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Objectives: This study aims to synthesize current knowledge and outcomes related to pediatric auditory brainstem implantation (ABI) in children with severe inner ear malformations (IEMs). It highlights the clinical management practices, challenges, and potential future directions for consensus development in this field. Methods: A systematic review of findings presented at the Third International Pediatric ABI Symposium organized by the Hacettepe Cochlear Implant team between 3 and 5 September 2020 was conducted, incorporating data from 41 departments across 19 countries. Relevant clinical outcomes, imaging techniques, surgical approaches, and rehabilitation strategies were analyzed to identify key trends and variability in practices. Results: The review indicates that children receiving ABIs exhibit diverse auditory outcomes influenced by individual anatomical variations and developmental factors. Early implantation, particularly before the age of three, positively correlates with better auditory and language development. Multicenter experiences underscore the necessity of tailored decision-making, which considers both surgical candidacy and comprehensive rehabilitation resources. Discussion:: The variability in outcomes emphasizes the need for improved consensus and guidelines regarding eligibility, surgical techniques, and multidisciplinary rehabilitation approaches. Notable complications and the necessity for thorough imaging assessments were also identified as critical components affecting clinical decisions. Conclusion: A formal consensus statement is warranted to standardize best practices in ABI management. This will not only enhance patient outcomes but also guide future research efforts to address the remaining challenges in the treatment of children with severe IEMs. Enhanced collaboration among team members will be pivotal in achieving these objectives. [ABSTRACT FROM AUTHOR]

Patient suitability for cochlear implant (CI) devices compatible with magnetic resonance imaging and CI processor configuration is dependent on their retro-auricular skin flap thickness. This is typically measured intra-operatively using a needle and therefore patients are not guaranteed their implant of choice prior to surgery. We aimed to identify an accurate method to measure skin flap thickness pre-operatively to streamline CI selection and simplify the consent process. Blinded prospective skin flap thickness measurements for patients undergoing CI surgery were recorded using pre-operative computed tomography (CT) and ultrasound (US), and intraoperative needle measurement. Fifty-six adult patients (36 females, 20 males; mean age 59 years) were included. The mean flap thickness was measured highest by CT (6.9 mm, 95% CI 6.5–7.3 mm), followed by US (6.3 mm, 95% CI 5.9–6.7 mm) and lastly needle (5.5 mm, 95% CI 5.1–5.9 mm) (p < 0.0001). A strong positive correlation (p < 0.001) was noted between all three modalities: CT vs needle (r = 0.869), US vs needle (r = 0.865), and CT vs US (r = 0.849). Accurate, non-invasive measurement of skin flap thickness prior to CI surgery can be achieved using CT or US. We recommend the routine use of US in the outpatient clinic to minimise unnecessary radiation exposure. [ABSTRACT FROM AUTHOR]

Many patients with cochlear implants (CI) and auditory brainstem implants (ABI) require magnetic resonance imaging (MRI) following implantation. This study explores the patient experience of MRI, identifying factors associated with pain, and the effect of interventions designed to enhance comfort and safety. A prospective observational case series from a tertiary referral unit. Tight head bandaging ± local anaesthetic injection (devices with non-MRI-compatible magnets) or observation alone (implants with MRI-compatible magnets) were employed for 1.5 T MRI of consecutive adult patients with CI or ABI without magnet removal. Pain was recorded via visual analogue scale (1 = no pain, 5 = extreme pain) at three time points; (1) baseline, (2) head bandage applied (3) during scanning. Patient age, device type, body area imaged and total scan time were recorded as variables, alongside adverse events. Data were collected for 227 MRI scans (34 patients with ABI, 32 with CI). In patients managed with bandaging, pain score after bandaging but prior to scanning (median 2.2) did not differ from pain during scanning (2.1), but both were significantly higher than baseline (1.4, both P ≤ 0.001). Scanning areas other than the head/cervical spine was associated with higher pain scores (P = 0.036). Pain during MRI differed between different manufacturers implants (P ≤ 0.001). Adverse events occurred in 8/227 scans (3.5%), none occurring with devices containing an MRI-compatible magnet. MRI scanning with auditory implant magnets in situ is safe and well tolerated by patients. [ABSTRACT FROM AUTHOR]

