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.

Bilateral Vestibular Hypofunction in the Time of the Video Head Impulse Test.

OBJECTIVES: Bilateral vestibulopathy is a clinical syndrome in which laboratory testing plays a crucial diagnostic role. We aimed to establish the frequency of detection of that finding in a tertiary level hospital considering the new methods of laboratory vestibular examination nowadays in use, with respect to the conventional caloric and rotatory chair test approaches. DESIGN: Two similar time periods (5 years) were retrospectively evaluated, and the demographic, clinical data and test results from 4,576 patients were reviewed. In the first period, the diagnosis was based on caloric and rotatory chair tests and, in the second, on the video head impulse test. RESULTS: Of the patients included, 3.77% in the first period and 4.58% in the second met the criteria for bilateral vestibular hypofunction; there was no significant difference between both periods. CONCLUSIONS: The functional vestibular evaluation to detect bilateral deficiency makes no significant difference to the number of patients diagnosed with a bilateral vestibulopathy. New diagnostic categories probably depend not only on the availability and accessibility of complete vestibular and visual-vestibular evaluation, but also on recent advances in defining vestibular disorders. Bilateral vestibular hypofunction manifests with very different patterns. Progress in more detailed definition (clinical and laboratory) is needed, in particular when all 6 semicircular canals and both maculae are available for testing.

Effects of Implantation and Reimplantation of Cochlear Implant Electrodes in an In Vivo Animal Experimental Model (Macaca fascicularis).

OBJECTIVES: The objectives of this study were to evaluate the effect of reimplanting a cochlear implant electrode in animal normal-hearing cochlea to propose measures that may prevent cochlear injury and, given its close phylogenetic proximity to humans, to evaluate the macaque as a model for electroacoustic stimulation. DESIGN: Simultaneous, bilateral surgical procedures in a group of 5 normal-hearing specimens (Macaca fascicularis) took place in a total of 10 ears. Periodic bilateral auditory testing (distortion product otoacoustic emissions and auditory brainstem evoked responses [ABR]) took place during a 6-month follow-up period. Subsequently, unilateral explantation and reimplantation was performed. Auditory follow-up continued up to 12 months, after which animals were sacrificed and both temporal bones extracted for histological analysis. RESULTS: Implantation and reimplantation surgeries were performed without complications in 9 of 10 cases. Full insertion depth was achieved at reimplantation in four of five ears. Auditory evaluation: Statistically significant differences between implanted and reimplanted were observed for the frequencies 2000 and 11,000 Hz, the remaining frequencies showed no differences for distortion product otoacoustic emission. Before the procedure, average thresholds with click-stimuli ABR of the five animals were 40 dB SPL (implanted group) and 40 dB SPL (reimplanted group). One week after first implantation, average thresholds were 55 dB SPL and 60 dB, respectively. After 12 months of follow-up, the average thresholds were 72.5 dB SPL (implanted group) and 65 dB SPL (reimplanted group). Hearing loss appeared during the first weeks after the first implantation and no deterioration was observed thereafter. Differences for ABR under click stimulus were not significant between the two ear groups. Similar results were observed with tone-burst ABR. A 15 dB shift was observed for the implanted group preoperatively versus 1-week post surgery and an additional 17.5 dB shift was seen after 12-month follow-up. For the reimplanted group, a 20 dB shift was observed within the first week post reimplantation surgery and an additional 5 dB after 6 months follow-up. Statistical analysis revealed significant differences between the implanted and reimplanted ear groups for frequencies 4000 Hz (p = 0.034), 12000 Hz (p = 0.031), and 16,000 Hz (p = 0.031). The histological analysis revealed that the electrode insertion was minimally traumatic for the cochlea, mainly indicating rupture of the basilar membrane in the transition area between the basal turn and the first cochlear turn only in Mf1 left ear. CONCLUSIONS: With application of minimally traumatic surgical techniques, it is possible to maintain high rates of hearing preservation after implantation and even after reimplantation. Partial impairment of auditory thresholds may occur during the first weeks after surgery, which remains stable. Considering the tonotopic distribution of the cochlea, we found a correlation between the histological lesions sites and the auditory findings, suggesting that a rupture of the basilar membrane may impact hearing levels. The macaque was observed to be a functionally and anatomically an excellent animal model for cochlear implantation.

