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This study used magnetoencephalography to record oscillatory activity in a group of 17 patients with chronic tinnitus. Two methods, residual inhibition and residual excitation, were used to bring about transient changes in spontaneous tinnitus intensity in order to measure dynamic tinnitus correlates in individual patients. In residual inhibition, a positive correlation was seen between tinnitus intensity and both delta/theta (6/14 patients) and gamma band (8/14 patients) oscillations in auditory cortex, suggesting an increased thalamocortical input and cortical gamma response, respectively, associated with higher tinnitus states. Conversely, 4/4 patients exhibiting residual excitation demonstrated an inverse correlation between perceived tinnitus intensity and auditory cortex gamma oscillations (with no delta/theta changes) that cannot be explained by existing models. Significant oscillatory power changes were also identified in a variety of cortical regions, most commonly midline lobar regions in the default mode network, cerebellum, insula and anterior temporal lobe. These were highly variable across patients in terms of areas and frequency bands involved, and in direction of power change. We suggest a model based on a local circuit function of cortical gamma-band oscillations as a process of mutual inhibition that might suppress abnormal cortical activity in tinnitus. The work implicates auditory cortex gamma-band oscillations as a fundamental intrinsic mechanism for attenuating phantom auditory perception. [ABSTRACT FROM AUTHOR]

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.

Background: Age-related hearing loss and mild cognitive impairment (MCI) independently increase dementia risk. The Ageing and Cognitive Health Evaluation in Elders randomised controlled trial (RCT) found hearing aids reduce cognitive decline in high-risk older adults with poor hearing., Methods: This pilot RCT in London memory clinics randomised people with MCI (aged ≥55, untreated hearing loss defined as Pure Tone Average 0.5-4 KHz between 25-70 dB) into two groups. The intervention group received 4 sessions of hearing aid fitting and support. The control group received healthy ageing education and a GP letter recommending audiological referral. Both were followed for 6 months. Primary outcomes were recruitment (feasibility target: 50%; 95% CI: 39%-61%) and retention (feasibility target: 80%; 95% CI: 71%-89%); intervention completion (≥2 visits) and hearing aid use (acceptability target: 80%; 95% CI: 71%-89%) for the intervention group and 50% difference between arms (95% CI: 31%-69%). Secondary outcomes included hearing aid fitting, cognition and other measures., Results: From October 2018 to March 2020, 58 participants were recruited (29 per group, 95% [86%-99%]). Twenty-four participants were fitted with hearing aids in the intervention arm, and 6 in the control arm (difference: 62% [42%-82%]). At 6 months, retention was 81% [69%-90%]. Hearing intervention completion (≥2 visits) was achieved by 24 (83%). Daily hearing aid use was reported by 18 (75%) intervention versus 5 (22%) control participants, a difference of 53% [29%-77%]., Conclusion: Randomisation of people with MCI to a personalised hearing intervention versus control is feasible. These findings support proceeding to a fully-powered multicentre RCT., (© The Author(s) 2025. Published by Oxford University Press on behalf of the British Geriatrics Society.)

Objectives: The objectives of this study were to evaluate the relationships between auditory processing, speech discrimination, and listening and communication abilities in children diagnosed with auditory neuropathy spectrum disorder (ANSD) compared to those with normal hearing., Methods: This was a case-control study involving seventeen participants with a diagnosis of ANSD who used spoken language as their primary mode of communication and wore hearing aids or were unaided and 21 normally hearing controls. All underwent a battery of behavioural measures of hearing, speech perception and auditory processing skills. Their parents filled in a series of questionnaires to evaluate listening and communication abilities. Group differences were evaluated, and hierarchical linear regression was carried out to assess the extent to which auditory measures predicted parent report scores., Results: Slow-rate amplitude modulation detection (AMD) was an important predictor of listening and communication abilities over and above the pure-tone audiogram in children diagnosed with ANSD. There was a significant relationship between the pure-tone audiogram and the ability to discriminate speech in quiet but not in noise. Children in the ANSD group had more difficulty completing tests of auditory processing ability or performed significantly more poorly than the control group for most tasks., Conclusions: This study confirms that outcomes for children diagnosed with ANSD are heterogeneous, and provides new information about real world listening and communication abilities. These findings will be useful for clinicians counselling families of newly diagnosed infants and providing management for children with a diagnosis of ANSD., Competing Interests: Declaration of competing interest This work was supported by a NIHR/CSO Healthcare Science Doctoral Research Fellowship, Hannah Cooper, NIHR-HCS-D12–03–05. We also acknowledge funding from the NIHR Biomedical Research Centre at Great Ormond Street Hospital and the Great Ormond Street Institute of Child Health, University College London, as well as at University College London Hospitals. There are no conflicts of interest, financial, or otherwise., (Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved.)

Previous research suggests that emotional prosody perception is impaired in neurodegenerative diseases like Alzheimer's disease (AD) and primary progressive aphasia (PPA). However, no previous research has investigated emotional prosody perception in these diseases under non-ideal listening conditions. We recruited 18 patients with AD, and 31 with PPA (nine logopenic (lvPPA); 11 nonfluent/agrammatic (nfvPPA) and 11 semantic (svPPA)), together with 24 healthy age-matched individuals. Participants listened to speech stimuli conveying three emotions in clear and noise-vocoded forms and had to identify the emotion being conveyed. We then conducted correlation analyses between task performance and measures of socio-emotional functioning. All patient groups showed significant impairments in identifying clear emotional prosody compared to healthy individuals. These deficits were exacerbated under noise-vocoded conditions, with all patient groups performing significantly worse than healthy individuals and patients with lvPPA performing significantly worse than those with svPPA. Significant correlations with social cognition measures were observed more consistently for noise-vocoded than clear emotional prosody comprehension. These findings open a window on a dimension of real-world emotional communication that has often been overlooked in dementia, with particular relevance to social cognition, and begin to suggest a novel candidate paradigm for investigating and quantifying this systematically., Competing Interests: Declarations. Competing interests: The authors declare no competing interests. Ethics approval: All participants gave informed consent to take part in the study, in accordance with Declaration of Helsinki guidelines. Ethical approval was granted by the University College London-National Hospital for Neurology and Neurosurgery Joint Research Ethics Committees., (© 2024. The Author(s).)

