Causative factors and epidemiology of bilateral vestibulopathy in 255 patients

Zingler, V. C.; Cnyrim, C.; Jahn, Klaus; Weintz, Eva; Fernbacher, Julia; Frenzel, Claudia; Brandt, Thomas; Strupp, Michael

doi:10.1002/ana.21105

Cited by 241 publications

(229 citation statements)

References 33 publications

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“…In those cases with both warm-/cold-water caloric irrigation and vHIT available 174 (n=66), these two approaches were compared. Unilateral hypofunction was defined as a canal 175 paresis factor of >25% with a preserved response on the healthy side (Halmagyi et al , 1997), 176 while for bilateral hypofunction on caloric irrigation a nystagmus with a mean peak slow-177 phase velocity of less than 5°/sec on both sides was required (Zingler et al , 2007). 178 MATLAB was used for statistical analysis.…”

Section: Patient Identification and Statistical Analysis 140mentioning

confidence: 99%

Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy

Tarnutzer

Bockisch

Buffone

et al. 2016

Clinical Neurophysiology

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OBJECTIVE: Bilateral vestibular loss (BVL) is often diagnosed with great delay and an underlying cause is only identified in 50-80%. We measured horizontal and vertical semicircular canal function using the video-head-impulse test (vHIT) and hypothesized that specific vHIT-patterns may be linked to certain etiologies. METHODS: We retrospectively analyzed 109 BVL-patients linked to aminoglycoside vestibulotoxicity (n=16), Menière's disease (n=10), infectious inner-ear disorders (n=11), sensorineural hearing-loss (n=11), cerebellar-ataxia-neuropathy-vestibular-areflexia-syndrome (CANVAS, n=5), other causes (n=19) as well as those with unknown origin (n=47). Vestibulo-ocular reflex gains and cumulative saccade amplitudes were measured with vHIT, and the functional integrity of all semicircular canals was rated. RESULTS: Overall, anterior canal hypofunction (n=86/218) was identified significantly (p<0.001) less often than horizontal (n=186/218) and posterior (n=194/218) hypofunction. Preserved anterior canal function was associated with aminoglycoside vestibulotoxicity, Menière's disease and BVL of unknown origin, while no such sparing was found for inner-ear infections, CANVAS and sensorineural hearing loss. CONCLUSIONS: Semicircular canal function in BVL shows disease-specific dissociations, potentially related to reduced vulnerability or superior recovery of the anterior canals. SIGNIFICANCE: In patients with suspected BVL we recommend quantifying vHIT gains and saccade amplitudes for all semicircular canals as the pattern of canal hypofunction may help identifying the underlying disorder.

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Section: Patient Identification and Statistical Analysis 140mentioning

confidence: 99%

Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy

Tarnutzer

Bockisch

Buffone

et al. 2016

Clinical Neurophysiology

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“…That the association of cerebellar ataxia with bilateral vestibular failure was not mere coincidence was recently confirmed. 5 These workers reported progressive ataxia in 54 of 255 patients with bilateral vestibular failure. An unspecified "peripheral polyneuropathy" accompanied the bilateral vestibulopathy in 17% of 54.…”

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confidence: 99%

Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome

Szmulewicz¹,

Waterston²,

Hálmagyi³

et al. 2011

Neurology

120

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Objective: The syndrome of cerebellar ataxia with bilateral vestibulopathy was delineated in 2004. Sensory neuropathy was mentioned in 3 of the 4 patients described. We aimed to characterize and estimate the frequency of neuropathy in this condition, and determine its typical MRI features. Methods:Retrospective review of 18 subjects (including 4 from the original description) who met the criteria for bilateral vestibulopathy with cerebellar ataxia. Results:The reported age at onset range was 39-71 years, and symptom duration was 3-38 years. The syndrome was identified in one sibling pair, suggesting that this may be a late-onset recessive disorder, although the other 16 cases were apparently sporadic. All 18 had sensory neuropathy with absent sensory nerve action potentials, although this was not apparent clinically in 2, and the presence of neuropathy was not a selection criterion. In 5, the loss of pinprick sensation was virtually global, mimicking a neuronopathy. However, findings in the other 11 with clinically manifest neuropathy suggested a length-dependent neuropathy. MRI scans showed cerebellar atrophy in 16, involving anterior and dorsal vermis, and hemispheric crus I, while 2 were normal. The inferior vermis and brainstem were spared.

