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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.)

Background and Objectives: Surveillance studies offer sparse knowledge of predictors of future growth in sporadic vestibular schwannomas (VS).Our aim was identification of these risk factors. We propose a scoring system to estimate the risk of growth in sporadic vestibular schwannoma., Methods: This retrospective study is based on the demographic and radiological data of 615 adult patients under the surveillance for single VS in our center. Univariate analysis, multivariate regression, and Kaplan-Meier analysis were used when appropriate. The regression coefficient-based "VS score" was calculated based on Cox proportional-hazards regression., Results: During surveillance, 285 tumors (46%) remained stable, 314 tumors (51%) grew, and 16 tumors (3%) shrank. The significant risks factors for future growth identified both in univariate and multivariate analyses were younger age at onset, cystic morphology, larger tumor volume, and cisternal location (as per Hannover grade). The proportion of growing tumors was 40%, 75%, and 96% among the homogeneous VS, primary cystic, and VS transformed to cystic, respectively. Moreover, tumor growth during the 1st year was significant predictor of continuous growth. Our "VS score" includes variables such as age, sex, morphology, and Hannover grade. The score extends between -3 and 6 points. Kaplan-Meier, confusion matrix, and receiver operating characteristic analysis proved high accuracy of our scoring model., Conclusion: Our retrospective study revealed that younger age, cystic morphology, cisternal extent, larger volume, and growth during 1st year were strong predictors of future growth. Moreover, we propose a scoring system that accurately estimates the risks of future tumor growth., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Introduction: Recent evidence supports the efficacy of surgical navigation (SN) in improving outcomes of sinonasal and craniofacial oncologic surgery. This study aims to demonstrate the utility of SN as a tool for integrating surgical, radiologic, and pathologic information. Additionally, a system for recording and mapping biopsy samples has been devised to facilitate sharing of spatial information., Materials and Methods: SN was utilized for biopsy mapping in 10 sinonasal/craniofacial oncologic procedures. Twenty-five raters with experience in anterior skull base oncology were interviewed to identify 15 anatomical structures in preoperative imaging, relying on topographical descriptions and surgical video clips. The difference in the localization of anatomical structures by raters was analyzed, using the SN-mapped coordinates as a reference (this difference was defined as spatial error)., Results: The analysis revealed an average spatial error of 9.0 mm (95 % confidence interval: 8.3-9.6 mm), with significant differences between surgeons and radiation oncologists (7.9 mm vs 12.5 mm, respectively, p < 0.0001). The proposed model for transferring SN-mapped coordinates can serve as a tool for consultation in multidisciplinary discussions and radiotherapy planning., Conclusions: The current standard method to evaluate disease extension and margin status is associated with a spatial error approaching 1 cm, which could affect treatment precision and outcomes. The study emphasizes the potential of SN in increasing spatial precision and information sharing. Further research is needed to incorporate this method into a multidisciplinary workflow and measure its impact on outcomes., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Objective: To summarize current knowledge on the indications, surgical strategies, and auditory outcomes of revision stapes surgery (RSS) for otosclerosis., Data Sources: The search was conducted in PubMed, Scopus, and Web of Science online databases, including papers published since 2000., Review Methods: The study was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. We considered original series describing RSS for otosclerosis, reporting postoperative hearing outcomes according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines for conductive hearing loss (CHL) for at least 30 consecutive cases. The quality of included studies was assessed with the Joanna Briggs Institute checklist. Pooled proportion and odds ratio (OR) meta-analysis were conducted using random effect models., Results: Nineteen studies were included, for a total of 2602 patients (2735 ears) and 2359 postoperative hearing results. RSS was mostly performed for recurrent CHL with air-bone gap (ABG)>20 dB. Multiple concomitant causes were often present, with prosthesis placement defect and incus necrosis as the most common causes. The pooled proportion of postoperative ABG<10 dB and ABG<20 dB was 57.2% (95% confidence interval [CI]: 52.8%-61.4%) and 79% (95% CI: 76.0%-81.8%), respectively. Dead ears were 1.2% (95% CI: 0.7%-2.1%). Compared to primary surgery, RSS had a significantly lower rate of ABG<10 dB (OR = 0.36, 95% CI: 0.24%-0.54%; P < .001). Both revision incudostapedotomy with/without incus reconstruction and malleovestibulopexy are viable options with nonsignificant differences in ABG closure rates (P = .182)., Conclusion: RSS is challenging and requires the surgeon to carefully evaluate all potential causes of previous failure and tailor the intervention according to intraoperative findings. In most cases, ABG closure within 20 dB can be achieved with a relatively low risk of dead ears., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Objectives: This systematic review and meta-analysis aimed to estimate the rate of taste disturbance following cochlear implantation., Methods: The review was designed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Included studies psychophysically measured taste. DerSimonian and Laird random-effects models were used. An overall mean from studies reporting a single mean of taste strip performance was calculated using inverse variance method for pooling., Results: Of 380 studies identified, 9 were included across which 55 cases of postoperative taste disturbance were reported in 498 patients. Taste was tested at variable timepoints, from <1 week to ≥6 months postoperatively. The overall rate of postoperative taste disturbance was 13.5% (95% CI, 7.6-20.7%) with high heterogeneity between studies (I 2  = 62%)., Discussion: 13.5% might indicate a higher prevalence of taste disturbance following cochlear implantation compared to the general population. However, the confidence we can assign to our calculated rate is limited by significant heterogeneity and potential publication bias. Studies reporting mean taste strip scores generally found reduced taste function on the side of the tongue ipsilateral to implantation, but this reduction wasn't statistically significant., Conclusion: Further research, employing more robust and standardised methodologies, is necessary to accurately ascertain the rate and nature of taste disturbance following cochlear implantation.

