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.

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.

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.

Personalised virtual gene panels reduce interpretation workload and maintain diagnostic rates of proband-only clinical exome sequencing for rare disorders.

PURPOSE: The increased adoption of genomic strategies in the clinic makes it imperative for diagnostic laboratories to improve the efficiency of variant interpretation. Clinical exome sequencing (CES) is becoming a valuable diagnostic tool, capable of meeting the diagnostic demand imposed by the vast array of different rare monogenic disorders. We have assessed a clinician-led and phenotype-based approach for virtual gene panel generation for analysis of targeted CES in patients with rare disease in a single institution. METHODS: Retrospective survey of 400 consecutive cases presumed by clinicians to have rare monogenic disorders, referred on singleton basis for targeted CES. We evaluated diagnostic yield and variant workload to characterise the usefulness of a clinician-led approach for generation of virtual gene panels that can incorporate up to three different phenotype-driven gene selection methods. RESULTS: Abnormalities of the nervous system (54.5%), including intellectual disability, head and neck (19%), skeletal system (16%), ear (15%) and eye (15%) were the most common clinical features reported in referrals. Combined phenotype-driven strategies for virtual gene panel generation were used in 57% of cases. On average, 7.3 variants (median=5) per case were retained for clinical interpretation. The overall diagnostic rate of proband-only CES using personalised phenotype-driven virtual gene panels was 24%. CONCLUSIONS: Our results show that personalised virtual gene panels are a cost-effective approach for variant analysis of CES, maintaining diagnostic yield and optimising the use of resources for clinical genomic sequencing in the clinic.

Acute paediatric mastoiditis in the UK before and during the COVID-19 pandemic: A national observational study.

OBJECTIVES: To explore the impact of COVID-19 on the management and outcomes of acute paediatric mastoiditis across the UK. DESIGN: National retrospective and prospective audit. SETTING: 48 UK secondary care ENT departments. PARTICIPANTS: Consecutive children aged 18 years or under, referred to ENT with a clinical diagnosis of mastoiditis. MAIN OUTCOME MEASURES: Cases were divided into Period 1 (01/11/19-15/03/20), before the UK population were instructed to reduce social contact, and Period 2 (16/03/20-30/04/21), following this. Periods 1 and 2 were compared for population variables, management and outcomes. Secondary analyses compared outcomes by primary treatment (medical/needle aspiration/surgical). RESULTS: 286 cases met criteria (median 4 per site, range 0-24). 9.4 cases were recorded per week in period 1 versus 2.0 in period 2, with no winter increase in cases in December 2020-Febraury 2021. Patient age differed between periods 1 and 2 (3.2 vs 4.7 years respectively, p < 0.001). 85% of children in period 2 were tested for COVID-19 with a single positive test. In period, 2 cases associated with P. aeruginosa significantly increased. 48.6% of children were scanned in period 1 vs 41.1% in period 2. Surgical management was used more frequently in period 1 (43.0% vs 24.3%, p = 0.001). Treatment success was high, with failure of initial management in 6.3%, and 30-day re-admission for recurrence in 2.1%. The adverse event rate (15.7% overall) did not vary by treatment modality or between periods 1& 2. CONCLUSION: The COVID-19 pandemic led to a significant change in the presentation and case mix of acute paediatric mastoiditis in the UK.

Two-phase survey on the frequency of use and safety of MRI for hearing implant recipients.

PURPOSE: Magnetic resonance imaging (MRI) is often used to visualize and diagnose soft tissues. Hearing implant (HI) recipients are likely to require at least one MRI scan during their lifetime. However, the MRI scanner can interact with the implant magnet, resulting in complications for the HI recipient. This survey, which was conducted in two phases, aimed to evaluate the safety and performance of MRI scans for individuals with a HI manufactured by MED-EL (MED-EL GmbH, Innsbruck, Austria). METHODS: A survey was developed and distributed in two phases to HEARRING clinics to obtain information about the use of MRI for recipients of MED-EL devices. Phase 1 focused on how often MRI is used in diagnostic imaging of the head region of the cochlear implant (CI) recipients. Phase 2 collected safety information about MRI scans performed on HI recipients. RESULTS: 106 of the 126 MRI scans reported in this survey were performed at a field strength of 1.5 T, on HI recipients who wore the SYNCHRONY CI or SYNCHRONY ABI. The head and spine were the most frequently imaged regions. 123 of the 126 scans were performed without any complications; two HI recipients experienced discomfort/pain. One recipient required reimplantation after an MRI was performed using a scanner that had not been approved for that implant. There was only one case that required surgical removal of the implant to reduce the imaging artefact. CONCLUSION: Individuals with either a SYNCHRONY CI or SYNCHRONY ABI from MED-EL can safely undergo a 1.5 T MRI when it is performed according to the manufacturer's safety policies and procedures.