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).)

The Iowa Test of Consonant Perception is a single-word closed-set speech-in-noise test with well-balanced phonetic features. The current study aimed to establish a U.K. version of the test (ITCP-B) based on the Southern Standard British English. We conducted a validity test in two sessions with 46 participants. The ITCP-B demonstrated excellent test-retest reliability, cross-talker validity, and good convergent validity. These findings suggest that ITCP-B is a reliable measure of speech-in-noise perception. The test can be used to facilitate comparative or combined studies in the U.S. and U.K. All materials (application and scripts) to run the ITCP-B/ITCP are freely available online., (© 2024 Author(s). All article content, except where otherwise noted, is licensed under a Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).)

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.

Background: Hearing efficiency is known to influence and interact with communication and mental health. Hearing impairment may be hidden when co-occurring with neurological disorders., Purpose: We performed a systematic review and meta-analysis in order to address the following questions: 1) which specific tools of auditory processing show clear deficits, separating Temporal Lobe Epilepsy (TLE) patients from normal controls,2) How well is TLE evaluated in terms of hearing and auditory processing?, Methods: The study inclusion criteria were: 1) patients diagnosed with temporal lobe epilepsy, 2) presence of a normal control group, 3) auditory processing assessment using auditory stimuli with behavioral tests and/or P300 or Mitch Match Negativity (MMN) latency and/or amplitude, 4) publications written in English, 5) publication date after 2000. 132 articles were retrieved and based on PRISMA & PICO criteria 23 articles were analyzed., Results: Temporal resolution and processing as measured by the behavioral tests of Gaps-In-Noise (GIN) and Duration Pattern Test (DPT) document deficiencies in TLE patients and separate them from normal controls. Electrophysiology as measured by MMN & P300 shows statistically significant differences in TLE patients compared to controls with patients showing deficient auditory processing. A clear difference between studies with psychoacoustic assessment as opposed to electrophysiology ones may be due to lacking or incomplete evaluation of peripheral hearing by gold standard tools (76.9% in electrophysiology studies)., Conclusion: Auditory processing is deficient in patients with TLE. There is a clear need to evaluate hearing efficiency before proceeding to auditory processing evaluation with behavioral or electrophysiological tests., 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 Elsevier Inc. All rights reserved.)

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: Falls have high socioeconomic costs. Information and communication technologies may support provision and monitoring of multisensory (MSR) physiotherapy programmes. The HOLOBalance platform used augmented reality holograms to provide patient-centred, individualised MSR., Objectives: To determine the platform's safety, acceptability and feasibility, investigate functional gait and dynamic balance benefits and provide data for a definitive trial., Design and Setting: Single-blinded pilot randomised controlled feasibility study. Interventions were conducted at clinical sites or participants' homes in three European countries., Participants: Community-dwelling older adults (median age 73 years; 64.2% female) at risk of falls were enrolled (May 2020-August 2021)., Methods: Participants were randomised to an 8-week clinic or home-based telerehabilitation MSR or OTAGO (control group) programme. Compliance, satisfaction, and adverse events determined feasibility. Clinical outcomes, assessed (blinded) within one-week prior to and post-intervention, included functional gait assessment (FGA), Mini BESTest and cognitive function., Results: Randomisation to completion rate was 76.15% with 109 participants recruited (n = 289 screened). Drop-out rate was similar between groups. Adverse events were reported (n = 3) in the control group. Sixty-nine percent would recommend the HOLOBalance intervention. Findings were similar for the home and clinic-based arms of each intervention; data was combined for analysis. FGA (95%CI [1.63, 4.19]) and Mini-BESTest (95%CI [1.46, 3.93]) showed greater improvement in the HOLOBalance group with a clinically meaningful change of 4/30 noted for the FGA., Conclusions: HOLObalance was feasible to implement and acceptable to older adults at risk of falls, with FGA and Mini-BEST improvements exceeding those for the OTAGO programme. A definitive trial is warranted., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Geriatrics Society.)

Background: Hearing loss has been proposed as a modifiable risk factor for dementia. However, the relationship between hearing, neurodegeneration, and cognitive change, and the extent to which pathological processes such as Alzheimer's disease and cerebrovascular disease influence these relationships, is unclear., Methods: Data from 287 adults born in the same week of 1946 who underwent baseline pure tone audiometry (mean age=70.6 years) and two time point cognitive assessment/multimodal brain imaging (mean interval 2.4 years) were analysed. Hearing impairment at baseline was defined as a pure tone average of greater than 25 decibels in the best hearing ear. Rates of change for whole brain, hippocampal and ventricle volume were estimated from structural MRI using the Boundary Shift Integral. Cognition was assessed using the Pre-clinical Alzheimer's Cognitive Composite. Regression models were performed to evaluate how baseline hearing impairment associated with subsequent brain atrophy and cognitive decline after adjustment for a range of confounders including baseline β-amyloid deposition and white matter hyperintensity volume., Results: 111 out of 287 participants had hearing impairment. Compared with those with preserved hearing, hearing impaired individuals had faster rates of whole brain atrophy, and worse hearing (higher pure tone average) predicted faster rates of hippocampal atrophy. In participants with hearing impairment, faster rates of whole brain atrophy predicted greater cognitive change. All observed relationships were independent of β-amyloid deposition and white matter hyperintensity volume., Conclusions: Hearing loss may influence dementia risk via pathways distinct from those typically implicated in Alzheimer's and cerebrovascular disease in cognitively unimpaired older adults., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

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.)

