Paediatric percutaneous bone anchored hearing aid implant failures: Comparing the experience of a tertiary centre with a systematic review of the literature and meta-analysis.

BACKGROUND: Despite the proven audiological benefits of Percutaneous Bone Anchored Hearing Aids (BAHAs) in paediatric patients with conductive or mixed hearing loss, their adoption has been limited due to concerns over implant failure and associated complications. This paper conducts a systematic review and meta-analysis to assess the prevalence of implant failure in paediatric populations, combined with a case series from our tertiary referral centre. METHODS: A comprehensive literature search identified 562 articles, from which 34 were included in the review, covering 1599 implants in 1285 patients. Our retrospective case series included consecutive patients from our tertiary referral centre who underwent percutaneous BAHA implantation from 2003-2019. RESULTS: Meta-analysis revealed an overall implant failure rate of 11%, predominantly attributed to traumatic extrusion. Our retrospective case series comprised 104 implantations in 76 patients, with a 4.8% failure rate. DISCUSSION: Factors contributing to the lower-than-expected failure rates in the case series likely included consistent use of 4 mm fixtures from a single manufacturer and older age at implantation. The study underscores the need for standardised reporting formats in bone conduction implants research, given the systematic review's limitations in study design heterogeneity, especially with the expected rise in the adoption of novel active devices.

Hearing preservation in paediatric cochlear implantation with the Nucleus Slim Straight Electrode - our experience.

OBJECTIVE: to evaluate the levels of successful hearing preservation and preservation of functional hearing following cochlear implantation (HPCI) in children using the Cochlear Nucleus® Slim Straight Electrode (SSE). DESIGN: retrospective case note review of paediatric HPCI cases in our CI centre from 2013 to 2023. Inclusion criteria were attempted hearing preservation surgery, SSE used for implantation, pre-operative hearing thresholds ≤80dBHL at 250 Hz, CI before 18 years of age. Patients were excluded if no postoperative unaided PTA was obtained (poor attendance). Primairy outcome was hearing preservation using the HEARRING group formula; secondary outcome was residual functional hearing (≤80dBHL at 250 Hz/<90dB LFPTA). STUDY SAMPLE: 56 patients with 94 CI's were included for review. RESULTS: Hearing preservation was achieved in 94.7% (89/94) of ears and complete preservation in 72% (68/94)). Average functional hearing was preserved in 89% using both criteria for preservation. Long-term follow up data was available for 36 ears (average 35.2 months), demonstrating 88.9% (32/36) complete preservation. CONCLUSION: We have reliably achieved and maintained a high success rate of HPCI using the SSE in our paediatric population. The field of HPCI would benefit from unification of outcome reporting in order to optimise the evidence available to professionals, patients and their carers.

Exploring NICU nurses' views of a novel genetic point-of-care test identifying neonates at risk of antibiotic-induced ototoxicity: A qualitative study.

AIM: To explore the views of neonatal intensive care nursing staff on the deliverability of a novel genetic point-of-care test detecting a genetic variant associated with antibiotic-induced ototoxicity. DESIGN: An interpretive, descriptive, qualitative interview study. METHODS: Data were collected using semi-structured interviews undertaken between January and November 2020. Participants were neonatal intensive care nursing staff taking part in the Pharmacogenetics to Avoid Loss of Hearing trial. RESULTS: Thematic analysis resulted in four themes: perceived clinical utility; the golden hour; point-of-care device; training and support. Recommendations were made to streamline the protocol and ongoing training and support were considered key to incorporating the test into routine care. CONCLUSION: Exploring the views of nurses involved in the delivery of the point-of-care test was essential in its implementation. By the study endpoint, all participants could see the value of routine clinical introduction of the point-of care test. IMPLICATIONS FOR THE PROFESSION AND/OR PATIENT CARE: Nurses are in a key position to support the delivery of point-of-care genetic testing into mainstream settings. This study has implications for the successful integration of other genetic point-of-care tests in acute healthcare settings. IMPACT: The study will help to tailor the training and support required for routine deployment of the genetic point-of-care test. The study has relevance for nurses involved in the development and delivery of genetic point-of-care tests in other acute hospital settings. REPORTING METHOD: This qualitative study adheres to the Standards for Reporting Qualitative Research EQUATOR guidelines and utilizes COREQ and SRQR checklists. PATIENT OR PUBLIC CONTRIBUTION: All staff working on the participating neonatal intensive care units were trained to use the genetic point-of-care test. All inpatients on the participating units were eligible to have testing via the point-of-care test. The Pharmacogenetics to Avoid Loss of Hearing Patient and Public Involvement and Engagement group provided valuable feedback. TRIAL AND PROTOCOL REGISTRATION: Registered within the University of Manchester. Ethics approval reference numbers: IRAS: 253102 REC reference: 19/NW/0400. Also registered with the ISRCTN ref: ISRCTN13704894.