The benefits of preserving residual hearing following cochlear implantation: a systematic review.

OBJECTIVE: Systematically review the current literature for evidence on the "real-life" benefits of hearing preservation cochlear implantation (HPCI) for children and adults. DESIGN: Systematic search of Pubmed, MEDLINE, EMBASE, CINHAL and Cochrane Library for MesH terms hearing¸ preservation and cochlear implantation. Inclusion criteria were the "real-life" benefit of HPCI i.e. other than pre- and post-operative pure tone thresholds. Exclusion criteria were non-English language, conference abstracts, reviews and animal and cadaveric studies. Risk of bias was assessed using the Evidence Project Tool. STUDY SAMPLE: 37 studies that matched criteria for review with 8/37 including children and 29/37 including adults. RESULTS: HPCI was associated with better speech perception in noise in 18/26 papers and better music perception in 4/5 papers. There was no significant benefit reported in speech perception in quiet (14/20 papers) or binaural cues (3/4 papers), nor was there convincing evidence of HPCI outperforming bimodal users (5/7 papers). QoL scores were high amongst HPCI patients (2/2 papers). Interpretation of findings was hindered by small study groups and significant heterogeneity in various parameters. CONCLUSION: Current literature on the "real-life" benefit of HPCI, although limited, supports the existence of meaningful benefit, especially in speech perception in noise and music perception.

Recording Obligatory Cortical Auditory Evoked Potentials in Infants: Quantitative Information on Feasibility and Parent Acceptability.

OBJECTIVES: With the advent of newborn hearing screening and early intervention, there is a growing interest in using supra-threshold obligatory cortical auditory evoked potentials (CAEPs) to complement established pediatric clinical test procedures. The aim of this study was to assess the feasibility, and parent acceptability, of recording infant CAEPs. DESIGN: Typically developing infants (n = 104) who had passed newborn hearing screening and whose parents expressed no hearing concerns were recruited. Testing was not possible in 6 infants, leaving 98, age range 5 to 39 weeks (mean age = 21.9, SD = 9.4). Three short duration speech-like stimuli (/m/, /g/, /t/) were presented at 65 dB SPL via a loudspeaker at 0° azimuth. Three criteria were used to assess clinical feasibility: (i) median test duration <30 min, (ii) >90% completion rate in a single test session, and (iii) >90% response detection for each stimulus. We also recorded response amplitude, latency, and CAEP signal to noise ratio. Response amplitudes and residual noise levels were compared for Fpz (n = 56) and Cz (n = 42) noninverting electrode locations. Parental acceptability was based on an 8-item questionnaire (7-point scale, 1 being best). In addition, we explored the patient experience in semistructured telephone interviews with seven families. RESULTS: The median time taken to complete 2 runs for 3 stimuli, including preparation, was 27 min (range 17 to 59 min). Of the 104 infants, 98 (94%) were in an appropriate behavioral state for testing. A further 7 became restless during testing and their results were classified as "inconclusive." In the remaining 91 infants, CAEPs were detected in every case with normal bilateral tympanograms. Detection of CAEPs in response to /m/, /g/, and /t/ in these individuals was 86%, 100%, and 92%, respectively. Residual noise levels and CAEP amplitudes were higher for Cz electrode recordings. Mean scores on the acceptability questionnaire ranged from 1.1 to 2.6. Analysis of interviews indicated that parents found CAEP testing to be a positive experience and recognized the benefit of having an assessment procedure that uses conversational level speech stimuli. CONCLUSIONS: Test duration, completion rates, and response detection rates met (or were close to) our feasibility targets, and parent acceptability was high. CAEPs have the potential to supplement existing practice in 3- to 9-month olds.

Parental Experience of Sleep-Disordered Breathing in Infants With Cleft Palate: Comparing Parental and Clinical Priorities.

