HEARING & EQUILIBRIUM

The external ear, the middle ear, and the cochlea of the inner ear are concerned with hearing. The semicircular canals, the utricle, and the saccule of the inner ear are concerned with equilibrium. Receptors in the semicircular canals detect rotational acceleration, receptors in the utricle detect linear acceleration in the horizontal direction, and receptors in the saccule detect linear acceleration in the vertical direction. The receptors for hearing and equilibrium are hair cells, and there are six groups of hair cells in each inner ear: one in each of the three semicircular canals, one in the utricle, one in the saccule, and one in the cochlea. ANATOMIC CONSIDERATIONS External & Middle Ear

The external ear funnels sound waves to the external auditory meatus. From the meatus,
the external auditory canal passes inward to the tympanic membrane (eardrum). The middle ear is an air-filled cavity in the temporal bone that opens via the auditory (eustachian) tube into the nasopharynx and through the nasopharynx to the exterior. The tube is usually closed, but during swallowing, chewing, and yawning it opens, keeping the air pressure on the two sides of the eardrum equalized. The three auditory ossicles, the malleus, incus, and stapes, are located in the middle ear. The manubrium (handle of the malleus) is attached to the back of the tympanic membrane. Its head is attached to the wall of the middle ear, and its short process is attached to the incus, which in turn articulates with the head of the stapes. Foot plate of stapes is attached by an annular ligament to the walls of the oval window. Two small skeletal muscles, the tensor tympani {its Contraction pulls the manubrium of the malleus medially and decreases the vibrations of the tympanic membrane} and the stapedius, {its contraction pulls the footplate of the stapes out of the oval window} Inner Ear The inner ear (labyrinth) is made up of two parts, one within the other. a. The bony labyrinth is a series of channels in the petrous portion of the temporal bone. b. Inside these channels, surrounded by a fluid called perilymph, is the membranous labyrinth. This membranous structure more or less duplicates the shape of the bony channels. It is filled with a fluid called endolymph, and there is no communication between the spaces filled with endolymph and those filled with perilymph. Cochlea The cochlear portion of the labyrinth is a coiled tube which in humans is 35 mm long and makes 23/4 turns. Throughout its length, the basilar membrane and Reissner's membrane divide it into three chambers (scalae) The upper scala vestibuli and the lower scala tympani contain perilymph and communicate with each other at the apex of the cochlea through a small opening called the helicotrema. At the base of the cochlea, the scala vestibuli ends at the oval window, which is closed by the footplate of the stapes.

 The scala tympani ends at the round window, a foramen on the medial wall of the middle ear that is closed by the flexible secondary tympanic membrane. The scala media, the middle cochlear chamber, is continuous with the membranous labyrinth and does not communicate with the other two scalae. It contains endolymph Organ of Corti Located on the basilar membrane is the organ of Corti, the structure that contains the hair cells which are the auditory receptors. This organ extends from the apex to the base of the cochlea and consequently has a spiral shape. The processes of the hair cells pierce the tough, membrane-like reticular lamina that is supported by the rods of Corti The hair cells are arranged in four rows: three rows of outer hair cells lateral to the tunnel formed by the rods of Corti, and one row of inner hair cells medial to the tunnel. There are 20,000 outer hair cells and 3500 inner hair cells in each human cochlea. Covering the rows of hair cells is a thin, viscous, but elastic tectorial membrane in which the tips of the hairs of the outer but not the inner hair cells are embedded. The cell bodies of the afferent neurons that arborize around the bases of the hair cells are located in the spiral ganglion within the modiolus, the bony core around which the cochlea is wound. Ninety to 95% of these afferent neurons innervate the inner hair cells; only 5-10% innervates the more numerous outer hair cells, and each neuron innervates several of these outer cells. By contrast, most of the efferent fibers in the auditory nerve terminate on the outer hair cells rather than on the inner hair cells. The axons of the afferent neurons that innervate the hair cells form the auditory (cochlear) division of the vestibulocochlear acoustic nerve and terminate in the dorsal and ventral cochlear nuclei of the medulla oblongata. The processes of the hair cells are bathed in endolymph, whereas their bases are bathed in perilymph.

