CHAPTER || Hearing & Equilibrium OBJECTIVES Pc U Ree em Lea = Describe the components and functions of the external, middle, and inner ear. m Explain the roles of the tympanic membrane, the auditory ossicles (malleus, incus, and stapes), and scala vestibule in sound transmission. = Describe the way that movements of molecules in the air are converted into impulses generated in hair cells in the cochlea. m Explain how pitch, loudness, and timbre are coded in the auditory pathways. m Describe the components of the auditory pathway from the cochlear hair cells to the cerebral cortex. = Compare the causes of conductive and sensorineural hearing loss and the tests used to distinguish between them. = Define the following terms: tinnitus, presbycusis, and syndromic and nonsyndromic deafness. = Explain how cochlear implants and hearing aids function. im Explain how the receptors in the semicircular canals detect rotational acceleration and how the receptors in the saccule and utricle detect linear acceleration.m List the major sensory inputs that provide the information that is synthesized in the brain into the sense of position in space. m Describe the neural mechanisms for vestibular nystagmus and how nystagmus can be used as a diagnostic indicator of the integrity of the vestibular system. m Describe the cause and clinical signs of the following vestibular disturbances: vertigo, Méniére disease, and motion sickness. INTRODUCTION Our ears not only let us detect sounds, but they also help us maintain balance. Receptors for two sensory modalities (hearing and equilibrium) are housed in the ear. The external ear, the middle ear, and the cochlea of the inner ear are involved with hearing. The semicircular canals, the utricle, and the saccule of the inner ear are involved with equilibrium. Both hearing and equilibrium rely on a very specialized type of receptor called a hair cell. 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. Receptors in the semicircular canals detect rotational acceleration, those in the utricle detect linear acceleration in the horizontal direction, and the ones in the saccule detect linear acceleration in the vertical direction. STRUCTURE & FUNCTION OF THE EAR EXTERNAL & MIDDLE EAR The external ear is composed of the auricle (pina) that captures sound waves, the external auditory meatus (ear canal) through which sound waves travel, and the tympanic membrane (eardrum) that moves in and out in response to sound (Figure 11-1), The tympanic membrane marks the beginning of the middle ear.[-——— Outer ear — -— Middle ear Inner ear Semicircular canals) Auditory ossicles FIGURE 11-1 The structures of the external, middle, and inner portions of the human ear. Sound waves travel from the external ear to the tympanic membrane via the external auditory meatus. The middle ear is an air-filled cavity in the temporal bone; it contains the auditory ossicles. The inner ear is composed of the bony and membranous labyrinths. To make the relationships clear, the cochlea has been turned slightly and the middle ear muscles have been omitted. (Reproduced with permission from Fox SI: Human Physiology. New York, NY: McGraw-Hill; 2008.) The middle ear is an air-filled cavity in the temporal bone that opens via the eustachian (auditory) 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. Figure 11-2 shows the three tiny auditory ossicles or bones of the middle ear: malleus (hammer), incus (anvil), and stapes (stirrup). 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 (stirrup). Its footplate is attached by an annular ligament to the walls of the ovalwindow that marks the beginning of the inner ear. Two small skeletal muscles (tensor tympani and stapedius) are also located in the middle ear. Contraction of the former pulls the manubrium of the malleus medially and decreases the vibrations of the tympanic membrane; contraction of the latter pulls the footplate of the stapes out of the oval window. The functions of the ossicles and muscles are detailed below. xmymtanic ReLlex Pyramis =] stapectus muscle FIGURE 11-2 The medial view of the middle ear containing three auditory ossicles (malleus, incus, and stapes) and two small skeletal muscles (tensor tympani muscle and stapedius). 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. The footplate of the stapes is attached by an annular ligament to the walls of the oval window. Contraction of the tensor tympani muscle pulls the manubrium medially and decreases the vibrations of the tympanic membrane; contraction of the stapedius muscle pulls the footplate of the stapes out of the oval window. (Reproduced with permission from Fox SI: Human Physiology. New York, NY: McGraw-Hill; 2008.)INNER EAR The inner ear (labyrinth) is made up of two parts, one within the other. The bony labyrinth is a series of channels in the petrous portion of the temporal bone and is filled with a fluid called perilymph that has a relatively low concentration of K’, similar to that of plasma or the cerebrospinal fluid. The membranous labyrinth is inside of these bony channels, surrounded by the perilymph; it more or less duplicates the shape of the bony channels and is filled with a K*-rich fluid called endolymph. The labyrinth has three components: the cochlea that contains hair cells (receptors) for hearing, semicircular canals that contain hair cells that respond to head rotation, and the otolith organs that contain hair cells that respond to changes in gravity and head tilt (Figure 11-3).LA w, Leal Vea oe & acceda tater Ve Wet iqeaTtor? Clove Semicircular canals Vestibular labyrinth Cochlea Awy Vort Ac (elatota: Supporting cells Haircells —- Nétves FIGURE 11-3 Th gGSiabranous APTI of the inner ear has three components: semicircular canals, cochlea, and otolith organs. ‘The semicircular canals are sensitive to angular accelerations that deflect the gelatinous cupula and associated hair cells. In the cochlea, hair cells spiral along the basilar membrane within the organ of Corti. Airborne sounds set the eardrum in motion, which is conveyed to the cochlea by bones of the middle ear. This flexes the membrane up and down. Hair cells in the organ of Corti are stimulated