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Objective: To investigate (1) the current level of awareness and knowledge on Auditory Processing Disorder (APD) among Audiologists and other hearing healthcare professionals; (2) current practices in screening, diagnosis, and management of APD in children and adults across the UK; (3) professional's acceptance of APD assessment and diagnosis., Design: An online survey was disseminated through the British Academy of Audiology and ENT UK., Study Sample: A total of 191 hearing healthcare professionals responded to the survey., Results: Overall, while 63% of the respondents considered themselves to be adequately informed about APD, only 4% viewed themselves as very informed on the topic. Fewer than half of the respondents report screening (31%), diagnosing (14%), or managing (36%) cases of APD. For screening APD, professionals most commonly use auditory processing tests in adults and take case histories in children, whereas routine audiological procedures are the primary method for diagnosing APD in both adults and children. Although modifying the listening environment is a widely recommended management strategy for APD, half of the respondents indicated that a diagnosis of APD has no implications for patient management., Conclusions: There is a critical need to promote APD-related training to ensure they can provide appropriate referrals and management.

Acute vertigo or dizziness is a frequent presentation to the emergency department (ED), making up between 2.1% and 4.4% of all consultations. Given the nature of the ED where the priority is triage, diagnostic delays and misdiagnoses are common, with as many as a third of vertebrobasilar strokes presenting with acute vertigo or dizziness being missed. Here, we review diagnostic errors identified in the evaluation and treatment of the acutely dizzy patient and discuss strategies to overcome them. Lessons learned include focusing on structured history taking, asking about timing and triggers to inform a targeted examination, assessing subtle ocular motor findings (e.g., by use of HINTS(+)), and avoiding overreliance on brain imaging (including early magnetic resonance imaging including diffusion-weighted sequences [DWI-MRI]). Importantly, up to 20% of DWI-MRI may be false negatives if obtained within the first 24-48 h after symptom onset. Likewise, overreliance on focal neurologic findings to confirm a stroke diagnosis should be avoided because isolated dizziness, vertigo, or even unsteadiness may be the only symptoms in some patients with vertebrobasilar stroke. Furthermore, in patients with triggered episodic vestibular symptoms provocation maneuvers should be preferred over HINTS(+), and a potential diagnosis of stroke should not be immediately dismissed in younger patients presenting with a headache (where migraine may be more common), but the possibility of a vertebral artery dissection should be further evaluated. Importantly, moderate training of non-experts allows for significant improvement in diagnostic accuracy in the acutely dizzy patient and thus should be prioritized.

Background: Stroke is the second leading cause of disability worldwide. Stroke results in focal neurological deficit and often leads to auditory problems due to its impact on the auditory pathway. Altered connections in the auditory pathway, caused by stroke, can result in hearing difficulties ranging from impaired sound detection to altered auditory perception. A better understanding of how stroke affects these early sound processing mechanisms will provide valuable insights into stroke recovery and rehabilitation options., Methods: We recruited forty consecutive adult patients with stroke (30 males, 10 females) due to ischemic or intracerebral hemorrhage > 3 and up to 12 months after stroke (subacute stage). Brain MRIs were performed on all patients, and we calculated a central auditory nervous system stroke severity index (CANS SSI) according to number of CANS areas involved and an extended CANS definition of auditory responsive areas. All patients underwent cognitive screening assessment, basic audiological assessments, and a hierarchical central auditory processing assessment battery with the Queen Square Tests of Auditory Cognition (early perceptual processing, apperceptive processing, semantic Processing) and Gaps in Noise tests., Results: When comparing patients with auditory responsive cortical lesions and with versus without Heschl's gyrus involvement (primary auditory cortex), patients with Heschl's gyrus involvement exhibited worse early perceptual scores. The CANS SSI showed a significant negative correlation with early perceptual test scores., Conclusion: This study demonstrates a correlation between stroke severity, characterized by a higher number of lesions involving auditory areas in patients with subacute stroke, and worse early perceptual scores. Heschl's gyrus involvement is associated with poorer early perceptual score., Competing Interests: Declarations. Conflicts of interest: None declared., (© 2024. The Author(s).)