Purpose: The aim of this study was to provide an updated European narrative review spanning the last decade, focusing on the cost-effectiveness of cochlear implants (CIs) for adults with severe to profound post-lingual hearing loss., Methods: This review encompasses both prospective and retrospective approaches, as well as cross-sectional and longitudinal trials conducted on CIs in adults. All studies related to European countries (Austria, Germany, Switzerland, the Netherlands, Sweden, the UK and Poland) were conducted in English and were published between 2012 and June 2023., Results: Nine studies were included in the analysis. The patients' ages ranged from 18 years to over 67 years, with sample sizes ranging from 20 to 100 patients; two of these studies were focused on single-sided deafness in adults. The Markov model was identified as the most commonly utilized analysis method., Conclusions: This review identified a general consensus on CI cost-effectiveness, despite substantial variability among countries in factors such as observation time horizons, cost-effectiveness thresholds, methods of cost collection, discount rates, CI eligibility criteria and country-specific health systems. Generally, CIs yield positive societal benefits for working-age individuals, potentially less for seniors. Early unilateral CI enhances cost-effectiveness, highlighting the importance of prompt candidate identification. A consistent undersupply of CIs relative to the percentage of potential recipients emerged across countries. Therefore, further investigation into subcategories such as single-sided deafness is warranted, along with country-specific cost analyses. Emphasizing the significance of detailed information on health systems and associated costs and benefits is crucial for facilitating comparisons across different settings., (© 2024. The Author(s).)

Background: Sinonasal malignancies with orbital invasion have dismal prognosis even when treated with orbital exenteration (OE). Sugawara et al. developed a surgical strategy called "extended-OE (EOE)," showing encouraging outcomes. We hypothesized that a similar resection is achievable under endoscopic guidance through the exenterated orbit (endoscopic-EOE)., Methods: The study was conducted in three institutions: University of Vienna; Mayo Clinic; University of Insubria; 48 orbital dissections were performed. A questionnaire was developed to evaluate feasibility and safety of each step, scoring from 1 to 10, ("impossible" to "easy," and "high risk" to "low risk," respectively), most likely complication(s) were hypothesized., Results: The step-by-step technique is thoroughly described. The questionnaire was answered by 25 anterior skull base surgeons from six countries. Mean, median, range, and interquartile range of both feasibility and safety scores are reported., Conclusions: Endoscopic-EOE is a challenging but feasible procedure. Clinical validation is required to assess real-life outcomes., (© 2024 Wiley Periodicals LLC.)