by shearing motion. The otolithic organs (saccule and utricle) are sensitive to linear acceleration in vertical and horizontal planes. Hair cells are attached to the otolithic membrane. Information from the cochlear hair cells is carried by thecochlear division of the auditory (VIII cranial) nerve. Information from the hair cells in the semicircular canals and otolith organs is carried by the vestibular divisions of the auditory nerve. The cochlea is a 35-mm-long coiled tube that makes two and three quarter turns. The basilar membrane and Reissner membrane divide it into three chambers or scalae (Figure 11-4). The upper scala vestibuli and lower scala tympani contain perilymph and communicate with each other at the apex of the cochlea via a small opening (helicotrema). At the base of the cochlea, the scala vestibuli ends at the oval window that is closed by the footplate of the stapes. The scala tympani ends at the round dow, 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.Spiral prominence Spiral ganglion s | Spiral ligament Modiolus: Spiral a Basilar trina 3 onane Reticular Outer lamina —_haircells Basilar perforata (rod of Cort) _ membrane la FIGURE 11-4 Schematic of the cochlea and organ of Corti in the membranous labyrinth of the inner ear. Top: The cross section of the cochlea shows the organ of Corti and the three scalae of the cochlea. Bottom: This shows the structure of the organ of Corti as it appears in the basal turn of the cochlea. DC, outer phalangeal cells (Deiters cells) supporting outer hair cells;IPG, inner phalangeal cells supporting inner hair cell. (Reproduced with permission from Pickels JO: An Introduction to the Physiology of Hearing, 2nd ed. Academic Press; 1988.) The spiral-shaped organ of Corti on the basilar membrane extends from the apex to the base of the cochlea. It contains the highly specialized auditory receptors (hair cells) whose processes pierce the tough, membrane-like reticular Jamina that is supported by the pillar cells or rods of Corti (Figure 11-4). 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 sensory 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. Most (90-95%) of these sensory neurons innervate the inner hair cells; only 5~10% innervate the more numerous outer hair cells. By contrast, most of the efferent fibers in the auditory nerve terminate on the outer rather than inner hair cells. The axons of the afferent neurons that innervate the hair cells form the auditory (cochlear) division of the eighth cranial nerve. In the cochlea, gap junctions between the hair cells and the adjacent phalangeal cells prevent endolymph from reaching the base of the cell. However, the basilar membrane is relatively permeable to perilymph in the scala tympani and the tunnel of the organ of Corti. The bases of the hair cells are bathed in perilymph. Because of similar gap junctions, the arrangement is similar for the hair cells in other parts of the inner ear; that is, the processes of the hair cells are bathed in endolymph, whereas their bases are bathed in perilymph. 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. The crista ampullaris (sensory organ of rotation) is located in the expanded end (ampulla) of each of the membranous canals (Figure 11-3). Each crista consists of hair cells and supporting 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 eighth cranial nerve. A pair of otolith organs, the saccule and utricle, are located near the center of the membranous labyrinth. The macula, the sensory epithelium of these organs, are vertically oriented in the saccule and horizontally located in the utricle whenthe head is upright. The maculae contain supporting cells and hair cells, surrounded by an otolithic membrane in which are embedded crystals of calcium carbonate, the otoliths (Figure 11-3). The otoliths or otoconia range from 3 to 19 pum in length. 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 eighth cranial nerve. SENSORY RECEPTORS IN THE EAR: HAIR CELLS The specialized sensory mechanoreceptors in the ear consist of six patches of hair cells in the membranous labyrinth (Figure 11-5). The hair cells in the organ of Corti signal hearing; the hair cells in the utricle signal horizontal acceleration; the hair cells in the saccule signal vertical acceleration; and a patch in each of the three semicircular canals signal rotational acceleration. Each hair cell is embedded in an epithelium made up of supporting cells, with the basal end in close contact with afferent neurons. A hair bundle projects from the apical end. It has one large kinocilium, a true but nonmotile cilium, with nine pairs of microtubules around a core and a central pair of microtubules. The kinocilium is lost from the cochlear hair cells in adults. The other 30-150 processes (stereocilia) are found in all hair cells; they have cores composed of parallel filaments of actin. There is an orderly structure within the clump of processes on each cell. Along an axis toward the kinocilium, the stereocilia increase progressively in height; along the perpendicular axis, all the stereocilia are of the same height.FIGURE 11-5 Structure of hair cell in the saccule. Left: Hair cells in the membranous labyrinth of the ear have a common structure, and each is within an epithelium of supporting cells (SC) surmounted by an otolithic membrane (OM) embedded with crystals of calcium carbonate, the otoliths (OL). Projecting from the apical end are rod-shaped processes, or hair cells (RC), in contact with afferent (A) and efferent (E) nerve fibers. Except in the cochlea, one of these, kinocilium (K), is a true but nonmotile cilium with nine pairs of microtubules around its circumference and a central pair of microtubules. The other processes, stereocilia (S), are found in all hair cells; they have cores of actin filaments coated with 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. (Reproduced with permission from Llinas R, Precht W (eds): Frog Neurobiology. Springer; 1976.) Right: Scanning electron micrograph of processes on a hair cell in the saccule. The otolithic membrane has been removed. The small projections around the hair cell are microvilli on supporting