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Background: Although gray matter (GM) volume alterations have been extensively documented in previous voxel-based morphometry studies on vestibular migraine (VM), little is known about the impact of this disease on the topological organization of GM morphological networks. This study investigated the altered network patterns of the GM connectome in patients with VM., Methods: In this study, 55 patients with VM and 57 healthy controls (HCs) underwent structural T1-weighted MRI. GM morphological networks were constructed by estimating interregional similarity in the distributions of regional GM volume based on the Kullback-Leibler divergence measure. Graph-theoretical metrics and interregional morphological connectivity were computed and compared between the two groups. Partial correlation analyses were performed between significant GM connectome features and clinical parameters. Logistic regression (LR), support vector machine (SVM), and random forest (RF) classifiers were used to examine the performance of significant GM connectome features in distinguishing patients with VM from HCs., Results: Compared with HCs, patients with VM exhibited increased clustering coefficient and local efficiency, as well as reduced nodal degree and nodal efficiency in the left superior temporal gyrus (STG). Furthermore, we identified one connected component with decreased morphological connectivity strength, and the involved regions were mainly located in the STG, temporal pole, prefrontal cortex, supplementary motor area, cingulum, fusiform gyrus, and cerebellum. In the VM group, several connections in the identified connected component were correlated with clinical measures (i.e., symptoms and emotional scales); however, these correlations did not survive multiple comparison corrections. A combination of significant graph- and connectivity-based features allowed single-subject classification of VM versus HC with significant accuracy of 77.68%, 77.68%, and 72.32% for the LR, SVM, and RF models, respectively., Conclusion: Patients with VM had aberrant GM connectomes in terms of topological properties and network connections, reflecting potential dizziness, pain, and emotional dysfunctions. The identified features could serve as individualized neuroimaging markers of VM., (© 2024. The Author(s).)

Background: Prior neuroimaging studies on vestibular migraine (VM) have extensively certified the functional and structural alterations in multiple brain regions and networks. However, few studies have assessed the cerebral blood flow (CBF) in VM patients using arterial spin labeling (ASL). The present study aimed to investigate CBF and functional connectivity (FC) alterations in VM patients during interictal periods., Methods: We evaluated 52 VM patients and 46 healthy controls (HC) who received resting-state pseudo-continuous ASL and functional magnetic resonance imaging (fMRI) scanning. Comparisons of voxel-based CBF and seed-based FC were performed between the two groups. Brain regions showed significant group differences in CBF analyses were chosen as seeds in FC analyses. Additionally, the associations between abnormal imaging results and clinical features were explored., Results: Compared with HC, VM patients showed higher normalized CBF in the right precentral gyrus (PreCG), left postcentral gyrus (PostCG), left superior frontal gyrus and bilateral insular (p < 0.05, FDR corrected). Furthermore, VM patients exhibited increased FC between the right PreCG and areas of the left PostCG, left cuneus and right lingual gyrus (p < 0.05, FDR corrected). In addition, we observed decreased FC between the left insular and regions of the left thalamus and right anterior cingulate cortex, as well as increased FC between the left insular and right fusiform gyrus in VM patients (p < 0.05, FDR corrected). Moreover, these variations in brain perfusion and FC were significantly correlated with multiple clinical features including frequency of migraine symptoms, frequency of vestibular symptoms and disease duration of VM (all p < 0.05)., Conclusions: Patients with VM during interictal period showed hyperperfusion and abnormal resting-state FC in brain regions potentially contributed to disrupted multi-sensory and autonomic processing, as well as impaired ocular motor control, pain modulation and emotional regulation. Our study provided novel insights into the complex neuropathology of VM from a CBF perspective., (© 2024. The Author(s).)

Background: Chronic unilateral vestibulopathy (CUVP) is a common chronic vestibular syndrome; the mechanisms of central vestibular compensation in CUVP are rarely studied. Methods: This study analyzed the data of 18 patients with CUVP and 18 healthy controls (HCs) and used seed-based functional connectivity (FC) and voxel-mirrored homotopic connectivity (VMHC) analyses to explore the FC alterations. Results: Compared with HCs, patients with CUVP showed decreased FC between the left dorsolateral superior frontal gyrus and the right hippocampus; the left middle frontal gyrus and the right posterior cingulate gyrus, the right hippocampus, the right parahippocampal gyrus. There is also a reduction in FC between the left and right insula. There was enhanced FC between the left supplementary motor area (SMA) and the bilateral superior occipital gyrus, the left hippocampus and the left posterior cingulate gyrus, as well as a the left middle temporal gyrus ( p = 0.03). Additionally,VMHC was decreased between the bilateral medial superior frontal gyrus, the bilateral precentral gyrus, and the bilateral postcentral gyrus ( p = 0.001). The zVMHC values in the bilateral superior frontal gyrus and the precentral gyrus were both negatively corrected with the Dizziness Handicap Inventory (DHI) score.well as Conclusions: Altered FC in regions of bilateral multisensory vestibular cortex existed in patients with CUVP. Decreased FC and VMHC in the bilateral multisensory vestibular cortex may affect vestibular information integration, thus affecting self-motion perception, spatial orientation, and postural control.

Objectives: Otitis media/interna (OMI) is the most common cause of peripheral vestibular disease in cats. The inner ear contains endolymph and perilymph, with perilymph being very similar in composition to cerebrospinal fluid (CSF). As a very-low-protein fluid, it would be expected that normal perilymph should suppress on fluid-attenuated inversion recovery (FLAIR) MRI sequences. Based on this, we hypothesized that MRI FLAIR sequences should provide a non-invasive way of diagnosing inflammatory/infectious diseases such as OMI in cats, something that has previously been demonstrated in humans and, more recently, in dogs., Methods: This was a retrospective cohort study in which 41 cats met the inclusion criteria. They were placed into one of four groups, based on presenting complaint: clinical OMI (group A); inflammatory central nervous system (CNS) disease (group B); non-inflammatory structural disease (group C); and normal brain MRI (control group; group D). Transverse T2-weighted and FLAIR MRI sequences at the level of the inner ears bilaterally were compared in each group. The inner ear was selected as a region of interest using Horos, with a FLAIR suppression ratio calculated to account for variability in signal intensity between MRIs. This FLAIR suppression ratio was then compared between groups. Statistical analyses were performed by an experienced statistician, with a general linear model used to compare mean FLAIR suppression ratio, CSF nucleated cell count and CSF protein concentration between groups., Results: The OMI group (group A) had significantly lower FLAIR suppression scores compared with all other groups. The CSF cell count was also significantly increased in the OMI (group A) and inflammatory CNS disease (group B) groups compared with the control group (group D)., Conclusions and Relevance: This study demonstrates the utility of MRI FLAIR sequences in diagnosing presumptive OMI in cats, similarly to in humans and dogs. This study is relevant to practicing veterinary neurologists and radiologists in interpreting MRI findings in cats with suspected OMI.