Central Auditory Pathways From the cochlear nuclei, auditory impulses pass via a variety of pathways to the inferior colliculi, the centers for auditory reflexes, and via the medial geniculate body in the thalamus to the auditory cortex. Others enter the reticular formation. Information from both ears converges on each superior olive, and at all higher levels most of the neurons respond to inputs from both sides. The primary auditory cortex, Brodmann's area 41, is in the superior portion of the temporal lobe. In humans, it is located in the sylvian fissure and is not normally visible on the surface of the brain. In the primary auditory cortex, most neurons respond to inputs from both ears, but there are also strips of cells that are stimulated by input from the contralateral ear and inhibited by input from the ipsilateral ear. There are several additional auditory receiving areas, just as there are several receiving areas for cutaneous sensation. The auditory association areas adjacent to the primary auditory receiving area are widespread. The olivocochlear bundle is a prominent bundle of efferent fibers in each auditory nerve that arises from both the ipsilateral and the contralateral superior olivary complex and ends primarily around the bases of the outer hair cells of the organ of Corti. Semicircular Canals On each side of the head, the semicircular canals are perpendicular to each other, so that they are oriented in the three planes of space. Inside the bony canals, the membranous canals are suspended in perilymph.

 A receptor structure, the crista ampullaris, is located in the expanded end (ampulla) of each of the membranous canals. Each crista consists of hair cells and sustentacular cells surmounted by a gelatinous partition (cupula) that closes off the ampulla The processes of the hair cells are embedded in the cupula, and the bases of the hair cells are in close contact with the afferent fibers of the vestibular division of the vestibulocochlear nerve. Utricle & Saccule Within each membranous labyrinth, on the floor of the utricle, there is an otolithic organ (macula). Another macula is located on the wall of the saccule in a semivertical position. The maculas contain sustentacular cells and hair cells, surmounted by an otolithic membrane in which are embedded crystals of calcium carbonate, the otoliths. The otoliths, which are also called otoconia or ear dust, range from 3 to 19 um in length in humans and are denser than the endolymph. The processes of the hair cells are embedded in the membrane. The nerve fibers from the hair cells join those from the cristae in the vestibular division of the vestibulocochlear nerve.

Neural Pathways The cell bodies of the 19,000 neurons supplying the cristae and maculas on each side are located in the vestibular ganglion. Each vestibular nerve terminates in the ipsilateral four-part vestibular nucleus and in the flocculonodular lobe of the cerebellum Fibers from the semicircular canals end primarily in the superior and medial divisions of the vestibular nucleus and project mainly to nuclei controlling eye movement. Fibers from the utricle and saccule end predominantly in the lateral division (Deiters' nucleus), which projects to the spinal cord. They also end in the descending nucleus, which projects to the cerebellum and the reticular formation. The vestibular nuclei also project to the thalamus and from there to two parts of the primary somatosensory cortex. HAIR CELLS Structure Each is embedded in an epithelium made up of supporting or sustentacular cells. Except for the outer hair cells in the cochlea, the basal end is in close contact with afferent neurons. Projecting from the apical end is 30-150 rod-shaped processes, or hairs. Except in the cochlea, one of these, the kinocilium, is a true but nonmotile cilium with nine pairs of microtubules around its circumference and a central pair of microtubules It is one of the largest processes and has a clubbed end. The kinocilium is lost in the hair cells of the cochlea in adult mammals. However, the other processes, which are called stereocilia, are present in all hair cells. They have cores composed of parallel filaments of actin. The actin is coated with various isoforms of myosin. Within the clump of processes on each cell, there is an orderly structure. Along an axis toward the kinocilium, the stereocilia increase progressively in height; along the perpendicular axis, all the stereocilia are the same height.

Electrical Responses The membrane potential of the hair cells is about -60 mV. When the stereocilia are pushed toward the kinocilium, the membrane potential is decreased to about -50 mV. When the bundle of processes is pushed in the opposite direction, the cell is hyperpolarized. Displacing the processes in a direction perpendicular to this axis provides no change in membrane potential, and displacing the processes in directions that are intermediate between these two directions produces depolarization or hyperpolarization that is proportionate to the degree to which the direction is toward or away from the kinocilium. Thus, the hair processes provide a mechanism for generating changes in membrane potential proportionate to the direction of displacement.

