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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.

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.

Conclusions: Codeine shows a similar association with profound deafness to other opiates. Good cochlear implant outcomes suggest a sensory disorder. Although macrocytosis could be due to confounding factors, the lack of other consistent findings may signify a vascular pathology., Objectives: To describe a series of patients identified as codeine users after referral for cochlear implantation., Patients and Methods: This was a retrospective case series review. Patients were identified by the senior audiologist. Information regarding mode of onset of deafness, past medical history, drug and alcohol history, investigations and audiological outcomes following cochlear implantation was collected from hospital records and patient questionnaires., Results: Ten patients were included in the study. All patients had taken codeine phosphate and paracetamol in combination for several years, usually at greater than recommended daily dose. All patients presented with sudden or rapidly progressive bilateral deafness. All patients had a significant macrocytosis at the time of deafness (mean cell volume (MCV): mean 115 fL; range 105-132 fL). No other investigation was consistently abnormal. Four patients had a history of alcoholism. Seven patients had abnormal liver function tests. Patients usually performed well with cochlear implants (CUNY sentence scores without lip reading >90% in 9 of 10 patients).