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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.)

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.)

Objective: The objective of this study is to determine the rate of postoperative meningitis after cochlear implantation in those with inner ear malformations (IEMs) via meta-analysis., Data Sources: Medline, EMBASE, and the Cochrane Library., Methods: This study was reported following the preferred reporting items for systematic reviews and meta-analyses (PRISMA) checklist. Proportion meta-analysis was conducted through an inverse variance random-effect model based on arcsin transformation and presented as forest plots. Quality assessment of the included studies was performed through the National Institutes of Health Quality Assessment Tool., Results: Overall, 38 of 2966 studies met the inclusion criteria and were included in the analysis. There were 10 cases of meningitis after cochlear implantation in 1300 malformed ears. The overall rate of meningitis after cochlear implantation in IEMs was 0.12% (95% confidence interval, 0.006-0.380%; I2 = 0%). Cases occurred in incomplete partition (n = 5), Mondini deformity (n = 2), common cavity (n = 2), and enlarged internal auditory canal (n = 1). Six of 10 cases of postoperative meningitis occurred with an intraoperative cerebrospinal fluid leak., Conclusion: In those with IEMs, the risk of meningitis after cochlear implantation is very low., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Purpose of Review: The introduction of antiretroviral therapy has significantly impacted the incidence of head and neck squamous cell carcinoma (HNSCC) in people living with HIV (PLWH). This manuscript aims to give an overview of the evidence in the literature about this population., Recent Findings: PLWH have an increased incidence of HNSCC, with earlier age and more advanced stage at diagnosis. This epidemiologic trend may be explained by combining traditional and HIV-related risk factors. With the improvement of global health status, more patients are candidate for complex therapeutic strategies with curative intent. To date, it is still debated whether HIV-infected patients possess a profile of increased risk regarding treatment-related toxicity and survival outcomes, with the literature still lacking substantial evidence. Among the prognostic factors that can guide the clinician in selecting the most appropriate treatment strategy, age, site/subsite, stage, HIV viral load, and CD4+ T-cell count at diagnosis are the most relevant., Summary: Pathogenesis, treatment characteristics, oncologic outcomes, and prognostic factors of HNSCC in PLWH are still debated. Given the increasing incidence of HNSCC in PLWH, the need for dedicated evidence-based studies represents a significant research gap to be addressed., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The current study systematically reviewed the literature to compare auditory outcomes of patients treated for labyrinthine fistula (LF) based on characteristics of disease and surgical management., Databases Reviewed: PubMed, Scopus, Web of Science., Methods: Original series (at least five cases) published from 2000 reporting management and hearing results of LF secondary to cholesteatoma were included. Proportion and odds-ratio (OR) meta-analyses were conducted through inverse variance random-effects models based on logit transformation., Results: The prevalence of LF is estimated to be 7% (95% confidence interval [CI], 5-9%). Fistulae involving the lateral semicircular canal (90%; 95% CI, 87-93%) and larger than 2 mm (53%; 95% CI, 43-64%) were common, whereas membranous involvement was less frequent (20%; 95% CI, 12-30%). Complete removal of the cholesteatoma matrix overlying the LF was mostly applied. Bone conduction (BC) preservation was frequently achieved (81%; 95% CI, 76-85%); new-onset postoperative anacusis was rarely reported (5%; 95% CI, 4-8%). A higher chance of BC preservation was associated with sparing the perilymphatic space (OR, 4.67; 95% CI, 1.26-17.37) or membranous labyrinth (OR, 4.56; 95% CI, 2.33-8.93), exclusive lateral semicircular canal involvement (OR, 3.52; 95% CI, 1.32-9.38), smaller size (<2 mm; OR, 3.03; 95% CI, 1.24-7.40), and intravenous steroid infusion (OR, 7.87; 95% CI, 2.34-26.42)., Conclusion: LF occurs in a significant proportion of patients with cholesteatoma. In the past two decades, complete removal of the cholesteatoma matrix followed by immediate sealing has been favored, supported by the high proportion of BC preservation. Hearing preservation depends primarily on characteristics of the LF, and specific surgical strategies should be pursued. Intraoperative and postoperative intravenous steroid infusion is recommended., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To determine if cochlear implantation without removal of inner ear schwannomas (IES) is an effective treatment option for Neurofibromatosis 2 (NF2) patients. To determine how the presence of an intracochlear schwannoma might impact cochlear implant (CI) outcomes and programming parameters., Study Design: Retrospective chart review., Setting: Tertiary center for cochlear and auditory brainstem implantation., Patients: Of 10 NF2 patients with IES, 8 are reported with no previous tumor removal on the implanted ear., Interventions: Cochlear implant without tumor removal., Main Outcome Measures: Performance outcomes with CI at least 1-year post intervention. Programming parameters, including impedances, for patients with IES., Results: All patients had full insertion of the electrode arrays with round window approaches. Performance outcomes ranged from 0 to 100% for Bamford-Kowal-Bench sentences. Impedance measurements for active electrodes for patients with IES were comparable to those measured in patients without vestibular schwannoma (VS). Only patients who had radiation treatment before receiving their implant had elevated threshold requirements for CI programming compared with CI recipients without VS., Conclusion: Cochlear implantation without tumor removal is an effective option for treating NF2 patients with IES. The presence of an intracochlear tumor did not have an impact on CI performance or programming requirements compared with patients without VS; however, previous treatment with radiation may be related to elevated current requirements in the CI settings., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: Management of vestibular schwannomas (VS) involves surgery, radiotherapy, or surveillance, based on patient and tumor factors. We recently described conditional probability as a more accurate method for stratifying VS growth risk. Building on this, we now describe determinants of VS growth, allowing clinicians to move toward a more personalized approach to growth-risk profiling., Methods: Retrospective analysis of a prospectively collected database in a tertiary referral skull base unit between 2005 and 2014. Inclusion of patients with unilateral VS managed on surveillance protocol for a minimum of 5 years. Analysis of patient age, sex, tumor location, tumor size, and symptomology using conditional probability., Results: A total of 340 patients met inclusion criteria. The conditional probability of growth of extracanalicular VS was significantly higher versus intracanalicular (IC) VS (30% versus 13%, p < 0.001) as was small-sized VS versus IC VS (28 versus 13%, p = 0.002), but only in the first year after diagnosis. Sex, age, and presenting symptoms did not significantly affect VS growth., Conclusion: In our series, extracanalicular VS were more likely to grow than IC VS and small-sized VS more likely to grow than IC VS, but only in the first year after diagnosis. Conversely, sex, age, and presenting symptoms did not affect the conditional probability of VS growth., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: To review our experience with cochlear implant infections over the past 5 years, the management strategy and to identify predictive factors that led to explantation., Study Design: Retrospective record-base case series of cochlear implant infections., Setting: Tertiary otology and implant center., Patients: All patients who had cochlear implantation over a period of 5 years., Intervention(s): None., Main Outcome Measure(s): To identify risk factors, rates and outcomes of cochlear implant infections, and to formulate strategies to develop clearer management protocols to prevent cochlear implant explantation., Results: Of 704 implanted patients, 22 suffered a postoperative soft tissue infection (3%). Fifty-nine percent of these infected patients resulted in explantation, giving an explantation rate of 1.8% over the whole study population. One hundred percent of the infected implants identified as having either Staph. Aureus or Pseudomonas spp. as the single causative organism resulted in explantation., Conclusions: There is a high rate of explantation when infection is detected. Currently there is no clear consensus on medical management, such as choice of antibiotics or length of antibiotic course. A registry of cochlear implants would facilitate standard reporting methods for severity and type of infection, to be able to pool data across centers and form a more robust management protocol for cochlear implant infections.