Hearing is multifaceted, and the relative contributions of peripheral and central hearing loss are rarely considered together in the context of dementia. Here, we assessed peripheral (as measured with pure-tone audiometry) and central (as measured with dichotic listening) hearing in 19 patients with typical amnestic Alzheimer's disease (tAD), 10 patients with logopenic variant primary progressive aphasia (lvPPA), 11 patients with nonfluent/agrammatic variant PPA (nfvPPA), 15 patients with semantic variant PPA (svPPA), and 28 healthy age-matched individuals. Participants also underwent neuropsychological assessment and magnetic resonance image scanning, allowing us to use voxel-based morphometry to assess associations between hearing scores and grey matter volume. Dichotic listening was impaired in all patient groups relative to healthy controls. In the combined patient (but not healthy control) cohort, dichotic listening scores were significantly correlated with measures of global cognitive functioning and speech-based neuropsychological tasks. Pure-tone audiometry scores were not significantly elevated in any patient group relative to the healthy control group, and no significant correlations were observed between peripheral hearing and neuropsychological task performance in either the combined patient or healthy control cohorts. Neuroanatomically, dichotic listening performance was associated with grey matter volume in a bilateral fronto-temporoparietal network over the combined patient cohort, but no correlates were identified for pure-tone audiometry. Our findings highlight the importance of speech parsing mechanisms beyond elementary sound detection in driving cognitive test performance, underline the importance of assessing central hearing alongside peripheral hearing in people with dementia, and further delineate the complex auditory profiles of neurodegenerative dementias., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Primary mitochondrial diseases, with diverse systemic manifestations, often present with auditory impairments due to mitochondrial dysfunction. This study provides an in-depth exploration of auditory deficits in primary mitochondrial diseases, highlighting the impact of various pathogenic variants on both cochlea and neural/central auditory functions. An observational study involving 72 adults with primary mitochondrial diseases was conducted. Participants underwent extensive audiological evaluations including pure-tone audiometry, tympanometry, acoustic reflex thresholds, quick speech-in-noise test, listening in spatialized noise-sentences test, auditory-evoked brainstem responses and distortion product otoacoustic emissions. Multivariate analysis of covariance and logistic regression analyses assessed the influence of various pathogenic DNA variants, accounting for age, cognitive status via the Montreal Cognitive Assessment and disease severity through the Newcastle Mitochondrial Disease Adult Scale. Participants with the pathogenic m.3243A>G/T variants (m.3243A>G n = 40; m.3243A>T n = 1) exhibited significant elevations in pure-tone audiometry thresholds, especially at high frequencies, suggesting cochlea involvement. Notably, the listening in spatialized noise-sentences test showed significant spatial processing deficits in the m.3243A>G/T group, possibly indicating a unique mutation-specific impact on central auditory processing. Auditory-evoked brainstem response results highlighted a higher likelihood of auditory brainstem response abnormalities in this group, further substantiating neural/central auditory pathway involvement. This study emphasizes the heterogeneous nature of hearing impairment in primary mitochondrial diseases, with a genotype-phenotype correlation, particularly in the m.3243A>G/T group. These insights advocate for personalized, genotype-specific auditory assessments and targeted management strategies. Conventional hearing aids and cochlear implants are ineffective for those with central auditory dysfunctions related to mitochondrial mutations. There is an urgent need for innovative rehabilitation strategies catering for both cochlear and neural/central auditory pathways., Competing Interests: The authors report no competing interests., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Background: Vestibular Migraine (VM) is a prevalent vestibular disorder, affecting up to 2.7% of the general population. Despite the establishment of diagnostic criteria by the Bárány Society and its inclusion in the International Classification of Headache Disorders, the clinical diagnosis of VM remains challenging due to its complex pathophysiology and symptom overlap with other dizziness disorders. Motion sickness is a core feature of migraine and can be interrogated through simple questionnaires., Objective: This study aims to identify to what extent motion sensitivity can predict VM compared to other causes of dizziness., Methods: We conducted a cross-sectional study involving 113 patients from the vestibular neurology clinics at University College London Hospitals. Participants were categorized into VM, Persistent Postural Perceptual Dizziness (PPPD), combined VM and PPPD, and 'other' dizziness etiologies. Data on motion sickness history and dizziness during car travel were collected through structured interviews and analyzed using logistic regression to assess the predictive value of these symptoms for VM., Results: A substantial portion of patients with VM (91.2%) reported nausea or dizziness when reading as a passenger, a symptom significantly more prevalent than in those with PPPD or other dizziness diagnoses. Logistic regression indicated that VM patients are significantly more likely to experience these symptoms compared to non-VM patients, with an odds ratio suggesting a strong predictive value for this symptom in diagnosing VM., Conclusion: The findings highlight increased motion sensitivity while reading in a moving vehicle as a promising diagnostic tool for VM, offering a practical aid in clinical settings to distinguish VM from other vestibular disorders., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Rova, Joffily, Carvalho, Cortese, Koohi and Kaski.)

The induction of immunological tolerance is a promising strategy for managing autoimmune diseases, allergies, and transplant rejection. Tregitopes, a class of peptides, have emerged as potential agents for this purpose. They activate regulatory T cells, which are pivotal in reducing inflammation and promoting tolerance, by binding to MHC II molecules and facilitating their processing and presentation to Treg cells, thereby encouraging their proliferation. Moreover, Tregitopes influence the phenotype of antigen-presenting cells by attenuating the expression of CD80, CD86, and MHC class II while enhancing ILT3, resulting in the inhibition of NF-kappa B signaling pathways. Various techniques, including in vitro and in silico methods, are applied to identify Tregitope candidates. Currently, Tregitopes play a vital role in balancing immune activation and tolerance in clinical applications such as Pompe disease, diabetes-related antigens, and the prevention of spontaneous abortions in autoimmune diseases. Similarly, Tregitopes can induce antigen-specific regulatory T cells. Their anti-inflammatory effects are significant in conditions such as autoimmune encephalomyelitis, inflammatory bowel disease, and Guillain-Barré syndrome. Additionally, Tregitopes have been leveraged to enhance vaccine design and efficacy. Recent advancements in understanding the potential benefits and drawbacks of IVIG and the discovery of the function and mechanism of Tregitopes have introduced Tregitopes as a popular option for immune system modulation. It is expected that they will bring about a significant revolution in the management and treatment of autoimmune and immunological diseases. This article is a comprehensive review of Tregitopes, concluding with the potential of these epitopes as a therapeutic avenue for immunological disorders., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Background: For adults with auditory processing disorder (APD), listening and communicating can be difficult, potentially leading to social isolation, depression, employment difficulties and certainly reducing the quality of life. Despite existing practice guidelines suggesting treatments, the efficacy of these interventions remains uncertain due to a lack of comprehensive reviews. This systematic review and meta-analysis aim to establish current evidence on the effectiveness of interventions for APD in adults, addressing the urgent need for clarity in the field., Methods: Following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, we conducted a systematic search across MEDLINE (Ovid), Embase (Ovid), Web of Science and Scopus, focusing on intervention studies involving adults with APD. Studies that met the inclusion criteria were grouped according to intervention with a meta-analysis only conducted where intervention, study design and outcome measure were comparable., Results: Out of 1,618 screened records, 13 studies were included, covering auditory training (AT), low-gain hearing aids (LGHA), and personal remote microphone systems (PRMS). Our analysis revealed: AT, Mixed results with some improvements in speech intelligibility and listening ability, indicating potential benefits but highlighting the need for standardized protocols; LGHA, The included studies demonstrated significant improvements in monaural low redundancy speech testing ( p < 0.05), suggesting LGHA could enhance speech perception in noisy environments. However, limitations include small sample sizes and potential biases in study design. PRMS, Demonstrated the most consistent evidence of benefit, significantly improving speech testing results, with no additional benefit from combining PRMS with other interventions., Discussion: PRMS presents the most evidence-supported intervention for adults with APD, although further high-quality research is crucial for all intervention types. The establishment and implementation of standardized intervention protocols alongside rigorously validated outcome measures will enable a more evidence-based approach to managing APD in adults., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor VI declared a past co-authorship with the author D-EB., (Copyright © 2024 Crum, Chowsilpa, Kaski, Giunti, Bamiou and Koohi.)

