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Introduction: Very few papers investigated the etiologic breakdown and demographic characteristics of patients with facial nerve (FN) palsy. Our paper aims to present the etiologic breakdown and demographic characteristics of patients with FN palsy, presenting at a private care center between 2014 and 2019, along with the treatment modalities that were offered to them., Methods: Charts of 800 patients with facial palsy (FP) were reviewed. Data included the etiology of their diagnosis, family history, recurrent FP, demographic information, and treatment provided before and after presentation., Results: Seventy-five percent of our study population were females. The average period between diagnosis with FP and presentation at our center was 10.8 years. The most commonly identified etiology was Bell's palsy, followed by acoustic neuroma. Eighty-one percent of the study subjects were prescribed steroids and/or antivirals. Facial neuromuscular retraining, electrical stimulation, chemodenervation, and surgical intervention were also part of some treatment plans for our population., Discussion: Recommendations for the treatment of idiopathic FP include steroids with adjuvant antiviral medications. Data remains uncertain whether the combination therapy of steroids and antivirals has better results compared to steroids alone. Electrical stimulation is still a controversial therapeutic tool for facial paralysis with a potential role in exacerbating synkinesis. The difference in referral patterns between tertiary and private care centers can explain the disparity in the ranking of the etiologies between our study and what has been published., Conclusion: Management of FP is a complex process. The FN community must develop a common database to improve its understanding of the different presentations., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Objective: Acute sensorineural hearing loss represents a spectrum of conditions characterized by sudden onset hearing loss. The "Clinical Practice Guidelines for the Diagnosis and Management of Acute Sensorineural Hearing Loss" were issued as the first clinical practice guidelines in Japan outlining the standard diagnosis and treatment. The purpose of this article is to strengthen the guidelines by adding the scientific evidence including a systematic review of the latest publications, and to widely introduce the current treatment options based on the scientific evidence., Methods: The clinical practice guidelines were completed by 1) retrospective data analysis (using nationwide survey data), 2) systematic literature review, and 3) selected clinical questions (CQs). Additional systematic review of each disease was performed to strengthen the scientific evidence of the diagnosis and treatment in the guidelines., Results: Based on the nationwide survey results and the systematic literature review summary, the standard diagnosis flowchart and treatment options, including the CQs and recommendations, were determined., Conclusion: The guidelines present a summary of the standard approaches for the diagnosis and treatment of acute sensorineural hearing loss. We hope that these guidelines will be used in medical practice and that they will initiate further research., Competing Interests: Declaration of competing interest All authors declare no conflicts of interest in this study., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: This study aimed to determine if hypointense cochlear magnetic resonance imaging (MRI) constructive interference in steady-state (CISS) signal correlates with hearing outcomes in conservatively managed vestibular schwannoma (VS) patients., Study Design: Retrospective review of 657 cases from 1992 to 2020., Setting: Tertiary academic referral center., Patients: A retrospective review was performed to identify conservatively managed VS patients with appropriate baseline MRI, audiology, and at least 12-month audiological follow-up. Patients were excluded if they progressed to surgery or radiotherapy in less than 12 months, bilateral tumors, or surgery on the contralateral ear., Intervention: Conservatively managed patients with CISS imaging studies and audiology testing., Main Outcome Measures: Primary outcome measure change in pure-tone average (PTA) and word recognition score (WRS). Secondary outcome measures tumor size, presence of lateral fluid cap, or cystic changes., Results: A total of 92 individuals (47% male, 58 ± 11.6 yr) met the inclusion criteria, with 36 (39%) of patients demonstrating abnormal cochlear CISS signal. At baseline, abnormal cochlear CISS signal was associated with higher intracanalicular (IC) length (7.9 versus 6.6 mm, p = 0.0177) and lower WRS (55.7 versus 78.8 dBHL, p = 0.0054). During follow-up, individuals with abnormal cochlear CISS signal had significantly higher PTA (62.4 versus 46.4 dBHL, p = 0.0010). After adjusting for baseline covariates, abnormal cochlear CISS signal was consistently associated with a greater increase in PTA of 8.3 dBHL (95% confidence interval, 2.9-13.7; p = 0.0032) from baseline when compared with the normal group., Conclusions: Abnormal cochlear signal on MRI CISS sequences is associated with poorer hearing outcomes in conservatively managed VS patients., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Purpose: While some factors have been well-shown to affect the decision-making in treating patients with vestibular schwannomas (VS), little is known on the role of deprivation. Our objective was to assess the effect of socioeconomic background on the management of patients with VS., Methods: This retrospective cohort study included 460 patients with sporadic VS from West of Scotland. The postcode-based, multifactorial Scottish Index of Multiple Deprivation (SIMD) was used to assess the socioeconomic background of each patient. We performed a multivariate analysis including tumour size, growth and patient age with management modality (observation, stereotactic radiotherapy, microsurgery) being the main outcome measure and outcome (need for additional treatment) an additional measure., Results: We found no significant difference in the demographics, tumour characteristics and primary treatment choice between patients with different SIMD scores. In addition, there was no statistically significant difference in the growth occurrence rates following first-line treatment (p = 0.964) and in the second-line treatment choice (p = 0.460)., Conclusions: Multiple deprivation does not affect decision making in patients with VS in the examined cohort. This is probably linked to the centralisation and uniformity of the service and might not necessarily be applicable to other health services without centralisation., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Vestibular schwannomas (VS) are benign intracranial tumors posing significant management challenges. This study aims to compare the outcomes of stereotactic radiosurgery (SRS) and watchful waiting (WW) in the management of newly diagnosed VS, integrating findings from both retrospective and the pioneering V-REX prospective trial. Adhering to PRISMA guidelines, a systematic review was conducted using MEDLINE, Embase, and Cochrane databases. Studies directly comparing SRS with WW for newly diagnosed VS were included. Primary outcomes focused on hearing preservation assessed through the AAO-HNS or Gardner-Robertson hearing classification scales and tumor progression, with secondary outcomes focusing on neurological symptoms, and the need for further treatment. Thirteen studies encompassing 1,635 patients (WW: 891; SRS: 744) were included.While no significant difference was found in serviceable hearing loss at last follow-up (RR = 1.51, [95%CI: 0.98, 2.32], p = 0.06), significant differences favoring WW were observed in pure tone audiometry (PTA) (MD = -13.51 [95%CI: -22.66, -4.37], p = 0.004) and word recognition score (WRS) (MD = 20.48 [95%CI: 9.72, 31.25], p = 0.0002). Analysis of tumor progression indicated no overall significant difference in risk between SRS and WW (RR = 0.40, [95%CI 0.07, 2.40], p = 0.32), but subgroup analysis suggested a lower risk with SRS in certain contexts. The need for further treatments favored SRS (RR = 0.24, [95%CI: 0.07, 0.74], p = 0.007). No significant differences were found in tinnitus and imbalance between the two groups. This comprehensive analysis suggests no marked difference in functional hearing preservation between SRS and WW in managing VS. However, untreated tumors commonly necessitate additional interventions. These findings highlight the need for individualized treatment decisions and underscore the importance of continued monitoring. The study advocates for further prospective trials to refine management strategies for VS., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Treatment of vestibular schwannoma (VS) has been extensively studied, but a gap in knowledge exists demonstrating how racial and socioeconomic status influence VS presentation. Our institution has a unique setting with a public safety net hospital (PSNH) and tertiary academic medical center (TAMC) in the same zip code, which we study to evaluate initial VS presentation disparities in patient populations presenting to these hospital settings., Methods: Retrospective chart review was performed of all adult patients (n = 531) presenting 2010 to 2020 for initial VS evaluation at TAMC (n = 462) and PSNH (n = 69). Ethnicity, insurance, maximum tumor size, audiometry, initial treatment recommendation, treatment received, and follow up were recorded and statistical analysis performed to determine differences., Results: Average age at diagnosis (51.7 ± 13.6 TAMC vs 52.3 ± 12.4 PSNH) and gender (58.4% TAMC vs 52.2% PSNH female) were similar. Patients' insurance (TAMC 75.9% privately insured vs PSNH 82% Medicaid) and racial/ethnic profiles (TAMC 67.7% White and 10.0% Hispanic/Latinx, vs PSNH 4.8% White but 59.7% Hispanic/Latinx) were significantly different. Tumor size was larger at PSNH (20.2 ± 13.3 mm) than TAMC (16.6 ± 10.0 mm). Hearing was more impaired at PSNH than TAMC (mean pure tone average 58.3 dB vs 43.9 dB, word recognition scores 52.3% vs 68.2%, respectively). Initial treatment recommendations and treatment received may include more than 1 modality. TAMC patients were offered 66.7% surgery, 31.2% observation, and 5.2% radiation, while PSNH patients offered 50.7% observation, 49.3% surgery, and 8.7% radiation. TAMC patients received 62.9% surgery, 32.5% observation, and 5.3% radiation, while PSNH patients received 36.2% surgery, 59.4% observation, and 14.5% radiation. Follow up and treatment at the same facility was not significantly different between hospitals., Conclusions: Hearing was worse and tumor size larger in patients presenting to PSNH. Despite worse hearing status and larger tumor size, the majority of PSNH patients were initially offered observation, compared to TAMC where most patients were initially offered surgery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Vestibular Schwannoma (VS), previously known as acoustic neuroma, constitutes the majority of tumours found in the cerebellopontine angle (CPA). Most guidelines for managing CPA tumours have been developed by high-income countries (HICs). However, these guidelines often fall short in addressing the unique challenges encountered in low- and middle-income countries (LMICs), such as Pakistan. In LMICs, issues related to a limited healthcare workforce, inadequate infrastructure, and constrained financial resources hinder the effective implementation of these HIC-derived guidelines. Additionally, it has been observed that VS tends to present at a larger size in LMICs compared to HICs. Given that VS is the predominant type of CPA tumour and other types are covered under separate guidelines, this article aims to provide practical, contextspecific recommendations for the screening, diagnosis, and management of Vestibular Schwannoma in LMIC settings. Our focus is to bridge the gap in care strategies and adapt them to the resource constraints and clinical realities of LMICs.