Successful communication in daily life depends on accurate decoding of speech signals that are acoustically degraded by challenging listening conditions. This process presents the brain with a demanding computational task that is vulnerable to neurodegenerative pathologies. However, despite recent intense interest in the link between hearing impairment and dementia, comprehension of acoustically degraded speech in these diseases has been little studied. Here we addressed this issue in a cohort of 19 patients with typical Alzheimer's disease and 30 patients representing the three canonical syndromes of primary progressive aphasia (non-fluent/agrammatic variant primary progressive aphasia; semantic variant primary progressive aphasia; logopenic variant primary progressive aphasia), compared to 25 healthy age-matched controls. As a paradigm for the acoustically degraded speech signals of daily life, we used noise-vocoding: synthetic division of the speech signal into frequency channels constituted from amplitude-modulated white noise, such that fewer channels convey less spectrotemporal detail thereby reducing intelligibility. We investigated the impact of noise-vocoding on recognition of spoken three-digit numbers and used psychometric modelling to ascertain the threshold number of noise-vocoding channels required for 50% intelligibility by each participant. Associations of noise-vocoded speech intelligibility threshold with general demographic, clinical and neuropsychological characteristics and regional grey matter volume (defined by voxel-based morphometry of patients' brain images) were also assessed. Mean noise-vocoded speech intelligibility threshold was significantly higher in all patient groups than healthy controls, and significantly higher in Alzheimer's disease and logopenic variant primary progressive aphasia than semantic variant primary progressive aphasia (all P < 0.05). In a receiver operating characteristic analysis, vocoded intelligibility threshold discriminated Alzheimer's disease, non-fluent variant and logopenic variant primary progressive aphasia patients very well from healthy controls. Further, this central hearing measure correlated with overall disease severity but not with peripheral hearing or clear speech perception. Neuroanatomically, after correcting for multiple voxel-wise comparisons in predefined regions of interest, impaired noise-vocoded speech comprehension across syndromes was significantly associated (P < 0.05) with atrophy of left planum temporale, angular gyrus and anterior cingulate gyrus: a cortical network that has previously been widely implicated in processing degraded speech signals. Our findings suggest that the comprehension of acoustically altered speech captures an auditory brain process relevant to daily hearing and communication in major dementia syndromes, with novel diagnostic and therapeutic implications., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain.)

The recognition of Auditory Processing Disorder (APD) as a distinct clinical condition that impacts hearing capacity and mental health has gained attention. Although pure tone audiometry is the gold standard for assessing hearing, it inadequately reflects everyday hearing abilities, especially in challenging acoustic environments. Deficits in speech perception in noise, a key aspect of APD, have been linked to an increased risk of dementia. The World Health Organization emphasizes the need for evaluating central auditory function in cases of mild hearing loss and normal audiometry results. Specific questionnaires play a crucial role in documenting and quantifying the difficulties faced by individuals with APD. Validated questionnaires such as the Children's Auditory Processing Performance Scale, the Fisher's Auditory Problems Checklist, and the Auditory Processing Domains Questionnaire are available for children, while questionnaires for adults include items related to auditory functions associated with APD. This systematic review and meta-analysis identified six questionnaires used for screening and evaluating APD with a total of 783 participants across 12 studies. The questionnaires exhibited differences in domains evaluated, scoring methods, and evaluation of listening in quiet and noise. Meta-analysis results demonstrated that individuals with APD consistently exhibited worse scores compared to healthy controls across all questionnaires. Additionally, comparisons with clinical control groups showed varying results. The study highlights (i) the importance of standardized questionnaires in identifying and assessing APD, aiding in its diagnosis and management, and (ii) the need to use sub-scores as well as overall scores of questionnaires to elaborate on specific hearing and listening situations. There is a need to develop more APD specific questionnaires for the adult population as well as for more focused research on APD diagnosed individuals to further establish the validity and reliability of these questionnaires., Competing Interests: Author MS received honoraria as a consultant or for lectures for Lundbeck and Viatris. The remaining 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 Samara, Thai-Van, Ptok, Glarou, Veuillet, Miller, Reynard, Grech, Utoomprurkporn, Sereti, Bamiou and Iliadou.)

Close links exist between vestibular function and cognition. Dual-task (DT) tests may have ecological validity to assess the impact of daily life cognitive-motor demands in people with vestibular dysfunction (PwVD), functional gait and falls risk. The present paper aimed at building predictive models for functional gait under DT conditions, while clarifying the impact of vestibular dysfunction, individual characteristics, varying task types and motor-cognitive demands. Case-controlled observational study with 39 PwVD and 62 healthy participants. The Functional Gait Assessment (FGA), with and without an additional motor, numeracy, or literacy task, was completed. Multiple linear regression was used to fit models to predict FGA under single and DT performance. Dual task cost (DTC, %) was calculated to assess DT interference on FGA performance using the equation: 100*(single task score-dual task score)/single-task score. Following Bonferroni corrections for multiple comparisons (corrected alpha level of 0.003), PwVD had poorer performance than controls for all FGA conditions (p < 0.001), motor (- 3.94%; p = 0.002) and numeracy (- 22.77%; p = 0.001) DTCs and spatial working memory (p = 0.002). The literacy DTC was marginally significant (- 19.39% p = 0.005). FGA single and DT motor, numeracy, and literacy models explained 76%, 76%, 66% and 67% of the variance respectively for PwVD. Sustained attention, visual memory and sex contributed to all models; short-term visual recognition memory, balance confidence, and migraine contributed to some models. Cognitive performance is impaired in PwVD. Motor, numeracy and literacy tasks impair functional gait performance. Cognitive assessment and FGA with a numeracy or literacy cognitive component should be included within assessment protocols and considered in the provision of targeted interventions for PwVD., (© 2023. The Author(s).)

Objectives: Vestibular rehabilitation clinical guidelines document the additional benefit offered by the Mixed Reality environments in the reduction of symptoms and the improvement of balance in peripheral vestibular hypofunction. The HOLOBalance platform offers vestibular rehabilitation exercises, in an Augmented Reality (AR) environment, projecting them using a low- cost Head Mounted Display. The effect of the AR equipment on the performance in three of the commonest vestibular rehabilitation exercises is investigated in this pilot study., Methods: Twenty-five healthy adults (12/25 women) participated, executing the predetermined exercises with or without the use of the AR equipment., Results: Statistically significant difference was obtained only in the frequency of head movements in the yaw plane during the execution of a vestibular adaptation exercise by healthy adults (0.97 Hz; 95% CI=(0.56, 1.39), p<0.001). In terms of difficulty in exercise execution, the use of the equipment led to statistically significant differences at the vestibular-oculomotor adaptation exercise in the pitch plane (OR=3.64, 95% CI (-0.22, 7.50), p=0.049), and in the standing exercise (OR=28.28. 95% CI (23.6, 32.96), p=0.0001)., Conclusion: Τhe use of AR equipment in vestibular rehabilitation protocols should be adapted to the clinicians' needs., (Copyright: © 2023 Hylonome Publications.)