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“…Average ages of patients with predominantly axonal (66 Ϯ 10 y; n ϭ 18, 8 women) and patients with predominantly demyelinating PNP (65 Ϯ 13 y; n ϭ 19, 4 women) were similar. Average duration of symptoms, as recorded in the medical histories, was 6.7 Ϯ 5.5 years (range [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Abnormal clinical findings on patients with PNP included the following: 1) unilaterally (n ϭ 12) or bilaterally (n ϭ 10) abnormal bedside headimpulse tests; 2) stand and gait ataxia (n ϭ 29); 3) sensory deficits (touch: n ϭ 37; pain: n ϭ 33; temperature; n ϭ 33; position: n ϭ 19; vibration: n ϭ 25); and 4) abnormal motor signs (muscle atrophy: n ϭ 20; muscle weakness: n ϭ 19; hyporeflexia or areflexia: n ϭ 33 patients).…”

Section: Resultsmentioning

confidence: 99%

“…This impression is supported by a recent study in which 22% of patients with bilateral vestibulopathy were also diagnosed with PNP. 18,19 Among the patients with bilateral vestibulopathy and PNP, about one third had additional cerebellar signs, which led the authors to speculate that the combination of vestibular, polyneuropathic, and cerebellar deficits may be due to a common neurodegenerative or autoimmune pathomechanism. The patients with PNP in our study lacked cerebellar signs; nevertheless, the association of vestibular and polyneuropathic deficits was evident.…”

Section: Discussionmentioning

confidence: 99%

Deficient high-acceleration vestibular function in patients with polyneuropathy

Palla¹,

Schmid-Priscoveanu²,

Studer³

et al. 2009

Neurology

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BACKGROUND: Unsteadiness during standing and walking is a frequent complaint of patients with polyneuropathy (PNP). OBJECTIVE: To determine whether balance disorders in patients with PNP may be caused by reduced proprioceptive input from the feet alone or whether impaired vestibular input, resulting from involvement of the vestibular nerve, can be an additional factor. METHODS: A total of 37 patients (mean age 65 years +/-12 SD; 12 women) with electrodiagnostically confirmed PNP (predominantly axonal: 18; predominantly demyelinating: 19) underwent horizontal search-coil head-impulse testing, which assesses the high-acceleration vestibulo-ocular reflex (VOR). RESULTS: Relative to a healthy comparison group, the gains (eye velocity divided by head velocity) of the horizontal VOR were reduced in 27 of 37 patients (unilateral: 13; bilateral: 14). The percentages of patients with unilateral or bilateral VOR deficits were not significantly different between patients with axonal or demyelinating PNP. CONCLUSIONS: Two thirds of patients with axonal or demyelinating polyneuropathy (PNP) showed unilateral (approximately 50%) or bilateral (approximately 50%) gain reductions of the horizontal high-acceleration vestibulo-ocular reflex. This finding suggests that, in many patients with PNP, the neuropathic process includes the vestibular nerve. Such information is highly relevant for subsequent physical therapy, since vestibular exercise improves balance control and reduces disability.

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Causative factors and epidemiology of bilateral vestibulopathy in 255 patients

Cited by 241 publications

References 33 publications

Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy

Disease-specific sparing of the anterior semicircular canals in bilateral vestibulopathy

Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome

Deficient high-acceleration vestibular function in patients with polyneuropathy

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