Peripheral vestibular disorders are caused by pathology of the inner ear. The majority of these disorders are diagnosed with a detailed history and vestibular physical exam. Imaging is rarely a part of diagnostic work up as the pathologies of these disorders are "invisible," or undetectable on imaging. However, these "invisible" diagnoses are becoming increasingly visible with advancements in imaging technology. Developments in magnetic resonance imaging are allowing for increased spatial resolution and better image contrast, improving our ability to see soft tissue structures including the membranous labyrinth, sensory epithelia and nerves. With these improvements in imaging, clinicians will be able to understand better atypical presentations of peripheral vestibular disorders, disease intractable to traditional therapy, disorders with unclear pathoetiology and disease only seen on histopathological studies. This review assesses the current state of imaging in the neurotology clinic with a special focus on magnetic resonance imaging and then gathers diseases identified by vestibular testing and histopathological studies that could be better understood with developments in imaging. In doing so, we hope to guide advancement in neurotologic imaging and apprise clinicians of the utility of imaging in peripheral vestibular disease diagnosis., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objectives: Persistent postural-perceptual dizziness (PPPD) is a chronic functional vestibular disorder that may have normal physical examination, clinical laboratory testing and vestibular evaluation. However, advances in neuroimaging have provided new insights in brain functional connectivity and structure in patients with PPPD. This systematic review was aimed at identifying significant structural or alterations in functional connectivity in patients with PPPD., Databases Reviewed: Science Direct, Pubmed, Embase via Ovid databases, and Cochrane library., Methods: This review following the guidelines of PRISMA, systematically and independently examined papers published up to March 2021 which fulfilled the predetermined criteria. PROSPERO Registration (CRD42020222334)., Results: A total of 15 studies were included (MRI = 4, SPECT = 1, resting state fMRI = 4, task-based fMRI = 5, task-based fMRI + MRI = 1). Significant changes in the gray matter volume, cortical folding, blood flow, and connectivity were seen at different brain regions involved in vestibular, visual, emotion, and motor processing., Conclusion: There is a multisensory dimension to the impairment resulting in chronic compensatory changes in PPPD that is evident by the significant alterations in multiple networks involved in maintaining balance. These changes observed offer some explanation for the symptoms that a PPPD patient may experience.Systematic Review Registration: This study is registered with PROSPERO (CRD42020222334)., Competing Interests: The authors disclose no conflicts of interest, financial, or otherwise., (Copyright © 2021, Otology & Neurotology, Inc.)

Recognition of distinct phenotypic features is an important component of genetic diagnosis. Although CHARGE syndrome, Kabuki syndrome, and a recently delineated KMT2D Ex 38/39 allelic disorder exhibit significant overlap, differences on neuroimaging may help distinguish these conditions and guide genetic testing and variant interpretation. We present an infant clinically diagnosed with CHARGE syndrome but subsequently found to have a de novo missense variant in exon 38 of KMT2D, the gene implicated in both Kabuki syndrome and a distinct KMT2D allelic disorder. We compare her brain and inner ear morphology to a retrospective cohort of 21 patients with classic Kabuki syndrome and to typical CHARGE syndrome findings described in the literature. Thirteen of the 21 Kabuki syndrome patients had temporal bone imaging (5/13 CT, 12/13 MRI) and/or brain MRI (12/13) which revealed findings distinct from both CHARGE syndrome and the KMT2D allelic disorder. Our findings further elucidate the spectrum of inner ear dysmorphology distinguishing Kabuki syndrome and the KMT2D allelic disorder from CHARGE syndrome, suggesting that these three disorders may be differentiated at least in part by their inner ear anomalies., (© 2021 Wiley Periodicals LLC.)

Unilateral damage to the inner ear results in an acute vestibular syndrome, which is compensated within days to weeks due to adaptive cerebral plasticity. This process, called central vestibular compensation (VC), involves a wide range of functional and structural mechanisms at the cellular and network level. The short-term dynamics of whole-brain functional network recruitment and recalibration during VC has not been depicted in vivo. The purpose of this study was to investigate the interplay of separate and distinct brain regions and in vivo networks in the course of VC by sequential [ 18 F]-FDG-PET-based statistical and graph theoretical analysis with the aim of revealing the metabolic connectome before and 1, 3, 7, and 15 days post unilateral labyrinthectomy (UL) in the rat. Temporal changes in metabolic brain connectivity were determined by Pearson's correlation (|r| > 0.5, p < 0.001) of regional cerebral glucose metabolism (rCGM) in 57 segmented brain regions. Metabolic connectivity analysis was compared to univariate voxel-wise statistical analysis of rCGM over time and to behavioral scores of static and dynamic sensorimotor recovery. Univariate statistical analysis revealed an ipsilesional relative rCGM decrease (compared to baseline) and a contralesional rCGM increase in vestibular and limbic networks and an increase in bilateral cerebellar and sensorimotor networks. Quantitative analysis of the metabolic connections showed a maximal increase from baseline to day 3 post UL (interhemispheric: 2-fold, ipsilesional: 3-fold, contralesional: 12-fold) and a gradual decline until day 15 post UL, which paralleled the dynamics of vestibular symptoms. In graph theoretical analysis, an increase in connectivity occurred especially within brain regions associated with brainstem-cerebellar and thalamocortical vestibular networks and cortical sensorimotor networks. At the symptom peak (day 3 post UL), brain networks were found to be organized in large ensembles of distinct and highly connected hubs of brain regions, which separated again with progressing VC. Thus, we found rapid changes in network organization at the subcortical and cortical level and in both hemispheres, which may indicate an initial functional substitution of vestibular loss and subsequent recalibration and reorganization of sensorimotor networks during VC., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Persistent postural-perceptual dizziness (PPPD) is a functional vestibular disorder, and is the most common cause of chronic vestibular syndrome. However, the pathogenesis of PPPD is currently unclear. This study aimed to analyze the changes of brain spontaneous functional activities in PPPD patients during the resting state, and to explore the underlying pathogenesis of PPPD, particularly the abnormal integration of visual and vestibular information. Ten PPPD patients and 10 healthy controls were enrolled from January to June 2018, and baseline data were collected from all subjects. Videonystagmography (VNG), the vestibular caloric test, the video head impulse test (vHIT) and vestibular evoked myogenic potentials (VEMPs) were measured to exclude peripheral vestibular lesions. Functional MRI (fMRI) was conducted in PPPD patients and healthy controls. The amplitude of low frequency fluctuation (ALFF) and regional homogeneity (ReHo), and functional connectivity were calculated to explore changes in brain spontaneous functional activity during the resting state. Compared with healthy controls, ALFF and ReHo values in the right precuneus and cuneus were significantly lower in PPPD patients (both P < 0.05). Further seed-based functional connectivity analysis showed decreased functional connectivity between precuneus, cuneus and left precentral gyrus (P < 0.05). Our findings suggest that the spontaneous functional activity of cuneus and precuneus in PPPD patients were altered, potentially leading to abnormal integration of visual and vestibular information. Weakened functional connectivity between the precuneus and the precentral gyrus may be associated with aggravated symptoms during upright posture, active or passive movements.

