including absorption, secretion, ce ...
.Caceci, T. VM8054 Veterinary Histology: Male Reproductive System. http://education.vetmed.vt.edu/Curriculum/VM8054/Labs/Lab27/Lab27.htm (accessed 2/16/06). The hair cells turn the fluid pressure and other mechanical stimuli into electric stimuli via the many microvilli that make up stereocilia rods.Alberts, B., Johnson, A., Lewis, J., Raff, M., Roberts, K. and Walter, P. (2002) The Molecular Biology of the Cell. Garland Science Textbooks. Stereocilia exist in the auditory and
vestibular The Vestibular (from pt, vestíbulo, "entrance hall") is a competitive examination and is the primary and widespread entrance system used by Brazilian universities to select the students admitted. The Vestibular usually takes place from Nov ...
systems.


Morphology

Resembling hair-like projections, the stereocilia are arranged in bundles of 30-300. Within the bundles the stereocilia are often lined up in several rows of increasing height, similar to a staircase. At the core of these hair-like stereocilia are rigid cross-linked
actin Actin is a family of globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in muscle fibrils. It is found in essentially all eukaryotic cells, where it may be present at a concentration of ov ...
filaments, which can renew every 48 hours. These actin filaments face their positive ends at the tips of the stereocilia and their negative ends at the base and can be up to 120 micrometres in length. Filamentous structures, called
tip link Tip links are extracellular filaments that connect stereocilia to each other or to the kinocilium in the hair cells of the inner ear.Pickles JO, Comis SD, Osborne MP. 1984.Cross-links between stereocilia in the guinea pig organ of Corti, and their ...
s, connect the tips of stereocilia in adjacent rows in the bundles. The tip links are made up of nearly vertical fine filaments that run upward from the top end of a shorter stereocilia to its taller neighbor. Tip links are analogous to tiny springs, which, when stretched, open
cation An ion () is an atom or molecule with a net electrical charge. The charge of an electron is considered to be negative by convention and this charge is equal and opposite to the charge of a proton, which is considered to be positive by convent ...
selective channels thus allowing ions to flow across the
cell membrane The cell membrane (also known as the plasma membrane (PM) or cytoplasmic membrane, and historically referred to as the plasmalemma) is a biological membrane that separates and protects the interior of all cells from the outside environment ( ...
into the hair cells. They also are involved in the force transmission across the bundle and the maintenance of the hair bundle structure.


Auditory pathway

As acoustic sensors in
mammals Mammals () are a group of vertebrate animals constituting the class Mammalia (), characterized by the presence of mammary glands which in females produce milk for feeding (nursing) their young, a neocortex (a region of the brain), f ...
, stereocilia are lined up in the
organ of Corti The organ of Corti, or spiral organ, is the receptor organ for hearing and is located in the mammalian cochlea. This highly varied strip of epithelial cells allows for transduction of auditory signals into nerve impulses' action potential. Transd ...
within the
cochlea The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus. A core component of the cochlea is the Organ of Corti, the sensory org ...
of the inner ear. 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 The cochlear nerve (also auditory nerve or acoustic nerve) is one of two parts of the vestibulocochlear nerve, a cranial nerve present in amniotes, the other part being the vestibular nerve. The cochlear nerve carries auditory sensory information ...
. Stereocilia are composed of cytoplasm with embedded bundles of cross-linked
actin Actin is a family of globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in muscle fibrils. It is found in essentially all eukaryotic cells, where it may be present at a concentration of ov ...
filaments. The actin filaments anchor to the terminal web and the top of the cell membrane and are arranged in grade of height. As sound waves propagate in the cochlea, the movement of endolymph fluid bends the stereocilia. If the direction of movement is towards the taller stereocilia, tension develops in the tip links, mechanically opening transduction channels near the tips. Cations from the
endolymph Endolymph is the fluid contained in the membranous labyrinth of the inner ear. The major cation in endolymph is potassium, with the values of sodium and potassium concentration in the endolymph being 0.91  mM and 154  mM, respectively. ...
flow into the cell, depolarizing the
hair cell Hair cells are the sensory receptors of both the auditory system and the vestibular system in the ears of all vertebrates, and in the lateral line organ of fishes. Through mechanotransduction, hair cells detect movement in their environment. ...
and triggering the release of neurotransmitters to nearby nerves, which send an electrical signal to the central nervous system.


