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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]

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]

Abstract: Over the past two decades, there has been increasing interest in the diagnosis and management of schwannomas of the inner ear including hearing rehabilitation with cochlear implants. However, tumor nomenclature and classification within the literature have been variable and oftentimes cumbersome. The term "intralabyrinthine schwannoma" is in common use when describing these tumors but is a potential source of confusion given that people often use the term "labyrinth" or "labyrinthine" to refer to the vestibular component of the inner ear only (i.e., labyrinthectomy or the translabyrinthine approach).During the Ninth Quadrennial Conference on Vestibular Schwannoma and Other Cerebellopontine Angle Lesions in Bergen, Norway, in May 2023, a multidisciplinary group of conference participants met and discussed issues pertaining to current terminology and classifications to enhance clarity and to reflect recent advances in tumor management and hearing rehabilitation.Although a variety of terms have been previously used to describe inner ear schwannomas, consensus was achieved on the term "inner ear schwannoma (IES)" to describe eighth nerve schwannomas of the cochlea, vestibule, or semicircular canals. Subgroups under this term comprise intravestibular, intracochlear, or intravestibulocochlear inner ear schwannomas (low complexity tumors), inner ear schwannomas with transfundal extension into the internal auditory canal but without modiolar involvement (intermediate complexity tumors), and inner ear schwannomas with transfundal extension with modiolar involvement (high complexity tumors).The details of the recommendations for an updated and simplified tumor nomenclature centered around tumor control and hearing rehabilitation with cochlear implantation are presented., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

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).

This study aimed to assess the extent and implications of short-term hearing fluctuation in Meniere's disease. Thirty-six subjects diagnosed with Meniere's were recruited to measure their own hearing using in-situ audiometry via a hearing aid (Widex Diva) and a portable programmer (SP3). Self-hearing tests measuring up to 14 frequency bands were conducted three times a day over eight weeks using the expanded Sensogram. Twenty-three ears showed low frequency fluctuation while ten fluctuated in mid frequencies with some 'double peak' audiogram configurations. Eight ears in the later stages of Meniere's, contrary to expected, also recorded fluctuation across all frequencies. Self-hearing testing Meniere's ears over eight weeks revealed great hearing fluctuation with significant changes in audiogram configuration. It suggests that as endolymphatic hydrops progresses through the cochlea, low frequency fluctuation is followed by fluctuation in the mid frequencies, leading to fluctuation across all frequencies. Use of a self-hearing test may facilitate diagnosis and hearing aid fitting for this population, as clinical audiograms may not provide accurate information of hearing fluctuation.