Recent evidence shows that it is possible to identify the elements responsible for sensorineural hearing loss, such as pro-inflammatory cytokines and macrophages, by performing perilymph sampling. However, current studies have only focused on the diagnosis of such as otologic conditions. Hearing loss is a feature of certain neuroinflammatory disorders such as multiple sclerosis, and sensorineural hearing loss (SNHL) is widely detected in Alzheimer's disease. Although the environment of the inner ear is highly regulated, there are several communication pathways between the perilymph of the inner ear and cerebrospinal fluid (CSF). Thus, examination of the perilymph may help understand the mechanism behind the hearing loss observed in certain neuroinflammatory and neurodegenerative diseases. Herein, we review the constituents of CSF and perilymph, the anatomy of the inner ear and its connection with the brain. Then, we discuss the relevance of perilymph sampling in neurology. Currently, perilymph sampling is only performed during surgical procedures, but we hypothesize a simplified and low-invasive technique that could allow sampling in a clinical setting with the same ease as performing an intratympanic injection under direct visual check. The use of this modified technique could allow for perilymph sampling in people with hearing loss and neuroinflammatory/neurodegenerative disorders and clarify the relationship between these conditions; in fact, by measuring the concentration of neuroinflammatory and/or neurodegenerative biomarkers and those typically expressed in the inner ear in aging SNHL, it could be possible to understand if SNHL is caused by aging or neuroinflammation.

The COVID-19 pandemic dramatically changed health service delivery with vulnerable patients advised to isolate and appointments provided virtually. This change affected recruitment into an observational cohort study, undertaken at a single site, where participants with mitochondrial disorders were due to have specialist hospital-based audiovestibular tests. To ensure study viability, the study protocol was amended to allow home-based assessment for vulnerable participants. Here, we report outcomes of an online survey of participants who underwent home-based assessment, related to the experience, perceived benefits, and drawbacks of home audiovestibular assessments. Seventeen participants underwent home-based neuro-otological assessment, due to the need to isolate during COVID-19. Following the assessment, 16 out of 17 participants completed an anonymised online survey to share their experiences of the specialist home-based assessment. One hundred percent of participants rated the home-based assessment 'very positively' and would recommend it to others. Sixty-three percent rated it better than attending hospital outpatient testing settings. The benefits included no travel burden (27%) and reduced stress (13%). A majority reported no drawbacks in having the home visit. The patient-reported feedback suggests a person-centred approach where audiovestibular assessments are conducted in their homes is feasible for patients, acceptable and seen as beneficial to a vulnerable group of patients.