Introduction: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy., Material and Methods: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC., Results: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found., Conclusions: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts., (Copyright © 2023 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Published by Elsevier España, S.L.U. All rights reserved.)

The treatment of vestibular schwannomas (VS) has always posed a challenge for physicians. Three essential treatment principles are available: wait-and-scan, surgery, and stereotactic radiotherapy. In addition to the type of treatment, decisions must be made regarding the optimal timing of therapy, the combination of different treatment modalities, the potential surgical approach, and the type and intensity of radiation. Factors influencing the therapy decision include tumor location and size or stage, patient age, comorbidities, symptoms, postoperative hearing rehabilitation options, patient preferences, and, not least, the experience of the surgeons and the personnel and technical capabilities of the clinical site. This article begins with a brief overview of vestibular schwannomas, then outlines the fundamental interdisciplinary treatment options, and finally discusses the ENT (ear, nose, and throat)-relevant factors in the therapy decision., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Background: Socioeconomic variables including race, education, and income have been shown to affect vestibular schwannoma incidence, treatment, and outcomes. We sought to determine the impact of socioeconomic factors on quality of life at the time of vestibular schwannoma diagnosis., Methods: Retrospective cohort study conducted at a tertiary academic center. All patients evaluated for vestibular schwannoma from March 1, 2010 to December 31, 2021 who completed at least one Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire at presentation or prior to any intervention were included. PANQOL scores were compared across income quintiles, racial groups, and health insurance categories., Results: Two-hundred and ninety-six patients who had non-missing information on variables of interest were included. Compared to White/Caucasian patients (84.5 %), Black/African American patients (4.7 %) had significantly lower PANQOL total scores (b = -12.8[-21.7, -4.0], p = 0.005). Compared to patients with Commercial insurance (53 %), patients who were Uninsured/ Self-pay (1.7 %) had significantly lower PANQOL total scores (b = -16.7[-31.4, -1.9], p = 0.027). Patients in higher income quintiles had significantly higher PANQOL total scores (b = 11.7[3.9, 19.5], p = 0.004 comparing highest income quintile to lowest). After controlling for potential confounders, income quintile (b = 9.6[1.3, 17.9], p = 0.023 comparing highest quintile to lowest) and insurance (b = -17.0[-31.9, -21], p = 0.025 comparing Uninsured/Self-pay to Commercial insurance) remained predictors of total PANQOL score., Conclusions: Socioeconomic factors including race, health insurance, and income appear to contribute to quality of life at the time of vestibular schwannoma diagnosis. These variables are interrelated and the effects of race may be mediated in part by differences in income and health insurance coverage., 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 © 2023 Elsevier Ltd. All rights reserved.)

Background Acoustic neuromas (ANs) are consistently associated with decreased quality of life (QOL) related to the physical and psychosocial impacts of symptoms experienced from the tumour and its treatment. This study explored patient-reported experiences of ANs in New Zealand, with a focus on the impact on QOL and the provision of information, support and services. Methods A mixed methods approach was taken, conducting an online community survey that included the Penn Acoustic Neuroma Quality of Life Scale (N  = 52). Those who indicated interest were offered semi-structured interviews after the survey (N  = 17), which were analysed using content analysis. Results A negative impact on QOL was found, highlighting five key themes in the experiences of people: (1) ongoing physical, social and psychological impacts; (2) information and support from the medical system; (3) autonomy and decision-making; (4) the importance of peer support; and (5) remaining positive - life goes on. Conclusions Our findings indicate areas for improvement that may benefit people's healthcare experience and QOL. Both quantitative and qualitative results identified gaps associated with person-centred care and the need for information, education, emotional support and access to services. Recommendations include a need for more information (verbal and written) during all stages of diagnosis and treatment, shared decision-making and increased access to allied health, including psychological services and support groups.