Objectives: The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Menière's disease (MD)., Methods: A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). MRI exploration was carried out on a 3-T Achieva® TX scanner. We analyzed the presence of the RWS, defined as a nodular FLAIR high signal in the round window (RW) and the presence of associated saccular hydrops. When this RWS was present, a temporal bone CT scan was performed and the RW was analyzed., Results: Of the 164 patients with definite MD (85 patients) or probable MD (79 patients), we found the RWS in 18 (11%), and 17/18 were classified into the group of probable MD. All these 18 patients showed other MR sequences considered as normal, including heavily weighted T2 imaging. Among these 18 patients, the temporal bone CT examination presented a filling of the RW in 13 patients (72%) and no filling of the RW in 5 patients (28%). Seven patients were surgically managed confirming in vivo the PLF diagnosis. The RWS was associated with the presence of a saccular hydrops in 4 cases., Conclusion: Delayed postcontrast 3D-FLAIR may reveal perilymphatic fistulae in patients with probable Menière's disease using the round window sign., Key Points: • MRI with delayed acquisition can detect perilymphatic fistulae with perfect sensitivity, based on the presence of the round window sign. • This visual sign is only visible on a 3D-FLAIR sequence. • 3D-FLAIR sequence with delayed acquisition is more sensitive than temporal bone CT scan examination in detecting PLF.

Idiopathic vestibular syndrome (IVS) is the most common cause of acute unilateral peripheral vestibular dysfunction in older dogs. The purpose of this retrospective, cross-sectional study was to characterize morphological changes in the utricle of dogs affected by IVS, using MRI. To evaluate differences between affected and unaffected utricles, the ratio of the largest to the smallest utricle diameter was obtained, as measured on transverse T2-weighted images, and defined as the utricle asymmetricity ratio (UAR). Out of 137 patients diagnosed with IVS after excluding other vestibular diseases by MRI, 101 were eligible for inclusion. Additionally, 31 older dogs with no signs of vestibular disorders or other intracranial diseases were included as a control group. The disease group was divided into two subgroups in which the direction of head tilt and nystagmus symptoms versus the decreased utricle diameters were consistent or inconsistent. The medians of UARs of the IVS and control groups were 0.83 (range 0.37-1.00) and 0.98 (0.70-1.00), respectively. The medians of the UARs of the consistent and inconsistent IVS subgroups were 0.82 (0.37-0.99) and 0.90 (0.74-1.00), respectively. The UAR of the IVS group was significantly decreased than that of the control group and UAR of the consistent sub-group was significantly decreased than that of the inconsistent sub-group (P < .01). In conclusion, significant asymmetry of utricle diameter was identified in dogs with IVS versus unaffected dogs. We propose that canine IVS may possibly be correlated with structural atrophy of the vestibular system., (© 2020 American College of Veterinary Radiology.)

The inner ear contains endolymph and perilymph. The second is comparable and in continuity with the cerebrospinal fluid (CSF) so it is expected to suppress in fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) if normal. Even though inner ear FLAIR abnormalities have been extensively described in humans with inner ear disease, its diagnostic value in dogs is yet to be proven. The goal of this retrospective cohort study was to investigate the diagnostic utility of FLAIR MRI in dogs with vestibular disease. A review of medical records identified 101 dogs that had brain MRI performed because of vestibular signs. Based on the final diagnosis, patients were allocated to three groups: otitis media/interna, idiopathic vestibular disease, and central vestibular disease. Additionally, a control group (n = 73) included dogs with normal MRI and without vestibular signs. Inner ears were delineated using a region of interest, and signal intensity was measured in FLAIR and T2-weighted images. The percentages of suppression in FLAIR were calculated and compared between affected and unaffected sides of each individual and between groups using a general linear mixed model. Correlation between suppression and CSF cell count and protein concentration was assessed. Affected inner ears in dogs with otitis media/interna had decreased suppression in FLAIR compared to the unaffected side (P < .001), and all other groups (P < .01). No significant correlation was detected between CSF results and suppression. These results show the diagnostic value of FLAIR in otitis media/interna due to lack of suppression in the affected inner ear., (© 2020 American College of Veterinary Radiology.)

The ASPECTS determines middle cerebral artery stroke severity using available computed tomography data.

Background: Vestibular dysfunction is relatively common in dogs, with a prevalence of 0.08% reported in primary veterinary care in the UK. There are several studies investigating how to differentiate between peripheral and central vestibular disease but only limited information regarding the possible underlying causes for peripheral vestibular dysfunction in dogs. This study therefore aimed to describe the clinical signs, magnetic resonance imaging findings (MRI), underlying causes and outcome in a large population of dogs diagnosed with peripheral vestibular disease., Results: One hundred eighty-eight patients were included in the study with a median age of 6.9 years (range 3 months to 14.6 years). Neurological abnormalities included head tilt (n = 185), ataxia (n = 123), facial paralysis (n = 103), nystagmus (n = 97), positional strabismus (n = 93) and Horner syndrome (n = 7). The most prevalent diagnosis was idiopathic vestibular disease (n = 128), followed by otitis media and/or interna (n = 49), hypothyroidism (n = 7), suspected congenital vestibular disease (n = 2), neoplasia (n = 1) and cholesteatoma (n = 1). Long-term follow-up revealed persistence of head tilt (n = 50), facial paresis (n = 41) and ataxia (n = 6) in some cases. Recurrence of clinical signs was observed in 26 dogs. Increasing age was associated with a mild increased chance of diagnosis of idiopathic vestibular syndrome rather than otitis media and/or interna (P = 0.022, OR = 0.866; CI 0.765-0.980). History of previous vestibular episodes (P = 0.017, OR = 3.533; CI 1.251-9.981) was associated with an increased likelihood of resolution of the clinical signs whilst contrast enhancement of cranial nerves VII and/or VIII on MRI (P = 0.018, OR = 0.432; CI 0.251-0.868) was associated with a decreased chance of resolution of the clinical signs., Conclusions: Idiopathic vestibular disease is the most common cause of peripheral vestibular dysfunction in dogs and it is associated with advanced age. Incomplete recovery from peripheral vestibular disease is common, especially in dogs presenting with cranial nerve enhancement on MRI but less so if there is previous history of vestibular episodes.