Genesis of Action Potentials in Afferent Nerve Fibers

Endolymph is formed by the stria vascularis and has a high concentration of K+ and a low concentration of Na+ Cells in the stria vascularis have a high concentration of Na+-K+ ATPase. In addition, it appears that there is a unique electrogenic K pump in the stria vascularis, which accounts for the fact that the scala media is electrically positive by 85 mV relative to the scala vestibuli and scala tympani. Very fine processes called tip links tie the tip of each stereocilium to the side of its higher neighbor, and at the junction there appear to be mechanically sensitive cation channels in the higher process. When the shorter stereocilia are pushed toward the higher, the open time of these channels increases. It is postulated that tension on each of the channels is adjusted by an "adaptation motor" made up of myosin in the higher stereocilium Displacement of the stereocilia in the opposite direction reduces channel open time. K+ enters the hair cell when they are open, producing depolarization. Ca2+ also enters, and a synaptic transmitter is released that depolarizes the afferent neuron or neurons in contact with the hair cell. The K+ that enters hair cells via the mechanically sensitive cation channels is recycled. It enters sustentacular cells and then passes on to other sustentacular cells by way of tight junctions. The K+ that enters hair cells via the mechanically sensitive cation channels is recycled. It enters sustentacular cells and then passes on to other sustentacular cells by way of tight junctions. HEARING Sound Waves Sound is the sensation produced when longitudinal vibrations of the molecules in the external environment, i.e., alternate phases of condensation and rarefaction of the molecules, strike the tympanic membrane. The speed of sound increases with temperature and with altitude Generally speaking, the loudness of a sound is correlated with the amplitude of a sound wave and its pitch with the frequency (number of waves per unit of time). frequency affects loudness, since the auditory threshold is lower at some frequencies than others

 Sound waves that have repeating patterns, even though the individual waves are complex, are perceived as musical sounds; aperiodic nonrepeating vibrations cause a sensation of noise. Most musical sounds are made up of a wave with a primary frequency that determines the pitch of the sound plus a number of harmonic vibrations (overtones) that give the sound its characteristic timbre (quality). The intensity of a sound in bels is the logarithm of the ratio of the intensity of that sound and a standard sound. A decibel (dB) is 0.1 bel. Sound intensity is proportionate to the square of sound pressure. The sound frequencies audible to humans range from about 20 to a maximum of 20,000 cycles per second (cps, Hz). The threshold of the human ear varies with the pitch of the sound the greatest sensitivity being in the 1000- to 4000-Hz range The number of pitches that can be distinguished by an average individual is about 2000, but trained musicians can improve on this figure considerably. Pitch discrimination is best in the 1000- to 3000-Hz range and is poor at high and low pitches.

Masking
It is common knowledge that the presence of one sound decreases an individual's ability to hear other sounds. This phenomenon is known as masking. It is believed to be due to the relative or absolute refractoriness of previously stimulated auditory receptors and nerve fibers to other stimuli. The degree to which a given tone masks other tones is related to its pitch. The masking effect of the background noise in all but the most carefully soundproofed environments raises the auditory threshold by a definite and measurable amount.

Sound Transmission The ear converts sound waves in the external environment into action potentials in the auditory nerves. The waves are transformed by the eardrum and auditory ossicles into movements of the footplate of the stapes. These movements set up waves in the fluid of the inner ear. The action of the waves on the organ of Corti generates action potentials in the nerve fibers.

Functions of the Tympanic Membrane & Ossicles In response to the pressure changes produced by sound waves on its external surface, the tympanic membrane moves in and out. The membrane therefore functions as a resonator that reproduces the vibrations of the sound source. It stops vibrating almost immediately when the sound wave stops; i.e., it is very nearly critically damped. The motions of the tympanic membrane are imparted to the manubrium of the malleus. The malleus rocks on an axis through the junction of its long and short processes, so that the short process transmits the vibrations of the manubrium to the incus. The incus moves in such a way that the vibrations are transmitted to the head of the stapes. Movements of the head of the stapes swing its footplate to and fro like a door hinged at the posterior edge of the oval window. The auditory ossicles thus function as a lever system that converts the resonant vibrations of the tympanic membrane into movements of the stapes against the perilymph-filled scala vestibuli of the cochlea