Importance: Over the past decades, the number of patients, especially in the older adult patient group, diagnosed with vestibular schwannoma (VS) has increased. Assuming that older adult patients have more comorbidities, a longer recovery period after surgery, a higher rate of surgical complications, and a higher mortality rate after VS surgery, a treatment strategy for this group of patients is warranted, based on clinical evidence on postsurgical survival., Objective: To evaluate the survival after diagnosis of a VS in patients 70 years and older, treated with either observation or surgery, and to compare these findings with the life span of an age-matched background population in Denmark., Design, Setting, and Participants: This was a retrospective cohort study of 624 patients 70 years and older diagnosed with VS in Denmark from 1976 to 2016. Since 1976, all patients with a VS have been registered in a national database, which contains 3637 patients. Of the included patients in this study, 477 were treated conservatively with the "wait-and-scan" strategy, and 147 were treated surgically with removal of the tumor. The survival of the patients was compared with a matched background population in Denmark. Data analysis was performed from January 1976 to January 2017., Exposures: Surgery, radiotherapy, or none., Main Outcomes and Measures: The main outcome was survival among the patients and compared with the matched background population., Results: A total of 624 patients were included (317 female patients [50.8%] and 307 male patients [49.2%]). The mean (SD) survival in the observed patients was 9.2 (4.7) years after diagnosis, whereas for the background population, the expected survival was 11 years from the mean age at diagnosis. For the surgically treated patients, the mean (SD) survival was 11.8 (6.6) years, and expected survival was 11 years for the matched background population. The mean (SD) survival was 10.7 (5.5) years in female patients and 8.9 (5.0) years in male patients. There was no significant difference in survival between treatment modalities, irrespective of tumor size., Conclusions and Relevance: In this cohort study, survival after diagnosis of a VS in patients 70 years and older was similar in the surgical group compared with the age-matched background population. In the wait-and-scan group, the survival after diagnosis was marginally shorter, which may be associated with increased comorbidity.

Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma., Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions., Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy., Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team., (Copyright © 2023 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier España S.L.U. All rights reserved.)

Purpose: During counseling and management of patients with vestibular schwannoma (VS), the emphasis is shifting from tumour control and nerve preservation towards maintaining or improving health-related quality of life (HRQoL). Understanding the patients' perspective and impact of VS is, therefore, of utmost importance. The current study aimed to identify treatment outcomes preferred by patients and to explore the patient-reported VS symptoms and management-related side effects and their impact on HRQoL., Methods: Patients with VS were contacted through the Dutch VS association Stichting Hoormij and questioned using a semi-structured, cross-sectional online survey. Patients were asked to report and rank symptoms and side effects, with their impact on HRQoL and frequency of occurrence. Results were structured through qualitative content analysis. Coded symptoms, side effects, impacts, frequencies, and patient-preferred outcomes were analysed and summarized with descriptive statistics., Results: Of the 231 respondents, 71% were actively treated. Hearing (symptoms vs. side effects: 78.8% vs. 63.6%), balance (62.3%; 48.8%), and energy issues (33.8%; 32.6%) were the most frequently mentioned symptoms and management-related side effects. Fatigue, deafness, headaches, and hearing loss had the highest impact on HRQoL. The majority of patients identified hearing preservation (61%), balance preservation (38.5%), and reduced tinnitus (34.6%) to be the patient-preferred outcomes., Conclusion: This qualitative study demonstrates that in this population many patients with VS encounter participation difficulties in their daily physical and social activities and value hearing and balance preservation, reduced tinnitus, and restored energy as preferred outcomes as they are hampered by symptoms and side effects related to hearing, balance, and energy. Healthcare professionals should consider these key points and use these and the patient-preferred outcomes in consultation, shared decision making, treatment, and follow-up to optimize patient-centred care., (© 2023. The Author(s).)

Introduction: Acoustic neuroma (AN) research largely employs a medical framework to understand health outcomes. An alternative is to examine quality of life (QOL) outcomes. This study explored whether mental well-being (i.e., anxiety and depression) were predictive of QOL in those with AN over and above symptomatology., Methods: A nationwide online survey was distributed to 24 community organisations. The inclusion criteria were a diagnosis of AN irrespective of the treatment approach. There were 52 respondents. Mental well-being was assessed using the Hospital Anxiety and Depression Scale (HADS), and quality of life was assessed using Penn Acoustic Neuroma QOL scale (PANQOL)., Results: The most frequently reported symptoms reported were poor balance, tinnitus, hearing loss, and headache. Preliminary analyses suggested that headaches, tinnitus and mental well-being were significantly correlated with QOL. Hierarchical regression revealed that these two symptoms and mental well-being accounted for 18.7% and 51.1% of the variance in QOL, respectively. In addition, there was a significant difference in depression scores between management types, with the surgery group having a significantly higher depression score than the radiation group., Conclusion: Symptoms and mood contribute to QOL for those diagnosed with AN. This can be understood through the common-sense model and fear of cancer recurrence. Screening for psychological difficulties should be provided from the point of diagnosis to post-treatment to allow for targeted management plans to mitigate the effects of these on QOL., 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 © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Importance: Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically., Objective: To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma., Design, Setting, and Participants: Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021., Interventions: Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires., Main Outcomes and Measures: The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications., Results: Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed., Conclusion and Relevance: Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed., Trial Registration: ClinicalTrials.gov Identifier: NCT02249572.