Vestibular pathway

In the vestibular system, the stereocilia are located in the
otolithic organs An otolith ( grc-gre, ὠτο-, ' ear + , ', a stone), also called statoconium or otoconium or statolith, is a calcium carbonate structure in the saccule or utricle of the inner ear, specifically in the vestibular system of vertebrates. The sa ...
and the
semicircular canals The semicircular canals or semicircular ducts are three semicircular, interconnected tubes located in the innermost part of each ear, the inner ear. The three canals are the horizontal, superior and posterior semicircular canals. Structure The ...
. 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 A kinocilium is a special type of cilium on the apex of hair cells located in the sensory epithelium of the vertebrate inner ear. Anatomy in humans Kinocilia are found on the apical surface of hair cells and are involved in both the morphogenesis ...
. Bending the stereocilia toward the kinocilium depolarizes the cell and results in increased afferent activity. Bending the stereocilia away from the kinocilium hyperpolarizes the cell and results in a decrease in afferent activity. In the semicircular canals, the hair cells are found in the
crista ampullaris The crista ampullaris is the sensory organ of rotation. They are found in the ampullae of each of the semicircular canals of the inner ear, meaning that there are three pairs in total. The function of the crista ampullaris is to sense angular acc ...
, and the stereocilia protrude into the
ampullary cupula The ampullary cupula, or cupula, is a structure in the vestibular system, providing the sense of spatial orientation. The cupula is located within the ampullae of each of the three semicircular canals. Part of the crista ampullaris, the cupula h ...
. Here, the stereocilia are all oriented in the same direction. In the otoliths, the hair cells are topped by small, calcium carbonate crystals called
otoconia An otolith ( grc-gre, ὠτο-, ' ear + , ', a stone), also called statoconium or otoconium or statolith, is a calcium carbonate structure in the saccule or utricle of the inner ear, specifically in the vestibular system of vertebrates. The sa ...
. Unlike the semicircular ducts, the kinocilia of hair cells in the otoliths are not oriented in a consistent direction. The kinocilia point toward (in the utricle) or away from (in the
saccule The saccule is a bed of sensory cells in the inner ear. It translates head movements into neural impulses for the brain to interpret. The saccule detects linear accelerations and head tilts in the vertical plane. When the head moves verticall ...
) a middle line called the striola.


Mechanoelectrical transduction

In the
cochlea The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus. A core component of the cochlea is the Organ of Corti, the sensory org ...
, a shearing movement between the
tectorial membrane The tectoria membrane (TM) is one of two acellular membranes in the cochlea of the inner ear, the other being the basilar membrane (BM). "Tectorial" in anatomy means forming a cover. The TM is located above the spiral limbus and the spiral org ...
and the
basilar membrane The basilar membrane is a stiff structural element within the cochlea of the inner ear which separates two liquid-filled tubes that run along the coil of the cochlea, the scala media and the scala tympani. The basilar membrane moves up and down i ...
deflects the stereocilia, affecting the tension on the tip-link filaments, which then open and close the non-specific ion channels. When tension increases, the flow of ions across the membrane into the hair cell rises as well. 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. The identity of the mechanosensitive channels in the stereocilia is still unknown. The transduction channels associated with stereocilia are thought to lie at the distal ends of the stereocilia. Deflections of the stereocilia in the direction of the tallest stereocilia leads to an increased rate of opening of nonspecific cation channels. This, in turn, causes receptor
depolarization In biology, depolarization or hypopolarization is a change within a cell, during which the cell undergoes a shift in electric charge distribution, resulting in less negative charge inside the cell compared to the outside. Depolarization is e ...
and leads to the excitement of the cochlear nerve afferents that are located at the base of the
hair cell Hair cells are the sensory receptors of both the auditory system and the vestibular system in the ears of all vertebrates, and in the lateral line organ of fishes. Through mechanotransduction, hair cells detect movement in their environment. ...
. Deflections of the stereocilia in the opposite direction toward the shortest stereocilia causes transduction channels to close. In this situation, the hair cells become hyperpolarized and the nerve afferents are not excited. There are two different types of fluid that surround the hair cells of the inner ear. The
endolymph Endolymph is the fluid contained in the membranous labyrinth of the inner ear. The major cation in endolymph is potassium, with the values of sodium and potassium concentration in the endolymph being 0.91  mM and 154  mM, respectively. ...
is the fluid that surrounds the apical surfaces of hair cells.
Potassium Potassium is the chemical element with the symbol K (from Neo-Latin ''kalium'') and atomic number19. Potassium is a silvery-white metal that is soft enough to be cut with a knife with little force. Potassium metal reacts rapidly with atmosphe ...
is the major cation in the endolymph and is thought to be responsible for carrying the receptor currents in the
cochlea The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus. A core component of the cochlea is the Organ of Corti, the sensory org ...
.
Perilymph Perilymph is an extracellular fluid located within the inner ear. It is found within the scala tympani and scala vestibuli of the cochlea. The ionic composition of perilymph is comparable to that of plasma and cerebrospinal fluid. The major cati ...
is found surrounding the sides and the bases of the hair cells. Perilymph is low in
potassium Potassium is the chemical element with the symbol K (from Neo-Latin ''kalium'') and atomic number19. Potassium is a silvery-white metal that is soft enough to be cut with a knife with little force. Potassium metal reacts rapidly with atmosphe ...
and high in
sodium Sodium is a chemical element with the symbol Na (from Latin ''natrium'') and atomic number 11. It is a soft, silvery-white, highly reactive metal. Sodium is an alkali metal, being in group 1 of the periodic table. Its only stable ...
. The different ionic makeups of the surrounding fluid in addition to the
resting potential A relatively static membrane potential which is usually referred to as the ground value for trans-membrane voltage. The relatively static membrane potential of quiescent cells is called the resting membrane potential (or resting voltage), as opp ...
of the hair cell creates a potential difference across the apical membrane of the hair cell, so potassium enters when transduction channels open. An influx of potassium ions depolarizes the cell and causes the release of a
neurotransmitter A neurotransmitter is a signaling molecule secreted by a neuron to affect another cell across a synapse. The cell receiving the signal, any main body part or target cell, may be another neuron, but could also be a gland or muscle cell. Neuro ...
that can initiate nerve impulses in the sensory neurons that synapse on the base of the hair cell.