Pathogenic variants in KMT2D, which encodes lysine specific methyltransferase 2D, cause autosomal dominant Kabuki syndrome, associated with distinctive dysmorphic features including arched eyebrows, long palpebral fissures with eversion of the lower lid, large protuberant ears, and fetal finger pads. Most disease-causing variants identified to date are putative loss-of-function alleles, although 15-20% of cases are attributed to missense variants. We describe here four patients (including one previously published patient) with de novo KMT2D missense variants and with shared but unusual clinical findings not typically seen in Kabuki syndrome, including athelia (absent nipples), choanal atresia, hypoparathyroidism, delayed or absent pubertal development, and extreme short stature. These individuals also lack the typical dysmorphic facial features found in Kabuki syndrome. Two of the four patients had severe interstitial lung disease. All of these variants cluster within a 40-amino-acid region of the protein that is located just N-terminal of an annotated coiled coil domain. These findings significantly expand the phenotypic spectrum of features associated with variants in KMT2D beyond those seen in Kabuki syndrome and suggest a possible new underlying disease mechanism for these patients., (© 2020 Wiley Periodicals, Inc.)

Purpose: To compare audiologic results according to vestibular aqueduct (VA) diameter in patients who have undergone cochlear implantation and were diagnosed with LVAS., Methods: This was a retrospective study detailing the outcomes of 18 patients with LVAS and 18 patients undergone cochlear implants. VA diameter was assessed by magnetic resonance imaging and computed tomography. Categories of Auditory Perception (CAP) and Speech Intelligibility Rating (SIR) were assessed in all patients, and speech audiometry, including speech recognition thresholds (SRT) and word discrimination scores, was applied for all subjects who were able to perform these tests. All audiologic parameters were compared between patients with and without LVAS, and the relationship of these parameters with VA diameter was investigated., Results: The control group consisted of 18 subjects (5 males, 13 females), ranging in age between 2 and 34 years (mean 13.17 ± 8.97 years). The research group consisted of 18 subjects (8 males, 10 females), ranging in age between 2 and 35 years (mean 13.28 ± 8.96 years). There was a statistically significant difference between the groups in terms of SIR and CAP pre-post differences (Mann-Whitney U test, p < 0.05), with higher averages in the LVAS group. No statistically significant correlations were found between VA diameter on computed tomography and magnetic resonance imaging and the audiologic variables collected., Conclusions: Patients with LVAS benefit from cochlear implant surgery and VA parameters do not affect audiologic parameters.

Kabuki syndrome is a rare, multi-systemic disorder of chromatin regulation due to mutations in either KMT2D or KDM6A that encode a H3K4 methyltransferase and an H3K27 demethylase, respectively. The associated clinical phenotype is a direct result of temporal and spatial changes in gene expression in various tissues including the brain. Although mild to moderate intellectual disability is frequently recognized in individuals with Kabuki syndrome, the identification of brain anomalies, mostly involving the hippocampus and related structures remains an exception. Recently, the first two cases with alobar holoprosencephaly and mutations in KMT2D have been reported in the medical literature. We identified a de novo, pathogenic KMT2D variant (c.6295C > T; p.R2099X) using trio whole-exome sequencing in a 2-year-old female with lobar holoprosencephaly, microcephaly and cranio-facial features of Kabuki syndrome. This report expands the spectrum of brain anomalies associated with Kabuki syndrome underscoring the important role of histone modification for early brain development., (© 2019 Wiley Periodicals, Inc.)

Objective: To explore the imaging characteristics of large vestibular aqueduct syndrome (LVAS) patients and their relationship with the acoustically evoked short latency negative response (ANSR), so as to provide reference for the diagnosis of LVAS. Methods: Clinical data of 174 patients(334 ears) with LVAS diagnosed and treated by the Department of Otorhinolaryngology Head and Neck Surgery of the First Affiliated Hospital of Guangxi Medical University, from October 2009 to December 2017 were retrospectively analyzed, including 117 males and 57 females, aged from 5 months to 47 years old, with the median age of 4 years and 4 months. ABR and imaging data of patients were collected. Midpoint diameter and the outlet diameter of the vestibular aqueduct were measured on CT images, the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac(EES) were measured on MRI images. The correlation between the above measurements was analyzed by Pearson test using SPSS 17.0. According to whether ASNR was detected in ABR, the above data were divided into two groups, and the differences of the above imaging measurements were compared by the Independent-Sample Test. Results: The average midpoint diameter of the vestibular aqueduct was (1.87±0.58) mm ( x ± s , the following was the same), and the outlet diameter was (3.07±0.99) mm on CT; the average midpoint diameter of the intraosseous parts in enlarged endolymphatic sac(EES) was (2.39±1.37) mm, and the extraosseous parts was (2.50±2.18) mm on MRI. There was a correlation between the four measurements ( P< 0.05), among which the midpoint diameter of vestibular aqueduct was strongly positively correlated with the outlet diameter ( r= 0.760), and the remaining pairs were weakly correlated. ASNR was detected in 241 ears (72.16%,241/334) and undetected in 93 ears (27.84%, 93/334) of the 334 ears with LVAS. Midpoint diameter and the outlet diameter of the vestibular aqueduct in no ASNR group were smaller than the ASNR group, and the difference was statistically significant ( t value was 2.814 and 2.754, P< 0.05). There was no significant difference in the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac between the two groups, and the difference was no statistically significant( t value was 0.101 and 0.683, P> 0.05). Conclusions: There is a strong positive correlation between the midpoint diameter of vestibular aqueduct and the outlet diameter in LVAS patients. There is a certain correlation between the size of vestibular aqueduct and the size of endolymphatic sac. The smaller the diameter of vestibular aqueduct, the lower the occurrence rate of ASNR.

A prospective study focused on whether vestibular symptoms are seen in acute hemispheric strokes, and if so, the frequency and lateralization of causative lesions on MRI. Among 668 patients with hemispheric infarction, we prospectively included those with chief complaints of acute vestibular symptoms, such as vertigo/dizziness, nausea/vomiting and gait instability, in the "VS" group. We also retrospectively reviewed MRI of all stroke patients, and included cases with the findings of parieto-insular vestibular cortex (PIVC) or temporo-periSylvian vestibular cortex (TPSVC) lesion by diffusion-weighted MRI, in the "PIVC" group. Eight patients were found to belong to the VS group, and six other patients to the PIVC group. In the VS group, six patients had the responsible lesion on the right hemisphere, in the middle cerebral artery (MCA) territory except one case and two on the left MCA territory, particularly in the insula, retro-insular region, superior/middle temporal gyrus, angular gyrus, supra-marginal gyrus, putamen and hippocampus/para-hippocampal gyrus. In contrast, none of the six other patients of the PIVC group had vestibular symptoms. One of them had a lesion in the right hemisphere and five in the left hemisphere. Four lesions were located in the insular area and two within the temporal lobe. In conclusion, cerebral hemispheric infarction limited to the PIVC or TPSVC does not necessarily cause vertigo. However, unilateral hemispheric infarctions, restricted to the areas belonging to the vestibular cortical network may cause vestibular symptoms. The lesions responsible for vestibular symptoms are located more often in the right hemisphere.