Objectives: This review summarizes current evidence on causes and management strategies for delayed pain post-cochlear implantation (CI) surgery, without clinical evidence of inflammation or infection., Methods: The systematic review was undertaken in line with Preferred Reporting Items for Systematic review and Meta-Analysis Protocols 2015 guidelines. A literature search was undertaken, with inclusion of patients who underwent CI and presented with delayed pain (>3 months post-operatively) around their device site without an identifiable cause. Analysis was undertaken using MATLAB (MathWorks, Natick, MA) and the R-software package (www.r-project.org)., Results: 4 articles (48 patients), all retrospective case series, met inclusion criteria. The mean onset of pain post-CI was 60 months and mean follow-up was 15.8 months, there was no difference in the prevalence of pain between device brands (p=0.13). The majority (90%) did not have any hearing deterioration, and investigations did not reveal a cause for the pain in any of the patients. In terms of management, medical therapies, including oral therapy (analgesia, non-steroidal anti-inflammatories, antibiotics) and local treatments (topical, injections) resolved pain in 41% and 63%, respectively. Surgical intervention (explantation, magnet replacement, tympanic neurectomy), where undertaken, resolved pain in 100%. A minority had an identifiable infective microorganism cultured from intra-operative soft tissue or biofilm samples., Conclusions: Evidence for the causes and management of delayed pain post-CI without clinical evidence of inflammation is scarce. A stepwise approach is deemed best, with decisions being made on an individual basis, evaluating each patient's specific circumstances and priorities. Further evaluation of explanted devices would allow for better understanding of the causes and treatment of this group of patients.