Destruction of stereocilia

Stereocilia (along with the entirety of the hair cell) in
mammal Mammals () are a group of vertebrate animals constituting the class Mammalia (), characterized by the presence of mammary glands which in females produce milk for feeding (nursing) their young, a neocortex (a region of the brain), fu ...
s can be damaged or destroyed by excessive loud noises, disease, and
toxin A toxin is a naturally occurring organic poison produced by metabolic activities of living cells or organisms. Toxins occur especially as a protein or conjugated protein. The term toxin was first used by organic chemist Ludwig Brieger (1849� ...
s and are not regenerable.
Environmental noise Environmental noise is an accumulation of noise pollution that occurs outside. This noise can be caused by transport, industrial, and recreational activities. Noise is frequently described as 'unwanted sound'. Within this context, environmenta ...
induced
hearing impairment Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spoken ...
is probably the most prevalent noise health effect according to the U.S.
Environmental Protection Agency A biophysical environment is a biotic and abiotic surrounding of an organism or population, and consequently includes the factors that have an influence in their survival, development, and evolution. A biophysical environment can vary in scal ...
. Abnormal structure/organization of a bundle of stereocilia can also cause deafness and in turn create balance problems for an individual. In other vertebrates, if the hair cell is harmed, supporting cells will divide and replace the damaged hair cells.