Background and Purpose: Identifying vestibular causes of dizziness and unsteadiness in multi-sensory neurological disease can be challenging, with problems typically attributed to central or peripheral nerve involvement. Acknowledging vestibular dysfunction as part of the presentation provides an opportunity to access targeted vestibular rehabilitation, for which extensive evidence exists. A diagnostic framework was developed and validated to detect vestibular dysfunction, benign paroxysmal positional vertigo or vestibular migraine. The specificity and sensitivity of the diagnostic framework was tested in patients with primary mitochondrial disease., Methods: Adults with a confirmed diagnosis of primary mitochondrial disease were consented, between September 2020 and February 2022. Participants with and without dizziness or unsteadiness underwent remote physiotherapy assessment and had in-person detailed neuro-otological assessment. The six framework question responses were compared against objective neuro-otological assessment or medical notes. The output was binary, with sensitivity and specificity calculated., Results: Seventy-four adults completed the study: age range 20-81 years (mean 48 years, ±SD 15.05 years); ratio 2:1 female to male. The framework identified a vestibular diagnosis in 35 participants, with seven having two diagnoses. The framework was able to identify vestibular diagnoses in adults with primary mitochondrial disease, with a moderate (40-59) to very high (90-100) sensitivity and positive predictive value, and moderate to high (60-74) to very high (90-100) specificity and negative predictive value., Conclusions: Overall, the clinical framework identified common vestibular diagnoses with a moderate to very high specificity and sensitivity. This presents an opportunity for patients to access effective treatment in a timely manner, to reduce falls and improve quality of life., (© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background: Cognitive impairment is highly prevalent in multiple sclerosis (MS) with poorly understood underlying mechanisms. Lipids are considered to be associated with MS progression through the inflammatory and oxidative stress pathways, brain atrophy, cellular signaling, and tissue physiology. In addition, serum lipids are proposed as a modifiable factor affecting the neuropsychiatric condition; therefore, this study aims to assess the association between serum lipid levels and cognitive outcomes in MS., Methods: This study was carried out following the PRISMA 2020 statement. A systematic search was conducted in PubMed, Scopus, Web of Science, and Embase in March 2023, and the Joanna Briggs Institute (JBI)'s critical appraisal tools were utilized for risk of bias (RoB) assessments in the included studies. The quantitative synthesis was performed with the comprehensive meta-analysis (CMA3) software., Results: Out of 508 screened records, 7 studies were eventually found to meet our inclusion criteria. In two studies, the course of MS in the sample of the study was only Relapsing-Remitting MS (RRMS), whereas the other five studies' sample was a combination of different phenotypes. Studies utilized different scales such as Minimal Assessment of Cognitive Function in MS (MACFIMS), Brief International Cognitive Assessment for MS (BICAMS), Montreal Cognitive Assessment (MoCA), Brief Repeatable Battery of Neuropsychological Tests (BRB-N) for cognitive evaluations. Dealing with possible confounders such as age, disease duration and level of disability was the most common possible source of bias in the included studies. One study revealed an inverse relationship between serum levels of apolipoproteins (including ApoA-I, ApoB, and ApoB/ApoA-I) and Symbol Digit Modalities Test (SDMT) scores. Also, a correlation between 24S-hydroxycholesterol (24OHC) serum concentrations and SDMT score was reported in one study. The association between serum total cholesterol (TC) and low-density lipoprotein cholesterol (LDL) and different aspects of cognitive function was reported in the studies; however, serum levels of high-density lipoprotein cholesterol (HDL) were not found to be associated. The quantitative synthesis revealed a significant correlation between TC and the MoCA scores (r =-0.238; 95 %CI: -0.366 to -0.100; p-value = 0.001); however, the correlation between TG levels and MoCA were not statistically significant (r:-0.070; 95 %CI: -0.209 to 0.072; p-value: 0.334). In addition, the mata-analyses were not associated with significant findings regarding the correlation between lipid profiles (including HDL, LDL, TG, and TC) and other cognitive assessment scales including SDMT, Brief Visuospatial Memory Test (BVMT), and California Verbal Learning Test (CVLT) (p-values>0.05)., Discussion: Available evidence suggested a link between TC and LDL with cognitive outcomes of MS patients which was not evident in our quantitative synthesis. The limited number of studies, high RoB, different cognitive assessment scales and reporting methods, and the cross-sectional design of the included studies, were the main limitations that alleviate the clinical significance of the findings of this study and suggested further investigations on this topic., Funding and Registration: The research protocol was approved and supported by the Student Research Committee, Tabriz University of Medical Sciences (grant number: 71,909). This study is registered in the international prospective register of systematic reviews (PROSPERO ID: CRD42023441625)., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier B.V.)

Acute vestibular syndrome (AVS) is characterised by a sudden vertigo, gait instability, nausea and nystagmus. Accurate and rapid triage of patients with AVS to differentiate central (potentially sinister) from peripheral (usually benign) root causes is a challenge faced across emergency medicine settings. While there exist bedside exams which can reliably differentiate serious cases, they are underused due to clinicians' general unfamiliarity and low confidence interpreting results. Nystagmus is a fundamental part of AVS and can facilitate triaging, but identification of relevant characteristics requires expertise. This work presents two quantitative digital biomarkers from nystagmus analysis, which capture diagnostically-relevant information. The directionality biomarker evaluates changes in direction to differentiate spontaneous and gaze-evoked (direction-changing) nystagmus, while the intensity differential biomarker describes changes in intensity across eccentric gaze tests. In order to evaluate biomarkers, 24 sets of three gaze tests (left, right, and primary) are analysed. Both novel biomarkers were found to perform well, particularly directionality which was a perfect classifier. Generally, the biomarkers matched or eclipsed the performance of quantitative nystagmus features found in the literature. They also surpassed the performance of a support vector machine classifier trained on the same dataset, which achieved an accuracy of 75%. In conclusion, these biomarkers simplify the diagnostic process for non-specialist clinicians, bridging the gap between emergency care and specialist evaluation, ultimately benefiting patients with AVS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Duvieusart, Leung, Koohi and Kaski.)

Introduction: Benign paroxysmal positional vertigo (BPPV) is the most common cause of positional vertigo. However, positional vertigo can also be due to diseases affecting the central vestibular pathways, such as vestibular migraine. Accurate and timely diagnosis enables effective triage and management., Objectives: To evaluate diagnoses made by emergency clinicians compared to acute vertigo specialists, in patients presenting to an emergency department (ED) with positional vertigo., Methods: Following routine ED care, patients with a primary complaint of dizziness, vertigo, light-headedness or unsteadiness, underwent detailed neuro-otological assessment by acute vertigo specialists. Demographics and final diagnoses were recorded and analyzed., Results: Of 71 consented patients (21-91 years; mean 56 years, ±16.7 years, 40 females), ED identified 13 with a peripheral cause of positional vertigo (mean 48.85 years, ±16.19, 8 females). Central positional nystagmus was not noted in any of the patients with positional vertigo seen by the ED clinicians. Acute vertigo specialists diagnosed nine patients with BPPV (age range 50-88 years, mean 66 years, ±12.22, 5 females), and six with central positional nystagmus (age range 23-59 years, mean 41.67 years, ±15.78, 6 females)., Conclusion: Positional vertigo should be assessed with positional maneuvers such as Dix-Hallpike and Roll tests in the ED to identify peripheral and central nystagmus features. Central causes are more common in younger females, with the presence of vomiting, and/or a background of motion sensitivity., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Koohi, Male and Kaski.)