OBJECTIVE: To identify outcomes relating to sleep-disordered breathing (SDB) that are relevant to parents, during the early weeks of caring for infants with cleft palate (CP), and compare these with clinical outcomes identified in a systematic search of research literature. DESIGN: A qualitative study using telephone/face-to-face interviews with parents explored their understanding of breathing and respiratory effort in infants with CP. SETTING: Care provided by 3 specialist cleft centers in the United Kingdom, with study conducted in parents' homes. PARTICIPANTS: Criteria for participation were parents of infants with isolated CP aged 12 to 16 weeks. Thirty-one parents of infants with CP (over 12 weeks) were invited to participate in the interview. Interviews were completed with 27 parents; 4 parents could not be contacted after completing the sleep monitoring. RESULTS: Parents' description of infants' sleep suggests that breathing is not considered as a separate priority from their principal concerns of feeding and sleeping. They observe indicators of infants' breathing, but these are not perceived as signs of SDB. Parents' decision to use lateral or supine sleep positioning reflects their response to advice from specialists, observation of their infants' comfort, ease of breathing, and personal experience. Outcomes, identified in published research of SDB, coincide with parents' concerns but are expressed in medical language and fit into distinct domains of "snoring," "sleep," "gas exchange," and "apnea." CONCLUSIONS: Parents' description of sleeping and respiration in infants with CP reflect their everyday experience, offering insight into their understanding, priorities, and language used to describe respiration. Understanding parents' individual priorities and how these are expressed could be fundamental to selecting meaningful outcomes for future studies of airway interventions.

A mixed-methods study of the management of hearing loss associated with otitis media with effusion in children with Down syndrome.

OBJECTIVES: To scope current service provision across England for management of otitis media with effusion and hearing loss in children with Down syndrome; to explore professional decision-making about managing otitis media with effusion and hearing loss; and to explore patient and public views on the direction of future research. DESIGN: Mixed methods including a service evaluation of NHS clinical practice through a structured telephone survey; a qualitative study of professional decision-making with in-depth interviews collected and analysed using grounded theory methods; patient/public involvement consultations. PARTICIPANTS: Twenty-one audiology services in England took part in the evaluation; 10 professionals participated in the qualitative study; 21 family members, 10 adults with Down syndrome and representatives from two charities contributed to the consultations. RESULTS: There was variation across services in the frequency of routine hearing surveillance, approaches to managing conductive hearing loss in infancy and provision of hearing aids and grommets. There was variation in how professionals describe their decision-making, reflecting individual treatment preferences, differing approaches to professional remit and institutional factors. The consultations identified that research should focus on improving practical support for managing the condition and supporting decision-making about interventions. CONCLUSIONS: There is system-level variation in the provision of services and individual-level variation in how professionals make clinical decisions. As a consequence, there is inequity of access to hearing health care for children with Down syndrome. Future research should focus on developing core outcomes for research and care, and on improving decision support for families.

Safe sleeping positions: practice and policy for babies with cleft palate.

Guidance recommends 'back to sleep' positioning for infants from birth in order to reduce the risk of sudden infant death. Exceptions have been made for babies with severe respiratory difficulties where lateral positioning may be recommended, although uncertainty exists for other conditions affecting the upper airway structures, such as cleft palate. This paper presents research of (i) current advice on sleep positioning provided to parents of infants with cleft palate in the UK; and (ii) decision making by clinical nurse specialists when advising parents of infants with cleft palate. A qualitative descriptive study used data from a national survey with clinical nurse specialists from 12 regional cleft centres in the UK to investigate current practice. Data were collected using semi-structured telephone interviews and analysed using content analysis. Over half the regional centres used lateral sleep positioning based on clinical judgement of the infants' respiratory effort and upper airway obstruction. Assessment relied upon clinical judgement augmented by a range of clinical indicators, such as measures of oxygen saturation, heart rate and respiration. CONCLUSION: Specialist practitioners face a clinical dilemma between adhering to standard 'back to sleep' guidance and responding to clinical assessment of respiratory effort for infants with cleft palate. In the absence of clear evidence, specialist centres rely on clinical judgement regarding respiratory problems to identify what they believe is the most appropriate sleeping position for infants with cleft palate. Further research is needed to determine the best sleep position for an infant with cleft palate. What is Known • Supine sleep positioning reduces the risk of sudden infant death in new born infants. • There is uncertainty about the benefits or risks of lateral sleep positioning for infants with upper airway restrictions arising from cleft palate. What is New • Variability exists in the information/advice provided to parents of infants with cleft palate regarding sleep positioning. • Over half the national specialist centres for cleft palate in the UK advise positioning infants with CP in the lateral position as a routine measure to reduce difficulties with respiration.