Risk factors associated with the development of chronic suppurative otitis media in children: Systematic review and meta-analysis.

OBJECTIVES: Chronic suppurative otitis media (CSOM) is defined as persistent discharge through a tympanic membrane perforation for greater than 2 weeks. It is associated with a significant disease burden, including hearing loss, and reducing its incidence could significantly improve short- and long-term health. We aimed to identify risk factors associated with the development of CSOM in children. DESIGN AND SETTING: Systematic review and meta-analysis of studies set in community, primary and secondary care settings, identified from Medline, Embase and Cochrane databases from 2000 to 2022. PARTICIPANTS: Children 16 years old and below. MAIN OUTCOME MEASURES: Clinical diagnosis of CSOM. RESULTS: In total, 739 papers were screened, with 12 deemed eligible for inclusion in the systematic review, of which, 10 were included in the meta-analysis. Risk factors examined included perinatal, patient, dietary, environmental and parental factors. Meta-analysis results indicate that atopy (RR = 1.18, 95% CI [1.01-1.37], p = .04, 2 studies); and birth weight <2500 g (RR = 1.79 [1.27-2.50], p < .01, 2 studies) are associated with an increased risk of CSOM development. Factors not associated were male sex (RR = 0.96 [0.82-1.13], p = .62, 8 studies); exposure to passive smoking (RR = 1.27 [0.81-2.01], p = .30, 3 studies); and parental history of otitis media (RR = 1.14 [0.59-2.20], p = .69, 2 studies). CONCLUSION: Optimal management of risk factors associated with CSOM development will help reduce the burden of disease and prevent disease progression or recurrence. The current quality of evidence in the literature is variable and heterogeneous. Future studies should aim to use standardised classification systems to define risk factors to allow meta-analysis.

Mobile audiometry for hearing threshold assessment: A systematic review and meta-analysis.

OBJECTIVES: Technological advancements in mobile audiometry (MA) have enabled hearing assessment using tablets and smartphones. This systematic review (PROSPERO ID: CRD42021274761) aimed to identify MA options available to health providers, assess their accuracy in measuring hearing thresholds, and explore factors that might influence their accuracy. DESIGN AND SETTING: A systematic search of online databases including PubMed, Embase, Cochrane, Evidence Search and Dynamed was conducted on 13th December 2021, and repeated on 30th October 2022, using appropriate Medical Subject Headings (MeSH) terms. Eligible studies reported the use of MA to determine hearing thresholds and compared results to conventional pure-tone audiometry (CA). Studies investigating MA for hearing screening (i.e. reporting just pass/fail) were ineligible for inclusion. Two authors independently reviewed studies, extracted data, and assessed methodological quality and risk of bias using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool. PARTICIPANTS: Adults and children, with and without diagnosis of hearing impairment. MAIN OUTCOME MEASURES: A meta-analysis was performed to obtain the mean difference between thresholds measured using MA and CA in dB HL. RESULTS: Searches returned 858 articles. After systematic review, 17 articles including 1032 participants were analysed. The most used software application was ShoeboxTM (6/17) followed by Hearing TestTM (3/17), then HearTestTM (2/17). Tablet computers were used in ten studies, smartphones in six, and a computer in one. The mean difference between MA and CA thresholds was 1.36 dB (95% CI, 0.07-2.66, p = 0.04). Significant differences between mobile audiometry (MA) and conventional audiometry (CA) thresholds were observed in thresholds measured at 500Hz, in children, when MA was conducted in a sound booth, and when MA was self-administered. However, these differences did not exceed the clinically significant threshold of 10 decibels (dB). Included studies exhibited high levels of heterogeneity, high risk of bias and low concerns about applicability. CONCLUSIONS: MA compares favourably to CA in measuring hearing thresholds and has role in providing access to hearing assessment in situations where CA is not available or feasible. Future studies should prioritize the integration of pure-tone threshold assessment with additional tests, such as Speech Recognition and Digits-in-Noise, for a more rounded evaluation of hearing ability, assesses acceptability and feasibility, and the cost-effectiveness of MA in non-specialist settings.

