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Disorders of smell

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  • In the event that the hearing aid provides inadequate rehabilitation, cochlear implants may be appropriate. Criteria for implantation include severe to profound hearing loss with word recognition score ≤30% under best aided conditions. Worldwide, 20,000 deaf individuals (including 4000 children) have received cochlear implants. Cochlear implants are neural prostheses that convert sound energy to electrical energy and can be used to stimulate the auditory division of the eighth nerve directly.

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  • Evoked Responses Electrocochleography measures the earliest evoked potentials generated in the cochlea and the auditory nerve. Receptor potentials recorded include the cochlear microphonic, generated by the outer hair cells of the organ of Corti, and the summating potential, generated by the inner hair cells in response to sound. The whole nerve action potential representing the composite firing of the firstorder neurons can also be recorded during electrocochleography.

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  • Approach to the Patient: Disorders of the Sense of Hearing The goal in the evaluation of a patient with auditory complaints is to determine (1) the nature of the hearing impairment (conductive vs. sensorineural vs. mixed), (2) the severity of the impairment (mild, moderate, severe, profound), (3) the anatomy of the impairment (external ear, middle ear, inner ear, or central auditory pathway), and (4) the etiology. The history should elicit characteristics of the hearing loss, including the duration of deafness, unilateral vs. bilateral involvement, nature of onset (sudden vs.

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  • The responses are measured in decibels. An audiogram is a plot of intensity in decibels of hearing threshold versus frequency. A decibel (dB) is equal to 20 times the logarithm of the ratio of the sound pressure required to achieve threshold in the patient to the sound pressure required to achieve threshold in a normal hearing person. Therefore, a change of 6 dB represents doubling of sound pressure, and a change of 20 dB represents a tenfold change in sound pressure.

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  • Presbycusis (age-associated hearing loss) is the most common cause of sensorineural hearing loss in adults. In the early stages, it is characterized by symmetric, gentle to sharply sloping high-frequency hearing loss. With progression, the hearing loss involves all frequencies. More importantly, the hearing impairment is associated with significant loss in clarity. There is a loss of discrimination for phonemes, recruitment (abnormal growth of loudness), and particular difficulty in understanding speech in noisy environments.

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  • Disorders of the Sense of Hearing Hearing loss can result from disorders of the auricle, external auditory canal, middle ear, inner ear, or central auditory pathways (Fig. 30-4). In general, lesions in the auricle, external auditory canal, or middle ear cause conductive hearing losses, whereas lesions in the inner ear or eighth nerve cause sensorineural hearing losses. Figure 30-4 An algorithm for the approach to hearing loss.

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  • The contribution of genetics to presbycusis (see below) is also becoming better understood. In addition to GJB2, several other nonsyndromic genes are associated with hearing loss that progresses with age. Sensitivity to aminoglycoside ototoxicity can be maternally transmitted through a mitochondrial mutation. Susceptibility to noise-induced hearing loss may also be genetically determined. There are 400 syndromic forms of hearing loss.

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  • Ear anatomy. A. Drawing of modified coronal section through external ear and temporal bone, with structures of the middle and inner ear demonstrated. B. High-resolution view of inner ear. Stereocilia of the hair cells of the organ of Corti, which rests on the basilar membrane, are in contact with the tectorial membrane and are deformed by the traveling wave. A point of maximal displacement of the basilar membrane is determined by the frequency of the stimulating tone. High-frequency tones cause maximal displacement of the basilar membrane near the base of the cochlea.

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  • Disorders of the Sense of Taste Disorders of the sense of taste are caused by conditions that interfere with the access of the tastant to the receptor cells in the taste bud (transport loss), injure receptor cells (sensory loss), or damage gustatory afferent nerves and central gustatory pathways (neural loss) (Table 30-2). Transport gustatory losses result from xerostomia due to many causes, including Sjögren's syndrome, radiation therapy, heavy-metal intoxication, and bacterial colonization of the taste pore.

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  • Approach to the Patient: Disorders of the Sense of Taste Patients who complain of loss of taste should be evaluated for both gustatory and olfactory function. Clinical assessment of taste is not as well developed or standardized as that of smell. The first step is to perform suprathreshold whole-mouth taste testing for quality, intensity, and pleasantness perception of four taste qualities: sweet, salty, sour, and bitter. Most commonly used reagents for taste testing are sucrose, citric acid or hydrochloric acid, caffeine or quinine (sulfate or hydrochloride), and sodium chloride.

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  • Definitions Disturbances of the sense of taste may be categorized as total ageusia, total absence of gustatory function or inability to detect the qualities of sweet, salt, bitter, or sour; partial ageusia, ability to detect some but not all of the qualitative gustatory sensations; specific ageusia, inability to detect the taste quality of certain substances; total hypogeusia, decreased sensitivity to all tastants; partial hypogeusia, decreased sensitivity to some tastants; and dysgeusia or phantogeusia, distortion in the perception of a tastant, i.e.

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  • Disorders of the Sense of Smell These are caused by conditions that interfere with the access of the odorant to the olfactory neuroepithelium (transport loss), injure the receptor region (sensory loss), or damage central olfactory pathways (neural loss). Currently no clinical tests exist to differentiate these different types of olfactory losses. Fortunately, the history of the disease provides important clues to the cause.

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  • Approach to the Patient: Disorders of the Sense of Smell Unilateral anosmia is rarely a complaint and is only recognized by testing of smell in each nasal cavity separately. Bilateral anosmia, on the other hand, brings patients to medical attention. Anosmic patients usually complain of a loss of the sense of taste even though their taste thresholds may be within normal limits. In actuality, they are complaining of a loss of flavor detection, which is mainly an olfactory function.

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  • Harrison's Internal Medicine Chapter 30. Disorders of Smell, Taste, and Hearing Smell The sense of smell determines the flavor and palatability of food and drink and serves, along with the trigeminal system, as a monitor of inhaled chemicals, including dangerous substances such as natural gas, smoke, and air pollutants. Olfactory dysfunction affects ~1% of people under age 60 and more than half of the population beyond this age. Definitions Smell is the perception of odor by the nose. Taste is the perception of salty, sweet, sour, or bitter by the tongue.

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