 This system increases the sound pressure that arrives at the oval window, because the lever action of the malleus and incus multiplies the force 1.3 times and the area of the tympanic membrane is much greater than the area of the footplate of the stapes. There are losses of sound energy as a result of resistance, but it has been calculated that at frequencies below 3000 Hz, 60% of the sound energy incident on the tympanic membrane is transmitted to the fluid in the cochlea. Tympanic Reflex When the middle ear musclesthe tensor tympani and the stapediuscontract, they pull the manubrium of the malleus inward and the footplate of the stapes outward. This decreases sound transmission. Loud sounds initiate a reflex contraction of these muscles generally called the tympanic reflex. Its function is protective, preventing strong sound waves from causing excessive stimulation of the auditory receptors. However, the reaction time for the reflex is 40-160 ms, so it does not protect against brief intense stimulation such as that produced by gunshots.

Bone & Air Conduction

Conduction of sound waves to the fluid of the inner ear via the tympanic membrane and the auditory ossicles, the main pathway for normal hearing, is called ossicular conduction. Sound waves also initiate vibrations of the secondary tympanic membrane that closes the round window. This process, unimportant in normal hearing, is air conduction. A third type of conduction, bone conduction, is the transmission of vibrations of the bones of the skull to the fluid of the inner ear. Considerable bone conduction occurs when tuning forks or other vibrating bodies are applied directly to the skull. This route also plays a role in transmission of extremely loud sounds.

Traveling Waves The movements of the footplate of the stapes set up a series of traveling waves in the perilymph of the scala vestibuli. As the wave moves up the cochlea, its height increases to a maximum and then drops off rapidly. The distance from the stapes to this point of maximum height varies with the frequency of the vibrations initiating the wave. High-pitched sounds generate waves that reach maximum height near the base of the cochlea; low-pitched sounds generate waves that peak near the apex. The bony walls of the scala vestibuli are rigid, but Reissner's membrane is flexible. The basilar membrane is not under tension, and it also is readily depressed into the scala tympani by the peaks of waves in the scala vestibuli. Displacements of the fluid in the scala tympani are dissipated into air at the round window. Therefore, sound produces distortion of the basilar membrane and the site at which this distortion is maximal is determined by the frequency of the sound wave. The tops of the hair cells in the organ of Corti are held rigid by the reticular lamina, and the hairs of the outer hair cells are embedded in the tectorial membrane. When the stapes moves, both membranes move in the same direction, but they are hinged on different axes, so there is a shearing motion that bends the hairs. The hairs of the inner hair cells are not attached to the tectorial membrane, but they are apparently bent by fluid moving between the tectorial membrane and the underlying hair cells.

Functions of the Inner & Outer Hair Cells The inner hair cells are the primary sensory cells that generate action potentials in the auditory nerves, and presumably they are stimulated by the fluid movements noted above. The outer hair cells, on the other hand, are innervated by cholinergic efferent fibers from the superior olivary complexes. These cells are motile, shortening when depolarized and lengthening when hyperpolarized. The cells are hyperpolarized by the acetylcholine secreted by the efferent fibers. They improve hearing by increasing the amplitude and sharpening the peaks of the vibration of the basement membrane, though the process by which they do this is controversial.

Action Potentials in Auditory Nerve Fibers The frequency of the action potentials in single auditory nerve fibers is proportionate to the loudness of the sound stimuli. At low sound intensities, each axon discharges to sounds of only one frequency, and this frequency varies from axon to axon depending upon the part of the cochlea from which the fiber originates. At higher sound intensities, the individual axons discharge to a wider spectrum of sound frequencies particularly to frequencies lower than that at which threshold simulation occurs. The major determinant of the pitch perceived when a sound wave strikes the ear is the place in the organ of Corti that is maximally stimulated. The traveling wave set up by a tone produces peak depression of the basilar membrane, and consequently maximal receptor stimulation, at one point. As noted above, the distance between this point and the stapes is inversely related to the pitch of the sound, low tones producing maximal stimulation at the apex of the cochlea and high tones producing maximal stimulation at the base. The pathways from the various parts of the cochlea to the brain are distinct. An additional factor involved in pitch perception at sound frequencies of less than 2000 Hz may be the pattern of the action potentials in the auditory nerve. When the frequency is low enough, the nerve fibers begin to respond with an impulse to each cycle of a sound wave. The importance of this volley effect, however, is limited; the frequency of the action potentials in a given auditory nerve fiber determines principally the loudness, rather than the pitch, of a sound. Although the pitch of a sound depends primarily on the frequency of the sound wave, loudness also plays a part; low tones (below 500 Hz) seem lower and high tones (above 4000 Hz) seem higher as their loudness increases. Duration also affects pitch to a minor degree. The pitch of a tone cannot be perceived unless it lasts for more than 0.01 s, and with durations between 0.01 and 0.1 s, pitch rises as duration increases. Finally, the pitch of complex sounds that include harmonics of a given frequency is still perceived even when the primary frequency (missing fundamental) is absent.