cells. (Used with permission of AJ Hudspeth.)ELECTRICAL RESPONSES IN HAIR CELLS Very fine processes called tip links (Figure 11-6) tie the tip of each stereocilium to the side of its higher neighbor, and mechanically sensitive cation channels are at the junction in the taller process. When the shorter stereocilia are pushed toward the taller ones, the channel open time is increased. K*, the most abundant cation in endolymph, and Ca2* enter via the channel and induce depolarization. A myosin-based molecular motor in the taller neighbor then moves the channel toward the base, releasing tension in the tip link. This causes the channel to close and restores the resting state. Depolarization of hair cells causes them to release a neurotransmitter, probably glutamate, which initiates depolarization of neighboring afferent neurons. Myosin Tip link FIGURE 11-6 Schematic representation of the role of tip links in the responses of hair cells. When a stereocilium is pushed toward a taller stereocilium, the tip link is stretched and opens an ion channel in its taller neighbor. The channel next is moved down the taller stereocilium by a molecular motor, so the tension on the tip link is released. When the hairs return to the resting position, the motor moves back up the stereocilium. The K* that enters hair cells via the mechanically sensitive cation channels is recycled (Figure 11-7). It enters supporting cells and then passes on to other supporting cells by way of gap junctions. In the cochlea, it eventually reaches the stria vascularis and is secreted back into the endolymph, completing the cycle.ew dolyrl iN > Scala vestibulj SL and sv gerbes KS cr 125 eo my formation * A) cochlear nuciei/ Sr —E Vestbular ‘sens Vestibular nuclei: ‘superior, lateral (Deiters), medial, eat ‘oui va om = ‘AUDITORY VESTIBULAR FIGURE 11-12 Simplified diagram of main auditory (left) and vestibular (right) pathways superimposed on a dorsal view of the brainstem. Cerebellum and cerebral cortex have been removed. For the auditory pathway, eighth cranial nerve afferent fibers form the cochlea end in dorsal and ventral cochlear nuclei. From there, most fibers cross the midline and terminate in the contralateral inferior colliculus. From there, fibers project to the medial geniculate body in the thalamus and then to the auditory cortex located on the superior temporal gyrus of the temporal lobe. For the vestibular pathway, the vestibular nerve terminates in the ipsilateral vestibular nucleus. Most fibers from the semicircular canals terminate in the superior and medial divisions of the vestibular nucleus and project to nuclei controlling eye movement. Most fibers from the utricle and saccule terminate in the lateral division, which then projects to the spinal cord. They also terminate on neurons that project to the cerebellum and the reticular formation. The vestibular nuclei also project to the thalamus and from there to the primary somatosensory cortex. The ascending connections to cranial nerve nuclei are concerned with eye movements.The responses of individual second-order neurons in the cochlear nuclei to sound stimuli are 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 an inhibitory process in the brainstem. In the primary auditory cortex, most neurons respond to inputs from both ears, but strips of cells are stimulated by input from the contralateral ear and inhibited by input from the ipsilateral ear. The increasing availability of positron emission tomography (PET) scanning and functional magnetic resonance imaging (fMRI) has greatly improved the level of knowledge about auditory association areas in humans. The auditory pathways in the cortex resemble the visual pathways in that increasingly complex processing of auditory information takes place along them. An interesting observation is that although the auditory areas look very much the same on the two sides of the brain, there is marked hemispheric specialization. fernicke area on the righ , and sound intensity. The auditory pathways are also very plastic, and, like the visual and somatosensory pathways, they are modified by experience. Examples of auditory plasticity in humans include the observation that in individuals who become deaf before language skills are fully developed, sign language activates auditory association areas. Conversely, individuals who become blind early in life are demonstrably better at localizing sound than individuals with normal eyesight. SOUND LOCALIZATION Determination of the direction from which a sound emanates in the horizontalplane depends on 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 on the fact that the sound is louder on the side closest to the source. . which can be as little eurons 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. This fixed period varies from neuron to neuron. Sounds coming from directly in front of the individual differ in quality from those coming from behind because each pinna (the visible portion of the exterior ear) is turned slightly forward. Ref ec RETESET surface change as sounds move up or down, and the change in the sound waves is the primary factor in locating sounds in thelyeiti¢aliplane. Sound localization is markedly disrupted by lesions of the auditory cortex. HEARING LOSS There are two major categories of hearing loss or deafness: sensorineural and conductive (Clinical Box 11-1). Sensorineural hearing loss (deafness) is the most common type of hearing loss; it is usually due to the loss of cochlear hair cells but can be result from damage to the eighth cranial nerve or within central auditory pathways. It often impairs the ability to hear certain pitches while others are unaffected. |. Damage to the hair cells by prolonged exposure to noise is also associated with hearing loss. Other causes include autoimmune disorders, traumatic injuries, acoustic neuromas, tumors of the eighth cranial nerve and cerebellopontine angle, and vascular damage in the medulla. Conductive hearing loss refers to impaired sound transmission in the external or middle ear and impacts all sound frequencies. Among the causes of conduction hearing loss are plugging of the external auditory canals with wax (cerumen) or foreign bodies, otitis externa (inflammation of the outer ear, “swimmer’s ear”) and otitis media (inflammation of the middle ear) causing fluid accumulation or scarring, or perforation of the eardrum. Severe conductivedeafness can result from otosclerosis in which bone is resorbed and replaced with sclerotic bone that grows over the oval window. 