Background: Intracranial metastasis is a common complication of systemic malignancy. A rare subset of intracranial masses constitutes tumor-to-tumor metastasis, in which an extracranial neoplasm hematogenously spreads to an existing intracranial lesion., Case Description: Here we present the unique case of a 59-year-old man with known hepatocellular carcinoma who presented with acute headaches, double vision, vertigo, and gait instability in the context of 2 years of right-sided hearing loss. Head imaging showed a heterogeneous right cerebellopontine angle mass extending into the porus acousticus with adjacent cerebellar edema. Histopathologic analysis after resection found coexisting hepatocellular carcinoma within a vestibular schwannoma., Conclusions: Tumor-to-tumor metastasis is an important consideration in the diagnostic work-up and treatment of patients with known systemic malignancy who present with a new intracranial lesion. This pathologic entity could be missed if this patient were treated with single-fraction radiosurgery such as Gamma Knife., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: Because delayed post-contrast three-dimensional fluid-attenuated inversion recovery imaging sequences enable the distinction between the utricle and the saccule, we raised the hypothesis that patients with vestibular atelectasis (VA) could show unilateral collapse of the utricle and the ampullas on imaging., Study Design: Retrospective case series., Methods: We retrospectively reviewed 200 patients who underwent 3 T magnetic resonance imaging (MRI) after intravenous administration of gadolinium. MRI scans were assessed for the presence of VA. The endolymphatic space was considered as collapsed when the utricle and at least two ampullas were not visible or were barely visible., Results: We reported four patients with VA on MRI responsible for atypical clinical presentations of acute vestibular deficit. All patients presented a specific involvement of the pars superior sensory captors (utricle, ampullas), preserving the pars inferior sensory captors (cochlea and saccule). This was confirmed both clinically and on MRI., Conclusions: Our study is the first to describe in vivo unilateral collapse of the pars superior on delayed postcontrast MRI in patients with a clinical unilateral vestibular loss., Level of Evidence: 4 Laryngoscope, 129:1689-1695, 2019., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To assess the ictal symptoms, interictal symptoms, psychiatric comorbidities, and interictal neuro-otologic examination findings in vestibular migraine (VM)., Methods: Retrospective chart review of 491 patients seen from August 2014 until March 2018 at a tertiary neurology referral center for vestibular disorders to identify patients fulfilling the 2012 VM criteria., Results: One hundred and thirty-one patients (105 women) were identified. Mean age of VM onset was 44.3 (±13.7) years. Preceding the onset of vestibular symptoms, most had migraine (57.3%) and motion sickness (61.1%). It was common to have a family history of migraine (50.8%) and episodic vestibular symptoms (28.1%). Common ictal symptoms were triggered (visually induced and head-motion) and spontaneous vertigo, accompanied by photophobia and phonophobia (118/131 [90.1%] patients), nausea (105/131 [80.2%] patients), aural symptoms (79/131 [60.3%] patients), and headache (65/131 [49.6%] patients). Interictally, many experienced visually induced (116/131 [88.6%] patients), head-motion (86/131 [65.6%] patients), and persistent (67/131 [51.1%] patients) dizziness. Psychiatric comorbidities include anxiety (92/131 [70.2%] patients), depression (53/131 [40.5%] patients), insomnia (38/131 [29.0%] patients), phobic disorders (15/131 [11.5%] patients), and psychogenic disorders (11/131 [8.4%] patients). Common triggers were stress (52/131 [39.7%] patients), bright lights (35/131 [26.7%] patients), weather changes (34/131 [26.0%] patients), and sleep deprivation (34/131 [26.0%] patients). Interictal neuro-otologic examination was abnormal in 56/131 (42.7%), usually hyperventilation-induced, head-shaking-induced, vibration-induced, and positional nystagmus. The most common balance-test finding was impaired sharpened Romberg's test (22/130 [16.9%] patients)., Conclusions: In this single center study, we found that VM typically affects women in their 40s, with a personal and family history of migraine. Typical ictal symptoms were triggered and spontaneous vertigo, associated with photophobia and phonophobia, nausea, aural symptoms, and headache. Interictal vestibular symptoms, comorbid psychiatric disorders, and non-specific interictal neuro-otologic findings were common., (© 2019 American Headache Society.)

Objective: Middle ear extension of vestibular schwannomas is not a common occurrence, and only a few cases have been described so far in past publications. We report three new cases of vestibular schwannomas extending to the middle ear and reviewed the literature to specify the patterns of such an extension., Materials and Methods: We analysed databases of previously published articles to search for additional cases of middle ear extension of vestibular schwannomas and compared them to the cases we have documented. Extension patterns of the tumours were analysed, especially focusing on the extension through the round and oval windows., Results and Conclusion: Middle ear vestibular schwannomas are uncommon tumours and only 13 cases have been published so far. The vestibular schwannoma (internal auditory canal or intralabyrinthine) has to invade the labyrinth first (complete invasion in 88% of the cases, n = 14), before reaching the middle ear. In the majority of cases (69%, n = 11/16), internal auditory canal vestibular schwannomas or intralabyrinthine schwannomas extended in the middle ear though the round window.

Background and Purpose: We explored whether there was a difference between measurements obtained with CT and MRI for the diagnosis of large vestibular aqueduct syndrome or large endolymphatic sac anomaly, and whether this influenced diagnosis on the basis of previously published threshold values (Valvassori and Cincinnati). We also investigated whether isolated dilated extra-osseous endolymphatic sac occurred on MRI. Secondary objectives were to compare inter-observer reproducibility for the measurements, and to investigate any mismatch between the diagnoses using the different criteria., Materials/methods: Subjects diagnosed with large vestibular aqueduct syndrome or large endolymphatic sac anomalies were retrospectively analysed. For subjects with both CT and MRI available (n = 58), two independent observers measured the midpoint and operculum widths. For subjects with MRI (± CT) available (n = 84), extra-osseous sac widths were also measured. Results There was no significant difference between the width measurements obtained with CT versus MRI. CT alone diagnosed large vestibular aqueduct syndrome or large endolymphatic sac anomalies in 2/58 (Valvassori) and 4/58 (Cincinnati), whilst MRI alone diagnosed them in 2/58 (Valvassori). There was 93% CT/MRI diagnostic agreement using both criteria. Only 1/84 demonstrated isolated extra-osseous endolymphatic sac dilatation. The MRI-based LVAS/LESA diagnosis was less dependent on which criteria were used. Midpoint measurements are more reproducible between observers and between CT/MR imaging modalities., Conclusion: Supplementing MRI with CT results in additional diagnoses using either criterion, however, there is no net increased diagnostic sensitivity for CT versus MRI when applying the Valvassori criteria. Isolated enlargement of the extra-osseous endolymphatic sac is rare.