The advent of MRI has led to more sporadic vestibular schwannomas diagnosed today than ever before. Despite the average patient being diagnosed in their sixth decade of life with a small tumor and minimal symptoms, population-based data demonstrate that more tumors per capita are treated today than ever before. Emerging natural history data justify either an upfront treatment approach or the "Size Threshold Surveillance" approach. Specifically, if the patient elects to pursue observation, then existing data support the tolerance of some growth during observation in appropriately selected patients up until a specific size threshold range (about 15 mm of CPA extension). The current article discusses the rationale behind a shift in the existing observation management approach, where initial detection of growth typically begets treatment, and outlines the application of a more flexible and nuanced approach based on existing evidence., Competing Interests: Conflicts of interest No authors have relevant conflicts of interest to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome caused by a mutation in the NF2 suppressor gene and is characterized by the development of multiple benign tumors throughout the central nervous system. Bilateral vestibular schwannomas (VSs) are pathognomonic for NF2 and are associated with progressive hearing loss and eventual deafness in most patients. This review presents current management options for NF-2-associated VSs., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Vestibular schwannomas continue to cause hearing loss, facial nerve paralysis, imbalance, and tinnitus. These symptoms are compounded by germline neurofibromatosis type 2 (NF2) gene loss and multiple intracranial and spinal cord tumors associated with NF2-related schwannomatosis. The current treatments of observation, microsurgical resection, or stereotactic radiation may prevent catastrophic brainstem compression but are all associated with the loss of cranial nerve function, particularly hearing loss. Novel targeted treatment options to stop tumor progression include small molecule inhibitors, immunotherapy, anti-inflammatory drugs, radio-sensitizing and sclerosing agents, and gene therapy., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The current management of vestibular schwannomas (VS) includes observation, microsurgery (MS), and stereotactic radiosurgery (SRS) or radiotherapy, and treatment failures may occur with any primary modality. SRS is most often used for microsurgical failures, as it carries a low risk of adverse events. Salvage MS following previous MS is reserved for specific cases and can present certain surgical challenges. Irradiation failures can be managed with both salvage MS and repeat SRS. This article is intended to review an approach to the failure of primary interventions for VS, with a focus on the time interval between modalities, rates of tumor control, functional outcomes, and possible complications., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The future of the management of both sporadic and neurofibromatosis type 2-asscoiated vestibular schwannomas (VSs) will be shaped by cutting-edge technologic and biomedical advances to enable personalized, precision medicine. This scoping review envisions the future by highlighting the most promising developments published, ongoing, planned, or potential that are relevant for VS, including integrated omics approaches, artificial intelligence algorithms, biomarkers, liquid biopsy of the inner ear, digital medicine, inner ear endomicroscopy, targeted molecular imaging, patient-specific stem cell-derived models, ultra-high dose rate radiotherapy, optical imaging-guided microsurgery, high-throughput development of targeted therapeutics, novel immunotherapeutic strategies, tumor vaccines, and gene therapy., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To examine the utility of telemedicine in a tertiary otologic practice., Study Design: Retrospective case series., Setting: Tertiary neurotology clinic., Patients: Consecutive adult patients presenting via video visit between January 2020 and January 2021., Interventions: Televideo modality to conduct visits with patients seeking evaluation for new concerns, second opinions, or routine follow-up for established conditions., Main Outcome Measures: Success of the televideo visit defined by the televideo visit being sufficient for determining a definitive plan and not requiring deferment of recommendations for a subsequent in-person visit., Results: A total of 102 televideo visits were performed among 100 unique patients. Of those, 92 (90.2%) visits were for second opinions or evaluation of new concerns, most commonly for vestibular schwannoma (n = 32, 31.4%), followed by sensorineural hearing loss (n = 20, 19.6%). Other visits were conducted for early postoperative follow-up and established general follow-up. In 91.2% of cases (n = 93), patients were successfully evaluated and provided recommendations from the initial video visit. All visits with patients having a diagnosis of meningioma (n = 7), and nearly all with vestibular Schwannoma (97%, n = 31) and sensorineural hearing loss (95%, n = 19) were successful. Of the 79 patients offered surgery as one potential treatment option, 31 patients underwent surgery at our institution by time of review. Patients with unsuccessful visits (n = 9, 8.8%) were advised to schedule additional in-person diagnostic imaging, vestibular testing, or cochlear implant candidacy evaluation to establish a more definitive care plan., Conclusion: Virtual televideo visits were successful for a high percentage of selected patients seen at a tertiary neurotology practice, particularly those seeking evaluation of vestibular schwannoma or sensorineural hearing loss., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To report on the results of intracanalicular vestibular schwannomas (ICVS) that were managed by wait and scan and to analyze the possible predictors of tumor growth and hearing deterioration throughout the observation period., Study Design: A retrospective case series., Setting: Quaternary referral center for skull base pathologies., Patients: Patients with sporadic ICVS managed by wait and scan., Intervention: Serial resonance imaging (MRI) with size measurement and serial audiological evaluation., Main Outcome Measure: Tumor growth defined as 2 mm increase of maximal tumor diameter, further treatment, and hearing preservation either maintain initial modified Sanna hearing class, or maintain initial serviceable hearing (class A/B)., Results: 339 patients were enrolled. The mean follow-up was 36.5±31.7 months with a median of 24 months. Tumor growth occurred in 141 patients (40.6%) either as slow growth (SG) in 26.3% of cases or fast growth (FG) in 15.3% of cases. Intervention was performed in only 64 cases (18.8%). Out of 271 patients who underwent hearing analysis, 86 patients (33.5%) showed hearing deterioration to a lower hearing class of the modified Sanna classification. Tumor growth and older age were predictors of hearing deterioration. Of the 125 cases with initial serviceable hearing (Class A/B), 91 cases (72.8%) maintained serviceable hearing at last follow-up. Tumor growth and a worse initial pure tone average (PTA) were predictors of hearing deterioration., Conclusions: Wait and scan management of ICVS is a viable option and only 18.8% of patients needed further treatment. Hearing tends to deteriorate over time., Competing Interests: The authors disclose no conflicts of interest. Sponsored by Mario Sanna Foundation., (Copyright © 2022, Otology & Neurotology, Inc.)

Vestibular schwannoma (VS) is a benign tumor that originates from Schwann cells in the vestibular component. Surgical treatment for VS has gradually declined over the past few decades, especially for small tumors. Gamma knife radiosurgery has become an accepted treatment for VS, with a high rate of tumor control. For neurofibromatosis type 2 (NF2)-associated VS resistant to radiotherapy, vascular endothelial growth factor (VEGF)-A/VEGF receptor (VEGFR)-targeted therapy (e.g., bevacizumab) may become the first-line therapy. Recently, a clinical trial using a VEGFR1/2 peptide vaccine was also conducted in patients with progressive NF2-associated schwannomas, which was the first immunotherapeutic approach for NF2 patients. Targeted therapies for the gene product of SH3PXD2A-HTRA1 fusion may be effective for sporadic VS. Several protein kinase inhibitors could be supportive to prevent tumor progression because merlin inhibits signaling by tyrosine receptor kinases and the activation of downstream pathways, including the Ras/Raf/MEK/ERK and PI3K/Akt/mTORC1 pathways. Tumor-microenvironment-targeted therapy may be supportive for the mainstays of management. The tumor-associated macrophage is the major component of immunosuppressive cells in schwannomas. Here, we present a critical overview of targeted therapies for VS. Multimodal therapy is required to manage patients with refractory VS.

Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in this context. Varying growth rates of these benign tumors complicate a prediction of functional impairment of cranial nerves. Therefore, a regular update on current therapeutic strategies and alternative treatment options is relevant for both physicians and patients., (© 2021. The Author(s).)

Appropriate imaging strategies for the detection, treatment planning, and posttreatment monitoring of vestibular schwannomas will be discussed. The typical and variant imaging appearances of vestibular schwannomas, as well as the imaging features that should prompt consideration of differential diagnoses, will be illustrated. Understanding the natural history of vestibular schwannomas, optimal measurement and definition of tumour growth helps the radiologist evaluate for the failure of conservative management and requirement for surgery or radiotherapy. In order to determine the success of conservative management, the radiologist is required to understand the natural history of vestibular schwannomas and how tumour growth is defined. Finally, the imaging features which help guide appropriate treatment with surgery or radiotherapy will be highlighted, and the expected posttreatment imaging changes will be described., Competing Interests: Disclosure The author acknowledges funding support from Wellcome/Engineering and Physical Sciences Research Council Centre for Medical Engineering at King’s College London (WT 203148/Z/16/Z); National Institute for Health Research Biomedical Research Centre at Guy’s & St Thomas’ Hospitals and King’s College London; Cancer Research UK National Cancer Imaging Translational Accelerator (A27066); the UK Research & Innovation London Medical Imaging and Artificial Intelligence Centre., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

Decision making on the treatment of vestibular schwannoma (VS) is mainly based on the symptoms, tumor size, patient's preference, and experience of the medical team. Here we provide objective tools to support the decision process by answering two questions: can a single checkup predict the need of active treatment?, and which attributes of VS development are important in decision making on active treatment? Using a machine-learning analysis of medical records of 93 patients, the objectives were addressed using two classification tasks: a time-independent case-based reasoning (CBR), where each medical record was treated as independent, and a personalized dynamic analysis (PDA), during which we analyzed the individual development of each patient's state in time. Using the CBR method we found that Koos classification of tumor size, speech reception threshold, and pure tone audiometry, collectively predict the need for active treatment with approximately 90% accuracy; in the PDA task, only the increase of Koos classification and VS size were sufficient. Our results indicate that VS treatment may be reliably predicted using only a small set of basic parameters, even without the knowledge of individual development, which may help to simplify VS treatment strategies, reduce the number of examinations, and increase cause effectiveness., (© 2021. The Author(s).)