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. Conduction and sensorineural deafness can be differentiated by simple tests with a tuning fork. Three of these tests are outlined in Table 11-1, The Rinne test compares sound conduction through air and bone. Bone conduction is the transmission of vibrations of the bones of the skull to the fluid of the inner ear. The Weber and Schwabach tests demonstrate the important masking effect of environmental noise on the auditory threshold. ‘TABLE 11-1 Common tests with a tuning fork to distinguish between sensorineural and conduction hearing loss. Weber Sehwabach Method Bas of vibrating uring frkplsced Bose of vibrating uning ferkplced on Bane conduction of patent compared onvertoxof stl meso proces unt subject nolonger withthe ofheathy subject hears then etna next toe ermal Hears equatyon both sides Hears wibation naar bone ‘conduction over Conduction Sound louder in sessed et \Vivationsinaeaothesidafterbone Bone conduction beter han nonal eafoess(oneea because mashingeect of ‘conduction over (Conduction efectescodesmaskng toenail abeenton rote eens de Servotneval __Soundlouderinnormal cor “sation hearin iter bone Bone conduction were than normal estos (one ee) “onduetlonis eer solong a neve estes part CLINICAL Bi a Hearing Loss Hearing loss is the most common sensory defect in humans. According to the World Health Organization, over 270 million people worldwide have moderate to profound hearing loss, with one-fourth of these cases beginning in childhood. According to the National Institutes of Health, ~15% of Americans between 20 and 69 years of age have high-frequency hearing loss due to exposure to loud sounds or noise at work or in leisure activities (noise- induced hearing loss). Both inner and outer hair cells are damaged by excessive noise, but outer hair cells appear to be more vulnerable. The use ofvarious chemicals (ototoxins) also causes hearing loss. These include some antibiotics the gradual hearing loss associated with aging, affects more than one-third of those over age 75 and is probably due to gradual cumulative loss of hair cells and neurons. In most cases, hearing loss is a multifactorial disorder caused by both genetic and environmental factors. Single-gene mutations can cause hearing loss. This type of hearing loss is a monogenic disorder with an autosomal dominant, autosomal recessive, X-linked, or mitochondrial mode of inheritance. Monogenic forms of deafness can be defined as syndromic (hearing loss. associated with other abnormalities) or nonsyndromic (only hearing loss). About 0.1% of newborns have genetic mutations leading to deafness. Nonsyndromic deafness due to genetic mutations can first appear in adults rather than in children and may account for many of the 16% of all adults who have significant hearing impairment. It is estimated that the products of 100 or more genes are essential for normal hearing, and deafness loci have been identified in all but 5 of the 24 human chromosomes. The most common mutation leading to congenital hearing loss is that of the protein connexin 26. This defect prevents the normal recycling of K* through the sustentacular cells. Mutations in three nonmuscle myosins also cause deafness. These are myosin-VIla, associated with the actin in the hair cell processes; myosin-Ib, which is probably part of the “adaptation motor” that adjusts tension on the tip links; and myosin-VI, which is essential in some way for the formation of normal cilia. Deafness is also associated with mutant forms of a-tectin, one of the major proteins in the tectorial membrane. An example of syndromic deafness is Pendred syndrome, in which a mutant multifunctional anion exchanger causes deafness and goiter. Another example is one form of the Jong QT syndrome in which one of the K* channel proteins, KVLQTI, is mutated. In the stria vascularis, the normal form of this protein is essential for maintaining the high K* concentration in endolymph, and in the heart it helps maintain a normal QT interval. Individuals who are homozygous for mutant KVLQT1 are deaf and predisposed to the ventricular arrhythmias and sudden death that characterize the long QT syndrom: esCochlear implants are used to treat both children and adults with severe hearing loss. The US Food and Drug Administration reported that, as of December 2012, approximately 324,000 cochlear devices have been implanted worldwide and at least 50,000 are implanted every year. They may be used in children as young as 12 months old. These devices consist of a microphone (picks up environmental sounds), a speech processor (selects and arranges these sounds), a transmitter and receiver/stimulator (converts these sounds into electrical impulses), and an electrode array (sends the impulses to the auditory nerve). Although the implant cannot restore normal hearing, it provides a useful representation of environmental sounds to a deaf person. Those with adult-onset deafness who receive cochlear implants can learn to associate the signals it provides with sounds they remember. Children who receive cochlear implants in conjunction with intensive therapy have been able to acquire speech and language skills. Research is also underway to develop cells that can replace the hair cells in the inner ear. For example, researchers at Stanford University were able to generate cells resembling mechanosensitive hair cells from mouse embryonic and pluripotent stem cells. Hearing aids can also be used to treat sensorineural hearing loss in individuals who have significant residual hearing. The microphone component of an analog hearing aid receives sound that converts sound ‘waves (vibrations) to electrical signals; an amplifier then increases the power of the signal and sends it to the ear via a speaker. Digital hearing