Videonystagmography (VNG) and posturography are two vestibular assessment techniques that are still in use today. VNG: VNG allows clinicians to observe and record eye movements in real-time. Compared with electronystagmography (ENG), VNG tracings are more detailed and can capture subtle clinical findings. The utility of the monothermal caloric screen has been proposed in various studies. When appropriate cut-offs are used, the monothermal screen can be completed with a low false-negative rate. Air is often used as a medium for caloric testing, though not without some controversy. When air and water are compared, the response magnitudes for air are consistently smaller than for water. However, the overall classification of the result appears to be generally the same regardless of the caloric medium used. Posturography: Concerns have been raised about the reliability and validity of posturographic measurements. Approaches and analysis techniques vary widely, and there is little consensus regarding the best approaches for assessment or interpretation. There is a need for standardized protocols. The portable and low-cost Nintendo Wii Balance Board (WBB) has inspired a surge in publications looking at the validity of the system for quantifying posturography and for balance training; preliminary findings are very promising., (© 2019 S. Karger AG, Basel.)

Kabuki syndrome (KS) is a rare congenital disorder (1/32000 births) characterized by distinctive facial features, intellectual disability, short stature, and dermatoglyphic and skeletal abnormalities. In the last decade, mutations in KMT2D and KDM6A were identified as a major cause of kabuki syndrome. Although genetic abnormalities have been highlighted in KS, brain abnormalities have been little explored. Here, we have investigated brain abnormalities in 6 patients with KS (4 males; M age  = 10.96 years, SD = 2.97 years) with KMT2D mutation in comparison with 26 healthy controls (17 males; M age  = 10.31 years, SD = 2.96 years). We have used MRI to explore anatomical and functional brain abnormalities in patients with KS. Anatomical abnormalities in grey matter volume were assessed by cortical and subcortical analyses. Functional abnormalities were assessed by comparing rest cerebral blood flow measured with arterial spin labeling-MRI. When compared to healthy controls, KS patients had anatomical alterations characterized by grey matter decrease localized in the bilateral precentral gyrus and middle frontal gyrus. In addition, KS patients also presented functional alterations characterized by cerebral blood flow decrease in the left precentral gyrus and middle frontal gyrus. Moreover, subcortical analyses revealed significantly decreased grey matter volume in the bilateral hippocampus and dentate gyrus in patients with KS. Our results strongly indicate anatomical and functional brain abnormalities in KS. They suggest a possible neural basis of the cognitive symptoms observed in KS, such as fine motor impairment, and indicate the need to further explore the consequences of such brain abnormalities in this disorder. Finally, our results encourage further imaging-genetics studies investigating the link between genetics, anatomical and functional brain alterations in KS., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Kabuki syndrome (KS) is a rare congenital anomaly syndrome affecting multiple organs. Two genes have been shown to be mutated in patients with KS: lysine (K)-specific demethylase 6A (KDM6A) and lysine (K)-specific methyltransferase 2D (KMT2D, formerly MLL2). Although the congenital clinical characteristic is helpful in diagnosis of the KS, there are no reports of specific findings in fetuses that might suggest the syndrome prenatally., Case Presentation: In this study, we described a male patient with a novel KDM6A splicing in exon(exon4) and flanking intron(intron3)-exon boundaries characterized by congenital hydrocephalus which had never been reported before. The male patient had inherited the c.335-1G > T splice site mutation from his mother who had fewer dysmorphic features than the patient who displayed a more severe phenotype with multiple organ involvement. Our research suggests that congenital hydrocephalus may accompany KS type 2, which improve the knowledge on KS further more., Conclusions: Based on genetic and clinical features, suggest that the c.335-1G > T splicing mutation in KDM6A causing KS-2 disease. At least for this case, we suggest that congenital hydrocephalus is closely associated with KS type 2.

A significant number of patients who seek medical treatment in an emergency department because of vertigo or dizziness, suffer from acute vestibular syndrome. This is characterized by sustained vertigo, horizontal or horizontal rotatory jerk nystagmus, and unsteady stance and gait. In the acute situation it is crucial to differentiate patients with a peripheral vestibular disorder from those with a central disease. A number of recent studies have shown that a structured clinical examination enables a reliable differential diagnosis of central or peripheral disorders. Such an examination includes the head impulse test, an alternating cover test to detect a skew deviation of the eyes, and observation of nystagmus in different positions of gaze and using Frenzel goggles. This examination is more sensitive for the detection of brainstem stroke than magnetic resonance imaging (MRI), at least within 48 h after symptom onset. As these facts are still little known, in practice there is an overuse of cost-intensive imaging with computed tomography and MRI, and a number of patient brainstem strokes in the vertebrobasilar circulation may be missed. This paper describes the relevant studies on this topic.

Objective: To investigate the morphology and dimensions of the vestibular aqueduct on axial, single-oblique and double-oblique computed tomography images., Methods: The computed tomography temporal bone scans of 112 patients were retrospectively evaluated. Midpoint and opercular measurements were performed using axial, single-oblique and double-oblique images. Morphometric analyses were also conducted. The vestibular aqueduct sizes on axial, single-oblique and double-oblique images were compared., Results: At the midpoint, the mean (± standard deviation) vestibular aqueduct measured 0.61 ± 0.23 mm, 0.74 ± 0.27 mm and 0.82 ± 0.38 mm on axial, single-oblique and double-oblique images, respectively; at the operculum, the vestibular aqueduct measured 0.91 ± 0.30 mm, 1.11 ± 0.45 mm and 1.66 ± 1.07 mm on the respective images. The co-efficients of variation of the vestibular aqueduct measured at the midpoint were 37.4 per cent, 36.5 per cent and 47.5 per cent on axial, single-oblique and double-oblique images, respectively; at the operculum, the measurements were 33.0 per cent, 40.5 per cent and 64.5 per cent. Regarding morphology, the vestibular aqueduct was fissured (33.5 per cent), tubular (64.3 per cent) or invisible (2.2 per cent)., Conclusion: The morphology and dimensions of the vestibular aqueduct were highly variable among axial, single-oblique and double-oblique images.

Kabuki syndrome (KS) is a rare disorder characterized by somatic and psychological disturbances including special face morphology, skeletal anomalies, and other systemic disorders. Because of the diverse clinical manifestation, the management of a patient with KS may involve several medical and dental specialists, including orthodontics. The aim of the article is to present successful orthodontic treatment performed in a 14-year-old boy diagnosed with the KS. Dental relations and smile aesthetics were normalized after orthodontic treatment; however, problems with patient compliance and cooperation and an increased risk of root resorption may influence treatment outcomes. Interdisciplinary cooperation between medical and dental specialists is essential in patients with KS.