Background: Pharmacologic immunosuppression regimes are commonly employed in stem cell clinical trials to mitigate host immune rejection and promote survival and viability of transplanted cells. Immunosuppression and cell survival has been extensively studied in retinal and spinal tissues. The applicability of stem cell therapy is rapidly expanding to other sensory organs such as the ear and hearing. As regenerative therapy is directed to new areas, a greater understanding of immunosuppression strategies and their efficacy is required to facilitate translation to organ-specific biologic microenvironments., Objective: This systematic review appraises the current literature regarding immunosuppression strategies employed in stem cell trials of retinal and neural cells., Methods: This systematic review was performed in line with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Inclusion criteria included studies presenting data on neural or retinal cells as part of an in-human clinical trial that detailed the immunosuppression regime used. Exclusion criteria included non-English language studies, animal studies, review articles, case reports, editorials, and letters. The databases Medline, Embase, Scopus, Web of Science, and the Cochrane Library were searched from inception to February 2024. Risk of bias was evaluated using the ROBINS-I tool., Results: Eighteen articles fit the inclusion criteria. Nine articles concerned retinal cells, 5 concerned spinal cord injury, and 4 concerned amyotrophic lateral sclerosis. A multi-drug and short-term immunosuppression regime were commonly employed in the identified studies. Detected immune responses in treated patients were rare. Common immunosuppression paradigms included tacrolimus, mycophenolate mofetil and tapering doses of steroids. Local immunosuppression with steroids was employed in some studies concerning retinal diseases., Discussion: A short-term course of systemic immunosuppression seemed efficacious for most included studies, with some showing grafted cells viable months to years after immunosuppression had stopped. Longer-term follow-up is required to see if this remains the case. Side effects related to immunosuppression were uncommon., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Gowrishankar et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Purpose: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection., Methods: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare., Results: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7)., Conclusions: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3-4 years of age, and at least annually, should be recommended., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Pure tone audiometry (PTA) is the gold standard for hearing assessment. However, it requires access to specialized equipment. Smartphone audiometry applications (apps) have been developed to perform automated threshold audiometry and could allow patients to perform self-administered screening or monitoring. This study aimed to assess the validity and feasibility of patients using apps to self-assess hearing thresholds at home, with comparison to PTA., Methods: A multi-center, prospective randomized study was conducted amongst patients undergoing PTA in clinics. Participants were randomly allocated to one of four publicly-available apps designed to measure pure tone thresholds. Participants used an app once in optimal sound-treated conditions and a further three times at home. Ear-specific frequency-specific thresholds and pure tone average were compared using Pearson correlation coefficient. The percentage of app hearing tests with results within ±10 dB of PTA was calculated. Patient acceptability was assessed via an online survey., Results: One hundred thirty-nine participants submitted data. The results of two at-home automated smartphone apps correlated strongly/very strongly with PTA average and their frequency-specific median was within ±10 dB accuracy. Smartphone audiometry performed in sound-treated and home conditions were very strongly correlated. The apps were rated as easy/very easy to use by 90% of participants and 90% would be happy/very happy to use an app to monitor their hearing., Conclusion: Judicious use of self-performed smartphone audiometry was both valid and feasible for two of four apps. It could provide frequency-specific threshold estimates at home, potentially allowing assessments of patients remotely or monitoring of fluctuating hearing loss., Level of Evidence: 2 Laryngoscope, 134:2864-2870, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Despite the spread of novel-generation cochlear-implant (CI) magnetic systems, access to magnetic resonance imaging (MRI) for CI recipients is still limited due to safety concerns. The aim of this study is to assess and record the experiences of Hires Ultra 3D (Advanced Bionics) recipients who underwent an MRI examination. A multicentric European survey about this topic was conducted focusing on safety issues, and the results were compared with the current literature. We collected a total of 65 MRI scans performed in 9 otologic referral centers for a total of 47 Hires Ultra 3D recipients, including, for the first time, 2 children and 3 teenagers. Preventive measures were represented by scanning time and sedation for children. Head wrapping was used in eight cases, and six of the eight cases received local anesthesia, even if both measures were not needed. Only three patients complained of pain (3/65 examinations, 4.6%) due to the tight head bandage, and one of the three cases required MRI scan interruption. No other adverse events were reported. We believe that these results should encourage MRI execution in accordance with manufacturer recommendations for Ultra 3D recipients.