Outcome measures for use in trials of paediatric otorrhoea: A systematic review.

INTRODUCTION: Paediatric otorrhoea (PO) describes a middle ear infection that results in a perforation of the tympanic membrane and ear discharge, in children and young people (CYP). Prolonged infection may be associated with hearing loss and developmental delay. The current management of paediatric otorrhoea is variable, including non-invasive treatments (conservative, oral antibiotics, topical antibiotics) and surgery, reflecting the lack of a sufficiently strong evidence base. Outcome reporting is fundamental to producing reliable and meaningful evidence to inform best practice. OBJECTIVES: Primary objective: to determine which outcome measures are currently used to evaluate treatment success in studies of non-surgical treatments for paediatric otorrhoea. SECONDARY OBJECTIVES: to identify outcome measurement instruments used in the literature and assess their applicability for use in clinical trials of PO. METHODS: This systematic review was registered with PROSPERO (CRD42023407976). Database searches of EMBASE, MEDLINE and Cochrane was performed on June 6, 2023, covering from Jan 1995 to May 2023. Randomised controlled trials or study protocols involving CYP with PO were included following PRISMA guidelines. Risk of bias was assessed with Cochrane's tool. RESULTS: Of the 377 papers identified, six were included in the systematic review. The primary outcome of five of the studies related to otorrhoea cessation; both time to cessation and proportion recovered at various time points were used as measures. Two measurement instruments were identified: Otitis Media-6 Questionnaire and the Institute for Medical Technology Assessment Productivity Cost Questionnaire. Both were shown to be applicable measurement instruments when used in clinical trials of PO. CONCLUSIONS: To promote homogeneity and facilitate meaningful comparison and combination of studies, we propose that time to cessation of otorrhoea from onset of otorrhoea should be used as the primary outcome in future studies. Further research is needed to establish if this is the most important outcome to children and their caregivers.

Enlarged Vestibular Aqueduct and Associated Inner Ear Malformations: Hearing Loss Prognostic Factors and Data Modeling from an International Cohort.

ACKGROUND: There is a need to operationalize existing clinical data to support precision medicine in progressive hearing loss (HL). By utilizing enlarged vestibular aqueduct (EVA) and its associated inner ear abnormalities as an exemplar, we model data from a large international cohort, confirm prognostic factors for HL, and explore the potential to generate a prediction model to optimize current management paradigms. METHODS: An international retrospective cohort study. Regression analyses were utilized to model frequency-specific HL and identify prognostic factors for baseline average HL severity and progression. Elastic-net regression and machine learning (ML) techniques were utilized to predict future average HL progression based upon routinely measurable clinical, genetic, and radiological data. RESULTS: Higher frequencies of hearing were lost more severely. Prognostic factors for HL were the presence of incomplete partition type 2 (coefficient 12.95 dB, P=.011, 95% CI 3.0-22 dB) and presence of sac signal heterogeneity (P=.009, 95% CI 0.062-0.429) on magnetic resonance imaging. Elastic-net regression outperformed the ML algorithms (R2 0.32, mean absolute error 11.05 dB) with coefficients for baseline average hearing level and the presence of sac heterogeneity contributing the most to prediction outcomes. CONCLUSION: Incomplete partition type 2 and endolymphatic sac signal heterogeneity phenotypes should be monitored closely for hearing deterioration and need for early audiological rehabilitation/cochlear implant. Preliminary prediction models have been generated using routinely collected health data in EVA. This study showcases how international collaborative research can use exemplar techniques to improve precision medicine in relatively rare disease entities.

Virtual reality in simulation-based emergency skills training: A systematic review with a narrative synthesis.

