VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words "bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy." Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

Comparison between high-resolution 3D-IR with real reconstruction and 3D-flair sequences in the assessment of endolymphatic hydrops in 3 tesla.

BACKGROUND: The main sequences used to assess degree of endolymphatic hydrops (EH) are the 3D- inversion-recovery (IR) with real reconstruction and the 3D- fluid-attenuated inversion recovery (FLAIR). OBJECTIVES: The purpose of this study is to describe and compare both sequences. MATERIAL AND METHODS: Forty-two consecutive patients diagnosed with probable or definite Ménièr´s disease were referred for hydrops MR examination. Vestibular and cochlear EH in both sequences were read by two independent radiologists, unaware of the patient's clinical status. The primary study endpoint was the concordance in the hydrops detection and severity with both imaging sequences. This was assessed using the Cohen's kappa Κ statistic for disease grading and Pearson Χ2 test to test the difference in detection rates of hydrops. Statistical significance was defined as two-sided p < .05. RESULTS: We obtained an excellent overall concordance, with a kappa of 0.821, (p < .001) when comparing hydrops detection. The degree of concordance is higher in vestibular hydrops than in cochlear hydrops. The 3D-IR sequence detected more hydrops than the 3D FLAIR, (62 vs. 39.5%, p < .03). CONCLUSION: The 3D-IR sequence seems to be superior to the 3D-FLAIR for the assessment of EH. Significance: The 3D-IR sequence should be considered as a standalone parameter for a shorter and optimized EH magnetic resonance imaging protocol.

The vestibulo-ocular reflex assessment in patients with Ménière's disease: examining all semicircular canals.

UNLABELLED: Abstract Conclusion: The distribution of abnormal results is not uniform between different canals in each patient; the most frequent gain reduction is obtained for the posterior canal. Gain reduction reflects the disease duration and amount of hearing loss. OBJECTIVE: To test the hypothesis that the vestibulo-ocular reflex (VOR) evoked after stimulation of each semicircular canal behaves in a different manner in patients with unilateral definite Ménière's disease. METHODS: We studied the VOR evoked by rapid head-impulses in the plane of the 6 semicircular canals in 36 patients. It was evaluated with a video system that analyzes the head and eye velocity and the gain was the objective measure. RESULTS: In 12 (33.3%) patients the examination of both ears was normal for all the semicircular canals, in 12 patients the results from the affected ear were abnormal in at least 1 of the semicircular canals, in 11 (30.5%) patients the results were abnormal in at least 1 of the semicircular canals in both the affected and unaffected ears, and in 1 (2.9%) patient the results were abnormal only in the unaffected ear. The most frequent abnormal result was obtained from the posterior canal of the affected ear and from the coupled superior canal of the unaffected ear. The distribution of abnormal findings was dependent on the disease duration and hearing loss.

Auditory and vestibular assessment of patients with Ménière's disease who suffer Tumarkin attacks.

Tumarkin attacks are a feature of Ménière's disease that generate a significant degree of disability. The surprising nature of these events is the main reason behind their associated morbidity. In this study we set out to evaluate auditory and vestibular function, as well as disability, in a population of patients who suffer Tumarkin attacks. We found that patients who suffer Tumarkin attacks are more disabled and experience severer and more frequent autonomic symptoms and that their hearing level is significantly worse in the asymptomatic ear, especially at low frequencies. Accordingly, we consider that treatment must be carefully planned to be as conservative as possible in terms of hearing and that psychiatric and/or psychological treatment must always be considered as an adjuvant therapy.