Purpose of Review: The purpose of this review is to outline the temporal bone management of external and middle ear carcinoma. The review will outline the current evidence involved in deciding which surgical approach to take, as well as new advances in auditory rehabilitation and immunotherapy., Recent Findings: Traditional surgical approaches include lateral temporal bone resection, subtotal temporal bone resection and total temporal bone resection. They can also involve parotidectomy and neck dissection depending on extension of disease into these areas. Options for auditory rehabilitation include osseointegrated hearing aids, transcutaneous bone-conduction implants, and active middle ear implants. Recent advances in immunotherapy have included the use of anti-PD-1 monoclonal antibodies., Summary: The mainstay of management of temporal bone disease involves surgical resection. Early-stage tumours classified according to the Pittsburgh staging tool can often be treated with lateral temporal bone resection, whereas late-stage tumours might need subtotal or total temporal bone resection. Parotidectomy and neck dissection might also be indicated if there is a risk of occult regional disease. Recent advances in immunotherapy have been promising, particularly around anti-PD-1 inhibitors. However, larger clinical trials will be required to test the extent of efficacy, particularly around combination use with surgery., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Extrusion of electrodes outside the cochlea and tip fold overs may lead to suboptimal outcomes in cochlear implant (CI) recipients. Intraoperative measures such as Trans-Impedance Matrix (TIM) measurements may enable clinicians to identify electrode malposition and direct surgeons to correctly place the electrode array during surgery., Objectives: To assess the current literature on the effectiveness of TIM measurements in identifying extracochlear electrodes and tip fold overs., Methods: A scoping review of studies on TIM-based measurements were carried out using the Databases-Medline/PubMed, AMED, EMBASE, CINAHL and the Cochrane Library following PRISMA guidelines. Eleven full texts articles met the inclusion criteria. Only human studies pertaining to TIM as a tool used in CI were included in the review. Further, patient characteristics, electrode design, and TIM measurement outcomes were reported., Results: TIM measurements were available for 550 implanted ears with the subjects age ranged between 9 months to 89 years. Abnormal TIM measurements were reported for 6.55% (36). Tip fold over was detected in 3.64% (20) of the cases, extracochlear electrodes in 1.45% (8), and 1.45% (8) were reported as buckling. Slim-modiolar electrode array designs were more common (54.71%) than pre-curved (23.34%) or lateral wall (21.95%) electrode array. Abnormal cochlear anatomy was reported for five ears (0.89%), with normal cochlear anatomy for all other patients., Conclusion: TIM measurement is a promising tool for the intraoperative detection of electrode malposition. TIM measurement has a potential to replace intraoperative imaging in future. Though, TIM measurement is in its early stages of clinical utility, intuitive normative data sets coupled with standardised criteria for detection of abnormal electrode positioning would enhance its sensitivity., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Ayas et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Introduction: The presence of cervical lymph node metastases is an unfavorable prognostic factor in head and neck squamous cell carcinoma (HNSCC) and a potential cause of treatment failure. Occult lymph node metastasis occurs in approximately 15-20% of HNSCC patients with a clinically negative neck (cN0), greatly impacting on their prognosis. The present study aimed to investigate the role of pre-treatment peripheral blood markers in predicting clinically occult cervical lymph node metastasis., Methods: This multicenter, retrospective study was performed in a cohort of 472 patients diagnosed with cN0 HNSCC who underwent up-front surgery. Baseline neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic inflammatory marker (SIM), and systemic immune-inflammation index (SII) were calculated from available blood parameters., Results: Oro-hypopharyngeal and oral cancers, locally advanced stage, moderately (G2), and poorly (G3) differentiated grade were associated with an increased risk of pathological lymph node involvement. NLR, LMR, PLR, SIM, and SII were significantly associated at multivariable analysis. NLR &gt;2.12 was the most reliable at predicting occult lymph node metastasis (OR = 5.22; 95% CI: 2.14-12.75). We describe a predictive score integrating cancer site, local stage, and NLR which is effective at predicting positive lymph node pathological status., Conclusions: The present study provides evidence that pre-treatment peripheral blood markers, in particular NLR, represent reliable predictors of clinically occult cervical lymph node metastasis in cN0 HNSCC. Therefore, the present study provides a novel useful predictive score for directing the elective management of the neck in patients with cN0 HNSCC., (© 2023 S. Karger AG, Basel.)

Purpose: Heterogeneous reporting in baseline variables in patients undergoing transsphenoidal resection of pituitary adenoma precludes meaningful meta-analysis. We therefore examined trends in reported baseline variables, and degree of heterogeneity of reported variables in 30 years of literature., Methods: A systematic review of PubMed and Embase was conducted on studies that reported outcomes for transsphenoidal surgery for pituitary adenoma 1990-2021. The protocol was registered a priori and adhered to the PRISMA statement. Full-text studies in English with > 10 patients (prospective), > 500 patients (retrospective), or randomised trials were included., Results: 178 studies were included, comprising 427,659 patients: 52 retrospective (29%); 118 prospective (66%); 9 randomised controlled trials (5%). The majority of studies were published in the last 10 years (71%) and originated from North America (38%). Most studies described patient demographics, such as age (165 studies, 93%) and sex (164 studies, 92%). Ethnicity (24%) and co-morbidities (25%) were less frequently reported. Clinical baseline variables included endocrine (60%), ophthalmic (34%), nasal (7%), and cognitive (5%). Preoperative radiological variables were described in 132 studies (74%). MRI alone was the most utilised imaging modality (67%). Further specific radiological baseline variables included: tumour diameter (52 studies, 39%); tumour volume (28 studies, 21%); cavernous sinus invasion (53 studies, 40%); Wilson Hardy grade (25 studies, 19%); Knosp grade (36 studies, 27%)., Conclusions: There is heterogeneity in the reporting of baseline variables in patients undergoing transsphenoidal surgery for pituitary adenoma. This review supports the need to develop a common data element to facilitate meaningful comparative research, trial design, and reduce research inefficiency., (© 2023. The Author(s).)