Exposure to high- and moderate-intensity sound is inevitable for professional singers during their working day, the majority of which is spent in rehearsal, preparing for a performance. The impact of self-produced sound exposure on singers' hearing within the rehearsal setting has not been examined., Objectives: This original pilot field study investigates the feasibility of data collection and hypothesis testing of singers' hearing within the rehearsal environment., Methods: 18 professional choir singers are examined for hearing threshold changes following routine rehearsal sound exposure. Pure Tone Audiometry is measured before, immediately after, and approximately 24 hours after rehearsal., Results: This study does not identify definitive Temporary Threshold Shift in this population under these conditions. That said, mean temporary threshold shift was found 3.61 dB higher than the recovery threshold shift in the right ear at 3000 Hz (P = 0.06), and this may be important to look at for future studies., Conclusions: Methodological challenges of this field study include dynamic experimental conditions intrinsic to the rehearsal process, environmental and musical influence on Pure Tone Audiometry results and estimation of sound intensity exposure., (Copyright © 2021 The Voice Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Established features of classical infratentorial superficial siderosis (iSS) include hearing loss, impaired balance, myelopathy and, less commonly, cognitive compromise. Olfactory function may be affected but dedicated studies are lacking. This study aimed to assess the prevalence of olfactory dysfunction in iSS and correlate it with auditory and cognitive functions., Methods: Ten participants with iSS completed the University of Pennsylvania Smell Identification Test (UPSIT). The scores were compared with population norms; regression analysis was performed to evaluate associations between the scores and hearing thresholds (3-frequency average, 3FA) or the number of cognitive domains impaired. Imaging was reviewed for haemosiderin distribution and to exclude other causes of olfactory and hearing dysfunction., Results: Eight of ten participants were male; the mean (standard deviation, SD) age was 52.5 (14.5) years. Olfactory hypofunction was identified in all participants and in six (60%) was moderate or completely absent. The mean UPSIT score of 25.5 (7.8) was significantly worse than population norms (difference in means - 10.0; 95% CI - 15.6 to - 4.4). Linear regression identified an association between UPSIT and hearing thresholds (R = 0.75; p = 0.013). The score decreases by 0.157 units (95% CI - 0.31 to - 0.002; p = 0.048) per unit increase in 3FA, after adjusting for hearing loss risk factors. There was no statistically significant association between UPSIT and cognitive function (R = 0.383; p = 0.397)., Conclusion: We report a high prevalence of olfactory dysfunction in iSS, the severity of which correlated with hearing loss. Olfaction appears to be a core feature of the iSS clinical syndrome that should be assessed routinely., (© 2022. The Author(s).)

Background and Objectives: Infratentorial superficial siderosis (iSS) is a rare but disabling neurologic condition characterized by progressive hearing loss and balance and mobility problems. The functional decline in these neurologic domains with iSS progression is likely to adversely affect health-related quality of life (HRQoL). We studied the HRQoL of adults with iSS using 2 common generic HRQoL measures (Health Utilities Index Mark III [HUI3] and EuroQoL EQ5D [5 Level]) to determine the most affected domains and evaluate the association between HRQoL scores and disease duration., Methods: This observational study was an anonymous online survey. Following institutional Research Ethics Committee approval, we contacted dedicated international organizations, charities, and patient groups identified through online searches, social media, and collaborative networks, to distribute the study information and study link, inviting their members diagnosed with iSS to participate. Participation required access to a digital device connected to the Internet, confirmation of eligibility (aged 18 years and older and previously diagnosed with iSS), and informed consent to participate in the survey, which included study-specific questions (demographics, iSS, and hearing) and HRQoL questionnaires. Survey responses were captured by the Research Electronic Data Capture survey software and analyzed using the SPSS statistical package. Linear regression analysis was performed to investigate the association between HRQoL scores and disease duration., Results: Of 50 participants, 60% were male; the median (interquartile range [IQR]) age was 60 (15) years. The median (IQR) multiattribute scores for HUI3 and EQ5D were 0.36 (0.53) and 0.64 (0.33), respectively. The most frequently affected domains (moderate or worse category) were hearing (64%) and pain (48%) for HUI3 and mobility (54%) and pain (50%) for EQ5D. There was a weak association between disease duration and multiattribute scores for HUI3 (R = 0.353; adjusted R 2 = 0.096; b = -0.008; p = 0.047) but not EQ5D., Discussion: Our findings demonstrate low HRQoL scores that capture low functional status in several domains typically affected in iSS, suggesting that iSS has a major adverse effect on quality of life in multiple functional domains. Measures of HRQoL in iSS should be included in clinical and research settings, including treatment trials., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Introduction: Auditory neuropathy spectrum disorder (ANSD) is a term for a collection of test results which indicate disruption of the auditory signal at some point along the neural pathway. This results in a spectrum of functional outcomes, ranging from reasonably normal hearing to profound hearing loss. This study assessed brain structure changes and behavioral correlates in children diagnosed with ANSD., Methods: Seventeen children who had previously been diagnosed with ANSD were recruited to the study and underwent a battery of behavioral measures of hearing, language, and communication, along with structural MR imaging. Analysis of cortical thickness of temporal lobe structures was carried out using FreeSurfer. Tract-based spatial statistics were performed on standard diffusion parameters of fractional anisotropy and diffusivity metrics. The control group comprised imaging data taken from a library of MRI scans from neurologically normal children. Control images were matched as closely as possible to the ANSD group for age and sex., Results: Reductions in right temporal lobe cortical thickness were observed in children with ANSD compared to controls. Increases in medial diffusivity in areas including the corpus callosum and in the right occipital white matter were also seen in the group with ANSD compared to controls. Speech perception abilities, both in quiet and in noise, were correlated with cortical thickness measurements for several temporal lobe structures in children with ANSD, and relationships were also seen between diffusion metrics and measures of auditory function., Conclusion: This study shows that children with ANSD have structural brain differences compared to healthy controls. It also demonstrates associations between brain structure and behavioral hearing abilities in children diagnosed with ANSD. These results show that there is a potential for structural imaging to be used as a biomarker in this population with the possibility of predicting functional hearing outcome., (© 2022 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Background: Balance rehabilitation programs represent the most common treatments for balance disorders. Nonetheless, lack of resources and lack of highly expert physiotherapists are barriers for patients to undergo individualized rehabilitation sessions. Therefore, balance rehabilitation programs are often transferred to the home environment, with a considerable risk of the patient misperforming the exercises or failing to follow the program at all. Holobalance is a persuasive coaching system with the capacity to offer full-scale rehabilitation services at home. Holobalance involves several modules, from rehabilitation program management to augmented reality coach presentation., Objective: The aim of this study was to design, implement, test, and evaluate a scoring model for the accurate assessment of balance rehabilitation exercises, based on data-driven techniques., Methods: The data-driven scoring module is based on an extensive data set (approximately 1300 rehabilitation exercise sessions) collected during the Holobalance pilot study. It can be used as a training and testing data set for training machine learning (ML) models, which can infer the scoring components of all physical rehabilitation exercises. In that direction, for creating the data set, 2 independent experts monitored (in the clinic) 19 patients performing 1313 balance rehabilitation exercises and scored their performance based on a predefined scoring rubric. On the collected data, preprocessing, data cleansing, and normalization techniques were applied before deploying feature selection techniques. Finally, a wide set of ML algorithms, like random forests and neural networks, were used to identify the most suitable model for each scoring component., Results: The results of the trained model improved the performance of the scoring module in terms of more accurate assessment of a performed exercise, when compared with a rule-based scoring model deployed at an early phase of the system (k-statistic value of 15.9% for sitting exercises, 20.8% for standing exercises, and 26.8% for walking exercises). Finally, the resulting performance of the model resembled the threshold of the interobserver variability, enabling trustworthy usage of the scoring module in the closed-loop chain of the Holobalance coaching system., Conclusions: The proposed set of ML models can effectively score the balance rehabilitation exercises of the Holobalance system. The models had similar accuracy in terms of Cohen kappa analysis, with interobserver variability, enabling the scoring module to infer the score of an exercise based on the collected signals from sensing devices. More specifically, for sitting exercises, the scoring model had high classification accuracy, ranging from 0.86 to 0.90. Similarly, for standing exercises, the classification accuracy ranged from 0.85 to 0.92, while for walking exercises, it ranged from 0.81 to 0.90., Trial Registration: ClinicalTrials.gov NCT04053829; https://clinicaltrials.gov/ct2/show/NCT04053829., (©Vassilios Tsakanikas, Dimitris Gatsios, Athanasios Pardalis, Kostas M Tsiouris, Eleni Georga, Doris-Eva Bamiou, Marousa Pavlou, Christos Nikitas, Dimitrios Kikidis, Isabelle Walz, Christoph Maurer, Dimitrios Fotiadis. Originally published in JMIR Rehabilitation and Assistive Technology (https://rehab.jmir.org), 31.08.2022.)

