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Vestibular paresis by caloric testing, directional preponderance
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266 Ataxia and falling ipsilaterally Ataxia present with eyes open Aphasia, visual eld, hemimotor, hemisensory, and other cerebral abnormalities, seizures No change Usually absent
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Cerebellopontine angle (acoustic neuroma, glomus and other tumors)
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Gaze-paretic, positional, coarser to side of lesion
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Brainstem and cerebellum (infarcts, tumors, viral infections)
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Auditory eighth and seventh cranial nerve abnormalities; abnormal head impulse test to affected side Ipsilateral fth, seventh, ninth, tenth cranial nerves, cerebellar ataxia Increased intracranial pressure (late) Multiple cranial nerves, brainstem tract signs, cerebellar ataxia Coarse horizontal and vertical, gaze-paretic
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Usually normal
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Higher (cerebral) connections
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Normal
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Radiography and CT may be normal or abnormal Vestibular paresis on caloric testing Directional preponderance CT and MRI abnormal Vestibular paresis on caloric testing BAEPs abnormal Increased CSF protein Hyperactive labyrinths or directional preponderance on caloric testing CT, MRI, and BAEPs abnormal in most cases No change in caloric responses CT and EEG may be abnormal
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See text and Chap 14 for description of types of nystagmus
DEAFNESS, DIZZINESS, AND DISORDERS OF EQUILIBRIUM
Finally, mention should be made of a familial vestibulocerebellar syndrome, beginning in childhood or early adult life and characterized by recurrent episodes of vertigo and imbalance Diplopia and dysarthria complicate some attacks, which seem to be precipitated by extreme exertion and emotion Repeated attacks are followed by a mild, persistent ataxia, mainly of the trunk This disorder was rst described by Farmer and Mustian and more recently by Baloh and Winder, who have pointed out that both the episodic vertigo and ataxia are markedly reduced or abolished by the administration of acetazolamide This
process is most likely related to the inherited acetazolamideresponsive ataxic syndrome described in Chap 5 A form that is kinesogenic, ie, brought on by activity, has a similar appearance In summary, the nature of the nystagmus, instability of the eyes during the head impulse test, and the other features of the neurologic examination allow a distinction to be made between central and peripheral cases of vertigo Associated hearing loss favors a vestibular cause of vertigo The features of the various vertiginous syndromes are summarized in Table 15-2
REFERENCES
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and treatment of dizziness J Neurol Neurosurg Psychiatry 68:129, 2000 HAMMEKE TA, MCQUILLEN MP, COHEN BA: Musical hallucinations associated with acquired deafness J Neurol Neurosurg Psychiatry 46:570, 1983 HOTSON JR, BALOH RW: Acute vestibular syndrome N Engl J Med 339: 680, 1998 JAMIESON DRS, MANN C, O REILLY B, THOMAS AM: Ear click in palatal tremor caused by activity of the levator veli palatini Neurology 46:1168, 1996 JANNETTA PJ: Neurovascular decompression in cranial nerve and systemic disease Am J Surg 192:518, 1980 JANNETTA PJ, MOLLER MB, MOLLER AR: Disabling positional vertigo N Engl J Med 310:1700, 1984 KASAI K, ASADA T, YUMOTO M, et al: Evidence for functional abnormality in the right auditory cortex during musical hallucinosis Lancet 354:1703, 1999 KONIGSMARK BW: Hereditary deafness in man N Engl J Med 281:713, 774, 827, 1969 KONIGSMARK BW: Hereditary diseases of the nervous system with hearing loss, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology Vol 22 Amsterdam, North-Holland, 1975, chap 23, pp 499 526 KONIGSMARK BW: Hereditary progressive cochleovestibular atrophies, in
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