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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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indicate which one is paretic Recording of eye movements during the test, as described below, allows quanti cation of these responses Galvanic stimulation of the labyrinths offers no particular advantage over caloric stimulation Vestibular (labyrinthine) stimulation can also be produced by rotating the patient in a Barany chair or any type of swivel chair The patient s eyes are kept closed or blindfolded or defocused with Frenzel lenses during rotation to avoid the effects of optokinetic nystagmus Electronystagmography (ENG) provides a more re ned method of detecting disordered labyrinthine function because it permits the accurate recording of eye movements without visual xation ENG is usually coupled with caloric stimulation or with modern devices for rotational testing that allow precise control of the velocity, acceleration, and extent of rotation beyond what can be done with the traditional Barany chair
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Meniere Disease and Other Forms of ` Labyrinthine Vertigo
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Labyrinthine disease is the most common cause of true vertigo The classic variety, Meniere disease, is characterized by recurrent ` attacks of vertigo associated with uctuating tinnitus and deafness One or the other of the latter symptoms rarely both may be absent during the initial attacks of vertigo, but invariably they assert themselves as the disease progresses and increase in severity during an acute attack Meniere disease affects the sexes about equally and ` has its onset most frequently in the fth decade of life, although it may begin earlier or later Cases of Meniere disease are usually ` sporadic, but hereditary forms, both autosomal dominant and recessive, have been described (see reviews by Konigsmark) The main pathologic changes consist of an increase in the volume of endolymph and distention of the endolymphatic system (endolymphatic hydrops) It has been speculated that the paroxysmal attacks of vertigo are related to ruptures of the membranous labyrinth and a dumping of potassium-containing endolymph into the perilymph, changes that have a paralyzing effect on vestibular nerve bers and lead to degeneration of the delicate cochlear hair cells (Friedmann) In typical Meniere disease, the attacks of vertigo are charac ` teristically abrupt and last for several minutes to an hour or longer The vertigo is unmistakably whirling or rotational in type and usually so severe that the patient cannot stand or walk Varying degrees of nausea and vomiting, low-pitched tinnitus, a feeling of fullness in the ear, and a diminution in hearing are practically always associated Nystagmus is present during the acute attack; it is horizontal in type, usually with a rotary component and with the slow phase to the side of the affected ear On attempting to touch a target with the eyes closed, there is past pointing as well as a tendency to fall toward the affected ear The patient prefers to lie with the faulty ear uppermost and is disinclined to look toward the normal side, which exaggerates the nystagmus and dizziness As the attack subsides, hearing improves, as does the sensation of fullness in the ear; with further attacks, however, there is a progressive increase of deafness The attacks vary considerably in frequency and severity They may recur several times weekly for many weeks on end, or there may be remissions of several years duration Frequently recurring attacks may give rise to a mild chronic state of disequilibrium and a reluctance to move the head or to turn quickly With milder forms of the disease, the patient may complain more of head discomfort and of dif culty in concentrating than of vertigo and may be considered neurotic
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Symptoms of anxiety are common in patients with Meniere disease, ` particularly in those who suffer frequent severe attacks As indicated earlier, a small proportion of patients with Me niere disease experience sudden, violent falling attacks These ep` isodes have been referred to by the quaint name otolithic catastrophe of Tumarkin, who attributed them, with little evidence, to deformation of the otolithic membrane of the utricle and saccule Patients characteristically describe a sensation of being pushed or knocked to the ground without warning, or there may be a sudden movement or tilt of the environment just before the fall Consciousness is not lost, and vertigo of the usual type and its accompaniments are not part of the falling attack, although some patients become aware of these symptoms after falling The attacks may occur early or late in the course of the disease Typically, several attacks occur over a period of a year or less and remit spontaneously (Baloh et al) An initial attack must be distinguished from other types of drop attacks (see page 329), but the occurrence of the more typical vertiginous attacks of Meniere disease, with deafness and ` tinnitus, clari es the diagnosis The hearing loss in Meniere disease usually precedes the rst ` attack of vertigo, but it may appear later Episodic deafness without vertigo has been called cochlear Meniere syndrome As already men ` tioned, with recurrent attacks, there is a saltatory progressive unilateral hearing loss (in most series only 10 percent of cases involve both ears, but Baloh places the gure closer to 30 percent) Early in the disease, deafness affects mainly the low tones and uctuates in severity; actually tones below 500 Hz are affected early on, and this loss is not evident to the patient Without the uctuations in puretone audiometric thresholds, the diagnosis is uncertain Later the uctuations cease and high tones are affected Speech discrimination is relatively preserved The attacks of vertigo usually cease when the hearing loss is complete, but there may be an interval of months or longer before this occurs Audiometry reveals a sensorineural type of deafness, with air and bone conduction equally depressed Provided that deafness is not complete, loudness recruitment can be demonstrated in the involved ear (see above) Treatment During an acute attack of Meniere disease, rest in ` bed is the most effective treatment, since the patient can usually nd a position in which vertigo is minimal The antihistaminic agents cyclizine (Marezine), meclizine (Bonine, Antivert), or transdermal scopolamine are useful in the more protracted cases Promethazine (Phenergan) is an effective vestibular suppressant, as is trimethobenzamide (Tigan), given in 200-mg suppositories, which also suppresses nausea and vomiting For many years a low-salt diet in combination with ammonium chloride and diuretics have been used in the treatment of Meniere disease, but the value of this ` regimen has never been established The same is true for dehydrating agents such as oral glycerol and the more recently popular calcium channel blockers Mild sedative drugs may help the anxious patient between attacks If the attacks are very frequent and disabling, permanent relief can be obtained by surgical means Destruction of the labyrinth should be considered only in patients with strictly unilateral disease and complete or nearly complete loss of hearing In patients with bilateral disease or signi cant retention of hearing, the vestibular portion of the eighth nerve can be sectioned An endolymphaticsubarachnoid shunt is the operation favored by some surgeons, and selective destruction of the vestibule by a cryogenic probe or transtympanic injection of gentamicin is favored by others Decompression of the eighth cranial nerve, by separating it from an ad-
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