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The latest medical research on Otology Neurotology

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Endovascular therapy versus best medical management in distal medium middle cerebral artery acute ischaemic stroke: a multinational multicentre propensity score-matched study.

Neurology, Neurosurgery and Psychiatry

The efficacy of endovascular treatment (EVT) in acute ischaemic stroke due to distal medium vessel occlusion (DMVO) remains uncertain. Our study aimed to evaluate the safety and efficacy of EVT compared with the best medical management (BMM) in DMVO.

In this prospectively collected, retrospectively reviewed, multicentre cohort study, we analysed data from the Multicentre Analysis of primary Distal medium vessel occlusions: effect of Mechanical Thrombectomy registry. Patients with acute ischaemic stroke due to DMVO in the M2, M3 and M4 segments who underwent EVT or received BMM were included. Primary outcome measures comprised 10 co-primary endpoints, including functional independence (mRS 0-2), excellent outcome (mRS 0-1), mortality (mRS 6) and haemorrhagic complications. Propensity score matching was employed to balance the cohorts.

Among 2125 patients included in the primary analysis, 1713 received EVT and 412 received BMM. After propensity score matching, each group comprised 391 patients. At 90 days, no significant difference was observed in achieving mRS 0-2 between EVT and BMM (adjusted OR 1.00, 95% CI 0.67 to 1.50, p>0.99). However, EVT was associated with higher rates of symptomatic intracerebral haemorrhage (8.4% vs 3.0%, adjusted OR 3.56, 95% CI 1.69 to 7.48, p<0.001) and any intracranial haemorrhage (37% vs 19%, adjusted OR 2.61, 95% CI 1.81 to 3.78, p<0.001). Mortality rates were similar between groups (13% in both, adjusted OR 1.48, 95% CI 0.87 to 2.51, p=0.15).

Our findings suggest that while EVT does not significantly improve functional outcomes compared with BMM in DMVO, it is associated with higher risks of haemorrhagic complications. These results support a cautious approach to the use of EVT in DMVO and highlight the need for further prospective randomised trials to refine treatment strategies.

Sodium valproate is associated with cortical thinning of disease-specific areas in juvenile myoclonic epilepsy.

Neurology, Neurosurgery and Psychiatry

Juvenile myoclonic epilepsy (JME) is associated with cortical thinning of the motor areas. The relative contribution of antiseizure medication to cortical thickness is unknown. We aimed to investigate how valproate influences the cortical morphology of JME.

In this cross-sectional study, individuals with JME with and without valproate, with temporal lobe epilepsy (TLE) with valproate and controls were selected through propensity score matching. Participants underwent T1-weighted brain imaging and vertex-wise calculation of cortical thickness.

We matched 36 individuals with JME on valproate with 36 individuals with JME without valproate, 36 controls and 19 individuals with TLE on valproate. JME on valproate showed thinning of the precentral gyri (left and right, p<0.001) compared with controls and thinning of the left precentral gyrus when compared with JME not on valproate (p<0.01) or to TLE on valproate (p<0.001). Valproate dose correlated negatively with the thickness of the precentral gyri, postcentral gyri and superior frontal gyrus in JME (left and right p<0.0001), but not in TLE.

Valproate was associated with JME-specific and dose-dependent thinning of the cortical motor regions. This suggests that valproate is a key modulator of cortical morphology in JME, an effect that may underlie its high efficacy in this syndrome.

Socioeconomic burden of AQP4-antibody seropositive NMOSD: a nationwide registry-based study.

Neurology, Neurosurgery and Psychiatry

AQP4-antibody seropositive (AQP4-Ab+) neuromyelitis optica spectrum disorder (NMOSD) may cause reduced work capability due to disability. Here, we evaluated the socioeconomic status of patients with AQP4-Ab+NMOSD in off-label therapy era compared with the general population.

A longitudinal nationwide population-based study including all Danish patients with AQP4-Ab+NMOSD and matched controls from the general population. The cohort was linked to other Danish nationwide population-based databases. The study period was from 1992 to 2021. The main outcomes were loss of income from salary, limited work capability, disability pension and civil status. The longitudinal risks of outcomes were presented in cumulative incidence curves. Fisher's exact test, χ2 test or Wilcoxon test were applied for comparison.