Objective: An important role is predicted for virtual reality (VR) in the future of medical education. We performed a systematic review of the literature with a narrative synthesis, to examine the current evidence for VR in simulation-based emergency skills training. We broadly define emergency skills as any clinical skill used in the emergency care of patients across all clinical settings. Methods: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta Analyses (PRISMA) guidelines. The data sources accessed during this study included: PubMed, CINAHL, EMBASE, AMED, EMCARE, HMIC, BNI, PsychINFO, Medline, CENTRAL, SCOPUS, Web of Science, BIOSIS Citation Index, ERIC, ACM Digital Library, IEEE Xplore, and ProQuest Dissertations and Thesis Global. Cochrane's Rob 2 and ROBVIS tools were used during study quality assessment. No ethical review was required for this work. Results: Thirty-four articles published between 14th March 1998 and 1st March 2022 were included in this review. Studies were predominantly conducted in the USA and Europe and focussed on a variety of healthcare disciplines including medical, nursing, and allied health. VR education was delivered using head-mounted displays, Cave Automatic Virtual Environment systems, and bespoke setups. These systems delivered education in a variety of areas (emergency medicine, equipment training, obstetrics, and basic/advanced life support). Subjective potential advantages of this technology included realism, replayability, and time-effectiveness. Reports of adverse events were low in frequency across the included studies. Whilst clear educational benefit was generally noted, this was not reflected in changes to patient-based outcomes. Conclusion: There may be educational benefit to using VR in the context of simulation-based emergency skills training including knowledge gain and retention, skill performance, acceptability, usability, and validity. Currently, there is insufficient evidence to demonstrate clear cost-effectiveness, or direct improvement of patient or institutional outcomes, at this stage.

Protocol for the Paediatric Otorrhoea Study (POSt): a multi-methods study to understand the burden of paediatric otorrhoea in the UK.

INTRODUCTION: Paediatric otorrhoea (PO) refers to the leakage of fluid through a perforation in the ear drum, resulting from an infection of the middle ear of a child or young person (CYP). PO frequently results in hearing loss which may lead to developmental delay, restricted communication and reduced educational attainment.Epidemiological information for PO is largely derived from low-income countries. The aim of this study will be to establish the incidence of PO within the UK and to understand the impact of PO on CYP and their families' everyday lives. It will build the foundations for a randomised controlled trial investigating the best antibiotic treatment for PO. METHODS AND ANALYSIS: The study will consist of two work packages. (1) Data from the Clinical Practice Research Datalink (CPRD), January 2005 to July 2021, will be used to determine the incidence of patient presentations with PO to primary care in the UK. It will also explore the current antimicrobial prescribing practice for PO in primary care. (2) Thirty semi-structured interviews will be conducted from 13 July to 31 October 2023 with CYP and their parents/carers to help identify the impact of PO on everyday life, the patient journey and how service users define treatment success. Three medical professional focus groups will be used to understand the current management practice, how treatment success is measured and acceptability to randomise patients. Thematic analysis will be used. ETHICS AND DISSEMINATION: The Health Research Authority, The Health and Social Care Research Ethics Committee (23/NI/0082) and the CPRD's research data governance panel (22_002508) reviewed this study. Results will be disseminated at medical conferences, in peer-reviewed journals and via social media. The study will cocreate a webpage on healthtalk.org, with the Dipex Charity, about PO to ensure members of the public can learn more about the condition. TRIAL REGISTRATION NUMBER: ISRCTN46071200.

Children using a unilateral cochlear implant and contralateral hearing aid: bimodal hearing outcomes when one ear is outside the UK (NICE 2009) audiological criteria for cochlear implantation - a single site case-control study.

INTRODUCTION: In the new revised National Institute for Health & Care Excellence (NICE, TA566, 2019) guidelines for cochlear implantation (CI) have clearly stipulated that the hearing loss must be bilateral. Prior to this revision, children and young people (CYP) with asymmetrical thresholds have been considered for unilateral CI when one ear was in audiological criteria. Children with asymmetrical hearing loss represent an important cohort of potential CI candidates, who will continue to be prevented from benefiting from CI unless evidence is produced to support implantation and maximise subsequent benefit.The aim of this study is to evaluate the 'real-life' hearing performance in a group of children who have received a unilateral CI and who have hearing thresholds in the contralateral ear that are outside the current UK NICE 2019 audiological criteria for CI. The contralateral ear will be aided using a conventional hearing aid (HA). The outcomes from this 'bimodal' group will be compared with a group of children who have received bilateral CI, and a group of children using bilateral HA, to extend the current knowledge about the different performance levels between bilateral CI, bilateral HA and bimodal hearing in CYP. METHODS AND ANALYSIS: Thirty CYP aged 6-17 years old, 10 bimodal users, 10 bilateral HA users and 10 bilateral cochlear implant users will be subjected to a test battery consisting of: (1) spatial release from masking, (2) complex pitch direction discrimination, (3) melodic identification, (4) perception of prosodic features in speech and (5) TEN test. Subjects will be tested in their optimal device modality. Standard demographic and hearing health information will be collected. In the absence of comparable published data to power the study, sample size was determined on pragmatic grounds. Tests are exploratory and for hypothesis generating purposes. Therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This has been approved by the Health Research Authority and NHS REC within the UK (22/EM/0104). Industry funding was secured via a competitive researcher-led grant application process. Trial results will be subject to publication according to the definition of the outcome presented in this protocol.