Objective: To assess the compliance with cochlear implantation (CI) in children subsequently diagnosed with autism spectrum disorder (ASD).Methods: This was a retrospective case review and survey performed at a tertiary referral centre. Children meeting the criteria for CI who were implanted between 1989 and 2015 and who subsequently received a diagnosis of ASD were included. The primary outcome measure was to assess compliance with CI in children subsequently diagnosed with ASD. Secondary outcome measures included assessment of pre-CI risk factors that may have identified children at higher risk of a subsequent diagnosis of ASD, as well as the benefit obtained by these children following CI.Results: 1050 children were implanted between 1989 and 2015. Of these, 22 children were diagnosed with ASD after receiving their CI. The average age at implantation was 2.6 years (median 3, range 1-8 years). The average age for diagnosis of ASD was 5 years, approximately 2 years (median 22 months, range 2-85 months) following CI. Of these, 16/22 (712.7%) regularly use their CI. 6/22 (27.2%) children became non-users of their implant. Some degree of verbal communication was used by 13/22 (59%) of our studied group.Conclusion: There is a range of level of disabilities in ASD, with some relatively minor social communication difficulties through to severe language, cognitive, and behavioural difficulties. Compliance with CI is variable and appears to correlate with the severity of the ASD. Preoperative counselling should include information about the possible impact of later diagnosed disabilities such as ASD on performance. [ABSTRACT FROM AUTHOR]

In neurofibromatosis type 2 (NF2) bilateral vestibular schwannomas (VS) or their treatment usually results in bilateral hearing loss. Cochlear implantation (CI) was traditionally not used in these patients due to concern that retrocochlear disease would render the implant ineffective. This paper describes the auditory outcomes of CI in 13 patients with NF2 and includes patients with untreated VS and patients undergoing VS removal with cochlear nerve preservation. The non-user rate was 7.7%. Of the active users, median CUNY score was 98%, median BKB score in quiet was 90% and median BKB score in noise was 68%. CI is a viable option in selected patients with NF2. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to quantify the benefit gained from cochlear implantation in pre- or peri-lingually deafened patients who were implanted as adults Methods: This was a retrospective case-control study. Auditory (BKB/CUNY/3AFC/Environmental sounds), quality of life (GBI/HUI3) and cognitive (customized questionnaire) outcomes in 26 late implanted pre- or peri-lingually deafened adults were compared to those of 30 matched post-lingually deafened, traditional cochlear implant users.Results: There was a statistically significant improvement in all scores in the study group following cochlear implantation. BKB scores for cases was 49.8% compared to 83.6% for controls (p=0.037). CUNY scores for cases was 61.7% compared to 90.3% for controls (p=0.022). The 3AFC and environmental sounds scores were also better in controls compared to cases but the difference was not statistically significant. Quality of life scores improved following implantation in cases and controls but the improvement was only statistically significant in the controls. There was a 7.7% non-user rate in the cases. There were no non-users in the control group.Discussion: Early deafened,,late implanted patients can benefit audiologically from cochlear implantation and in this study the improvement in speech discrimination scores was greater than expected perhaps reflecting careful selection of patients. Nevertheless, audiological benefits are limited compared to traditional cochlear implant recipients with the implant acting as an aid to lip reading in most cases.Conclusion: With careful selection of candidates, cochlear implantation is beneficial in early deafened, late implanted patients. [ABSTRACT FROM AUTHOR]

Aim To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD). Materials and methods The pre- and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS). Results Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19–68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0–5) and 2.5 (range 0–6), while the post-CI scores 5.8 (range 2–9) and 7.7 (range 3–10), respectively. Conclusions Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids. [ABSTRACT FROM AUTHOR]

Electric Acoustic Stimulation is the combination of acoustic stimulation (via a hearing aid) and electric stimulation (via a cochlear implant) in the implanted ear. This paper summarises the preliminary speech perception scores and hearing preservation in adult patients (n = 9) implanted with the Sonata FlexEAS electrode array. Subjects were adult candidates who met the national guidelines for cochlear implantation yet had some usable low frequency hearing. The surgical procedure aimed to preserve this residual hearing in the ear to be implanted. Patients retaining this usable hearing post operatively were issued with the DUET speech processor which provides a combination of acoustic and electric stimulation. Patients whose residual hearing was not preserved used the OPUS 2 processor in conjunction with the FlexEAS electrode array. The aim of the study was to investigate the speech perception scores of these two patient groups in comparison to patients using the standard Sonata Flex Soft electrode array. We aim to analyse whether patients whose residual hearing was not preserved perform as well as those for whom hearing preservation was not attempted. Results show that hearing preservation is possible in this patient group and the outcomes in terms of aided speech discrimination are potentially excellent. Therefore, it may be prudent to review the criteria for implant candidature in this patient group in the future. [ABSTRACT FROM AUTHOR]