OBJECTIVES:Active transcutaneous bone conduction devices have expanded rehabilitation options for children with conductive, mixed, and single-sided deafness (SSD). The Cochlear™ OSIA® system improves hearing while preserving intact skin. We hypothesised that postoperative outcomes vary by operative context and that incision design may influence complication risk. METHODS:A prospectively maintained database of paediatric patients implanted between September 2021 and April 2026 was retrospectively analysed. Patients were grouped by operative context: (1) microtia/craniofacial anomalies, (2) surgically altered tissues (chronic ear disease/post-mastoidectomy or conversion from percutaneous devices), and (3) normal anatomy (SSD). The primary outcome was soft tissue complications. Secondary outcomes included audiological improvement and subjective benefit (SAC-A). RESULTS:Thirty-four children underwent 46 OSIA® implants (22 unilateral, 12 bilateral); they were aged between 7-19 years and 56% were female. Seventeen had microtia/craniofacial syndromes, twelve had chronic ear disease (including four conversions), and five had SSD. No perioperative complications occurred. Postoperative soft tissue complications occurred in 4/46 implants (8.7%), exclusively in the microtia/craniofacial and surgically altered tissue groups. One explantation was required for late infection; the remainder were managed conservatively or with revision surgery. Significant improvements were seen at 2 kHz (P = 0.009), 4 kHz (P < 0.001), and in speech recognition (86% to 95%, P = 0.004). SAC-A scores improved significantly (36 to 24; P = 0.002), with 94.1% reporting satisfactory usability. CONCLUSIONS:Paediatric OSIA® implantation provides substantial audiological benefit with low complication rates. Complications clustered in anatomically or surgically altered tissues, supporting context-specific counselling and surgical planning.

Cochlear implantation has significantly improved outcomes for children with severe-to-profound sensorineural hearing loss. Children and young people (CYP) with residual hearing may benefit from advances in surgical technique and electrode design, which has made preserving remaining natural hearing more achievable. This enables combined electric-acoustic and electric-natural stimulation, supporting a more natural listening experience. This article reviews current candidacy criteria for cochlear implants (CI) in CYP, including the concept of ‘borderline’ candidacy, where CYP are not achieving anticipated benefit from their conventional hearing aids (age-appropriate speech, language and listening skills), despite receiving some measurable benefit. The role of cochlear implantation in children with complex needs and those with single-sided deafness is also discussed with practical considerations such as magnetic resonance imaging (MRI) safety and the presence of ventriculoperitoneal shunts. Timely identification and referral are essential to ensure all eligible CYP are given the opportunity to benefit from cochlear implantation.

Objective: to evaluate the levels of successful hearing preservation and preservation of functional hearing following cochlear implantation (HPCI) in children using the Cochlear Nucleus (R) 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

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.

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.

Outcomes following adenotonsillectomy for paediatric obstructive sleep apnoea from our centre support the paediatric national safety strategy recommendations published in 2019 Age and significant comorbidities are key factors in determining post-operative complication risk following adenotonsillectomy in paediatric patients with obstructive sleep apnoea Day case surgery is safe for children aged 2 and over who do not have comorbidities There is no significant difference in post-operative bleeding rates for intracapsular coblation verses extracapsular dissection tonsillectomy techniques in our study.

Systematically review the current literature for evidence on the "real-life" benefits of hearing preservation cochlear implantation (HPCI) for children and adults.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.37 studies that matched criteria for review with 8/37 including children and 29/37 including adults.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.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.

Cleft lip and/or palate is the commonest congenital craniofacial abnormality affecting approximately 1 in 700 newborns each year. It comprises of a heterogenous group of disorders affecting facial growth and cosmesis, that are associated with increased risk of airway obstruction, sleep disordered breathing (SDB), glue ear and chronic ear disease, feeding difficulties, and failure to thrive (FTT). Cleft palate (CP) can be accompanied by an abnormally undersized jaw, known as micrognathia; although micrognathia can also be found in isolation. The craniofacial abnormalities found in these children can lead to a reduction in airway size due to the tongue falling backwards. The risk of airway obstruction ranges from intermittent airway collapse during sleep (obstructive sleep apnoea, OSA) to potentially life-threatening airway compromise necessitating intubation or a tracheostomy. This paper sets out to describe the pathophysiology of airway compromise in these children, recognising clinical symptoms and appropriate referral strategy, as well as a broad range of management options.