Can MRI biomarkers for hearing loss in enlarged vestibular aqueduct be measured reproducibly?

OBJECTIVE: Morphological features of an enlarged endolymphatic duct (ED) and sac (ES) are imaging biomarkers for genotype and hearing loss phenotype. We determine which biomarkers can be measured in a reproducible manner, facilitating further clinical prediction studies in enlarged vestibular aqueduct hearing loss. METHODS: A rater reproducibility study. Three consultant radiologists independently measured previously reported MRI ED & ES biomarkers (ED midpoint width, maximal ED diameter closest to the vestibule, ES length, ES width and presence of ES signal heterogeneity) and presence of incomplete partition Type 2 from 80 ears (T2 weighted axial MRI). Interclass correlation coefficients (ICC) and Gwet's Agreement Coefficients (AC) were generated to give a measure of reproducibility for both continuous and categorical feature measures respectively. RESULTS: ES length, width and sac signal heterogeneity showed adequate reproducibility (ICC 95% confidence intervals 0.77-0.95, Gwet's AC for sac heterogeneity 0.64). When determining ED midpoint width, measurements from multiple raters are required for "good" reliability (ICC 95% CI 0.75-0.89). Agreement on the presence of incomplete partition Type 2 ranged from "moderate" to "substantial". CONCLUSIONS: Regarding MR imaging, the opinion of multiple expert raters should be sought when determining the presence of an enlarged ED defined by midpoint width. ED midpoint, ES length, width and signal heterogeneity have adequate reproducibility to be further explored as clinical predictors for audiological phenotype. ADVANCES IN KNOWLEDGE: We report which ED & ES biomarkers are reproducibly measured. Researchers can confidently utilise these specific biomarkers when modelling progressive hearing loss associated with enlarged vestibular aqueduct.

'Real-life' benefit of hearing preservation cochlear implantation in the paediatric population: a single-site case-control study.

INTRODUCTION: Cochlear implantation with hearing preservation (HPCI) has allowed a cochlear implant (CI) electrode to be implanted while trying to preserve residual acoustic low-frequency hearing. The concept arises from the importance of this low-frequency information and the limitations of a CI in several auditory domains. The combination of electrical hearing with either preserved acoustic hearing or amplified 'natural' hearing has the potential to address these issues and enable children with HPCI to closely follow normal auditory development.The aim of this study is to evaluate the 'real-life' benefit of preserved acoustic low-frequency hearing in children with a CI, understand the benefits of preserved natural hearing in complex listening situations and so enable parents and children to make an informed choice about implantation. Ultimately, helping to ensure the maximum number of children benefit from this life-changing intervention. METHODS AND ANALYSIS: Nineteen ears in children and young people aged 6-17 years old with 'successful' HPCI will be subjected to a test battery consisting of: (1) spatial release from masking; (2) complex pitch direction discrimination; (3) melodic identification; (4) perception of prosodic features in speech and (5) threshold equalising noise test. Subjects will be tested in the electro-acoustic stimulation (EAS)/electro-natural stimulation (ENS) and the electric-only (ES) condition, thereby acting as their own control group. Standard demographic and hearing health information will be collected. In the absence of comparable published data to power the study, sample size was determined on pragmatic grounds. Tests are exploratory and for hypothesis-generating purposes. Therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This study has been approved by the Health Research Authority and NHS Research Ethics Committee (REC) within the UK (22/EM/0017). Industry funding was secured via a competitive researcher-led grant application process. Trial results will be subject to publication according to the definition of the outcome presented in this protocol.