We included 65 patients with a median follow-up of 8.6 years. Annual income declined significantly after disease onset (index year) compared with the general population. One year after the index year, the median annual income in 2015-indexed Euro for patients averaged 13 285 (IQR: 139 to 36 336) versus controls 33 035 (IQR: 6870 to 45 978); p=0.04. Five years postindex year, the average income for patients further dropped to 276 (IQR: 0 to 23 691) versus controls 22 141 (IQR: 0 to 42 986); p=0.03. At the end of follow-up, significantly higher proportion of patients were either in 'flexjob' (36.9% patients vs 14% controls, p<0.00) or receiving disability pension (16.9% patients vs 4.3% controls, p<0.00).

The socioeconomic status of patients with AQP4-Ab+NMOSD deteriorates rapidly following disease onset. A substantial proportion of these patients lose their work capacity leading to increased financial burden on both their families and society.

The evolving contribution of MRI measures towards the prediction of secondary progressive multiple sclerosis.

Neurology, Neurosurgery and Psychiatry

In multiple sclerosis (MS), both lesion accrual and brain atrophy predict clinical outcomes. However, it is unclear whether these prognostic features are equally relevant throughout the course of MS. Among 103 participants recruited following a clinically isolated syndrome (CIS) and followed up over 30 years, we explored (1) whether white matter lesions were prognostically more relevant earlier and brain atrophy later in the disease course towards development of secondary progressive (SP) disease; (2) if so, when the balance in prognostic contribution shifts and (3) whether optimised prognostic models predicting SP disease should include different features dependent on disease duration.

Binary logistic regression models were built using age, gender, brain lesion counts and locations, and linear atrophy measures (third ventricular width and medullary width) at each time point up to 20 years, using either single time point data alone or adjusted for baseline measures.

By 30 years, 27 participants remained CIS while 60 had MS (26 SPMS and 16 MS-related death). Lesions counts were prognostically significant from baseline and at all later time points while linear atrophy measure models reached significance from 5 years. When adjusted for baseline, in combined MRI models including lesion count and linear atrophy measures, only lesion counts were significant predictors. In combined models including relapse measures, Expanded Disability Status Scale scores and MRI measures, only infratentorial lesions were significant predictors throughout.

While SPMS progression is associated with brain atrophy, in predictive models only infratentorial lesions were consistently prognostically significant.

Machine learning classification of functional neurological disorder using structural brain MRI features.

Neurology, Neurosurgery and Psychiatry

Brain imaging studies investigating grey matter in functional neurological disorder (FND) have used univariate approaches to report group-level differences compared with healthy controls (HCs). However, these findings have limited translatability because they do not differentiate patients from controls at the individual-level.

183 participants were prospectively recruited across three groups: 61 patients with mixed FND (FND-mixed), 61 age-matched and sex-matched HCs and 61 age, sex, depression and anxiety-matched psychiatric controls (PCs). Radial basis function support vector machine classifiers with cross-validation were used to distinguish individuals with FND from HCs and PCs using 134 FreeSurfer-derived grey matter MRI features.

Patients with FND-mixed were differentiated from HCs with an accuracy of 0.66 (p=0.005; area under the receiving operating characteristic (AUROC)=0.74); this sample was also distinguished from PCs with an accuracy of 0.60 (p=0.038; AUROC=0.56). When focusing on the functional motor disorder subtype (FND-motor, n=46), a classifier robustly differentiated these patients from HCs (accuracy=0.72; p=0.002; AUROC=0.80). FND-motor could not be distinguished from PCs, and the functional seizures subtype (n=23) could not be classified against either control group. Important regions contributing to statistically significant multivariate classifications included the cingulate gyrus, hippocampal subfields and amygdalar nuclei. Correctly versus incorrectly classified participants did not differ across a range of tested psychometric variables.

These findings underscore the interconnection of brain structure and function in the pathophysiology of FND and demonstrate the feasibility of using structural MRI to classify the disorder. Out-of-sample replication and larger-scale classifier efforts incorporating psychiatric and neurological controls are needed.

Validation of the hotspot for dorsolateral subthalamic nucleus targeting in deep brain stimulation surgery for Parkinson's disease: a post hoc analysis of a randomised controlled trial.