Objective: The degree to which various treatment modalities modify vestibular schwannoma (VS)-associated symptoms has received limited attention. The purpose of this study was to determine how different treatment modalities affect subjective symptoms in those presenting with VS., Study Design: Prospective survey., Setting: Tertiary neurotology referral center., Methods: Patients with sporadic VS who received treatment at our institution were prospectively surveyed with a VS symptom questionnaire. Those who completed a baseline survey prior to treatment and at least 1 posttreatment survey were included. The prospective survey evaluated the severity of self-reported symptoms (Likert scale, 1-10), including tinnitus, dizziness or imbalance, headaches, and hearing loss., Results: A total of 244 patients were included (mean age, 57 years). The mean duration of follow-up was 2.1 years, and the median number of surveys completed was 2 (interquartile range, 1-3). Seventy-eight (32%) cases were managed with observation, 118 (48%) with microsurgery, and 48 (20%) with radiosurgery. Multivariable analyses revealed no statistically significant difference in the change in tinnitus ( P = .15), dizziness or imbalance ( P = 0.66), or headaches ( P = .24) among treatment groups. Evaluation of clinically important differences demonstrated that microsurgery leads to significant bidirectional changes in headaches., Conclusions: Limited prospective data exist regarding the progression or resolution of subjective symptoms in those presenting with VS. This study suggests that tinnitus, dizziness or imbalance, and headaches are unlikely to be significantly modified by treatment modality and generally should not be used to direct treatment choice.

Purpose: In cases of small- to medium-sized vestibular schwannomas, three management strategies can be opted for: active surveillance, surgery or radiotherapy. In these cases, the patient's preference is pivotal in decision-making. The aim of this study was to identify factors that influence a patient's decision for a particular management strategy., Methods: A qualitative inductive thematic analysis was performed based on semi-structured interviews. Eighteen patients with small- to medium-sized vestibular schwannomas were interviewed. All patients were diagnosed or treated at one of the two participating university medical centers in the Netherlands., Results: Ten themes were identified that influenced the decision, classified as either medical or patient-related. The medical themes that emerged were: tumor characteristics, the physician's recommendation, treatment outcomes and the perceived center's experience. The patient-related themes were: personal characteristics, anxiety, experiences, cognitions, logistics and trust in the physician., Conclusion: Knowledge of the factors that influence decision-making helps physicians to tailor their consultations to arrive at a true shared decision on vestibular schwannoma management., (© 2020. The Author(s).)

Purpose: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series., Materials and Methods: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019., Results: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections., Conclusion: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations., (Copyright © 2021. Published by Elsevier Inc.)

Background: Optimal management of vestibular schwannoma (VS) is still debated and thus international consensus has not been achieved. Treatment options are observation, radiotherapy, and surgery. Knowledge on the natural history of tumor growth is essential for choice of treatment modality. The aim is to present intra-/extrameatal tumor growth and management data from a prospective, unselected national cohort of patients diagnosed with VS during the period 1976-2015., Methods: Since 1976, all data from patients diagnosed with sporadic VS in Denmark have been referred to our national treatment center, where they have been entered prospectively into the national database. Data on tumor localization, growth, and treatment were retrieved. Growth definition: >2 mm by linear measurement, in accordance with the Tokyo 2001 consensus-meeting recommendations., Results: 3637 cases of VS were diagnosed, in which 1304 patients had surgery and 21 received radiotherapy post diagnosis. 2312 patients were observed with mean follow-up of 7.33 years. Of these, 434(19%; 102 intra-and 332 extrameatal tumors) changed to active treatment during the observation period due to tumor growth. 5 years after diagnosis, 21% of the intrameatal tumors exhibited growth during observation, whereas 37% of extrameatal tumors had grown, increasing to 25% intrameatal and 42% extrameatal after 10 years. Following growth, the intrameatal tumors were mostly observed further and the extrameatal mostly underwent surgery. Tumor growth occurred mainly within the first 5 years post diagnosis., Conclusion: This natural history study documents the growth occurrence of both intra-and extrameatal VS during the first 12 years after diagnosis and should be used in patient counseling, management, and treatment decision making., (© The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: The relationship between quantitative posturography results and growth of vestibular schwannomas (VSs) during conservative management has not been studied. We aimed to clarify the relationship between the presence of disequilibrium based on posturographic measurement and VS growth., Methods: This retrospective, single-center study included 53 patients with VSs (Koos stage I or II) managed conservatively after initial diagnosis. Radiographic progression was considered present if 20% volumetric growth was observed over the imaging interval. Posturography was performed at initial diagnosis, and sway velocity (SV) and sway area were calculated. Tumor growth-free survival was estimated using the Kaplan-Meier method., Results: Mean follow-up period was 2.87 ± 2.58 years, up to tumor growth detection or last follow-up magnetic resonance imaging. Tumor growth incidence was 40.8% and 61.2% at 2 and 5 years, respectively. Cerebellopontine angle extension and SV with eyes open were related to tumor growth. Tumor growth-free survival of patients with cerebellopontine angle extension and patients with intracanalicular tumor at 2 years was 37.3% and 76.4%, respectively. Tumor growth-free survival of patients with SV >2.06 cm/second and patients with SV ≤2.06 cm/second at 2 years was 30.8% and 68.9%, respectively. The Cox hazard model demonstrated a significant risk for future tumor growth with SV >2.06 cm/second (relative risk, 2.475; 95% confidence interval, 1.11-5.37, P = 0.027)., Conclusions: We demonstrated a positive correlation between SV with eyes open and future tumor growth. Posturographic data are objective and quantitative; thus, SV may be a potential predictor of future growth of VSs., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: To evaluate the management of sporadic vestibular schwannomas (VS) with a 12-year follow-up., Study Design: Retrospective study of all VS patients initially treated in 2005 in a tertiary referent center., Methods: Initial decision making for microsurgical resection (MSR) or wait-and-scan (WaS) was according to VS size and hearing; subsequently, MSR or stereotactic radiosurgery (SRS) was proposed dependent on VS growth and size, hearing, and patient's age or willingness., Results: Two hundred and one sporadic VS were included. The first management apportionment was 120 WaS (61.5%), 72 MSR (37%), three SRS (1.5%), and six others refused MSR and were lost to follow-up (LFU). Within 1 year, 95 (47%) VS were surgically removed; 17 (8.5%) were treated by SRS; and 35 (17.5%) were LFU. The proportions for SRS and LFU were virtually unchanged for the following years, and the proportion under MSR increased slightly within 3 years and then remained stable. Finally, at 12 years, 104 (51.5%) cases had been operated on, 21 (10.5%) treated by SRS, 23 (11.5%) still under WaS, and 53 (26.5%) LFU, which were mainly intracanalicular. The initially and subsequently operated cases presented similar hearing preservation rates and good facial nerve function outcomes., Conclusion: This longitudinal study of a large number of VS, which were diagnosed over a short period of time and followed for 12 years, provides new information on both the natural history of these benign tumors and individual patient concerns. This study recommends use of the WaS policy for small and mid-sized VS before active therapeutic decision making., Level of Evidence: 3 Laryngoscope, 131:E970-E976, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To examine the impact of treatment on disease-specific health-related quality of life (HRQOL) among patients with sporadic vestibular schwannoma (VS)., Study Design: National cross-sectional study., Setting: Tertiary referral center and the Acoustic Neuroma Association., Subjects and Methods: Adult patients with VS who completed the Penn Acoustic Neuroma Quality of Life (PANQOL) survey. Patients were classified by treatment at the most recent survey as microsurgery, radiosurgery, observation, or recently diagnosed (RD). PANQOL scores were compared among VS treatment groups and with nontumor controls using analysis of covariance., Results: A total of 1362 respondents with VS were analyzed. Total PANQOL scores differed significantly by treatment ( P = .024) with adjusted means of 60, 63, 65, and 62 for the microsurgery, radiosurgery, observation, and RD groups, respectively. Subdomain scores for facial function, balance, hearing loss, and pain differed significantly ( P < .05) by treatment. Comparisons that surpassed thresholds for clinical significance included microsurgery compared to radiosurgery and observation for facial function, microsurgery compared to observation and RD for hearing loss, and microsurgery compared to radiosurgery and observation for pain. Subdomain scores for anxiety, facial function, balance, hearing loss, energy, and pain and total PANQOL scores for nontumor controls were significantly higher (ie, more favorable) compared to the VS groups ( P < .05). General health scores did not differ significantly between the nontumor controls and the VS groups ( P = .19)., Conclusion: Differences in HRQOL among treatment modalities are small and variably exceed the minimal clinically important difference. The impact of a VS diagnosis on HRQOL supersedes that of the treatment type.