aids convert sound waves into numerical codes akin to a computer binary code before amplifying them. The code also includes information about pitch or loudness, so it can be programmed to amplify selectively those sound wave frequencies to which the wearer is least sensitive. Injury to inner ear hair cells can induce random electrical impulses that are then relayed to the auditory cortex, leading to an intermittent or steady, high- tus affects about 50 million Americans and can be a symptom of, , excessive exposure to loud noises, ear infections, or otosclerosis. In most cases, its cause is unknown.VESTIBULAR SYSTEM The vestibular system can be divided into the vestibular apparatus and central vestibular nuclei. The vestibular apparatus within the inner ear detects head motion and position and transduces this information to a neural signal (Figure 11-3), 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. CENTRAL VESTIBULAR PATHWAY The cell bodies of the 19,000 neurons supplying the cristae and maculae on each side are located in the vestibular ganglion. Each vestibular nerve terminates in the ipsilateral four-part vestibular nucleus (Figure 11-12) and in the flocculonedular lobe of the cerebellum (not shown in the figure). Fibers from the semicircular canals terminate primarily in the superior and medial divisions of the vestibular nucleus; neurons in this region project mainly to nuclei controlling eye movement (see Chapter 10). Fibers from the utricle and saccule project predominantly to the lateral division (Deiters nucleus) of the vestibular nucleus which then projects to the contralateral spinal cord, The descending vestibular nucleus receives input from the otolith and projects to the cerebellum, reticular formation, and the spinal cord. The ascending connections to cranial nerve nuclei are control eye movements; the vestibular nuclei also ascend to project to thalamocortical neurons. RESPONSES TO ROTATIONAL ACCELERATION . 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 (Figure 11-3), 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. It returns to mid position in 25-30 s. Movement of the cupula in one direction increases the firing rate of single nerve fibers from the crista; movement in the opposite direction inhibits neural activity (Figure 11-13).3 3 2 20 Bop ews 3 60 > 2 40 ») & 3 20 = $ of/——___------ i f Angular 0 10 20 30 40 50 680 Neon Time (s) FIGURE 11-13 Ampullary responses to rotation. Average time course of impulse discharge from the . Movement of the cupula in one Qe (Reproduced with permission from Adrian ED: Discharges from vestibular receptors in the cat. J Physiol 1943; Mar 25; 101(4):389-407.) Rotation causes maximal stimulation of the semicircular canals most nearly in the plane of rotation. Because the canals on one side of the head are a mirror image of those on the other side, the endolymph is displaced toward the ampulla on one side and away from it on the other. The pattern of stimulation reaching the brain varies with the direction as well as the plane of rotation. Linear acceleration probably fails to displace the cupula and therefore does not stimulate the cristae. However, when one part of the labyrinth is destroyed, other parts take over its functions. Clinical Box 11—2 describes the characteristic eye movements that occur during a period of rotation. RESPONSES TO LINEAR ACCELERATION = sean respectively. The otoliths in the surrounding membrane are denser an 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 vestibular nerve. The maculae also discharge in the absence of head movement due to the pull of gravity on the otoliths.The impulses generated from these receptors are partly responsible for labyrinth righting reflexes. The reflex is initiated by tilting of the head that stimulates the otolithic organs; the response is a compensatory contraction of the neck muscles to keep the head level. A vestibulo-ocular reflex stabilizes images on the retina during head movements. Vestibular stimulation during the rotation leads to inhibition of extraocular muscles on one side and activation on the extraocular muscles on the other side. CLINIC. Nystagmus Nystagmus is the characteristic jerky movement of the eye observed at the start and end of a period of rotation. It is actually a reflex that maintains visual fixation on stationary points while the body rotates. When rotation starts, the eyes move slowly in a direction opposite to the direction of rotation, maintaining visual fixation (vestibulo-ocular reflex). When the io anew Nystagmus is frequently horizontal (ie, the eyes move in the horizontal plane), but it can be Went (nee pe seas dina) oa ven de ead iS{ippedyforward). By convention, the direction of eye movement in nystagmus is identified by the direction of the quick component. The direction of the quick component during rotation is the same as that of the rotation, but the postrotatory nystagmus that occurs due to displacement of the cupula when rotation is stopped is in the apposite direction. When msgs sen ares it asin of apaholony Tria Sean ese eee congenital nystagmus that is seen at birth and @¢quired nystagmus that occurs later in life. In these clinical cases, nystagmus can persist for hours at rest. Acquired nys! or after damage to the Tt can also occur as a result of stroke, multiple sclerosis, head injury, and brain tumors. Some drugs (especially antiseizure drugs), alcohol, and sedatives can cause nystagmus. Nystagmus can be used as a diagnostic indicator of the integrity of the vestibular system.vestibular labyrinth. The semicircular canals are stimulated by instilling warm (40°C) or cold (30°C) water into the external auditory meatus. The temperature difference sets up convection currents in the endolymph, with consequent motion of the cupula. In healthy persons, warm water causes nystagmus that bears toward the stimulus, whereas cold water induces nystagmus that bears toward the opposite ear. This test is given the mnemonic aes ae SS Cs e case of a unilateral lesion in the epee yen] ular pathway, nystagmus is reduced or absent on the side of the lesion. To avoid nystagmus, vertigo, and nausea when irrigating the ear