We present a series of patients with vertical supranuclear gaze palsy, postural instability with falls, and progressive spasticity, who mimic Progressive Supranuclear Palsy - Richardson's syndrome (PSP-R) but have no parkinsonism, and in whom dopamine transporter imaging is normal. We suggest possible aetiologies for this constellation of symptoms, discuss the possible origin of these signs and highlight this phenotype as it may mimic atypical parkinsonism and in particular PSP., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The vestibular lesion (VL) is required to examine the physiological function of the vestibular system in animals. Toxic chemicals or electrical apparatus have been used for the VL, however, they are not ideal as they have low specificity, and can result in unintended damage, and systemic toxic effect., New Method: Localized vibration-induced VL, using an ultrasonicator, is expected to overcome the problems associated with chemical and electrical lesions. Thus, we examined the effect of the ultrasonication on the VL from the aspects of both the physiological function and histology in the present study., Results: and Comparison with Existing Method(s) Complete VL, which was evaluated by deterioration of swimming skills, righting reflex, and body stability, was induced using an ultrasonicator or electrical apparatus. Histological evaluation shows that hair cell layers in the saccule and utricle were completely destroyed in both methods Furthermore, significant drop in body mass was observed in VL. However, abscess at the cranial base was observed in VL induced by the electrical apparatus in ICR mice. Complete chemically-induced VL was observed in C57BL/6J but not ICR mice, and systemic leakage of the toxic chemicals (arsenic) was not detectable even 1day after surgery., Conclusions: Compared to the electrical apparatus, the ultrasonicator is useful for inducing VL in ICR and C57BL/6J mice, as it results in less non-specific damage. Toxic chemicals can be used for inducing VL in C57BL/6J mice; however, this method does not ensure complete disruption of the hair cells in the saccule and utricle., (Copyright © 2017 The Author(s). Published by Elsevier B.V. All rights reserved.)

Objective: The value of vestibular rehabilitation in patients with bilateral vestibular dysfunction was investigated., Materials and Methods: This study assessed 17 patients (9 males, 8 females) with bilateral vestibular dysfunction. Vestibular rehabilitation continued for 1.5 months. Videonystagmography tests (including oculomotor testing, positional testing, and caloric tests), vestibular evoked myogenic potential (VEMP) testing, and computerized dynamic posturography were performed during the pre-, mid-, and post-treatment periods. The patients underwent cranial and internal acoustic canal MRI. Consultant physicians from the neurology and physical medicine and rehabilitation departments reviewed all patients., Results: The post-treatment anteroposterior somatosensorial (APSO), anteroposterior global (APGLO), mediolateral visual (MLVI), and mediolateral global values and anteroposterior and mediolateral trials and conditions were significantly higher than those measured in the pre-treatment period. Similarly, mid-treatment values of the APSO, APGLO, and the anteroposterior sensory organization test (SOT) 2 were significantly higher than those measured in the pre-treatment period., Conclusion: Vestibular rehabilitation was effective in patients with bilateral vestibular dysfunction. As the vestibular rehabilitation duration increased, so did the efficacy of the treatment.

Objectives: The clinical approach to acute vestibular syndromes is often complex for the physician. Neurosonology offers a noninvasive method to study the cervicocephalic circulation when a vascular etiology is suspected. We aim to evaluate the diagnostic accuracy of a vascular neurosonological exam in isolated acute vestibular syndrome., Methods: All patients submitted to cerebrovascular ultrasound and magnetic resonance imaging during the period between 2011 and 2015 with acute isolated vestibular syndrome. Those with any clinical sign of brainstem lesion on presentation were excluded. All patients performed the neuroimaging study (brain computed tomography and magnetic resonance imaging) and neurologic surveillance. Neurosonological exam included all intra- and extracranial segments of the vertebrobasilar circulation. Positive ultrasound exam was defined as the presence of stenotic or occlusive disease in any of these segments related to the infarcted area., Results: A total of 108 patients were included: 60 (53.6%) were males (mean age: 60.75 years (standard deviation, 14.17)). In 27 patients (25.0%) a cerebral ischemic lesion was found to be the cause of the vertigo. Neurosonological assessment showed a sensitivity of 40.7% (95% confidence interval (CI): 22.4; 61.2), specificity of 100% (95% CI: 95.5; 100.0), positive predictive value (PPV) of 100% (95% CI: 71.5; 100.0), and negative predictive value (NPV) of 83.5% (95% CI: 74.6; 90.3)., Conclusions: Our study suggests that cerebrovascular ultrasound is a highly specific method for the diagnosis of cerebrovascular vertigo. However, its low sensitivity makes it a poor candidate for screening., (© 2017 by the American Institute of Ultrasound in Medicine.)

Background: Cerebellar infarction is an uncommon but serious cause of isolated acute vestibular symptoms, particularly in young, healthy individuals, and can easily be overlooked. We present two cases of cerebellar infarction in U.S. Air Force pilots, one of which occurred during flight., Case Reports: A 41-yr-old man developed acute vertigo, disequilibrium, nausea, and headache, with progressive slow symptomatic improvement, and presented to medical attention 4 d after symptom onset. Brain magnetic resonance imaging showed right inferomedial cerebellar infarction. Echocardiography discovered patent foramen ovale and atrial septal aneurysm. A 40-yr-old man developed severe vertigo, nausea, and vomiting during initial aircraft descent. Head computed tomography scan was performed acutely and was normal. Initial assessment was benign paroxysmal positional vertigo. Brain magnetic resonance imaging 1 mo after symptom onset showed a small right inferior cerebellar infarction. Patent foramen ovale and bilateral atrial enlargement were seen on echocardiography. Both pilots made full neurological recoveries and were eventually returned to flight status., Discussion: Central causes of isolated acute vestibular symptoms are uncommon and are often not considered in otherwise healthy individuals. Cerebellar infarction is one of these uncommon but increasingly recognized causes of acute vestibular symptoms. As evaluation and management of central causes are much different from peripheral conditions, prompt localization confirmation is paramount. Accurate evidence-based bedside screening methods are available for rapid localization. Awareness of the possibility of central etiologies and careful clinical evaluation with application of bedside screening methods in patients with acute vestibular symptoms will reduce the number of inaccurate diagnoses.Hesselbrock RR. Cerebellar infarction presenting with acute vestibular syndrome in two U.S. Air Force pilots. Aerosp Med Hum Perform. 2017; 88(9):880-883.

