VEMPs in a patient with cerebellar ataxia, neuropathy and vestibular areflexia (CANVAS)

Rust, Heiko; Peters, Nils; Allum, J.H.J.; Wagner, Benjamin; Honegger, F.; Baumann, Thomas

doi:10.1016/j.jns.2017.04.029

Cited by 15 publications

(10 citation statements)

References 9 publications

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“…VEMPs are considered to be only complementary to vHIT, caloric and sinusoidal rotation tests when diagnosing bilateral vestibular failure, however VEMP characteristics may provide clues to the aetiology of BVP. VEMPs may be preserved in cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) (Rust et al, 2017) but are likely to be impaired in bilateral MD and gentamicin toxicity (Agrawal et al, 2013) as well as superficial siderosis (Ushio et al, 2006) (Fig. 14).…”

Section: Clinical Application Of Vempsmentioning

confidence: 99%

Vestibular evoked myogenic potentials in practice: Methods, pitfalls and clinical applications

Rosengren

Colebatch

Young

et al. 2019

Clinical Neurophysiology Practice

195

208

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Section: Clinical Application Of Vempsmentioning

confidence: 99%

Vestibular evoked myogenic potentials in practice: Methods, pitfalls and clinical applications

Rosengren

Colebatch

Young

et al. 2019

Clinical Neurophysiology Practice

195

208

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show abstract

“…Nevertheless, it can be evaluated with cervical and ocular vestibular evoked myogenic potential (cVEMPs and oVEMPs respectively testing the saccule and the utricule). In CANVAS, similarly to BVH, studies suggest a great variability in responses for VEMP testing [45][46][47]. Subtypes are still to be defined.…”

Section: Vestibular Impairementmentioning

confidence: 99%

Update on Cerebellar Ataxia with Neuropathy and Bilateral Vestibular Areflexia Syndrome (CANVAS)

2020

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The syndrome of cerebellar ataxia with neuropathy and bilateral vestibular areflexia (CANVAS) has emerged progressively during the last 30 years. It was first outlined by the neurootology/neurophysiology community in the vestibular areflexic patients, through the description of patients slowly developing late-onset cerebellar ataxia and bilateral vestibulopathy. The characteristic deficit of visuo-vestibulo-ocular reflex (VVOR) due to the impaired slow stabilizing eye movements was put forward and a specific disease subtending this syndrome was suggested. The association to a peripheral sensory axonal neuropathy was described later on, with neuropathological studies demonstrating that both sensory neuropathy and vestibular areflexia were diffuse ganglionopathy. Clinical and electrophysiological criteria of CANVAS were then proposed in 2016. Besides the classical triad, frequent chronic cough, signs of dysautonomia and neurogenic pains were frequently observed. From the beginning of published cohorts, sporadic as well as familial cases were reported, the last suggestive of an autosomal recessive mode of transmission. The genetic disorder was discovered in 2019, under the form of abnormal biallelic expansion in the replication factor C subunit 1 (RFC1) in a population of late-onset ataxia. This pathological expansion was found in 100% of the familial form and 92% of sporadic ones when the triad was complete. But using the genetic criteria, the phenotype of CANVAS seems to expand, for exemple including patients with isolated neuronopathy. We propose here to review the clinical, electrophysiological, anatomical, genetic aspect of CANVAS in light of the recent discovery of the genetic aetiology, and discuss differential diagnosis, neuropathology and physiopathology.

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“…In a later study of 31 patients with CANVAS, only 7 had absent AC cVEMPs, while 17 had impaired caloric responses and all had a bilaterally positive bedside HIT ( 33 ). Finally, a recent case report described a patient with CANVAS with preserved AC cVEMPs and oVEMPs, but absent caloric and rotation responses and absent vHIT responses in all six canal planes ( 34 ).…”

Section: Concordance Of Otolith and Canal Function In Bvp Of Differenmentioning

confidence: 99%

Vestibular-Evoked Myogenic Potentials in Bilateral Vestibulopathy

2018

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Bilateral vestibulopathy (BVP) is a chronic condition in which patients have a reduction or absence of vestibular function in both ears. BVP is characterized by bilateral reduction of horizontal canal responses; however, there is increasing evidence that otolith function can also be affected. Cervical and ocular vestibular-evoked myogenic potentials (cVEMPs/oVEMPs) are relatively new tests of otolith function that can be used to test the saccule and utricle of both ears independently. Studies to date show that cVEMPs and oVEMPs are often small or absent in BVP but are in the normal range in a significant proportion of patients. The variability in otolith function is partly due to the heterogeneous nature of BVP but is also due to false negative and positive responses that occur because of the large range of normal VEMP amplitudes. Due to their variability, VEMPs are not part of the diagnosis of BVP; however, they are helpful complementary tests that can provide information about the extent of disease within the labyrinth. This article is a review of the use of VEMPs in BVP, summarizing the available data on VEMP abnormalities in patients and discussing the limitations of VEMPs in diagnosing bilateral loss of otolith function.

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VEMPs in a patient with cerebellar ataxia, neuropathy and vestibular areflexia (CANVAS)

Cited by 15 publications

References 9 publications

Vestibular evoked myogenic potentials in practice: Methods, pitfalls and clinical applications

Vestibular evoked myogenic potentials in practice: Methods, pitfalls and clinical applications

Update on Cerebellar Ataxia with Neuropathy and Bilateral Vestibular Areflexia Syndrome (CANVAS)

Vestibular-Evoked Myogenic Potentials in Bilateral Vestibulopathy

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