canals in the treatment of ear in Busta vues iC HIGHLIGHTS There is no cure for acquired nystagmus, and treatment depends on the cause. Correcting the underlying cause (stopping drug usage, surgical removal of a tumor) is often the treatment of choice. Also, rectus muscle surgery has been used successfully to treat some cases of acquired nystagmus. Short-term correction of nystagmus can result from injections of botulinum toxin (Botox) to paralyze the ocular muscles. Although most of the responses to stimulation of the maculae are reflex in nature, vestibular impulses also reach the cerebral cortex. These impulses may mediate conscious perception of motion and suppl information SPATIAL ORIENTATION Orientation in space depends in part on input from the vestibular receptors, but visual cues are also important. Spatial orientation also uses information from proprioceptors in joint capsules and from cutaneous touch and pressure receptors. These four inputs are synthesized at a cortical level into a continuous picture of the individual’s orientation in space. Clinical Box 11-3 describes some common vestibular disorders.CLINICAL Bi 3 Vestibular ders Vestibular balance disorders are the ninth most common reason for visits to a primary care clinician. It is one of the most common reasons elderly people seek medical advice. Patients often describe balance problems in terms of vertigo, dizziness, lightheadedness, and motion sickness. Neither lightheadedness nor dizziness is necessarily a symptom of vestibular problems, but vertigo is a prominent symptom of a disorder of the inner ear or vestibular system, especially when one labyrinth is inflamed. Benign paroxysmal positional vertigo is the most common vestibular disorder and is characterized by episodes of vertigo that occur with particular changes in body position (eg, turning over in bed and bending over). One possible cause is that otoconia from the utricle separate from the otolith membrane and become lodged in the canal or cupula of the semicircular canal. This causes abnormal deflections when the head changes position relative to gravity. Méniére disease is an abnormality of the inner ear causing vertigo or severe dizziness, tinnitus, fluctuating hearing loss, and the sensation of pressure or pain in the affected ear lasting several hours. Symptoms can occur suddenly and recur daily or very rarely. The hearing loss is initially transient but can become permanent. The pathophysiology may involve an immune reaction. An inflammatory response can increase fluid volume within the membranous labyrinth, causing it to rupture and allowing the endolymph and perilymph to mix together. The worldwide prevalence for Méniére disease is ~12 per 1000 individuals. It is diagnosed most often between the ages of 30 and 60, and it affects both sexes similarly. The nausea, blood pressure changes, sweating, pallor, and vomiting that are the well-known symptoms of motion sickness are produced by excessive vestibular stimulation and occur when conflicting information is fed into the vestibular and other sensory systems. Space motion sickness (ie, the nausea, vomiting, and vertigo experienced by 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 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.THERAPEUTIC HIGHLIGHTS Symptoms of benign paroxysmal positional vertigo often subside over weeks or months, but if treatment is needed, one option is a procedure called canalith repositioning. This consists of simple and slow maneuvers to position your head to move the otoconia from the semicircular canals back into the vestibule that houses the utricle. There is no cure for Méniére disease, but the symptoms can be controlled by reducing the fluid retention through dietary changes (low- salt or salt-free diet, no caffeine, no alcohol) or medications such as diuretics (eg, hydrochlorothiazide). Individuals with Méniére disease often respond to drugs used to alleviate the symptoms of vertigo. Vestibulosuppressants such as the antihistamine meclizine decrease the excitability of the middle ear labyrinth and block conduction in middle ear vestibular-cerebellar pathway. Motion sickness commonly can be prevented with the use of antihistamines or scopolamine, a cholinergic muscarinic receptor antagonist. CHAPTER SUMMARY m The external ear includes the auricle, external auditory meatus, and tympanic membrane; the external ear captures sound waves and directs them toward the middle ear. The middle ear contains three bones (malleus, incus, and stapes), the Eustachian tube, and the tensor tympani and stapedius muscles. The inner ear contains the cochlea with the receptors (hair cells) for hearing and the semicircular canals with hair cell receptors for balance. m The pressure changes produced by sound waves cause the tympanic membrane to move in and out; thus, it functions as a resonator to reproduce the vibrations of the sound source. The auditory ossicles serve as a lever system to convert the vibrations of the tympanic membrane into movements of the stapes against the perilymph-filled scala vestibuli of the cochlea. 1m Sound is the sensation produced when longitudinal vibrations of air molecules strike the tympanic membrane. The hair cells in the organ of Corti signal hearing. The stereocilia provide a mechanism for generating changes in membrane potential proportional to the direction and distance the hair moves. m= Loudness is correlated with the amplitude of a sound wave, pitch with the frequency, and timbre with harmonic vibrations.m The activity within the auditory pathway passes from the eighth cranial nerve afferent fibers to the dorsal and ventral cochlear nuclei to the inferior colliculi to the thalamic medial geniculate body and then to the auditory cortex. m Tinnitus, a high-pitched ringing in the ear, can result from injury to inner ear hair cells. Presbycusis is age-related hearing loss due to the gradual cumulative loss of hair cells. Single-gene mutations can cause hearing loss; these monogenic forms of deafness are defined as either syndromic (hearing loss associated with other abnormalities) or nonsyndromic (only hearing loss). = Sensorineural hearing loss