Objective: To analyze and summarize the effect of bilateral large vestibular aqueducts in peripheral vestibular organ function., Methods: Eighteen patients with bilateral large vestibular aqueduct syndrome (LVAS; Study Group) and 18 healthy volunteers (Control Group) were investigated using audiometry, caloric test, sensory organization test (SOT), and vestibular-evoked myogenic potential (VEMP) tests., Results: All 18 patients (36 ears) exhibited sensorineural hearing loss. For cervical VEMP (cVEMP), the Study Group showed lower thresholds (Study Group vs., Control Group: 71.4vs. 75.3dBnHL; p=0.006), N1 latencies (24.1vs. 25.2ms; p=0.026) and shorter P1 (15.3vs. 16.6ms; p=0.003), and higher amplitudes (400.7vs. 247.2µV; p<0.001) than the Control Group. For ocular VEMP (oVEMP), the Study Group had lower thresholds (79.3vs. 81.8dBnHL; p=0.046) and higher amplitudes (40.6vs. 14.4µV; p<0.001) than the Control Group. Fourteen of 16 patients (87.5%) who completed caloric tests had abnormal results, and 10 of 18 patients (55.6%) exhibited abnormal results in SOTs., Conclusions: The hyperfunction of vestibular test in otolithic organs and the hypofunction of vestibular test in semicircular canals, as well as the dysfunction in the balance test were demonstrated in patients with LVAS., Significance: Our findings can help clinicians gain a better understanding of the characteristics of vestibular organ function in patients with LVAS, which can facilitate optimal targeted treatment., (Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Objectives: Endolymphatic hydrops (EH) can be studied in patients by MRI. With the semi-quantitative grading system, previous imaging studies showed discrepancies in the occurrence and grading of EH in patients with Meniere's disease (MD). Here, we compared the inversion of the saccule to utricle area ratio (SURI) with the semi-quantitative method of grading conventionally used to diagnose MD., Methods: Imaging was carried out on a 3-T MRI scanner. We performed 3D-FLAIR sequences 4 h after a single intravenous dose of contrast agent. Two radiologists independently studied the morphology of the inner ear structures in the healthy subjects and MD patients. Each subject was then graded on the basis of the EH semi-quantitative analysis and on saccular morphology using axial and sagittal reference slices in the vestibule plane., Results: Thirty healthy subjects and 30 MD patients had MRI scans. Using the semi-quantitative method, we found no significant difference in the number of subjects with EH between the two groups. SURI was found in 15 out of 30 MD patients and in none of the 30 healthy subjects. In three MD patients the saccule was not visible., Conclusion: SURI is currently the most specific criterion for imaging diagnosis of MD., Key Points: • Half of MD patients presented with inversion of the saccule to utricle ratio. • Saccular analysis is crucial when assessing patients with Meniere's disease. • In some patients, the saccule is not visible, suggestive of intra-labyrinthine fistulae.

Our objective was to assess the capability of computerized dynamic posturography (CDP) to evaluate dizziness in blind patients who lack corneoretinal potential. We performed a retrospective analysis of 2 young blind subjects with intraorbital scarring who complained of dizziness and underwent CDP. Tests were carried out at our center for balance disorders using the NeuroCom's EquiTest System version 4.0. CDP demonstrated vestibular dysfunction and revealed it to be severe. We conclude that CDP can serve as a potentially reliable tool for assessing dizziness in blind patients who have no corneoretinal potential.

Introduction: We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy., Methods: The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compared with 7 age- and gender-matched controls and with the mean CSA of our reference population., Results: The nerve CSA of CANVAS patients was significantly smaller than that of controls at all sites (P < 0.01). All but 1 individual measurement of CSA at the mid-forearm level in the CANVAS patients fell outside the normal control range and was >2 standard deviations below the reference mean., Conclusions: The small nerves in CANVAS probably reflect nerve thinning from axonal loss secondary to ganglion cell loss. Our data show a role for ultrasound in the diagnosis of CANVAS ganglionopathy. This may also be applicable to ganglionopathy from other causes. Muscle Nerve 56: 160-162, 2017., (© 2016 Wiley Periodicals, Inc.)

Introduction: The aim of this study was to investigate the effects of high intensity resistance circuit (HIRC) and traditional strength training (TST) on neuromuscular fatigue and metabolic responses., Methods: Twelve trained young subjects performed HIRC and TST in a counterbalanced order with 1 week rest in-between. The amount of workload and the inter-set time for each local muscle group were matched (180 s), however, the time between successive exercises differed. The twitch interpolation technique was used to test neuromuscular function of the knee extensor muscles. Blood lactate concentration was used to evaluate metabolic responses., Results: Maximum voluntary contraction and resting potentiated twitch amplitude (Q tw ) were significantly reduced after HIRC, but there were not changes after TST, while reductions in voluntary activation were similar. Lactate concentration increased significantly more after HIRC., Conclusions: The higher lactate concentration after HIRC probably impaired excitation-contraction coupling, indicating larger peripheral fatigue than after TST. Muscle Nerve 56: 152-159, 2017., (© 2017 Wiley Periodicals, Inc.)

A 5 year-old boy was rather healthy until one month ago when loss of balance and muscle tone was noted by the parents. A battery of tests comprising electroencephalography, audiometry, caloric test and ocular vestibular evoked myogenic potential (oVEMP) test were performed, yet all tests showed normal responses. Conversely, cervical VEMP (cVEMP) test showed delayed responses bilaterally. MR imaging demonstrated bilaterally intrameatal vascular loops extending into both internal auditory canals. After a 3-month medication, delayed cVEMPs were resolved and returned to normal cVEMPs bilaterally, accompanied by subsidence of dysequilibrium., (Copyright © 2017 Elsevier B.V. All rights reserved.)

We sought to determine the cerebellar structures responsible for tilt suppression of post-rotatory nystagmus. We investigated ocular motor findings and MRI lesions in 73 patients with isolated cerebellar lesions who underwent recording of the vestibulo-ocular reflex (VOR) using rotatory chair tests. Tilt suppression of post-rotatory nystagmus was diminished in 27 patients (27/73, 37.0 %). The gains of the VOR and the TCs of per- and post-rotatory nystagmus did not differ between the patients with diminished and with normal tilt suppression. The patients with impaired tilt suppression showed perverted ("cross-coupled") head-shaking nystagmus (pHSN) and central positional nystagmus (CPN) more frequently than those with normal responses. Tilt suppression was impaired in five (71.4 %) of the seven patients with isolated nodulus and uvular infarction. Probabilistic lesion-mapping analysis showed that the nodulus and uvula are responsible for tilt suppression. Impaired tilt suppression may be ascribed to disruption of cerebellar contribution to the vestibular velocity-storage mechanism, which integrates information from the semicircular canals and otolith organs to help derive the brain's estimate of the head orientation relative to the pull of gravity.