Genetic studies

The methionine sulfoxide reductase B3 gene (MsrB3), a protein repair enzyme, has been implicated in large scale stereocilia bundle degeneration, as well as many other factors such as
gestational age In obstetrics, gestational age is a measure of the age of a pregnancy which is taken from the beginning of the woman's last menstrual period (LMP), or the corresponding age of the gestation as estimated by a more accurate method if available. ...
and tolerance to cold environments in plants. Although the exact process of pathogenesis is unknown, it seems to be related to apoptotic cell death. A study based on splicing morpholinos to down-regulate MsrB3 expression in
zebrafish The zebrafish (''Danio rerio'') is a freshwater fish belonging to the minnow family (Cyprinidae) of the order Cypriniformes. Native to South Asia, it is a popular aquarium fish, frequently sold under the trade name zebra danio (and thus often ...
showed shorter, thinner, and more crowded
cilia The cilium, plural cilia (), is a membrane-bound organelle found on most types of eukaryotic cell, and certain microorganisms known as ciliates. Cilia are absent in bacteria and archaea. The cilium has the shape of a slender threadlike pro ...
, as well as small, misplaced
otolith An otolith ( grc-gre, ὠτο-, ' ear + , ', a stone), also called statoconium or otoconium or statolith, is a calcium carbonate structure in the saccule or utricle of the inner ear, specifically in the vestibular system of vertebrates. The sa ...
s. Several stereocilia also underwent
apoptosis Apoptosis (from grc, ἀπόπτωσις, apóptōsis, 'falling off') is a form of programmed cell death that occurs in multicellular organisms. Biochemical events lead to characteristic cell changes ( morphology) and death. These changes inclu ...
. Injection with
wild-type The wild type (WT) is the phenotype of the typical form of a species as it occurs in nature. Originally, the wild type was conceptualized as a product of the standard "normal" allele at a locus, in contrast to that produced by a non-standard, "m ...
MsrB3 mRNA rescued auditory deficits, suggesting MsrB3 helps prevent
apoptosis Apoptosis (from grc, ἀπόπτωσις, apóptōsis, 'falling off') is a form of programmed cell death that occurs in multicellular organisms. Biochemical events lead to characteristic cell changes ( morphology) and death. These changes inclu ...
. Another gene, DFNB74, has been observed as a gene involved in
recessive In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and t ...
hearing loss. DFNB74 based hearing loss may be related to
mitochondrial A mitochondrion (; ) is an organelle found in the cells of most Eukaryotes, such as animals, plants and fungi. Mitochondria have a double membrane structure and use aerobic respiration to generate adenosine triphosphate (ATP), which is us ...
dysfunction. DFNB74 and MsrB3 based deafness may be related to one another. Research on these genes is based on families with recessive deafness, and multiple nonrelated families with this deafness have mutations on both DFNB74 and MsrB3. Damaged or abnormal stereocilia that are a result of genetic mutations often cause hearing loss and other complications, and can be passed down to children. In a recent study, researchers studied mice that inherited a mutated hair cell gene called whirlin, which leads to shorter and fatter stereocilia that are organized in additional rows and that often die off after birth. No current therapies or reparative measures exist to replace such defective hair cells in humans. In order to correct this mutation, researchers injected a
gene therapy Gene therapy is a medical field which focuses on the genetic modification of cells to produce a therapeutic effect or the treatment of disease by repairing or reconstructing defective genetic material. The first attempt at modifying human DN ...
containing the corrected gene into the inner ear of mice with the genetic mutation. The therapy restored stereocilia to normal lengths and eliminated the additional rows of stereocilia in newborn whirler mice. Despite the restoration of hair cells, the treated whirler mice exhibited no signs of improved hearing ability following testing after one month and after three months of treatment. Further studies are looking to understand why the restoration of the stereocilia did not improve the hearing ability of the mutated mice.


Current research

Sound above a certain decibel level can cause permanent damage to inner ear stereocilia. New research has shown that the damage can possibly be reversed if we can repair or recreate some of the proteins in the stereocilia. In this study, scientists used
zebrafish The zebrafish (''Danio rerio'') is a freshwater fish belonging to the minnow family (Cyprinidae) of the order Cypriniformes. Native to South Asia, it is a popular aquarium fish, frequently sold under the trade name zebra danio (and thus often ...
to examine the motion of proteins within live ear cells using a
confocal microscope Confocal microscopy, most frequently confocal laser scanning microscopy (CLSM) or laser confocal scanning microscopy (LCSM), is an optical imaging technique for increasing optical resolution and contrast of a micrograph by means of using a sp ...
. This has shown that proteins in stereocilia move quickly, indicating that the movement of the proteins within the hair cells may be a very important factor to maintaining the integrity of the hair bundles in the inner ear. Further research found
myosin Myosins () are a superfamily of motor proteins best known for their roles in muscle contraction and in a wide range of other motility processes in eukaryotes. They are ATP-dependent and responsible for actin-based motility. The first myosin (M ...
and
actin Actin is a family of globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in muscle fibrils. It is found in essentially all eukaryotic cells, where it may be present at a concentration of ov ...
, two proteins that are important for cell movement, move very quickly.
Fascin Fascin is an actin bundling protein. Species and tissue distribution It is a 54-58 kilodalton monomeric actin filament bundling protein originally isolated from sea urchin egg but also found in ''Drosophila'' and vertebrates, including ...
2b, a protein involved in actin cross-linking, moves even faster. Constant movement of proteins within cells, along with replacement and readjustment, helps cells repair damage. The fast movement of these proteins has changed our understanding of stereocilia and indicates that proteins within stereocilia are not immobile and static. Further research hopes to investigate manipulating protein dynamics to restore human hearing function after damage.


References

{{Auditory system Auditory system