Neurology, Neurosurgery and Psychiatry

Visualisation of the dorsolateral subthalamic nucleus (STN) remains challenging on 1.5 and 3Tesla T2-weighted MRI. Our previously defined hotspot, relative to the well-visualised medial STN border, serves as an MRI landmark for dorsolateral STN identification in deep brain stimulation (DBS). We aimed to validate this hotspot in a separate trial cohort of Parkinson's disease (PD) patients and refine its location.

In this post hoc analysis of a randomised controlled trial, in which the hotspot was taken into account during target planning, responses to DBS were evaluated using hemibody improvement on the Movement Disorder Society-Unified Parkinson's Disease Rating Scale motor examination and compared with our historical cohort, as well as dopaminergic medication reduction. Then, a refined hotspot was calculated and the Euclidean distance from individual active contacts to the refined hotspot was correlated with motor improvement.

The first quartile of the hemibodies (poor responders) showed an average improvement of 13%, which was higher than the -8% in the historical control group (p=0.044). Dopaminergic medication reduction was greater in the current cohort compared with the historical cohort (p=0.020). Overall variability of hemibody motor improvement was reduced in the current cohort compared with the historical control group (p=0.003). Motor improvement correlated to the Euclidean distance from active contact to the refined hotspot (2.8 mm lateral, 1.1 mm anterior and 2.2 mm superior to the medial STN border) (p=0.001).

We validated the hotspot for dorsolateral STN targeting in DBS for patients with PD and showed an improved motor response in poor responders, a reduced variability in motor improvement and a greater dopaminergic medication reduction. We then refined the hotspot at 2.8 mm lateral, 1.1 mm anterior and 2.2 mm superior relative to the medial STN border, which visualises a readily implementable target within the dorsolateral STN on lower field strength MRI.

Sudden Hearing Loss Before, During, and After the Pandemic: Investigating COVID-19 Illness and Vaccine-Related Symptoms.

Audiology and Neuro-Otology

Accumulating reports suggest an increase in sudden sensorineural hearing loss during the COVID-19 pandemic and vaccination periods. However, clear evidence is lacking. The goal of this study was to determine if sudden sensorineural hearing loss is associated with COVID-19 illness or its vaccine.

Retrospective chart review of fifty randomly selected patients from three, 6-month time periods: "pre-pandemic", "early pandemic", and "late pandemic". Group comparisons were performed for demographics, comorbid conditions, audiologic history, audiometric data, speech reception thresholds, and word recognition.

One hundred fifty patients were included in this study. A mean difference was observed in that the relative percentage of sensorineural hearing loss (SNHL) cases increased over time, corresponding to a relative decrease in conductive hearing loss cases. However, this change was not explained by proportional changes in sudden SNHL. Patients in the early pandemic time period were more likely to report tinnitus. Otherwise, the patient groups did not differ on demographic variables, hearing health history, hearing loss presentation, pure tone averages, speech reception thresholds, or word recognition performance.

Proportion of patients with sudden sensorineural hearing loss did not change over time from the pre-pandemic period to the early or late pandemic phases. Despite a randomized sample, these findings do not support the hypothesis that COVID-19 illness or vaccine is associated with sudden sensorineural hearing loss.

Exploring CHAPS as a Potential Measurement for Auditory Processing and Cognitive Ability in Children with Hearing Loss.

Audiology and Neuro-Otology

The primary goal is to investigate the suitability of CHAPS for assessing cognitive abilities and auditory processing in people with hearing loss, specifically in the domains of auditory processing, verbal working memory, and auditory attention.

The study comprised 44 individuals between the ages of seven and 14, 22 with hearing loss (N = 11 males), and 22 with normal hearing (N = 10 males). Individuals' auditory attention, working memory, and auditory processing skills were assessed in the study, and self-report questionnaires were used. The evaluation utilized the Sustained Auditory Attention Capacity Test (SAACT), Working Memory Scale (WMS), Filtered Words Test, Auditory Figured Ground Test (AFGT), and the Children's Auditory Performance Scale (CHAPS). Analyses were conducted, including group comparisons, correlation examinations, and Receiver Operating Characteristic (ROC) evaluations.