Auditory Responses of Neurons in the Medulla Oblongata The response of individual second-order neurons in the cochlear nuclei to sound stimuli is like those of the individual auditory nerve fibers. The frequency at which sounds of the lowest intensity evoke a response varies from unit to unit; with increased sound intensities, the band of frequencies to which a response occurs becomes wider. The major difference between the responses of the first- and second-order neurons is the presence of a sharper "cutoff" on the low-frequency side in the medullary neurons. This greater specificity of the second-order neurons is probably due to some sort of inhibitory process in the brain stem, but how it is achieved is not known. Primary Auditory Cortex Impulses ascend from the dorsal and ventral cochlear nuclei through complex paths that are both crossed and uncrossed. In humans, low tones are represented anterolaterally and high tones posteromedially in the auditory cortex. However, it is pitch and not frequency per se that is coded in the auditory cortex, because when a complex sound with a missing fundamental is presented, the part of the cortex that is stimulated is the part corresponding to the perceived pitch. Thus, processing of pure frequencies into pitch must occur at a subcortical level. Other Cortical Areas Concerned With Audition In the case of the auditory system, there seems to be a dorsal-parietal pathway concerned with sound localization (the "where" pathway). On the other hand, the human temporal lobes contain a ventral "what" pathway, including areas where the neurons respond selectively to voices. A portion of the posterior superior temporal gyrus known as the planum temporale is regularly larger in the left than in the right cerebral hemisphere, particularly in right-handed individuals. This area appears to be involved in language-related auditory processing. Other Cortical Areas Concerned With Audition They are organized in two general paths. 1. There seems to be a dorsal-parietal pathway concerned with sound localization (the "where" pathway). The parietal areas are concerned, for example, with sound movement 2. The human temporal lobes contain a ventral "what" pathway, including areas where the neurons respond selectively to voices. Examples of auditory plasticity in humans include the observation that in individuals who become deaf before language skills are fully developed; viewing sign language activates auditory association areas outside the primary auditory cortex. Conversely, individuals who become blind early in life are demonstrably better at localizing sound than individuals with normal eyesight. Musicians provide additional examples of cortical plasticity. In these individuals, there is an increase in the size of the auditory areas activated by musical tones. A portion of the posterior superior temporal gyrus known as the planum temporale is regularly larger in the left than in the right cerebral hemisphere, particularly in right-handed individuals. This area appears to be involved in language-related auditory processing. Planum temporale is even larger than normal on the left side, i.e., the asymmetry is greater, in musicians and others who have perfect pitch.