Endolymphatic hydrops severity in magnetic resonance imaging evidences disparate vestibular test results.

OBJECTIVES: It has been suggested that in Ménière's disease (MD) a dissociated result in the caloric test (abnormal result) and video head-impulse test (normal result) probably indicates that hydrops affects the membranous labyrinth in the horizontal semicircular canal (HSC). The hypothesis in this study is that based on endolymphatic hydrops' cochleocentric progression, hydrops should also be more severe in the vestibule of these patients than in those for whom both tests are normal. METHODS: 22 consecutive patients with unilateral definite MD were included and classified as NN if both tests were normal or AN if the caloric test was abnormal. MRI evaluation of endolymphatic hydrops was carried out with a T2-FLAIR sequence performed 4h after intravenous gadolinium administration. The laterality and degree of vestibular endolymphatic hydrops and the presence or absence of cochlear endolymphatic hydrops were recorded. Demographic data, audiometric and vestibular evoked myogenic potentials were collected, and video head-impulse and caloric tests were performed. RESULTS: Patients in both groups (NN and AN) were similar in terms of demographic data and hearing loss. The interaural asymmetry ratio was significantly higher for ocular and cervical VEMP in patients in the AN group. There was a significantly higher degree of hydrops in the vestibule of the affected ear of AN patients (χ2; p=0.028). CONCLUSION: Significant canal paresis in the caloric test is associated with more severe endolymphatic hydrops in the vestibule as detected with gadolinium-enhanced MRI and with a more severe vestibular deficit. LEVEL OF EVIDENCE: 2a.

Hypoglossal nerve stimulation surgery for obstructive sleep apnoea: Our preliminary experience. / Cirugía de implante de estimulador de nervio hipogloso en el síndrome de apnea obstructiva del sueño: nuestra experiencia preliminar.

The objective of this communication is to describe our preliminary results in upper airway stimulation surgery via hypoglossal nerve stimulation implantation for obstructive sleep apnoea. We describe 4 cases and the outcomes of the surgery were analysed using the Epworth scale, apnoea-hypopnoea index, minimal O2 Sat, average O2 Sat and snoring intensity. In all cases a significant reduction in Epworth scale values and apnoea-hypopnoea index were obtained (P<.05). The minimum and average oxygen saturation had better values after the surgery, however, there was no statistically significant difference. The snoring severity measured subjectively changed from «intense¼ to «absent¼ in all cases. The preliminary results obtained with the upper airway stimulation surgery via hypoglossal nerve stimulation showed objective and subjective improvement after the implant activation.

Vestibulo-Cochlear Function After Cochlear Implantation in Patients With Meniere's Disease.

OBJECTIVES: To measure the auditory (pure tone audiometry and word recognition scores) and vestibular (video head impulse test and vestibular myogenic potentials) outcomes in patients diagnosed with Meniere's disease (MD) who underwent cochlear implantation. MATERIAL AND METHODS: This prospective study included 23 cochlear implant users with MD and 29 patients diagnosed with far-advanced otosclerosis (the control group). RESULTS: The preoperative mean pure tone average thresholds were 99 and 122.5 dB for the Meniere's and control groups, respectively. Word recognition scores after cochlear implant yielded a median of 80% and 72% for the Meniere's and control groups, respectively. Semicircular canal gain was not observed to vary post implantation (mean variation for lateral, posterior, and anterior plane was 0, 0.03, and 0, respectively). The mean ocular and cervical myogenic potentials asymmetry varied as 9.65% and 18.39%, respectively. CONCLUSIONS: The auditory performance improved in patients with MD similar to the general cochlear implant population. No major dysfunction of otolithic or semicircular canal function was demonstrated after the implantation surgery.