There were significant differences in CHAPS total, attention, noise, quiet, and multiple inputs between groups. No significant differences were seen in CHAPS_ideal and CHAPS_auditory memory across groups. The study of SAACT and its subscores, WMS and its subscores, FWT, and AFGT revealed a significant difference between groups, caused by the poor performance of persons in the HL group compared to those in the NH group. The SAACT and its subscores correlated significantly with CHAPS_attention. The AUC calculation showed that The SAACT and CHAPS_attention distinguished persons with or without hearing loss (p&lt;0.05). WMS_stm and WMS_total correlated with CHAPS auditory memory subscale, however WMS_vwm did not. AUC values for WMS and its subscores showed significant discrimination in identifying children with or without hearing loss (p&lt;0.05), whereas CHAPS_auditory memory did not (AUC=0.665; p=0.060). FWT and AFGT had a significant relationship with CHAPS_noise and CHAPS_multiple inputs subscales. The CHAPS_quiet and CHAPS_ideal subtests only correlated with AFGT. CHAPS_quite and CHAPS_ideal did not exhibit significant discriminative values (p&lt;0.05) for identifying children with or without hearing loss, while CHAPS_noise, CHAPS_multiple inputs, FWT, and AFGT did.

The CHAPS_attention subscale could be a trustworthy instrument for assessing auditory attention in children with hearing loss. However, the CHAPS_auditory memory subscale may not be suitable for testing working memory. While performance-based auditory processing tests showed improved discrimination, the CHAPS_noise and CHAPS_multiple inputs subtests can still assess hearing-impaired auditory processing. The CHAPS_quiet and CHAPS_ideal subtests may not evaluate auditory processing.

Data-driven sequence of cognitive decline in people with Parkinson's disease.

Neurology, Neurosurgery and Psychiatry

Understanding the sequential progression of cognitive impairments in Parkinson's disease (PD) is crucial for elucidating neuropathological underpinnings, refining the assessment of PD-related cognitive decline stages and enhancing early identification for targeted interventions. The first aim of this study was to use an innovative event-based modeling (EBM) analytic approach to estimate the sequence of cognitive declines in PD. The second aim was to validate the EBM by examining associations with EBM-derived individual-specific estimates of cognitive decline severity and performance on independent cognitive screening measures.

This cross-sectional observational study included 99 people with PD who completed a neuropsychological battery. Individuals were classified as meeting the criteria for mild cognitive impairment (PD-MCI) or subtle cognitive decline by consensus. An EBM was constructed to compare cognitively healthy individuals with those with PD-MCI or subtle cognitive disturbances. Multivariable linear regression estimated associations between the EBM-derived stage of cognitive decline and performance on two independent cognitive screening tests.

The EBM estimated that tests assessing executive function and visuospatial ability become abnormal early in the sequence of PD-related cognitive decline. Each higher estimated stage of cognitive decline was associated with approximately 0.24 worse performance on the Dementia Rating Scale (p<0.001) and 0.26 worse performance on the Montreal Cognitive Assessment (p<0.001) adjusting for demographic and clinical variables.

Findings from this study will have important clinical implications for practitioners, on specific cognitive tests to prioritise, when conducting neuropsychological evaluations with people with PD. Results also highlight the importance of frontal-subcortical system disruption impacting executive and visuospatial abilities.

Complete nanopore repeat sequencing of SCA27B (GAA-FGF14 ataxia) in Japanese.

Neurology, Neurosurgery and Psychiatry

Although pure GAA expansion is considered pathogenic in SCA27B, non-GAA repeat motif is mostly mixed into longer repeat sequences. This study aimed to unravel the complete sequencing of FGF14 repeat expansion to elucidate its repeat motifs and pathogenicity.

We screened FGF14 repeat expansion in a Japanese cohort of 460 molecularly undiagnosed adult-onset cerebellar ataxia patients and 1022 controls, together with 92 non-Japanese controls, and performed nanopore sequencing of FGF14 repeat expansion.

