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BACKGROUND: Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) presents an unpredictable and uneven clinical development of cerebellar ataxia, neuropathy, and vestibular areflexia. The aim of this study is to report the variability of vestibular test results in genetically confirmed patients with cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. METHODS: Caloric testing, video head impulse test (vHIT), and rotatory chair testing were performed in 7 patients who presented pathogenic repeat expansions in the replication factor complex unit 1 gene related to cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. RESULTS: Reduced vestibulo-ocular reflex (VOR) gain was observed in 100% of the patients in rotatory chair testing. Three of them had bilateral areflexia in caloric testing while 2 showed unilateral hypofunction and 2 had no alterations in the test. Only 1 patient had bilateral abnormal vHIT with gains under 0.6 in both ears. CONCLUSION: Genetic testing allows an early diagnosis of cerebellar ataxia, neuropathy, and vestibular areflexia syndrome, whereby the vestibular system may be affected to different degrees. Rotatory chair testing has a higher sensitivity for the detection of vestibular hypofunction in these patients. Caloric testing can provide additional information. vHIT might underdiagnose patients with mild-to-moderate vestibulopathy. [ABSTRACT FROM AUTHOR]

Caloric irrigation (CI) is the gold standard to investigate peripheral vestibular dysfunction. The video head impulse test (vHIT) is faster and more accessible and may be useful during acute vertigo stroke risk differentiation. Comparative studies between the two methods are needed. The objective of this study was to compare vestibular function data derived from caloric irrigation with that from vHIT. This study included 80 patients with acute onset vertigo who underwent caloric irrigation and vHIT. CI derived sum of slow phase velocities (SPVs) and unilateral weakness (UW) were compared with vHIT vestibulo-ocular reflex (VOR) gain and gain asymmetry (GA) using correlation analyses. Optimal cut offs for vHIT VOR gain and GA were calculated using Youden indexes. There was a strong positive correlation between the asymmetry measures UW and GA whereas the correlation between the sum of SPVs and VOR gain was weaker. The optimal cut offs to diagnose unilateral vestibular weakness were 0.80 for VOR gain and 28% for GA; with specificities for predicting normal caloric irrigation results of 55% and 93%, respectively. In one third of cases the results from caloric irrigation and vHIT dissociated. The results from vHIT correlated with those from CI, still neither test seem to have the accuracy to replace the other. GA appears as an attractive measure in acute vertigo as the high specificity can be used to identify those with a substantial probability of normal vestibular function in need of more comprehensive work-up for central causes. To diagnose vestibular dysfunction, CI remains gold standard. [ABSTRACT FROM AUTHOR]

Objectives  The aim of this article was to evaluate the relationship between signal intensity of the labyrinth and vestibulocochlear function and morphologic features of vestibular schwannoma (VS). Design  Cross-sectional Study. Setting  Tertiary referral center. Participants  Fifty-four patients with sporadic, untreated VS. Main Outcome Measure  Signal intensity of the cochlea and vestibule (SIRc and SIRv: signal intensity of cochlea/vestibule compared with cerebellar signal intensity; AURc and AURv: SIRc/SIRv of the affected side compared with the unaffected side) in 1.5T T2-weighted images was correlated with size (Hannover classification), cystic status, distance from the fundus of the internal auditory canal, video head impulse test (vHIT), and audiometry. Results  Signal intensity of the vestibule was higher than that of the cochlea (p  < 0.01). Large tumors had lower SIRc than smaller tumors (p  = 0.03); Hannover T1 tumors had higher SIRc (p  < 0.01), SIRv (p  < 0.01), AURc (p  < 0.01) and AURv (p  < 0.01) than the rest; heterogenous and cystic tumors had higher SIRv than solid large tumors (p  = 0.02); superior vestibular nerve pattern on vHIT had higher SIRv and AURv than inferior vestibular nerve and mixed patterns (p  = 0.03 and 0.004, respectively); and there was a weak correlation between AURv and speech discrimination (r  = 0.33, p  = 0.04). Conclusion  A more abnormal signal intensity of the labyrinth is associated with larger size and solid status of VS. There was a positive relationship between signal intensity of the labyrinth and speech discrimination scores on audiogram. [ABSTRACT FROM AUTHOR]

Background: To investigate the association between disease duration and the severity of bilateral vestibulopathy in individuals with complete or incomplete CANVAS (Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome) and biallelic RFC1 repeat expansions. Methods: Retrospective analysis of clinical data and the vestibulo‐ocular reflex quantified by the video head impulse test in 20 patients with confirmed biallelic RFC1 repeat expansions. Results: Vestibulo‐ocular reflex gain at first admittance 6.9 ± 5.0 years after disease onset was 0.16 [0.15–0.31] (median [interquartile range]). Cross‐sectional analysis revealed that gain reduction was associated with disease duration. Follow‐up measurements were available for ten individuals: eight of them exhibited a progressive decrease of the vestibulo‐ocular reflex gain over time. At the first visit, six of all patients (30%) did not show clinical signs of cerebellar ataxia. Conclusions: Our data suggest a pathological horizontal head impulse test, which can easily be obtained in many outpatient clinics, as a sign of bilateral vestibulopathy in genetically confirmed CANVAS that can precede clinically accessible cerebellar ataxia at least in a subset of patients. The presumably continuous decline over time possibly reflects the neurodegenerative character of the disease. Thus, genetic testing for RFC1 mutations in (isolated) bilateral vestibulopathy might allow disease detection before the onset of cerebellar signs. Further studies including a wider spectrum of vestibular function tests are warranted in a prospective design. [ABSTRACT FROM AUTHOR]

Objective: To compare video head impulse test (vHIT) and caloric test efficacy in decompensated and compensated vertigo patients and to further investigate whether vHIT alone can be used as a diagnostic tool in vertigo. Methods: This study included 25 patients diagnosed with vertigo and without any previous history of vertigo or hearing loss before their admission to our clinic. The control group consisted of 16 healthy adult volunteers. Patients were classified into 2 groups, compensated and decompensated. Video head impulse test and caloric tests were performed and the results were compared between the groups. Results: The difference of caloric test values between control-compensated groups and compensated-decompensated groups was statistically significant (P <.001, Pearson χ2). However, there was no statistically significant difference between the compensated and control groups according to vHIT gain asymmetry values (P =.087). In the very early stages of the disease with spontaneous nystagmus, the diagnostic significance of vHIT was similar to that of the caloric test. When both sides were compared, vHIT gain asymmetry values were close to the caloric test asymmetry values. In the compensated stage, caloric test was superior to vHIT in differentiating compensated vestibular pathologies. When vHIT sensitivity was evaluated according to the bi-thermal caloric test results, the sensitivity of the vHIT gain asymmetry value was 85.71% and 23.08% for decompensated and compensated patients respectively. Conclusion: In the early decompensated stages of the disease with spontaneous nystagmus, vHIT shows similar diagnostic accuracy to that of the caloric test. Since patients can tolerate vHIT more easily, our results suggest that vHIT can be considered as a primary evaluation method in the early (decompensated) period of the disease and should be preferred over the caloric test during the acute phase. Caloric test is more reliable at the compensated stage. Video head impulse test is inadequate in evaluating the compensated vestibular hypofunctional states after compensation has been restored. Level of Evidence: Level 2b [ABSTRACT FROM AUTHOR]