Balance disorders and falls are common in the elderly population. Regular balance exercises are an evidence-based physical intervention to prevent falls in older adults, while patient motivation and adherence are important factors for intervention outcome. Exergames are a relatively new, alternative intervention for physical rehabilitation as they improve balance and strength in older adults. The aims of this systematic review and meta-analysis were to assess the (1) effect of motivation factors as per the Capability, Opportunity and Motivation model of Behavior change (COM-B) on the effectiveness of exergame interventions in healthy older adults, (2) effectiveness of exergames to improve balance in older healthy adults and, (3) impact of exergames on cognitive outcomes. Results show that motivation and capability components influence the general outcome of the exergame training. Motivational factors should thus be considered when setting-up an exergame intervention. Furthermore, exergame intervention appears to be a promising training method in comparison to traditional exercise training. However, exergame training in itself might not be sufficient to improve fall risk and cognitive performance., 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 Buyle, Jung, Pavlou, Gonzalez and Bamiou.)

The term superficial siderosis (SS) is derived from the Greek word 'sideros', meaning iron. It includes two subtypes, distinguished by their anatomical distribution, causes and clinical features: 'classical' infratentorial SS (iSS, which sometimes also affects supratentorial regions) and cortical SS (cSS, which affects only supratentorial regions). This paper considers iSS, a potentially disabling disorder usually associated with very slow persistent or intermittent subarachnoid bleeding from a dural defect, and characterised by progressive hearing and vestibular impairment, ataxia, myelopathy and cognitive dysfunction. The causal dural defect-most often spinal but sometimes in the posterior fossa-typically follows trauma or neurosurgery occurring decades before diagnosis. Increasing recognition of iSS with paramagnetic-sensitive MRI is leading to an unmet clinical need. Given the diagnostic challenges and complex neurological impairments in iSS, we have developed a multidisciplinary approach involving key teams. We discuss pathophysiology, diagnosis and management of iSS, including a proposed clinical care pathway., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: There is growing evidence supporting the use of third-wave psychological therapies, such as mindfulness-based interventions (MBIs) and acceptance and commitment therapy (ACT), for people with long-term or chronic physical health conditions. We conducted a systematic review and meta-analysis to critically evaluate the effectiveness of third-wave interventions for improving hearing-related distress and psychological well-being in people with audiological problems., Method: We searched online bibliographic databases and assessed study quality. We conducted random-effects meta-analyses if at least two randomized controlled trials (RCTs) examined hearing-related distress, depression, anxiety, or quality of life in people with audiological problems. Findings of pre-post studies were summarized narratively., Results: We identified 15 studies: six RCTs and nine pre-post studies. The methodological quality of studies was mostly poor to moderate, and sample sizes were typically small (overall n = 750). Most studies focused on tinnitus ( n = 12), MBIs ( n = 8), and ACT ( n = 6). Statistically significant improvements in hearing-related distress were found with ACT and MBIs versus controls and other treatments at post-intervention in people with tinnitus, while improvements in depression and anxiety were only found for ACT versus controls at post-intervention. However, gains were either not maintained or not examined at follow-up, and there was no evidence for improvements in quality of life., Conclusions: At present, there is insufficient evidence to recommend the use of third-wave interventions for improving hearing-related distress or psychological well-being in people with audiological problems. There is some evidence that ACT and MBIs may be useful in addressing hearing-related distress in people with tinnitus, but only in the short term. However, findings should be interpreted with caution given the small number of studies with generally small sample sizes and mostly poor-to-moderate methodological quality. More high-quality, adequately powered, double-blind RCTs, particularly in audiological problems other than tinnitus, are needed to draw firm conclusions and meaningful clinical recommendations., Supplemental Material: https://doi.org/10.23641/asha.19735975.