Tracheal Resection for Critical Airway Obstruction in Morquio A Syndrome.

Introduction: The primary cause of death in Morquio A syndrome (mucopolysaccharidosis (MPS) IVA) is airway obstruction, brought about by an inexorable and pathognomonic multilevel airway tortuosity, buckling, and obstruction. The relative pathophysiological contributions of an inherent cartilage processing defect versus a mismatch in longitudinal growth between the trachea and the thoracic cage are currently a subject of debate. Enzyme replacement therapy (ERT) and multidisciplinary management continue to improve life expectancy for Morquio A patients by slowing many of the multisystem pathological consequences of the disease but are not as effective at reversing established pathology. An urgent need has developed to consider alternatives to palliation of progressive tracheal obstruction to preserve and maintain these patients' hard-won good quality of life, as well as to facilitate spinal and other required surgery. Case Report. Following multidisciplinary discussion, transcervical tracheal resection with limited manubriectomy was successfully performed, without the need for cardiopulmonary bypass, in an adolescent male on ERT with the severe airway manifestations of Morquio A syndrome. His trachea was found to be under significant compressive forces at surgery. On histology, chondrocyte lacunae appeared enlarged, but intracellular lysosomal staining and extracellular glycosaminoglycan staining was comparable to control trachea. At 12 months, this has resulted in a significant improvement in respiratory and functional status, with corresponding enhancement to his quality of life. Conclusion: This addressing of tracheal/thoracic cage dimension mismatch represents a novel surgical treatment approach to an existing clinical paradigm and may be useful for other carefully selected individuals with MPS IVA. Further work is needed to better understand the role and optimal timing of tracheal resection within this patient cohort so as to individually balance considerable surgical and anaesthetic risks against the potential symptomatic and life expectancy benefits.

Aided Cortical Auditory Evoked Potentials in Infants With Frequency-Specific Synthetic Speech Stimuli: Sensitivity, Repeatability, and Feasibility.

OBJECTIVES: The cortical auditory evoked potential (CAEP) test is a candidate for supplementing clinical practice for infant hearing aid users and others who are not developmentally ready for behavioral testing. Sensitivity of the test for given sensation levels (SLs) has been reported to some degree, but further data are needed from large numbers of infants within the target age range, including repeat data where CAEPs were not detected initially. This study aims to assess sensitivity, repeatability, acceptability, and feasibility of CAEPs as a clinical measure of aided audibility in infants. DESIGN: One hundred and three infant hearing aid users were recruited from 53 pediatric audiology centers across the UK. Infants underwent aided CAEP testing at age 3 to 7 months to a mid-frequency (MF) and (mid-)high-frequency (HF) synthetic speech stimulus. CAEP testing was repeated within 7 days. When developmentally ready (aged 7-21 months), the infants underwent aided behavioral hearing testing using the same stimuli, to estimate the decibel (dB) SL (i.e., level above threshold) of those stimuli when presented at the CAEP test sessions. Percentage of CAEP detections for different dB SLs are reported using an objective detection method (Hotellings T 2 ). Acceptability was assessed using caregiver interviews and a questionnaire, and feasibility by recording test duration and completion rate. RESULTS: The overall sensitivity for a single CAEP test when the stimuli were ≥0 dB SL (i.e., audible) was 70% for the MF stimulus and 54% for the HF stimulus. After repeat testing, this increased to 84% and 72%, respectively. For SL >10 dB, the respective MF and HF test sensitivities were 80% and 60% for a single test, increasing to 94% and 79% for the two tests combined. Clinical feasibility was demonstrated by an excellent >99% completion rate, and acceptable median test duration of 24 minutes, including preparation time. Caregivers reported overall positive experiences of the test. CONCLUSIONS: By addressing the clinical need to provide data in the target age group at different SLs, we have demonstrated that aided CAEP testing can supplement existing clinical practice when infants with hearing loss are not developmentally ready for traditional behavioral assessment. Repeat testing is valuable to increase test sensitivity. For clinical application, it is important to be aware of CAEP response variability in this age group.

Column-based cortical depth analysis of the diffusion anisotropy and radiality in submillimeter whole-brain diffusion tensor imaging of the human cortical gray matter in vivo.