Airway-related symptoms and surgeries in patients with mucopolysaccharidosis I.

OBJECTIVE: Mucopolysaccharidosis I (MPS I) is a progressive, debilitating, and life-threatening genetic disease, which, owing to the nonspecific nature of the early symptoms, is often unrecognized and associated with significant diagnostic delays. To improve early recognition leading to early diagnosis and initiation of treatment, we characterized the extent of airway-related symptoms and surgeries among patients with MPS I. METHODS: Analysis of the frequency of airway-related symptoms and surgeries from 1041 patients enrolled in the MPS I Registry and correlation with other systemic manifestations of MPS I. RESULTS: Airway-related symptoms (macroglossia, enlarged tonsils, reactive airway disease/asthma, or sleep disturbances) were reported for as many as 85% of Hurler, 83% of Hurler-Scheie, and 65% of Scheie patients-very often before the diagnosis of MPS I was established. Surgeries for an airway indication were reported in 39% of patients and many had at least 1 airway-related surgery before the diagnosis of MPS I was confirmed. The mean percentage of patients with airway-related symptoms for whom hernias and/or dysostosis multiplex were also reported was 84% and 54%, respectively. CONCLUSION: Airway-related symptoms and surgeries are common and often the earliest presenting feature in MPS I. Improved recognition of early MPS I disease manifestations may lead to earlier diagnosis and treatment.

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.

Anaesthesia and airway management in mucopolysaccharidosis.

This paper provides a detailed overview and discussion of anaesthesia in patients with mucopolysaccharidosis (MPS), the evaluation of risk factors in these patients and their anaesthetic management, including emergency airway issues. MPS represents a group of rare lysosomal storage disorders associated with an array of clinical manifestations. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardiovascular manifestations poses a high anaesthetic risk to these patients. Typical anaesthetic problems include airway obstruction after induction or extubation, intubation difficulties or failure [can't intubate, can't ventilate (CICV)], possible emergency tracheostomy and cardiovascular and cervical spine issues. Because of the high anaesthetic risk, the benefits of a procedure in patients with MPS should always be balanced against the associated risks. Therefore, careful evaluation of anaesthetic risk factors should be made before the procedure, involving evaluation of airways and cardiorespiratory and cervical spine problems. In addition, information on the specific type of MPS, prior history of anaesthesia, presence of cervical instability and range of motion of the temporomandibular joint are important and may be pivotal to prevent complications during anaesthesia. Knowledge of these risk factors allows the anaesthetist to anticipate potential problems that may arise during or after the procedure. Anaesthesia in MPS patients should be preferably done by an experienced (paediatric) anaesthetist, supported by a multidisciplinary team (ear, nose, throat surgeon and intensive care team), with access to all necessary equipment and support.

Mixed Reality Enhanced Otolaryngology Case-Based Learning: A Randomized Educational Study.

INTRODUCTION: The COVID-19 pandemic has caused unprecedented disruptions to medical education. Education in medical specialties, such as otolaryngology faces multiple challenges, including reduced bedside and "hands-on" training opportunities at all levels. Educators are turning to technological advancements to deliver effective remote medical education. This study investigated the value of enhancing traditional remote case-based teaching with the HoloLens2™. METHODS: We present a randomized educational design study. All educational content, media, and learning outcomes were identical. Primary outcome measures included student performance as measured with pre- and post-intervention quizzes and student engagement as measured by a tally-mark system. Secondary outcome measures, collected using feedback questionnaires, included perceived enjoyment, engagement, and opinions regarding the educational role of this technology. RESULTS: The undergraduate medical students were randomized to either conventional or HoloLens2™ enhanced remote case-based teaching (n = 56). HoloLens2™ enhanced teaching improved student performance by an average of 3 marks of 15% (p < 0.001). It was engaging and encouraged questions 4-fold per session (p < 0.05) when compared to conventional remote case-based teaching. There was no significant difference in overall objective measurements of engagement. Students taught using HoloLens2™ agreed that the teaching was enjoyable, effective in concept demonstration, and encouraged engagement. CONCLUSIONS: Remote teaching has allowed for the continuation of medical education in uncertain times. Beyond COVID-19, we predict that there will be a paradigm shift toward remote learning as new technological advancements emerges. These novel technologies may prove invaluable in the future potentially enabling education to be delivered between different hospitals, universities, and even overseas. LEVEL OF EVIDENCE: NA Laryngoscope, 133:1606-1613, 2023.

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.

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.