Background: Hearing can be impaired in many neurological conditions and can even represent a forme fruste of specific disorders. Auditory function can be measured by either subjective or objective tests. Objective tests are more useful in identifying which auditory pathway (superior or inferior) is most affected by disease. The inner ear's perilymphatic fluid communicates with the cerebrospinal fluid (CSF) via the cochlear aqueduct representing a window from which pathological changes in the contents of the CSF due to brain inflammation could, therefore, spread to and cause inflammation in the inner ear, damaging inner hair cells and leading to hearing impairment identifiable on tests of auditory function., Methods: A systematic review of the literature was performed, searching for papers with case-control studies that analyzed the hearing and migraine function in patients with neuro-inflammatory, neurodegenerative disorders. With data extracted from these papers, the risk of patients with neurological distortion product otoacoustic emission (DPOAE) was then calculated., Results: Patients with neurological disorders (headache, Parkinson's disease, and multiple sclerosis) had a higher risk of having peripheral auditory deficits when compared to healthy individuals., Conclusion: Existing data lend credence to the hypothesis that inflammatory mediators transmitted via fluid exchange across this communication window, thereby represents a key pathobiological mechanism capable of culminating in hearing disturbances associated with neuroimmunological and neuroinflammatory disorders of the nervous system., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Di Stadio, De Luca, Koohi, Kaski, Ralli, Giesemann, Hartung, Altieri, Messineo, Warnecke, Frohman and Frohman.)

Background and Purpose: Differentiating between peripheral and central aetiologies can be challenging in patients with acute vertigo, given substantial symptom overlap. A detailed clinical history and focused physical eye movement examination such as the HINTS eye examination appear to be the most reliable approach to identify acute cerebellar/brainstem stroke, outperforming even acute brain imaging. We have observed, however, that isolated vertigo of central cause may be accompanied by acute truncal ataxia, in the absence of nystagmus., Methods: We explored the frequency of ataxia without concurrent nystagmus in a cross section of patients with acute vertigo who presented to the emergency department at two centres in Argentina (Group A) and the UK (Group B). Patients underwent detailed clinical neuro-otological assessments (Groups A and B), which included instrumented head impulse testing and oculography (Group B)., Results: A total of 71 patients in Group A and 24 patients in Group B were included in this study. We found acute truncal ataxia-without nystagmus-in 15% (n = 14) of our overall cohort. Lesions involved stroke syndromes affecting the posterior inferior cerebellar artery, anterior inferior cerebellar artery, and superior cerebellar artery, thalamic stroke, cerebral hemisphere stroke, multiple sclerosis, and a cerebellar tumour. Additional oculomotor deficits did not reliably identify a central cause in these individuals, even with oculography., Conclusions: We have identified a significant subpopulation of patients with acute vertigo in whom the current standard approaches such as the HINTS examination that focus on oculomotor assessment may not be applicable, highlighting the need for a formal assessment of gait in this setting., (© 2023 European Academy of Neurology.)

Background and Objectives: Saccades, rapid movements of the eyes towards a visual or remembered target, are useful in understanding the healthy brain and the pathology of neurological conditions such as progressive supranuclear palsy (PSP). We set out to investigate the parameters of horizontal reflexive and volitional saccades, both visually guided and memory-guided, over a 1 min epoch in healthy individuals and PSP patients., Methods: An experimental paradigm tested reflexive, volitional visually guided, and volitional memory-guided saccades in young healthy controls (n = 14; 20-31 years), PSP patients (n = 11; 46-75 years) and older age-matched healthy controls (n = 6; 56-71 years). The accuracy and velocity of saccades was recorded using an EyeBrain T2® video eye tracker and analyses performed using the MyEyeAnalysis® software. Two-way analysis of variance (ANOVA) was used to identify significant effects (p < 0.01) between young and older controls to investigate the effects of ageing upon saccades, and between PSP patients and age-matched controls to study the effects of PSP upon saccades., Results: In both healthy individuals and PSP patients, volitional saccades are slower and less accurate than reflexive saccades. In PSP patients, accuracy is lower across all saccade types compared to age-matched controls, but velocity is lower only for reflexive saccades. Crucially, there is no change in accuracy or velocity of consecutive saccades over short (one-minute) timescales in controls or PSP patients., Conclusions: Velocity and accuracy of saccades in PSP does not decrease over one-minute timescales, contrary to that previously observed in Parkinson's Disease (PD), suggesting a potential clinical biomarker for the distinction of PSP from PD., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

The video head impulse test (vHIT) assesses the vestibulo-ocular reflex (VOR) during a rapid high-velocity low amplitude (10°-20°) head rotation. Patients with peripheral vestibulopathy have a reduced VOR gain with corrective catch-up saccades during the head turn. There are several pitfalls, mainly technical, which may interfere with interpretation of vHIT data. In addition, intrusive eye movement disorders such as spontaneous nystagmus that affect normal eye position and tracking can affect the vHIT results. To date there has been little study of neurological saccadic eye movements that may interfere with the interpretation of vHIT data. Here, in ten patients with a range of central neurological disorders, we describe oculomotor abnormalities on vHIT in the presence of normal range VOR gain values, recorded at a tertiary vestibular neurology service., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Vestibular Migraine (VM) is the most common cause of non-positional episodic vestibular symptoms. Patients with VM commonly report increased motion sensitivity, suggesting that vestibular responses to head movement may identify changes specific to VM patients. Here we explore whether the vestibulo-ocular reflex (VOR) gain alters in response to a clinical "headshake" maneuver in patients with VM. Thirty patients with VM in the inter-ictal phase, 16 patients with Benign Positional Paroxysmal Vertigo (BPPV) and 15 healthy controls were recruited. Patients responded to the question "Do you feel sick reading in the passenger seat of a car?" and completed a validated motion sickness questionnaire as a measure of motion sensitivity. Lateral canal vHIT testing was performed before and after headshaking; the change in VOR gain was calculated as the primary outcome. Baseline VOR gain was within normal limits across all participants. There was no significant change in VOR gain after headshaking in any group ( p = 0.264). Patients were 4.3 times more likely to be in the VM group than in the BPPV group if they reported nausea when reading in the passenger seat of a car. We postulate that a headshake stimulus may be insufficient to disrupt cortical interactions and induce a change in VOR gain. Alternatively, changes in VOR gain may only be apparent in the acute phase of VM. Reading in the passenger seat of a car was considered uncomfortable in all VM patients suggesting that this specific question may be useful for the diagnosis of VM., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Patel, Castro, Koohi, Arshad, Gargallo, Carmona and Kaski.)

