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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]

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]

Objective: To assess the otolaryngologic manifestations of Sjögren's syndrome (SS). Design: A retrospective case study. Setting: The Toronto Hospital. Method: Case note review of 196 patients evaluated in a multidisciplinary clinic for this disease. Patients were retrospectively classified according to the revised international classification. Main Outcome Measures: The prevalence of subjective and objective audiologic and otorhinolaryngologic abnormalities. Results: One hundred eleven patients were diagnosed with primary and 26 with secondary SS, leaving 60 with unclassified sicca syndrome. There was minimal otologic pathology. There was a mildly increased prevalence of sensorineural deafness in secondary SS (41-60 years, 36%; 61-80 years, 70%). Approximately 50% of patients in each group complained of nasal symptoms, but only 20% had abnormal findings on rhinoscopy. Similarly, 60 to 70% in each group complained of throat symptoms, but only 20% had abnormal findings on indirect laryngoscopy. Thirty-eight percent of primary patients had parotid gland symptoms, and 25% had abnormally swollen glands, with eight subsequently diagnosed with lymphoma. No patients in the other two groups had abnormal parotid glands. Conclusions: SS does not appear to be associated with increased otologic or audiologic disease, except perhaps in conjunction with systemic autoimmunity. Nose and throat symptoms are common in SS, but the complications of mucosal dryness on examination are unusual (approximately 20%). Primary SS can cause serious parotid morbidity secondary to inflammation and infection. There is also a significant risk of lymphomas that often present as parotid masses, necessitating long-term follow-up. [ABSTRACT FROM AUTHOR]