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).)

Introduction: Vestibular migraine (VM) is a common condition; individuals experience dizziness with migraine symptoms. Vestibular rehabilitation therapy (VRT) has been reported as an effective treatment for VM, however, evidence is limited. VM and traumatic brain injury (TBI) can co-occur, and some suggest that TBI can induce VM. There is limited evidence on the effect a history of TBI has on VRT in patients with VM., Methods: Retrospective case series of 93 (f = 63, m = 30) participants with VM and underwent VRT (mean age 48.62; SD 15.92). Pre- and post-treatment self-reported outcome measures and functional gait assessment were extracted from the participants health records and evaluated. The impact of TBI on VRT outcome in participants with VM was analyzed. Individuals with TBI and no history of migraine (n = 40) were also extracted to act as a control., Results: VRT significantly improved self-reported dizziness on the Dizziness Handicap Inventory (DHI), with a mean change of -18 points (p < 0.000) and +5 points on the functional gait assessment (FGA) (p < 0.000) in patients with VM. A history of TBI significantly impacted outcome on the DHI (p = 0.018) in patients with VM.VRT significantly improved all outcome measures for individuals with TBI, with a mean change of -16 points on the DHI (p = 0.001) and +5 points on the FGA (p < 0.000). VM presence significantly impacted outcome., Conclusion: VRT should be considered as a treatment option to reduce dizziness and the risk of falls in individuals with VM. TBI may negatively impact VRT outcomes in individuals with VM., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Physical inactivity and sedentary time are associated with all-cause mortality, chronic non-communicable diseases and falls in the elderly. Objective of this review is to assess and summarize recommendations from clinical guidelines for physical activity (PA) of older adults in general and related to falls. A scoping review of the existing clinical guidelines was conducted. The included studies should have been developed under the auspices of a health organization and their methodology should be described in detail. Nine clinical guidelines providing specific recommendations for the elderly were identified. There was a strong agreement across the guidelines regarding goals, activities parameters, adverse effects of PA, in addition to reference for preventing falls. Keeping even the minimum of physical activity, introducing balance exercises and strengthening exercises for preventing falls, avoiding unexpected accelerations in the intensity of the activities, applying the necessary precautions and consulting a health professional are the main pillars of recommendations. Despite any deficiencies in definitions, monitoring and optimal dosage consistency of recommendations, is an ideal incentive for countries and organizations to adopt and enhance physical activity as an antidote to the degeneration of human's health and quality of life., (Copyright: © 2022 Hylonome Publications.)

Introduction: It has been proposed that hearing loss may result in improved visuospatial abilities. The evidence for this assertion is inconsistent, and limited to studies in congenitally deaf children, despite older adults with age-related hearing loss constituting the vast majority of the hearing impaired population. We assessed visuospatial (visuoconstruction and visuospatial memory) ability in older adult hearing aid users with and without clinically significant cognitive impairment. The primary aim of the study was to determine the effect of hearing loss on visuospatial abilities., Method: Seventy-five adult hearing aid users (HA) aged over 65 were recruited, out of whom 30 had normal cognition (NC-HA), 30 had mild cognitive impairment (MCI-HA), and 15 had dementia (D-HA). The Rey Osterrieth Complex figure test (ROCFT) copy, 3 min recall and 30 min recall tests were performed to evaluate the visuoconstructional and visuospatial memory abilities of the participants., Results: There were significant differences between the ROCFT copy, 3 min recall, and 30 min recall among the three cohorts ( p < 0.005). Compared with previously published normative data, the NC-HA performed significantly better in the ROCFT copy ( p < 0.001), immediate recall ( p < 0.001), and delay recall ( p = 0.001), while the MCI-HA performed similarly to the expected norms derived from population ( p = 0.426, p = 0.611, p = 0.697, respectively), and the D-HA performed below this norm., Conclusion: Though visuospatial abilities tend to decline when the global cognitive functioning declines, we found suggestive evidence for positive effects of age-related hearing loss on visuospatial cognitive ability. Participants with mild cognitive impairment and hearing loss, who would have been expected to perform worse than normative data, were in fact performing as well as cognitively healthy subjects without hearing loss. Visuospatial ability could be targeted when providing rehabilitation for the older adults with hearing loss., 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 Utoomprurkporn, Stott, Costafreda and Bamiou.)

Background: Hearing loss is a major public health challenge. Audiology services need to utilise a range of rehabilitative services and maximise innovative practice afforded by technology to actively promote personalized, participatory, preventative and predictive care if they are to cope with the social and economic burden placed on the population by the rapidly rising prevalence of hearing loss. Digital interventions and teleaudiology could be a key part of providing high quality, cost-effective, patient-centred management. There is currently very limited evidence that assesses the hearing impaired patient perspective on the acceptance and usability of this type of technology., Aim: This study aims to identify patient perceptions of the use of a hearing support system including a mobile smartphone app when used with Bluetooth-connected hearing aids across the everyday life of users, as part of the EVOTION project., Methods: We applied a questionnaire to 564 participants in three countries across Europe and analysed the following topics: connectivity, hearing aid controls, instructional videos, audiological tests and auditory training., Key Findings: Older users were just as satisfied as younger users when operating this type of technology. Technical problems such as Bluetooth connectivity need to be minimised as this issue is highly critical for user satisfaction, engagement and uptake. A system that promotes user-controllability of hearing aids that is more accessible and easier to use is highly valued. Participants are happy to utilise monitoring tests and auditory training on a mobile phone out of the clinic but in order to have value the test battery needs to be relevant and tailored to each user, easy to understand and use. Such functions can elicit a negative as well as positive experience for each user., Conclusion: Older and younger adults can utilise an eHealth mobile app to complement their rehabilitation and health care. If the technology works well, is tailored to the individual and in-depth personalised guidance and support is provided, it could assist maximisation of hearing aid uptake, promotion of self-management and improving outcomes., 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 Murdin, Sladen, Williams, Bamiou, Bibas, Kikidis, Oiknonomou, Kouris, Koutsouris and Pontoppidan.)