Objective: Management of small vestibular schwannomas has evolved to where observation with interval imaging is an accepted treatment strategy. Loss of residual hearing is a known complication of observation. Magnetic resonance imaging (MRI) may provide critical information to assist in determining which tumors are at highest risk of hearing loss. We wished to determine what effect fundal cap size and cochlear fluid-attenuated inversion recovery (FLAIR) signal had on the progression of hearing loss in a large cohort of observed subjects., Study Design: Retrospective chart review., Setting: Tertiary academic referral center., Patients: Three hundred ninety-three adults with a vestibular schwannoma who underwent expectant management with serial audiograms and MRI., Interventions: Audiogram and MRI., Main Outcome Measures: Hearing outcomes included pure-tone average and word discrimination score (WRS). Cochlear FLAIR signal was measured as a ratio between the affected and nonaffected cochlea. Cerebrospinal fluid fundal cap was measured from the most lateral aspect of the tumor to the fundus of the internal auditory canal., Results: An increased cochlear FLAIR ratio was associated with a worse initial WRS (p = 0.0001, β=-0.25). A multivariate regression analysis demonstrated the variables fundal cap and initial WRS to significantly predict change in WRS over time. The larger the fundal cap size, the smaller the change in the WRS (p = 0.047, β=-0.35)., Conclusions: Cerebrospinal fluid fundal cap size predicts the natural history of hearing in vestibular schwannoma patients. The presence of a smaller fundal cap is correlated with a greater risk of progression of hearing loss and should be a variable considered in the management of small vestibular schwannomas., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: Recently, treatment decision making for large vestibular schwannomas (VS) in patients with neurofibromatosis type 2 (NF2) has become increasingly challenging due to the availability of multiple therapeutic options including surgery, bevacizumab (an anti-VEGF), radiosurgery, and observation; and it often remains an arbitrary decision based on local practices without firm recommendations. Our objective is to discuss the multimodal treatment options for Koos IV VS in a national reference center for NF2., Study Design: Single-institution retrospective cohort study., Methods: All NF2 patients with Koos IV VS who visited our center, the National Reference Center for NF2 Rare Disease in Pitié-Salpétrière Hospital of Paris, between January 2016 and December 2018 were included. Clinical charts, radiology, operative reports, and audiograms were reviewed., Results: Among 54 NF2 patients with Koos IV VS (mean maximum extrameatal diameter: 34 mm; range:17-62 mm), 27 were operated on for 28 VS; 21 were treated with bevacizumab; and six were observed. In the surgical group, VS resections were gross total, near-total, subtotal, or partial in 32%, 25%, 32%, and 11%, respectively; and a good (House-Brackmann grades I-II) facial nerve function was achieved in 81.5% at 1 year. Hearing was preserved in 14%, 78%, and 66% of the surgical (n = 7), bevacizumab (n = 9), and observation (n = 3) patients, respectively., Conclusion: All therapeutic options, including surgery and/or bevacizumab and occasionally observation, should be proposed to NF2 patients with large VS in the setting of dedicated centers. A decision-making tree is proposed for Koos IV VS management based on tumor evolution, hearing and clinical status of the patient, and contralateral VS size., Level of Evidence: 4, case series study, historically controlled study Laryngoscope, 131:E98-E107, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Vestibular schwannoma (VS) is a Schwann cell-derived tumour arising from the vestibulocochlear nerve. Although benign, it represents a threat to intracranial structures due to mass effect and carries a small risk of malignant transformation. VS therefore represents an important healthcare burden. We review the literature regarding pathogenesis, risk factors, and diagnosis of VS. The current and future potential management strategies are also discussed. A narrative review of all relevant papers known to the authors was conducted. The majority of VS remain clinically stable and do not require interventional procedures. Nevertheless, various surgical techniques exist for removing VS, the most common of which are translabyrinthine and retrosigmoid approaches. Due to surgical risks such as hearing loss, facial nerve dysfunction, post-operative headache, and cerebrospinal fluid leakage, a "watch and rescan" approach is adopted for most patients. Radiotherapy is a useful alternative and has been shown to have a similar response for growth restriction. Due to the heterogeneous nature of VS, there is a lack of consensus regarding management of tumours that are too large for conservative management but too small to indicate surgery. Emerging biologic therapies, such as Bevacizumab, Everolimus, and Lapatinib, as well as anti-inflammatories like aspirin are promising potential treatments; however, long-term evidence of their efficacy is required. The knowledge base regarding VS continues to improve. With increased understanding of the pathogenesis of these tumors, we believe future work should focus on pharmacologic intervention. Biologic therapies aimed toward improved patient outcomes are particularly promising.