Background: Dizziness and imbalance are common symptoms that are often inadequately diagnosed or managed, due to a lack of dedicated specialists. Decision Support Systems (DSS) may support first-line physicians to diagnose and manage these patients based on personalised data., Aim: To examine the diagnostic accuracy and application of the EMBalance DSS for diagnosis and management of common vestibular disorders in primary care., Methods: Patients with persistent dizziness were recruited from primary care in Germany, Greece, Belgium and the UK and randomised to primary care clinicians assessing the patients with (+ DSS) versus assessment without (- DSS) the EMBalance DSS. Subsequently, specialists in neuro-otology/audiovestibular medicine performed clinical evaluation of each patient in a blinded way to provide the "gold standard" against which the + DSS, - DSS and the DSS as a standalone tool (i.e. without the final decision made by the clinician) were validated., Results: One hundred ninety-four participants (age range 25-85, mean = 57.7, SD = 16.7 years) were assigned to the + DSS (N = 100) and to the - DSS group (N = 94). The diagnosis suggested by the + DSS primary care physician agreed with the expert diagnosis in 54%, compared to 41.5% of cases in the - DSS group (odds ratio 1.35). Similar positive trends were observed for management and further referral in the + DSS vs. the - DSS group. The standalone DSS had better diagnostic and management accuracy than the + DSS group., Conclusion: There were trends for improved vestibular diagnosis and management when using the EMBalance DSS. The tool requires further development to improve its diagnostic accuracy, but holds promise for timely and effective diagnosis and management of dizzy patients in primary care., Trial Registration Number: NCT02704819 (clinicaltrials.gov)., (© 2021. The Author(s).)

Background: Acute vestibular syndrome (AVS) features continuous dizziness and may result from a benign inner ear disorder or stroke. The head impulse-nystagmus-test of skew (HINTS) bedside assessment is more sensitive than brain MRI in identifying stroke as the cause of AVS within the first 24 hours. Clinicians' perspectives of the test in UK secondary care remains unknown. Here, we explore front-line clinicians' perspectives of use of the HINTS for the diagnosis of AVS., Methods: Clinicians from two large UK hospitals who assess AVS patients completed a short online survey, newly designed with closed and open questions., Results: Almost half of 73 total responders reported limited (n=33), or no experience (n=19), reflected in low rates of use of HINTS (n=31). While recognising the potential utility of HINTS, many reported concerns about subjectivity, need for specialist skills and poor patient compliance. No clinicians reported high levels of confidence in performing HINTS, with 98% identifying training needs. A lack of formalised training was associated with onward specialist referrals and neuroimaging (p=0.044)., Conclusions: Although the low sample size in this study limits the generalisability of findings to wider sites, our preliminary data identified barriers to the application of the HINTS in AVS patients and training needs to improve rapid, cost-effective and accurate clinical diagnosis of stroke presenting with vertigo., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Vestibular migraine (VM) is a leading cause of episodic vertigo, affecting up to 1% of the general population. Despite established diagnostic criteria, there is currently no evidence-based approach for acute treatment of VM, with treatment recommendations generally extrapolated from studies on classical migraine headache. Several small-scale studies have identified flunarizine as a potentially effective prophylactic medication in VM. We conducted a single-centre observational service evaluation study exploring patient experiences of preventative medications over a 28-month period, including flunarizine, for control of VM symptoms. To compare patient experience of flunarizine with other medications, data from patients taking flunarizine were separately analysed. A total of 90% of VM patients taking flunarizine reported symptomatic improvement, compared to only 32% of patients on other medications. Whilst 50% of patients on flunarizine reported side effects. these were not deemed to outweigh the clinical benefits, with most patients deciding to continue treatment. Our data supports the use of flunarizine in VM.

Background and Purpose: In posterior circulation stroke, vertigo can be a presenting feature. However, whether isolated hemispheric strokes present with vertigo is less clear, despite a few single case reports in the literature. Here, (a) the prevalence of vertigo/dizziness in acute stroke is explored and (b) the cortical distribution of the lesions in relation to both the known vestibular cortex and the evolution of the symptoms, are considered., Methods: Structured interviews were conducted in 173 consecutive unselected patients admitted to the hyperacute stroke unit at the University College London Hospitals. The interview was used to evaluate whether the patient was suffering from dizziness and/or vertigo before the onset of the stroke and at the time of the stroke (acute dizziness/vertigo), and the nature of these symptoms., Results: In all, 53 patients had cortical infarcts, of which 21 patients reported acute dizziness. Out of these 21, five patients reported rotational vertigo. Seventeen of the total 53 patients had lesions in known vestibular cortical areas distributed within the insular and parietal opercular cortices., Conclusions: The prevalence of vertigo in acute cortical strokes was 9%, with no single locus of lesion overlap. There is growing evidence supporting a lateralized vestibular cortex, with speculation that cortical strokes affecting the right hemisphere are more likely to cause vestibular symptoms than left hemispheric strokes. A trend was observed for this association, with the right hemisphere affected in four of five patients who reported spinning vertigo at the onset of the stroke., (© 2021 European Academy of Neurology.)