is usually due to loss of cochlear hair cells but can result from damage to the eighth cranial nerve or central auditory pathway. Conductive hearing loss is due to impaired sound transmission in the external or middle ear and impacts all sound frequencies. Conductive and sensorineural deafness can be differentiated by simple tests (eg, Rinne test) with a tuning fork. = Cochlear implants consist of a microphone, a speech processor, a transmitter and receiver/stimulator that converts sounds into electrical impulses, and an electrode array that sends the impulses to the auditory nerve. Hearing aids consist of a microphone, an amplifier and a speaker. Analog hearing aids convert sound waves to electrical signals; digital hearing aids convert sound waves into numerical codes that provide information about pitch or loudness. = Rotational acceleration stimulates the crista in the semicircular canals, displacing the endolymph in a direction opposite to the direction of rotation, deforming the cupula and bending the hair cell. The utricle responds to horizontal acceleration and the saccule to vertical acceleration. Acceleration in any direction displaces the otoliths, distorting the hair cell processes and generating neural activity. m Spatial orientation is dependent on input from vestibular receptors, visual cues, proprioceptors in joint capsules, and cutaneous touch and pressure receptors. = Nystagmus is the characteristic jerky movement of the eye at the start and end of a period of rotation; it is actually a reflex that maintains visual fixation on stationary points while the body rotates (vestibulo-ocular reflex). A test that induces nystagmus (COWS) by instilling warm or cold water into the external auditory meatus can be used to evaluate the integrity of thevestibular system. m Benign paroxysmal positional vertigo is the most common vestibular disorder and is characterized by episodes of vertigo that occur with particular changes in body. Méniére disease is an abnormality of the inner ear that causes vertigo, tinnitus, hearing loss, and sensation of pressure or pain in the affected ear. Symptoms of motion sickness (eg, nausea, sweating, pallor, and vomiting) occur when conflicting information is fed into the vestibular and other sensory systems. MULTIPLE-CHOICE QUESTIONS For all questions, select the single best answer unless otherwise directed. 1. A9-year-old girl complained of ear pain due to an inflammation and build-up of fluids in the middle ear. She was diagnosed with a middle ear infection, acute otitis media of bacterial origin, and she was treated with an antibiotic. The middle ear contains A. hair cells that mediate linear acceleration. B. the membranous labyrinth containing endolymph. C. the bony labyrinth containing perilymph fluid. D. cochlear hair cells that mediate hearing. E. the auditory ossicles and the tensor tympani and stapedius muscles. 2. A 2-year-old girl was diagnosed with a type of sensorineural deafness. After being evaluated by an audiologist, she was determined to be a good candidate for a cochlear implant. A cochlear implant is comprised of A. a microphone, transmitter, and receiver that converts sound into vibrating waves. B. artificial hair cells that are able to replace damaged hair cells in the inner ear. C. a microphone, amplifier, and speaker that can convert sound waves into electrical signals. D. a microphone, speech processor, transmitter, and receiver that converts sound into electrical impulses. E. a microphone, transmitter, and receiver that converts sound into numerical codes that provide information about pitch or loudness. 3. After playing the violin for the Boston Symphony Orchestra for 18 years, a40-year-old man was given the opportunity to follow the dream he had as a child to be a physician like his father, Compared to other students in his medical school class, what distinctive features might be expressed in his auditory system? A. The pitch of his conversational voice will be about 120 Hz compared to his younger male classmates whose voices are likely to have a pitch of about 250 Hz. B. When presented with musical tones, a larger area of his auditory cortex will be activated compared to the area activated in the cortex of his classmates who are not musically inclined. C. As a musician, he will be better able to localize sound than most of his classmates. D. The Wernicke area on the left side of his brain will be more concerned with melody, pitch, and sound. E. He will be able to distinguish about 2000 pitches; in contrast, his younger classmates will be able to distinguish only about 1000 pitches. . A 40-year-old man, employed as a road construction worker for nearly 20 years, went to his clinician to report that he recently began to notice difficulty hearing during normal conversations. A Weber test showed that sound from a vibrating tuning fork was localized to the right ear. A Schwabach test showed that bone conduction was below normal. A Rinne test showed that both air and bone conductions were abnormal, but air conduction lasted longer than bone conduction. The diagnosis was A. sensorial hearing loss in both ears. B. conduction deafness in the right ear. C. sensorial deafness in the right ear. D. conduction deafness in the left ear. E. sensorineural deafness in the left ear. . A medical resident was asked to give a lecture to medical students on how sound is transmitted from the environment through the ear. He would have said the following about the roles of the auditory ossicles in this process. A. The movement of the malleus transmits the vibrations of the manubrium to the incus. B. The movement of the incus allows the sound wave to go through the oval window. C. The movement of the stapes transmits the vibrations of the manubrium tothe incus. D. The movement of the stapes allows the sound wave to go through the round window. E. The movement of the malleus transmits the vibrations of the tympanic membrane to the stapes. . A medical resident was asked to give a lecture to medical students on how the brain synthesizes information to provide the sense of the position of the body in space. He would have said that the following sensory