Objectives: This research aims to validate a modified visually based Montreal Cognitive Assessment for hearing-aid users (MoCA-HA). This population should be the target of cognitive screening due to high risk of developing dementia., Design: Case-control study., Setting: The participants were recruited from referral hearing-aid center and memory clinic in central London, United Kingdom., Participant: 75 hearing-aid users were recruited. Of these, thirty were cognitively intact controls with hearing impairment (NC-HI); thirty had mild cognitive impairment with hearing impairment (MCI-HI); fifteen had dementia with hearing impairment (D-HI)., Measurements: The baseline characteristics and analysis of the MoCA-HA for the NC-HI were recorded. The MoCA-HA performance of the MCI-HI cohort and D-HI cohort were also studied., Results: The cutpoint of <26 yields 93.3% sensitivity with 80% specificity in distinguishing MCI-HI from NC-HI. The specificity increased to 95.6% in screening for all cognitive impairment (MCI-HI and D-HI) from NC-HI., Conclusion: The MoCA-HA has been validated with a cutpoint which is comparable to the traditional MoCA. This tool may help clinicians to early identify older adult hearing-aid users for appropriate cognitive evaluation., 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 Utoomprurkporn, Stott, Costafreda, North, Heatley and Bamiou.)

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).)

Misophonia is a common disorder characterized by the experience of strong negative emotions of anger and anxiety in response to certain everyday sounds, such as those generated by other people eating, drinking, and breathing. The commonplace nature of these "trigger" sounds makes misophonia a devastating disorder for sufferers and their families. How such innocuous sounds trigger this response is unknown. Since most trigger sounds are generated by orofacial movements (e.g., chewing) in others, we hypothesized that the mirror neuron system related to orofacial movements could underlie misophonia. We analyzed resting state fMRI (rs-fMRI) connectivity ( N = 33, 16 females) and sound-evoked fMRI responses ( N = 42, 29 females) in misophonia sufferers and controls. We demonstrate that, compared with controls, the misophonia group show no difference in auditory cortex responses to trigger sounds, but do show: (1) stronger rs-fMRI connectivity between both auditory and visual cortex and the ventral premotor cortex responsible for orofacial movements; (2) stronger functional connectivity between the auditory cortex and orofacial motor area during sound perception in general; and (3) stronger activation of the orofacial motor area, specifically, in response to trigger sounds. Our results support a model of misophonia based on "hyper-mirroring" of the orofacial actions of others with sounds being the "medium" via which action of others is excessively mirrored. Misophonia is therefore not an abreaction to sounds, per se, but a manifestation of activity in parts of the motor system involved in producing those sounds. This new framework to understand misophonia can explain behavioral and emotional responses and has important consequences for devising effective therapies. SIGNIFICANCE STATEMENT Conventionally, misophonia, literally "hatred of sounds" has been considered as a disorder of sound emotion processing, in which "simple" eating and chewing sounds produced by others cause negative emotional responses. Our data provide an alternative but complementary perspective on misophonia that emphasizes the action of the trigger-person rather than the sounds which are a byproduct of that action. Sounds, in this new perspective, are only a "medium" via which action of the triggering-person is mirrored onto the listener. This change in perspective has important consequences for devising therapies and treatment methods for misophonia. It suggests that, instead of focusing on sounds, which many existing therapies do, effective therapies should target the brain representation of movement., (Copyright © 2021 Kumar et al.)

(1) Introduction: The validity of self-reported hearing disability measures has been assessed using their correlation with the pure-tone average (PTA) hearing loss for non-cognitively impaired adults. However, for people with cognitive impairment, factors in addition to the PTA can play a role in their self-reported difficulties. Patients with cognitive impairment may experience more hearing difficulties due to their brain processing sounds abnormally, irrespective of PTA. (2) Methods: Three groups of hearing aid users who had normal cognition, mild cognitive impairment and dementia were recruited. Self-reported hearing abilities were assessed with the modified Amsterdam inventory for auditory disability (mAIAD) and the speech, spatial and qualities of hearing scale (SSQ). (3) Results: The SSQ and mAIAD scores were highly correlated with each other for all three groups. However, a correlation with objective PTA was found in the normal cognition but not the cognitively impaired groups. Self-reported hearing scores were associated with cognitive scores for the dementia group (4) Discussion: In people with combined cognitive and hearing impairment, PTA alone may be a poor predictor of hearing abilities. Subjective hearing questionnaires together with hearing tests may provide a better understanding of their hearing difficulties.

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: A large proportion of older adults assessed for cognitive impairment likely have hearing loss, potentially affecting accuracy of cognitive performance estimations. This study aimed to develop a hearing-impaired version of the Addenbrooke's Cognitive Examination-III (HI-ACE-III) and to assess whether the HI-ACE-III can accurately distinguish people with mild cognitive impairment (MCI) and dementia from cognitively intact controls., Methods: The HI-ACE-III was developed by converting verbal instructions into a visual, timed PowerPoint presentation. Seventy-four participants over the age of 60 years were classified into three groups: 29 had MCI, 15 had mild to moderate dementia and 30 were cognitively intact controls. Receiver operating characteristic (ROC) curves were graphed to test screening accuracy. Concurrent validity was examined through correlations between HI-ACE-III domain scores and relevant, visually presented standardized neuropsychological measures., Results: ROC analysis for dementia revealed an area under the curve (AUC) of 0.99, achieving excellent sensitivity (100%) and good specificity (93.3%) at an optimum cut-off of <87. The AUC for MCI was 0.86, achieving reasonable sensitivity (75.9%) and good specificity (86.7%) at an optimum cut-off of <92. HI-ACE-III subtests shared anticipated and statistically significant correlations with established measures of cognitive functioning. Internal consistency of the HI-ACE-III was excellent as verified with Cronbach's alpha (α = 0.904)., Conclusions: Preliminarily, the HI-ACE-III showed good reliability, validity and screening utility for MCI and dementia in older adults in a hearing-impairment context. The adapted HI-ACE-III may offer accurate and reliable indication of cognitive performance, supporting timely diagnosis and research examining links between hearing loss and cognitive decline., (© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