Objective: To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma., Study Design: Modified Delphi method., Methods: The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round., Results: Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus., Conclusion: This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

Acoustic Neuromas (AN) are benign tumors of the vestibulocochlear nerve with symptomatology that includes unilateral sensorineural hearing loss, tinnitus, dizziness, facial and/or trigeminal neuropathy. There are different treatment options of AN: watchful waiting, microsurgical resection, stereotactic radiation and the choice depends by many variables such age, health and hearing of patients as size, location and growing status of tumor. The objective of this retrospective study is to better understand the differences in demographic, hearing status, symptoms, tumor characteristics in patients affected by AN presenting at our clinic and analyze the factors that influence the therapeutic choice. One-hundred three patients affected by AN were included in the study. All subjects underwent a detailed clinical interview and audio-vestibular examination, and Magnetic Resonance Imaging (MRI). Tumor status, growing or stable was estimated comparing new size to any previous MRI with at least a 6-month interval. Descriptive statistics were used for clinical and demographic features of patients. Therapeutic choices related to subjective symptoms were assessed with the chi-square test. Treatment options in our sample included watchful waiting, surgical resection and stereotactic radiosurgery. Overall, 17 patients (16.5%) pursued surgical resection via the retrosigmoid approach, 3 patients (2.9%) were treated with gamma knife stereotactic radiosurgery and 83 patients (80.6%) underwent watchful waiting. The decision-making process for AN treatment was based on size of tumor, age, and hearing loss; a statistically significant difference was found at Z test about size of tumor and PTA of patients that underwent retrosigmoid surgery. No statistically significant difference was found at chi-square test between the type of treatment and symptoms (p=0.719). The analysis of the data showed that the main elements taken into consideration for surgery were the size of tumor (p<0.000004) and, secondly, the PTA threshold; the latter may be due to the fact that patients with bigger tumors had greater hearing impairment (p<0.001). Disease progression influenced the therapeutic decision making with a positive correlation between tumor progression and surgery (p<0.001). In our sample, active surveillance was the most adopted option for small tumor, slow growth and old age. Microsurgical resection was the preferred treatment in patients with large tumors, hearing deterioration and rapid growth. Stereotactic radiation has been proposed in a few cases of elderly patients with slow growing tumor and mild hearing loss.

Introduction: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI)., Methods: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events., Results: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter., Conclusions: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

Background: Facial paresis is one of the complications after treatment for vestibular schwannoma (VS). Acupuncture has been used for Bell palsy but not in iatrogenic facial paresis. The objective of this study is to measure the efficacy of using acupuncture for iatrogenic facial nerve palsy and patients' satisfaction., Methods: This is a single-center retrospective study with patients from 2007-2019 received treatment for newly diagnosed or recurrent VS. Some patients who suffered facial paresis after surgery had self-initiated acupuncture. All patients who had facial paresis were included. Their facial nerve status before and immediately after surgery, postoperative 6 months and 12 months, were recorded. Those who received acupuncture also answered 6- and 12-month patient satisfaction surveys over the phone. Adverse effects were also assessed., Results: There were 123 patients in this period. Of these, 29 patients had iatrogenic facial paresis and 23 of them received acupuncture. There was significant improvement of facial paresis for the acupuncture group compared with the nonacupuncture group at 6 and 12 months. More than 80% of patients who received acupuncture were satisfied. They had motor improvement and experienced less pain and tightness. No adverse effects were reported., Conclusions: Acupuncture for postresection VS facial paresis seemed to speed up its recovery. Both patients' recovery and satisfaction were good after acupuncture, and it seemed to be a safe procedure in trained hands., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: To outline a possible decision-making process for sporadic vestibular schwannoma (VS) with contralateral nonserviceable hearing at diagnosis., Study Design: Retrospective case series., Methods: Diagnosed VS was studied in a tertiary referral center from 1995 to 2013., Results: Twenty-eight patients were included, with a mean follow-up of 6.9 years (range = 0.5-20 years). Ten were stage 1, 10 were stage 2, five were stage 3, and three were stage 4. Ipsilateral hearing levels were A (n = 3), B (n = 10), C (n = 7) and D (n = 8) American Academy of Otolaryngology-Head and Neck Surgery classification. Contralateral hearing levels were C (n = 11) and D (n = 17). Initial VS management included surveillance (n = 12) or surgery (n = 16), and four patients were later treated with surgery (n = 3) or stereotactic irradiation (n = 1). Hearing was improved by hearing aids and/or etiological treatment (n = 8), restored by contralateral (n = 15) or ipsilateral (n = 4) cochlear implants (CIs), or ipsilateral auditory brainstem implants (ABIs) (n = 3). Finally, 18 CIs were active daily; 14 of them presented high or moderate benefit with mean open-set dissyllabic word scores (WRS) of 58.1% and sentence recognition scores (SRS) of 69.7%, but only one ABI was still active (WRS of 70% and SRS of 87% with lip reading)., Conclusions: When early removal of VS was not necessary, contralateral CI or etiological treatment for hearing loss might be recommended initially. Ipsilateral CI is proposed, whereas VS should be operated on if previous hearing restoration was not successful. ABI should be reserved for the rare cases where a contralateral CI could not be implanted or the cochlear nerve was sectioned during VS removal., Level of Evidence: 3 Laryngoscope, 130:E407-E415, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To explore patient demographics as to predicting vestibular schwannoma (VS) size and treatment plan within a single institution., Methods: Using a large tertiary referral skull base center database, all patients with sporadic VS who presented to the center between 2009 and 2018 were reviewed., Results: A total of 816 patients with VS over 18 years of age were included. The median age was 56.8 years (range: 18.6-90.9 years). The median tumor diameter at diagnosis was 11.9 mm (range: 0.6-51.1 mm). With multivariate analysis, older age was associated with decreased tumor size (0.23 mm, 95% confidence interval [CI]: 0.17-0.29), whereas married patients had larger tumors (2.5 mm, 95% CI: 0.92-4.09). When comparing observation, radiation, or surgery, older patients are more likely to pursue observation as compared with surgery and radiation (odds ratio [OR]: 1.08, 95% CI: 1.06-1.10 and OR: 1.20, 95% CI: 1.08-1.33), respectively. Married patients were less likely to pursue observation as compared with surgery (OR: 0.49, 95% CI: 0.29-0.82). Each additional mile a patient lives farther from the center increases his or her odds of pursuing treatment (OR: 1.002, 95% CI: 1.001-1.003)., Conclusions: Older age is associated with smaller tumors, whereas married patients have larger tumors at diagnosis as compared with nonmarried patients. Furthermore, married patients are more likely to pursue treatment, specifically surgery, as compared with nonmarried patients, whereas patients who live farther from the center are more likely to pursue treatment., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Dizziness is a major contributing factor to poor quality of life for patients with vestibular schwannoma (acoustic neuroma). We wished to review the literature on interventions for balance dysfunction in these patients., Data Sources: A systematic literature review was performed identifying studies that measured balance function before and after treatment for vestibular schwannoma. Data sources include Medline (1950-present), EMBASE (1974-present), Cochrane Library (issue 3, 2008), NHS Centre of reviews and dissemination, Clinical Evidence, Cochrane central register of controlled trial, and CINAHL., Study Selection: A minimum follow-up of 6 months was required, to estimate long-term balance function. Eight articles were identified, including five studies with surgical intervention, two studies with stereotactic radiotherapy, and one comparing the two. Study design was generally poor with a high risk of bias. These studies all utilized the Dizziness Handicap Inventory (DHI) as a measure of pre- and postintervention balance function., Data Extraction: Results showed that overall DHI scores are not statistically affected by intervention irrespective of modality (surgery or stereotactic radiotherapy). Patients selected with severe dizziness, who undergo surgery, improved postoperatively. No other studies for severe dizziness were noted for comparison., Conclusions: Age, sex, and tumor size have no statistically significant effect on DHI outcomes, and no evidence to suggest which treatment modality has better dizziness related outcomes.No specific treatment modality was superior in terms of long-term balance function. Patients with severe dizziness may benefit from surgery, although no comparator studies were identified.