In the Japanese population, the GCA motif was predominantly observed as the non-GAA motif, whereas the GGA motif was frequently detected in non-Japanese controls. The 5'-common flanking variant was observed in all Japanese GAA repeat alleles within normal length, demonstrating its meiotic stability against repeat expansion. In both patients and controls, pure GAA repeat was up to 400 units in length, whereas non-pathogenic GAA-GCA repeat was larger, up to 900 units, but they evolved from different haplotypes, as rs534066520, located just upstream of the repeat sequence, completely discriminated them. Both (GAA)≥250 and (GAA)≥200 were enriched in patients, whereas (GAA-GCA)≥200 was similarly observed in patients and controls, suggesting the pathogenic threshold of (GAA)≥200 for cerebellar ataxia. We identified 14 patients with SCA27B (3.0%), but their single-nucleotide polymorphism genotype indicated different founder alleles between Japanese and Caucasians. The low prevalence of SCA27B in Japanese may be due to the lower allele frequency of (GAA)≥250 in the Japanese population than in Caucasians (0.15% vs 0.32%-1.26%).

FGF14 repeat expansion has unique features of pathogenicity and allelic origin, as revealed by a single ethnic study.

Academic Outcomes With Hearing Amplification Devices in Children With Unilateral Hearing Loss: A Systematic Review and Narrative Synthesis.

Audiology and Neuro-Otology

Many studies have shown increased academic problems in children with unilateral hearing loss (UHL). However, whether hearing devices can ameliorate the educational difficulties associated with UHL is not well studied. Therefore, the objective of the current systematic review was to answer the question: Do non-surgical amplification devices, bone-anchored hearing aids, and/or cochlear implants improve academic outcomes in school-aged children and adolescents with UHL?

Embase, MEDLINE, Scopus, CINAHL, APA PsycInfo, ClinicalTrials.gov, and Cochrane databases were searched from inception to 12/21/22. Published, peer-reviewed studies comparing academic outcomes in patients with UHL aged ≥ 5 and ≤ 19 years with and without hearing devices (non-surgical amplification devices, bone-anchored hearing aids, or cochlear implants) were included. Results of studies were qualitatively synthesized, and the risk of bias was evaluated with the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool.

A total of 5,644 non-duplicate publications were identified by the search, and four studies were included for synthesis, every one of which was investigating non-surgical amplification. One small, single-arm study demonstrated significant improvement in subjective classroom listening difficulties after a 3- to 4-month trial with a BTE hearing aid. The other three studies of non-surgical amplification devices showed no benefit across multiple academic outcomes with FM systems and conventional and CROS-style hearing aids.

The small sample sizes, heterogeneous and/or ill-defined study samples, and overall low quality of the available literature ultimately make it hard to draw definitive conclusions regarding non-surgical amplification devices' effectiveness in improving academic outcomes in children with UHL. No articles were identified that studied cochlear implants or bone-anchored hearing aids. Further studies with high-quality study design, large sample sizes, and long-term follow-up are needed to answer this clinically important question.

Development of a semi-automated approach for the quantification of neuronal cells in the spiral ganglion of the whole implanted gerbil cochlea, acquired by light-sheet microscopy.

Audiology and Neuro-Otology

Assessing cochlear implantation's impact on cell loss and preventing post-implant cochlear damage are key areas of focus for hearing preservation research. The preservation of auditory neuronal and sensory neural hearing cells has a positive impact on auditory perception after implantation. This study aimed to provide details on a semi-automated spiral ganglion neuronal cell counting method, developed using whole implanted gerbil cochlea acquisitions with light sheet microscopy.

Mongolian gerbils underwent right cochlear implantation with an electrode array whose silicone was loaded with dexamethasone or not and were euthanized 10 weeks after implantation. The cochleae were prepared according to a 29-day protocol, with the electrode array in place. Light sheet microscopy was used for acquisition, and Imaris software was employed for three-dimensional analysis of the cochleas and semi-automatic quantification of spiral ganglion cells. The imaJ software was used for manual quantification of these cells.

Six cochleae were acquired by light-sheet microscopy, allowing good identification of cells. There was no significant difference between the mean number of spiral ganglion cell obtained by manual and semi-automatic counting (p = 0,25).

Light sheet microscopy provided complete visualization of the spiral ganglion and cell identification. The semi-automated counting method developed using Imaris software tools proved reliable and efficient and could be applied to a larger sample to assess post-cochlear implant cell damage and the efficacy of protective drugs delivered to the inner ear.