Objective: To investigate the outcomes of hearing preservation surgery (HPS) for acoustic neuroma and quantify tumor and patient characteristics predictive of hearing preservation after surgery., Study Design: Retrospective study., Setting: Tertiary referral center., Patients: A total of 100 consecutive patients diagnosed with acoustic neuroma from 2000 to 2012., Intervention: Hearing preservation surgery through microscopic retrosigmoid approach combined with a retrolabyrinthine meatotomy., Main Outcome Measure: Pre- and postoperative hearing stratified according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Tokyo classifications. The most accurate cutoff was identified for each tumor and patients' variable affecting the outcome by calculating the Youden index. A multivariable analysis was undertaken at these cutoffs to identify prognostic factors for hearing preservation., Results: Preoperative hearing class was preserved after surgery in 31% (AAO-HNS), and 39% (Tokyo classification) of patients. According to the AAO-HNS classification, the tumor size in the cerebello-pontine angle, pure-tone average (PTA), and speech discrimination score cutoffs for predicting good postoperative hearing function were 7 mm, 21 dB, and 90%, respectively. With the Tokyo classification, only the PTA cutoff differed, with 27 dB. On multivariable analysis, tumor size and PTA were independent prognostic factors for postoperative hearing with high model's goodness of fit (area under the curve  = 0.784; 95% CI = 0.68-0.88 and area under the curve  = 0.813; 95% CI = 0.72-0.90), according to both the hearing classifications., Conclusions: The estimated cutoffs for tumor size and PTA were independently associated with HPS. These factors should be prospectively investigated before they are adopted as selection criteria for HPS.

Objective: The natural history of vestibular schwannomas (VS) is well documented in the literature, with tumour growth being paramount to decision making for both surveillance and treatment of these patients. Most previous studies refer to the risk of VS growth over a given period of time; however, this is not useful for counselling patients at different stages of their follow-up, as the risk of tumour growth is likely to be less following each subsequent year that a tumour does not grow. Accordingly, we investigated the conditional probability of VS growth at particular time-points, given a patient has not grown thus far. This Bayesian method of risk stratification allows for more tailored and accurate approximations of the risk of growth versus nongrowth of VS., Methods: Retrospective analysis of a prospectively collected database in a tertiary referral skull base unit, containing all patients diagnosed between 2005 and 2014 with sporadic unilateral VS and a minimum of 5-year surveillance., Results: A total of 341 patients met the inclusion criteria. The mean age at diagnosis was 67 years, the sizes of the VS at diagnosis were intracanalicular in 49%, small in 39%, medium in 11%, and large in 1%. Over the entire 5-year surveillance period, a total of 139 tumours were seen to grow (41%) and 202 did not grow (59%). At 1 year, the probability of growth given that the tumour had not grown to date was seen to be 21%, at 2 years 12%, at 3 years 9%, at 4 years 3%, and at 5 years 2%. The conditional probability of growth of extracanalicular VS was significantly higher in the first year when compared with intracanalicular VS (29% versus 13%, p = 0.01), but there was no such difference in years 2, 3, 4 or 5 (p = 0.60, 0.69, 0.36, 0.39, respectively)., Conclusion: This is the first study in the literature concerned specifically with the conditional probability of VS growth. The data presented here can be used to better inform VS patients of their risk of growth at particular time points in their disease-the longer VS have been observed to be stable, the lower the risk of subsequent growth in a given year. Further, an extracanalicular vestibular schwannoma is more likely to grow in the first year compared with an intracanalicular vestibular schwannoma. Our data also adds support to surveillance protocols with increasingly infrequent MRI scans, as after 4 years of not growing, the risk of growth in year 5 falls to <2%.