Auditory neural impairment is a key clinical feature of Friedreich's Ataxia (FRDA). We aimed to characterize the phenotypical spectrum of the auditory impairment in FRDA in order to facilitate early identification and timely management of auditory impairment in FRDA patients and to explore the relationship between the severity of auditory impairment with genetic variables (the expansion size of GAA trinucleotide repeats, GAA1 and GAA2), when controlled for variables such as disease duration, severity of the disease and cognitive status. Twenty-seven patients with genetically confirmed FRDA underwent baseline audiological assessment (pure-tone audiometry, otoacoustic emissions, auditory brainstem response). Twenty of these patients had additional psychophysical auditory processing evaluation including an auditory temporal processing test (gaps in noise test) and a binaural speech perception test that assesses spatial processing (Listening in Spatialized Noise-Sentences Test). Auditory spatial and auditory temporal processing ability were significantly associated with the repeat length of GAA1. Patients with GAA1 greater than 500 repeats had more severe auditory temporal and spatial processing deficits, leading to poorer speech perception. Furthermore, the spatial processing ability was strongly correlated with the Montreal Cognitive Assessment (MoCA) score. To our knowledge, this is the first study to demonstrate an association between genotype and auditory spatial processing phenotype in patients with FRDA. Auditory temporal processing, neural sound conduction, spatial processing and speech perception were more severely affected in patients with GAA1 greater than 500 repeats. The results of our study may indicate that auditory deprivation plays a role in the development of mild cognitive impairment in FRDA patients., (© 2021. The Author(s).)

Background: Auditory temporal processing tests are key clinical measures in order to diagnose central auditory processing disorder (CAPD). Although these tests have been used for decades, there is no up-to-date evidence to determine the effectiveness of detecting the abnormalities in central auditory processing in adults while the available national CAPD guidelines predominantly address CAPD in the pediatric population. Purpose: To determine the efficacy of the auditory temporal ordering tests [duration pattern test (DPT) and frequency pattern test (FPT)], and a temporal resolution test [gaps-in-noise (GIN) test] for detecting the central auditory processing abnormalities in adults with documented brain pathology. Research Design: Systematic reviews and meta-analyses. Study samples: Four databases, including PubMed, Web of Science, Embase, and Scopus, were systematically searched. The publications in the English language that recruited adults (above 16 years old) with pathologic brain conditions and described the diagnostic tests for auditory temporal processing were selected for review. Data Collections and Analysis: All data were systematically evaluated, extracted, categorized, and summarized in tables. The meta-analysis was done in order to determine the effectiveness of the DPT, FPT, and GIN tests. Results: The results showed significantly poorer performance of DPT and FPT, compared between participants with confirmed brain disease and normal controls, at the mean differences of percent correct -21.93 (95% CI, -26.58 to -17.29) and -31.37 (95% CI, -40.55 to -22.19), respectively. Subjects with brain pathology also performed poorer in GIN test at the mean difference of 3.19 milliseconds (95% CI, 2.51 to 3.87). Conclusion: The results from the meta-analysis provide evidence that DPT, FPT, and GIN clinical measures are effective in the diagnosis of CAPD in adults with neurological disorders. Poor performance on these tests is significantly related to the confirmed brain pathology. However, different units in results presentation and variety of testing strategies are limitations for this meta-analysis. The standard pattern of result reporting and international protocols test strategies should be developed in order to conduct better meta-analyses with a larger collection of suitable studies and less heterogeneity., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Chowsilpa, Bamiou and Koohi.)

Background: Auditory impairment post stroke is common and may be due to both peripheral hearing loss and or central auditory processing disorder (CAPD). When auditory impairment remains untreated, it may impact on patient communication and rehabilitation after stroke. Offering a comprehensive audiological assessment to all stroke patients would be both costly and time-consuming. A brief hearing screening is thus required. Objective: The aim of this study was to determine whether a two-tiered hearing screening approach, with use of a handheld hearing screener and two validated hearing questionnaires could be used as a hearing screening for peripheral hearing loss and CAPD in stroke survivors. The sensitivity and specificity of the screening method was analyzed. Methods: This was a prospective study conducted in a tertiary neurology hospital. Forty-two consecutive stroke patients were recruited and tested within 3-12 months post-onset of their stroke. Three screening tools for the identification of hearing impairment were evaluated in this study: A handheld hearing screener for determination of peripheral audiometric hearing loss and two validated questionnaires (The Amsterdam Inventory Auditory for Disability (AIAD) and the Hearing Handicap Inventory for Elderly (HHIE) questionnaires) for determination of peripheral hearing loss and/or CAPD. Results: The hearing screener had a sensitivity of 92. 59% detecting a hearing loss and specificity of 100%. The greatest test accuracy in identifying a central auditory processing type hearing impairment in stroke patients was found when the handheld hearing screener and the AIAD questionnaire were combined. Conclusion: This study is a first step toward addressing the complex auditory needs of stroke survivors in a systematic manner, with the ultimate aim to support their communication needs and long-term recovery and wellbeing. Registration: Project Identification number 11/0469 and REC ref 11/LO/1675.