The association between hearing impairment and dementia has emerged as a major public health challenge, with significant opportunities for earlier diagnosis, treatment and prevention. However, the nature of this association has not been defined. We hear with our brains, particularly within the complex soundscapes of everyday life: neurodegenerative pathologies target the auditory brain, and are therefore predicted to damage hearing function early and profoundly. Here we present evidence for this proposition, based on structural and functional features of auditory brain organization that confer vulnerability to neurodegeneration, the extensive, reciprocal interplay between 'peripheral' and 'central' hearing dysfunction, and recently characterized auditory signatures of canonical neurodegenerative dementias (Alzheimer's disease, Lewy body disease and frontotemporal dementia). Moving beyond any simple dichotomy of ear and brain, we argue for a reappraisal of the role of auditory cognitive dysfunction and the critical coupling of brain to peripheral organs of hearing in the dementias. We call for a clinical assessment of real-world hearing in these diseases that moves beyond pure tone perception to the development of novel auditory 'cognitive stress tests' and proximity markers for the early diagnosis of dementia and management strategies that harness retained auditory plasticity., (© The Author(s) (2020). Published by Oxford University Press on behalf of the Guarantors of Brain.)

Objective: To explore the views and experiences of individuals with Auditory Processing Disorder (APD) and/or their families in getting support from services and to receive their suggestions for improvement. Design: Cross-sectional random sample survey with descriptive analysis. Settings: Online survey. Participants: One hundred and fifty six individuals with APD and/or their family members from the APD Support UK patient support organization and four associated APD Facebook groups. Main Outcome Measure: A 16-item questionnaire on negative and positive experiences in getting a referral for diagnosis, funding for the FM system, and overall support for APD. Results: The key findings that emerged included reports of difficulty in getting a referral for diagnosis (54%), obtaining funding for an FM system (45%), getting support for APD (61%), and poor recognition and awareness of APD (63%) in Education, Health or Work settings. The positive experiences reported were ease in getting a referral for diagnosis (46%), in obtaining an FM system (20%), and with diagnosis leading to help at school or to a better understanding of the condition and the required adjustments. The recommended improvement areas were raising awareness of APD and related management in Education (30%), the Health sector (25%), and the public (18%). Conclusions: Individuals and families of individuals with APD overwhelmingly report a lack of awareness of APD across health, education, and work sectors, and difficulties in getting access to diagnosis and support. This information may provide an initial understanding of the patients' needs for clinical services for APD, identify research priorities, and influence longer-term public health decisions toward improved care., 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 Agrawal, Dritsakis, Mahon, Mountjoy and Bamiou.)

Introduction: Approximately one in three of all older adults fall each year, with wide ranging physical, psychosocial and healthcare-related consequences. Exercise-based interventions are the cornerstone for falls prevention programmes, yet these are not consistently provided, do not routinely address all components of the balance system and are often not well attended. The HOLOBalance system provides an evidence-based balance training programme delivered to patients in their home environment using a novel technological approach including an augmented reality virtual physiotherapist, exergames and a remote monitoring system. The aims of this proof-of-concept study are to (1) determine the safety, acceptability and feasibility of providing HOLOBalance to community dwelling older adults at risk for falls and (2) provide data to support sample size estimates for a future trial., Methods: A single (assessor) blinded pilot randomised controlled proof of concept study. 120 participants will be randomised to receive an 8-week home exercise programme consisting of either: (1) HOLOBalance or (2) The OTAGO Home Exercise Programme. Participants will be required to complete their exercise programme independently under the supervision of a physiotherapist. Participants will have weekly telephone contact with their physiotherapist, and will receive home visits at weeks 0, 3 and 6. Outcome measures of safety, acceptability and feasibility, clinical measures of balance function, disability, balance confidence and cognitive function will be assessed before and immediately after the 8 week intervention. Acceptability and feasibility will be explored using descriptive statistics, and trends for effectiveness will be explored using general linear model analysis of variance., Ethics and Dissemination: This study has received institutional ethical approvals in Germany (reference: 265/19), Greece (reference: 9769/24-6-2019) and the UK (reference: 19/LO/1908). Findings from this study will be submitted for peer-reviewed publications., Trial Registration Number: NCT04053829., Protocol Version: V.2, 20 January 2020., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.)

Background: The ageing process may degrade an individual's balance control, hearing capacity, and cognitive function. Older adults perform worse on simultaneously executed balance and secondary tasks (i.e., dual-task performance) than younger adults and may be more vulnerable to auditory distraction. Aim: The purpose of this study was to determine the effect of passive listening on functional gait in healthy older vs. younger adults, and to investigate the effect of age, functional gait, hearing ability and cognitive functioning on dual-task performance. Methods: Twenty young and 20 older healthy adults were recruited. Functional gait (Functional Gait Assessment in silent and noisy condition), hearing function (audiogram; Speech in Babble test), and cognitive ability (Cambridge Neuropsychological Test Automated Battery) were measured. Results: Overall, a significant difference between functional gait performance in silent vs. noisy conditions was found ( p = 0.022), with no significant difference in dual-task cost between the two groups ( p = 0.11). Correlations were found between increasing age, worse functional gait performance, poorer hearing capacity and lower performance on cognitive function tasks. Interestingly, worse performance on attention tasks appeared to be associated with a worse functional gait performance in the noisy condition. Conclusion: Passive listening to multi-talker babble noise can affect functional gait in both young and older adults. This effect could result from the cognitive load of the babble noise, due to the engagement of attention networks by the unattended speech., 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 Buyle, Azoidou, Pavlou, Van Rompaey and Bamiou.)