Objective: Brown–Vialetto–Van Laere syndrome (BVVL) is a rare neurodegenerative disorder associated with auditory neuropathy (AN). The decision process for CI in AN is evolving with increasing evidence of efficacy. We evaluated the benefit of CI in children with BVVL syndrome. Methods: A retrospective study reviewed the pre- and post-operative hearing outcomes of three patients with BVVL who presented for CI. A fourth patient with BVVL who was not suitable for CI is also discussed. The primary outcomes were hearing thresholds and auditory perception. Outcome measurement instruments included visual reinforcement audiometry (VRA) or Play Audiometry (PA), Categories of Auditory Performance (CAP) and Auditory Speech Sound Evaluation (ASSE). Secondary outcomes were parental report (BAPP questionnaire), the perception of our SaLT and compliance. Results: Patient 1 had ASSE levels of 40–45 dB HL 1 year post-operatively, and CAP score had improved from 2 to 5. At 2-year review, aided thresholds were 40 dB at 2–4 kHz. Three months following CI, the CAP score of Patient 2 had improved from 3 to 5. At 6 months, thresholds were 25–30 dB at 2–4 kHz. Single words/phrases are used by both patients and benefit is reported by both families. Patient 3 has recently undergone CI, having been previously rejected at another centre. Three months following CI, his thresholds were 35–40 dB at 2–4 kHz and increased use of sign and vocalization is reported. Conclusion: CI in children with AN complicating BVVL has a variable, but a positive effect. Other manifestations of BVVL make measuring benefit challenging, in the absence of a ‘bespoke’ measurement instrument for children with complex needs. This study provides further evidence for the benefit of CI in children with AN.

Background: Mucopolysaccharide (MPS) diseases are a heterogeneous group of inherited, metabolic disorders characterized by accumulation of partially degraded glycosaminoglycans (GAG) in multiple organ systems. Due to accumulation in the airway, patients often present with multilevel airway obstruction and obstructive sleep apnoea (OSA). Adenotonsillar surgery leads to a significant improvement in the severity of OSA in MPS patients. However, access to secure the airway and for conventional surgery can be challenging, due to limited neck extension, macroglossia and reduced mouth opening. This study was undertaken to evaluate the role of transnasal microdebridement and radiofrequent plasma ablation (Coblation) in adenoidectomy to treat OSA in patients with MPS and restricted airway access. Methods: A retrospective case review was performed including patients with MPS undergoing adenoidectomy for OSA in the period between June 2015 and March 2017. In all cases, either a microdebrider (Gyrus Diablo) or a Coblation wand (EVAC70, Smith&Nephew) was used via a transnasal approach guided by nasendoscopy. The primary outcome was effect upon OSA, measured by sleep oximetry and parental report of benefit. The secondary outcomes were surgical complications and risk factors for persistent OSA after surgery. Results: A total of nine patients were identified with a mean age of 9 years (range 3-14 years) at surgery. Postoperative sleep study data was available for eight patients (8/9). Six patients (6/8) had improvement in 4% oxygen desaturation index (ODI-4) with a mean of 8.11 pre-operatively (range 2.69-14.0) and 4.99 postoperatively (range 0.68-8.48). ODI-4 did not improve in two (2/8) patients. Irrespective of sleep oximetry results, improvement in OSA-related symptoms was noted by all parents postoperatively. No risk factors for persistent OSA were identified. Furthermore, no complications were noted in this cohort. Conclusion: Transnasal Coblation and Microdebrider adenoidectomy is a safe and effective surgical treatment for OSA in patients with Mucopolysaccharidosis and adenoidal hypertrophy. As lifespan increases for patients with the Mucopolysaccharidoses, greater emphasis is being given to optimising airway management over the longer-term. This technical note describes the novel application of endoscopic techniques for the management of primary adenoidal hypertrophy when transoral access is restricted, or to debulk recurrent disease that would be challenging to remove via the standard transoral route.