Sound Localization Determination of the direction from which a sound emanates in the horizontal plane depends upon detecting the difference in time between the arrival of the stimulus in the two ears and the consequent difference in phase of the sound waves on the two sides; it also depends upon the fact that the sound is louder on the side closest to the source. Neurons in the auditory cortex that receive input from both ears respond maximally or minimally when the time of arrival of a stimulus at one ear is delayed by a fixed period relative to the time of arrival at the other ear. The change in the sound waves is the primary factor in locating sounds in the vertical plane. Sound localization is markedly disrupted by lesions of the auditory cortex. Audiometry Auditory acuity is commonly measured with an audiometer. This device presents the subject with pure tones of various frequencies through earphones. At each frequency, the threshold intensity is determined and plotted on a graph as a percentage of normal hearing. This provides an objective measurement of the degree of deafness and a picture of the tonal range most affected. Deafness Clinical deafness may be due to impaired sound transmission in the external or middle ear (conduction deafness) or to damage to the hair cells or neural pathways (nerve deafness). Presbycusis, the gradual hearing loss associated with aging, affects more than one-third of those over 75 and is probably due to gradual cumulative loss of hair cells and neurons. VESTIBULAR FUNCTION Responses to Rotational Acceleration Rotational acceleration in the plane of a given semicircular canal stimulates its crista. The endolymph, because of its inertia, is displaced in a direction opposite to the direction of rotation. The fluid pushes on the cupula, deforming it. This bends the processes of the hair cells. When a constant speed of rotation is reached, the fluid spins at the same rate as the body and the cupula swings back into the upright position. When a constant speed of rotation is reached, the fluid spins at the same rate as the body and the cupula swings back into the upright position. When rotation is stopped, deceleration produces displacement of the endolymph in the direction of the rotation, and the cupula is deformed in a direction opposite to that during acceleration. Movement of the cupula in one direction commonly causes increased impulse traffic in single nerve fibers from its crista, whereas movement in the opposite direction commonly inhibits neural activity Rotation causes maximal stimulation of the semicircular canals most nearly in the plane of rotation. Linear acceleration probably fails to displace the cupula and therefore does not stimulate the cristae. The vestibular nuclei are primarily concerned with maintaining the position of the head in space. The tracts that descend from these nuclei mediate head-on-neck and head-on-body adjustments

Nystagmus The characteristic jerky movement of the eye observed at the start and end of a period of rotation is called nystagmus. It is actually a reflex that maintains visual fixation on stationary points while the body rotates, although it is not initiated by visual impulses and is present in blind individuals. When rotation starts, the eyes move slowly in a direction opposite to the direction of rotation, maintaining visual fixation (vestibulo-ocular reflex, VOR). Responses to Linear Acceleration In mammals, the utricular and saccular maculas respond to linear acceleration. In general, the utricle responds to horizontal acceleration and the saccule to vertical acceleration. The otoliths are denser than the endolymph, and acceleration in any direction causes them to be displaced in the opposite direction, distorting the hair cell processes and generating activity in the nerve fibers. The maculas also discharge tonically in the absence of head movement, because of the pull of gravity on the otoliths. Although most of the responses to stimulation of the maculas are reflex in nature, vestibular impulses also reach the cerebral cortex. These impulses are presumably responsible for conscious perception of motion and supply part of the information. Although most of the responses to stimulation of the maculas are reflex in nature, vestibular impulses also reach the cerebral cortex. These impulses are presumably responsible for conscious perception of motion and supply part of the information necessary for orientation in space. Vertigo is the sensation of rotation in the absence of actual rotation and is a prominent symptom when one labyrinth is inflamed.

Caloric Stimulation The semicircular canals can be stimulated by instilling water that is hotter or colder than body temperature into the external auditory meatus. The temperature difference sets up convection currents in the endolymph, with consequent motion of the cupula. This technique of caloric stimulation, which is sometimes, used diagnostically, causes nystagmus, vertigo, and nausea. Spatial Orientation Orientation in space depends in part upon input from the vestibular receptors, but visual cues are also important. Pertinent information is also supplied by impulses from proprioceptors in joint capsules, which supply data about the relative position of the various parts of the body, and impulses from cutaneous exteroceptors, especially touch and pressure receptors. These four inputs are synthesized at a cortical level into a continuous picture of the individual's orientation in space.

Motion Sickness The nausea, blood pressure changes, sweating, pallor, and vomiting that are the well-known symptoms of motion sickness are produced by excessive vestibular stimulation. They are probably due to reflexes mediated via vestibular connections in the brain stem and the flocculonodular lobe of the cerebellum Scopolamine treats motion sickness

Space motion sickness, The nausea, vomiting, and vertigo that occur in astronauts, develops when they are first exposed to microgravity and often wears off after a few days of space flight. It can then recur with reentry, as the force of gravity increases again. It is believed to be due to mismatches in neural input created by changes in the input from some parts of the vestibular apparatus and other gravity sensors without corresponding changes in the other spatial orientation inputs.