High-resolution diffusion tensor imaging (DTI) can noninvasively probe the microstructure of cortical gray matter in vivo. In this study, 0.9-mm isotropic whole-brain DTI data were acquired in healthy subjects with an efficient multi-band multi-shot echo-planar imaging sequence. A column-based analysis that samples the fractional anisotropy (FA) and radiality index (RI) along radially oriented cortical columns was then performed to quantitatively analyze the FA and RI dependence on the cortical depth, cortical region, cortical curvature, and cortical thickness across the whole brain, which has not been simultaneously and systematically investigated in previous studies. The results showed characteristic FA and RI vs. cortical depth profiles, with an FA local maximum and minimum (or two inflection points) and a single RI maximum at intermediate cortical depths in most cortical regions, except for the postcentral gyrus where no FA peaks and a lower RI were observed. These results were consistent between repeated scans from the same subjects and across different subjects. They were also dependent on the cortical curvature and cortical thickness in that the characteristic FA and RI peaks were more pronounced i) at the banks than at the crown of gyri or at the fundus of sulci and ii) as the cortical thickness increases. This methodology can help characterize variations in microstructure along the cortical depth and across the whole brain in vivo, potentially providing quantitative biomarkers for neurological disorders.

Listening effort and downstream effects due to hearing loss in children and young people: an online quantitative questionnaire-based observational study.

INTRODUCTION: The clinical application of listening effort (LE) is challenging due to the lack of consensus regarding measuring the concept. Correlational analysis between different measuring instruments shows conditional and weak relationships, indicating they capture different dimensions of LE. Current research has suggested possible links between LE and downstream consequences such as fatigue, stress and confidence. One way to clinically measure LE would be to focus on its corollaries. Further research is needed to explore whether tools used to measure these downstream effects can be applied to capture LE. This study explores using existing questionnaire-based outcome instruments to evaluate LE and its associated consequences in children and young people (CYP), with and without hearing loss. METHODS AND ANALYSIS: One hundred CYP aged 12-17 years with normal hearing and a range of hearing loss levels will be invited to complete a series of online questionnaires (Speech, Spatial and Qualities, Vanderbilt Fatigue Scale-Child, Perceived Stress Scale and Rosenberg Self-Esteem Scale) and a hearing test (Digits in Noise). They will complete the questionnaires at two time points (1) at the end of a rest day and (2) at the end of a workday. Standard demographic and hearing health information will be collected. The sample size was determined pragmatically due to a lack of comparable published data to power the study. Tests are exploratory and for generating hypotheses; therefore, the standard criterion of p<0.05 will be used. ETHICS AND DISSEMINATION: This study has been reviewed within the funding organisation (Cochlear Research and Development Limited) by an independent and relevant peer reviewer/committee. This study has had a favourable ethics committee review by both NHS ethics and University of Manchester ethics. The study will be disseminated through newsletters, publication and presentations at conferences. The results will be made available to participants on request.

Exploring the Correlations Between Measures of Listening Effort in Adults and Children: A Systematic Review with Narrative Synthesis.

Listening effort (LE) describes the cognitive resources needed to process an auditory message. Our understanding of this notion remains in its infancy, hindering our ability to appreciate how it impacts individuals with hearing impairment effectively. Despite the myriad of proposed measurement tools, a validated method remains elusive. This is complicated by the seeming lack of association between tools demonstrated via correlational analyses. This review aims to systematically review the literature relating to the correlational analyses between different measures of LE. Five databases were used- PubMed, Cochrane, EMBASE, PsychINFO, and CINAHL. The quality of the evidence was assessed using the GRADE criteria and risk of bias with ROBINS-I/GRADE tools. Each statistically significant analysis was classified using an approved system for medical correlations. The final analyses included 48 papers, equating to 274 correlational analyses, of which 99 reached statistical significance (36.1%). Within these results, the most prevalent classifications were poor or fair. Moreover, when moderate or very strong correlations were observed, they tended to be dependent on experimental conditions. The quality of evidence was graded as very low. These results show that measures of LE are poorly correlated and supports the multi-dimensional concept of LE. The lack of association may be explained by considering where each measure operates along the effort perception pathway. Moreover, the fragility of significant correlations to specific conditions further diminishes the hope of finding an all-encompassing tool. Therefore, it may be prudent to focus on capturing the consequences of LE rather than the notion itself.