Objectives: Increasingly, children are considered for a unilateral cochlear implant (CI), even if the contralateral ear falls outside current audiological guidelines, especially if they are not considered to be reaching their educational potential. Here we present the outcomes of CI in children with potentially useable hearing in the contralateral ear. Methods: A retrospective case note review was performed for a total of 57 patients. Primary outcome was speech and language (SaL) development, as measured by the Manchester Speech and Language Development Scale (MSLDS) and SaL age equivalent. Secondary outcomes were auditory perception, perceived parental benefit and compliance; respectively measured by Categories of Auditory Performance (CAP), Brief Assessment of Parental Perception (BAPP) and reported use. Results: SaL development improved after CI with a mean pre-operative MSLDS score of 5.8 to a postoperative score of 8.0 (n = 57) and a mean SaL age equivalent of 14 months in a one-year period (n = 14). Furthermore, CAP scores improved from 4.9 to 7.0 (n = 57). Analysis of BAPP scores showed improved quality of life in 18/19 patients (94.7%). With regards to compliance, 50/57 (87.7%) are fulltime users of both their CI and their HA. Conclusion: The present study indicates that despite one ear having potentially useable hearing outside national audiological criteria, the majority of participants received benefit from a CI in the poorer hearing ear. We suggest that assessment of each ear separately and treatment with the most appropriate amplification device, has given these children a benefit they may not otherwise have acquired if they only had bilateral HA.

The field of Cochlear Implantation (CI) continues to rapidly evolve, with professionals and industry exploring novel technologies, extended applications of existing technologies and other means of optimizing CI outcome. Such developments will have a financial impact upon healthcare systems and providers, and demand the development of evidence of efficacy, with evidence-based practice remaining the ultimate goal. Currently, effective comparison between CI technologies, populations, and evaluation of the added value of novel technologies is significantly hindered by heterogeneity in outcomes used and corresponding measurement instruments. Ultimately, this negatively impacts the shared decision-making (SDM) process between professionals and patients, because of the lack of definitive evidence. A previous Editorial in this journal stressed the importance of developing agreed sets of outcomes (e.g. Core Outcome Sets (COS), http://www.cometinitiative.org) to standardize reporting of effectiveness, and enable the meaningful combination and comparison of data from multiple studies (Bruce et al., 2015). Other fields of medicine, such as Rheumatology and Gynecology, have embraced this approach to good effect (Boers et al., 2014; The CROWN Initiative, 2014). To illustrate the extent of unmet need in the field of CI, we have conducted a review of outcome reporting in Cochlear Implants International (CIM). Studies published in this journal in 2015 (Vol 16: 1–6, S1, S2, S4) were included, with the exclusion of reviews with lack of new patient data, lack of effectiveness evaluation and those not involving CI recipients. All articles were reviewed for outcomes, corresponding choice of measurement instrument, time point of measurement, study design, study description, study sample, and age group of participants. A total of 43 articles were reviewed, in which 17 different outcomes were used. Even greater variability was seen in the corresponding measurement instruments used, with a total of 72 instruments/tools recorded (Table 1). The most common outcome was speech perception (24/43 publications), with 25 measurement instruments being used for this outcome (Table 1). Furthermore, there was a lack of standardized time points for outcome measurement, with time points varying between 0 and 20 years after CI surgery. This wide variation in outcome domains and corresponding measurement instruments is in agreement with the findings of the systematic review of adult CI studies by Vila et al. who found 11 domains in only 8 studies (Vila et al., 2016). Although, only publications in CIM in 2015 were included, the inclusion of more volumes was deemed unlikely to lead to any major changes in our findings. Review of the first three issues of CIM in 2015 resulted in a total number of 15 outcomes, with only 2 outcomes added after analyzing the other issues. This review also highlighted the significant numbers of different measurement instruments used in CI studies. To an extent, this variation is inevitable, reflecting the need to use speech and language tests in the patient’s native language. The nature of some measurement instruments allows for translation into different languages without affecting the validity of the measurement instrument. While other instruments (e.g. speech perception tests) may not be readily translated, as they are specific to the understanding of words and phrases in a particular language. In such circumstances, it may be useful to compare the proportion of CI patients within 1 and 2 standard deviations of the mean for normal hearing patients, when ‘non-translatable’ instruments are being compared. Alternatively, the design and use of novel measurement instruments not reliant upon understanding of language (e.g. phoneme tests), could address some of these difficulties. Similarly, speech understanding with digits may be an alternative to ‘traditional’ speech perception tests, with digits representing familiar stimuli known to people with even limited language ability, with testing having a smaller learning effect (Cullington and Aidi 2017).