inputs play an important role in spatial orientation. A. vestibular receptors, cochlear receptors, retinal receptors, and cutaneous pressure receptors B. cochlear receptors, retinal receptors, proprioceptors, and cutaneous touch and pressure receptors C. vestibular receptors, visual cues, proprioceptors in joints, and cutaneous touch and pressure receptors D. middle ear receptors, visual cues, [A and IB sensory fibers, and touch receptors E. organ of Corti hair cells, membranous labyrinth hair cells, visual clues, and touch receptors . The components of the auditory pathway are A. sensory fibers in the acoustic branch of the eighth cranial nerve, the lateral cochlear nucleus, the superior colliculus, and the auditory cortex. B. sensory fibers in the auditory branch of the eighth cranial nerve, the lateral cochlear nucleus, the inferior colliculus, lateral geniculate body, and the superior temporal gyrus of the cortex. C. afferent fibers of the eighth cranial nerve, the dorsal and ventral cochlear nuclei, the inferior colliculi, the lateral geniculate body, and the auditory cortex. D. sensory fibers in the cochlear branch of the eighth cranial nerve, the dorsal and ventral cochlear nuclei, the inferior colliculus, the medial geniculate body, and the auditory cortex. E. afferent fibers of the cochlear branch of the eighth cranial nerve, the ventral cochlear nuclei, the superior colliculi, the lateral geniculate body, and the superior temporal gyrus of the cortex. . Ahealthy male medical student volunteered to undergo evaluation of the function of his vestibular system for a class demonstration. The direction ofhis nystagmus is expected to be vertical when he is rotated A. after warm water is put in one of his ears. B. with his head tipped backward. C. after cold water is put in both of his ears. D. with his head tipped sideways. E. with his head tipped forward. 9. A 65-year old man had an acute injury to the utricle of the inner ear, causing problems with balance. In the utricle, tip links in hair cells are involved in A. formation of perilymph. B. depolarization of the stria vascularis. C. movements of the basement membrane. D. perception of sound. E. regulation of distortion-activated ion channels. .0. A 40-year old woman made an appointment with an otolaryngologist due to ringing in her ear that has been interfering with her ability to concentrate. What is a likely diagnosis and a potential cause of this symptom? A. Pendred syndrome due to inflammation of the middle ear hair cells that induce random electrical impulses in the auditory nerve. B. Syndromic hearing due to a single gene mutation. C. Presbycusis that can result from injury to inner ear hair cells that induce random electrical impulses in the eighth cranial nerve. D. Tinnitus that can be caused by injury to inner ear hair cells that induce random electrical impulses in the auditory nerve. E. Tinnitus due to an accumulation of perilymph moving over inner ear hair cells. 1. An MD/PhD candidate was doing research on the generation of changes in the membrane potential of cochlear inner hair cells. What steps are involved in this process? A. When the shorter stereocilia are pushed toward the taller ones, the channel open time is increased, and K* and Ca®* enter via the channel and induce hyperpolarization. B. When the shorter stereocilia are pushed toward the taller ones, the channel open time is increased, and K* and Ca?* enter via the channel and induce depolarization. C. When the taller stereocilia are pushed toward the shorter ones, the channelopen time is increased, and Na* and Ca?* exit via the channel and induce hyperpolarization. D. When the stereocilia move toward the sound source, the channel open time is decreased, and K* and CI exit via the channel and induce depolarization. E. When the stereocilia move toward the sound source, the channel open time is increased, and K* and CI enter via the channel and induce depolarization. . A.45-year-old woman sought medical advice when she experienced sudden onset of vertigo, tinnitus and hearing loss in her left ear, nausea, and vomiting. She was referred to an otolaryngologist to rule out Méniare disease. Which of the following are possible causes of Méniére disease? A. Méniére disease is autosomal dominant genetic disorder that weakens the membranous labyrinth of the inner ear. B. The hair cells of the cochlea are altered to give the sensation of motion even at rest. C. The otoliths dislodge, enter the semicircular canal, and stimulate the hair cells. D. An inflammatory response increases fluid volume within the membranous labyrinth, causing it to rupture and allowing the endolymph and perilymph to intermix. E. The membranous labyrinth on one side has become inflamed.D. The enteric nervous system contains motor neurons within the circular muscle, sensory neurons within the longitudinal muscle, and interneurons within the mucosa that relay sensory information to the central nervous system. E. The enteric nervous system the submucosal plexus comprised exclusively of sensory neurons transmit information about the contents of the gastrointestinal tract via interneurons to the myenteric plexus that is comprised exclusively of motor neurons. A respiratory therapist was giving a lecture on how the body responds to changes in arterial blood gases. As part of her lecture, she explained homeostasis as follows. eS A, Homeostasis prevents blood gases from deviating from normal values for even brief periods of time. B. Homeostasis maintains blood gases in a normal range by activation of chemoreceptors that sense the deviation from normal and then engages a positive feedback system to return blood gases to a normal level. C. Homeostasis maintains blood gases in a normal range by activation of sympathetic and parasympathetic chemoreceptors that then stimulate increased respiratory activity. D. Homeostasis maintains blood gases in a normal range by activation of chemoreceptors that sense the deviation from normal and then engages a negative feedback system to return blood gases to a normal level. eta fo W>-v2 Be) a ($-12 Ac ) g (a-t ha) po (e992 Veltaye Wi L nprnnn (Qo~ 27 KA Me mh & 6 ag