Background: Stroke survivors may suffer from a range of hearing impairments that may restrict their participation in postacute rehabilitation programs. Hearing impairment may have a significant impact on listening, linguistic skills, and overall communication of the affected stroke patient. However, no studies sought to systematically characterize auditory function of stroke patients in detail, to establish the different types of hearing impairments in this cohort of patients. Such information would be clinically useful in understanding and addressing the hearing needs of stroke survivors., Purpose: The present study aimed to characterize and classify the hearing impairments, using a detailed audiological assessment test battery, in order to determine the level of clinical need and inform appropriate rehabilitation for this patient population., Research Design: A case-control study., Study Sample: Forty-two recruited stroke patients who were discharged from a stroke unit and 40 control participants matched for age., Data Collection and Analysis: All participants underwent pure-tone audiometry and immittance measurements including acoustic reflex threshold, transient-evoked otoacoustic emissions, auditory-evoked brainstem response, and a central auditory processing assessment battery, performed in a single session. Hearing impairments were classified as peripheral hearing loss (cochlear and neural type), central auditory processing disorder (CAPD), and as a combination of CAPD and peripheral hearing loss., Results: Overall mean hearing thresholds were not significantly different between the control and stroke groups. The most common type of hearing impairment in stroke patients was the combination type, "peripheral and CAPD," in the 61- to 80-yr-old subgroup (in 55%), and auditory processing deficits in 18- to 60-yr-olds (in 40%), which were both significantly higher than in controls., Conclusions: This is the first study to examine hearing function in detail in stroke patients. Given the importance of hearing for the efficiency of communication, it is essential to identify hearing impairments and differentiate peripheral and central deficits to define an appropriate intervention plan., (American Academy of Audiology)

Objectives: Approximately one in five stroke survivors suffer from difficulties with speech reception in noise, despite normal audiometry. These deficits are treatable with personal frequency-modulated systems (FMs). This study aimed to evaluate long-term benefits in speech reception in noise, after daily 10-week use of personal FMs, in non-aphasic patients with stroke with auditory processing deficits., Design: This was a prospective non-randomised controlled trial study. Patients were allocated to an intervention care group or standard care subjects group according to their willingness to use the intervention or not., Setting: Tertiary care setting., Participants: Nine non-aphasic subjects with ischaemic stroke, normal/near-normal audiometry and auditory processing deficits and with reported difficulties understanding speech in background noise were recruited in the subacute stroke stage (3-12 months after stroke)., Interventions: Four patients (intervention care subjects) used the FMs in their daily life over 10 weeks. Five patients (standard care subjects) received standard care., Primary Outcome Measures: All subjects were tested at baseline (visit 1) and 10 weeks later (visit 2) on a sentences in noise test with the FMs (aided) and without the FMs (unaided)., Results: Speech reception thresholds showed clinically and statistically significant improvements in intervention but not in standard care subjects at 10 weeks in aided and unaided conditions., Conclusions: 10-week use of FMs by adult patients with stroke may lead to benefits in unaided speech in noise perception. Our findings may indicate auditory plasticity type changes and require further investigation., Trial Registration Number: Pre-results; NCT02889107., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Purpose: Auditory disability due to impaired auditory processing (AP) despite normal pure-tone thresholds is common after stroke, and it leads to isolation, reduced quality of life and physical decline. There are currently no proven remedial interventions for AP deficits in stroke patients. This is the first study to investigate the benefits of personal frequency-modulated (FM) systems in stroke patients with disordered AP., Methods: Fifty stroke patients had baseline audiological assessments, AP tests and completed the (modified) Amsterdam Inventory for Auditory Disability and Hearing Handicap Inventory for Elderly questionnaires. Nine out of these 50 patients were diagnosed with disordered AP based on severe deficits in understanding speech in background noise but with normal pure-tone thresholds. These nine patients underwent spatial speech-in-noise testing in a sound-attenuating chamber (the "crescent of sound") with and without FM systems., Results: The signal-to-noise ratio (SNR) for 50% correct speech recognition performance was measured with speech presented from 0° azimuth and competing babble from ±90° azimuth. Spatial release from masking (SRM) was defined as the difference between SNRs measured with co-located speech and babble and SNRs measured with spatially separated speech and babble. The SRM significantly improved when babble was spatially separated from target speech, while the patients had the FM systems in their ears compared to without the FM systems., Conclusions: Personal FM systems may substantially improve speech-in-noise deficits in stroke patients who are not eligible for conventional hearing aids. FMs are feasible in stroke patients and show promise to address impaired AP after stroke. Implications for Rehabilitation This is the first study to investigate the benefits of personal frequency-modulated (FM) systems in stroke patients with disordered AP. All cases significantly improved speech perception in noise with the FM systems, when noise was spatially separated from the speech signal by 90° compared with unaided listening. Personal FM systems are feasible in stroke patients, and may be of benefit in just under 20% of this population, who are not eligible for conventional hearing aids.

Background: vestibular disorders are common in the general population, increasing with age. However, it is unknown whether older adults who fall have a higher proportion of vestibular impairment compared with age-matched older adult non-fallers., Objective: to identify whether a greater proportion of older adult fallers have a peripheral vestibular impairment compared with age-matched healthy controls., Design: case-controlled study., Setting: tertiary falls and neuro-otology clinics and local community centres, London, UK., Participants and Methods: community-dwelling older adults experiencing: (i) ≥2 unexplained falls within the previous 12-months (Group F, n = 25), (ii) a confirmed peripheral vestibular disorder (Group PV, n = 15) and (iii) healthy non-fallers (Group H, n = 16). All the participants completed quantitative vestibular function tests, the functional gait assessment (FGA), physiological profile assessment (PPA) and subjective measures for common vestibular symptoms (i.e. giddiness), balance confidence during daily activities and psychological state., Results: a clinically significant vestibular impairment was noted for 80% (20/25) of Group F compared with 18.75% (3/16) for Group H (P < 0.01). Group F performed less well in complex gait tasks (FGA), and reported a greater number of falls than both Groups H and PV (P < 0.05). Vestibular symptom scores showed no significant difference between Groups F and PV., Conclusion: vestibular dysfunction is significantly more prevalent in older adult fallers versus non-fallers. Individuals referred to a falls clinic are older, more impaired and report more falls than those referred to a neuro-otology department. A greater awareness of vestibular impairments may lead to more effective management and treatment for older adult fallers.