Objective: To determine the current epidemiology and management trends for patients with vestibular schwannomas (VS)., Study Design: Retrospective cohort study., Setting: The Surveillance, Epidemiology, and End Results (SEER) tumor registry., Patients: The SEER database was queried to identify patients diagnosed with VS from 1973 to 2015. Demographics, patient and tumor characteristics, and treatment methods were analyzed., Results: A total of 14,507 patients with VS were identified. The mean age at diagnosis was 55 ± 14.9 years. Age-adjusted incidence from 2006 to 2015 was 1.4 per 100,000 per year and remained relatively stable. Incidence across age varied with sex, as younger women and older men had increased incidences comparatively. A higher percentage of patients underwent surgery alone (43%), followed by observation (32%), radiation alone (23%), and combined radiation and surgery (2%). Age 65 and older was associated with observation (odds ratio [OR] 1.417; p = 0.029) whereas age 20 to 39 and 40 to 49 were associated with surgery (OR 2.013 and 1.935; p < 0.001). Older age was associated with radiation. Larger tumor size was associated with surgery and combined treatment. African American patients and American Indian or Alaskan Native patients were more likely to undergo observation than surgery., Conclusions: The overall incidence of VS is 1.4 per 100,000 per year and has remained relatively stable. There is a trend toward more conservative management with observation, which may be secondary to earlier diagnosis given widespread use of magnetic resonance imaging. Further studies are necessary to investigate differences in disease patterns and disparities in management.

Objectives: To assess the current state of the diagnosis and management of vestibular schwannoma (VS) as well as treatment trends, and to evaluate the role of treatment setting and various specialists in treatment plan., Methods: Patients diagnosed with VS completed a voluntary and anonymous survey. The questionnaires were distributed through Acoustic Neuroma Association website, Facebook page, and e-mail newsletters from January to March 2017., Results: In total, 789 VS patients completed the survey. Of those, 414 (52%) underwent surgery; 224 (28%) underwent radiotherapy; and 121 (15%) were observed. General otolaryngologists diagnosed 62% of responders, followed by primary care (11%) and neurotologists (10%). Patients who underwent surgery were significantly younger and had larger tumors compared to those treated with radiation or observation. The ratio of patients having nonsurgical versus surgical resection changed from 1:2 to 1:1 for the periods of 1979 through 2006 versus 2007 through 2017, respectively. Neurosurgeons (40%) and neurotologists (38%) were the most influential in treatment discussion. Neurotologists (P < 0.001) and general otolaryngologists (P = 0.04) were more influential than neurosurgeons for the decision process in patients with smaller tumors. Patients treated at academic versus nonacademic private institutions reported similar tumor sizes (P = 0.27), treatment decisions (P = 0.09), and decision satisfaction (P = 0.78)., Conclusion: There is a continuing trend toward nonsurgical management, with approximately half of the patients opting for nonsurgical management. In this cohort, the patients commonly presented with otologic symptoms and otolaryngologists made the most diagnoses. Neurotologists and neurosurgeons were the most influential in treatment discussion., Level of Evidence: NA Laryngoscope, 130:482-486, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

The level of evidence to provide treatment recommendations for vestibular schwannoma is low compared with other intracranial neoplasms. Therefore, the vestibular schwannoma task force of the European Association of Neuro-Oncology assessed the data available in the literature and composed a set of recommendations for health care professionals. The radiological diagnosis of vestibular schwannoma is made by magnetic resonance imaging. Histological verification of the diagnosis is not always required. Current treatment options include observation, surgical resection, fractionated radiotherapy, and radiosurgery. The choice of treatment depends on clinical presentation, tumor size, and expertise of the treating center. In small tumors, observation has to be weighed against radiosurgery, in large tumors surgical decompression is mandatory, potentially followed by fractionated radiotherapy or radiosurgery. Except for bevacizumab in neurofibromatosis type 2, there is no role for pharmacotherapy., (© The Author(s) 2019. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: We sought to compare balance outcomes according to treatment modality of vestibular schwannoma (VS) via a meta-analysis that divided measuring tools of balance outcomes into three categories based on type., Methods: A comprehensive review of the literature from January 1966 to September 2017 was performed, looking for studies about long-term balance outcomes after microsurgery (MS), radiotherapy (RT), or observation for VS. A comprehensive meta-analysis was used to analyze effect sizes, explore possible causes of heterogeneity, and check publication bias with a funnel plot and Egger's regression., Results: Among 633 references, 34 were included in the meta-analysis. Perceived dizziness improvement rate was significantly higher in the MS group than in the RT group (odds ratio [OR]: 1.61; 95% confidence interval [CI]: 1.08 to 2.40; P < .05, I 2 = 4.18], but no significant difference was observed between the two groups with regard to validated dizziness questionnaire score (standardized mean difference: 0.04; 95% CI: -0.36 to 0.44; P = .84, I 2 = 69.61) or dizziness or disequilibrium-related symptom incidence rate (OR: 0.91; 95% CI: 0.50 to 1.68; P = .77, I 2 = 0). In a subanalysis conducted within the groups after intervention, the MS group demonstrated a lower vertigo incidence rate (P < .001), and the RT group experienced a significant reduction in validated dizziness questionnaire score (P < .05)., Conclusions: Our results indicate that MS should be considered at least equal to RT in regard to resolving long-term dizziness and improving balance outcomes. Furthermore, well-designed studies are necessary to predict balance outcomes after VS treatment and to choose from among possible treatment options., Level of Evidence: 2a Laryngoscope, 130:178-189, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Vestibular schwannomas (VS) are rare benign tumours of the vestibular nerve that cause hearing loss. Management strategies include watchful waiting, radiotherapy or surgical resection. Historically, the presence of retrocochlear disease has been considered to be a contra-indication to cochlear implantation (CI). The aim of this systematic review is to assess hearing rehabilitation outcomes for CI recipients with VS, either sporadic or associated with neurofibromatosis type 2, whose tumours have been managed with either observation or radiotherapy. Methods: PubMed, Embase, and Cochrane Library databases were searched from inception through to November 2018. 50 cases from 12 studies met the inclusion criteria. Patient demographics, VS characteristics, management strategy, pre-CI hearing status, electrical promontory stimulation testing, post-CI hearing status and speech perception scores, functional benefits and follow-up length are reported. Results: Radiotherapy and observation groups had similar patient demographics in terms of age at CI, tumour size and duration of deafness. Following CI, 64% and 60% of patients in the radiotherapy and observation groups achieved open-set speech perception, respectively. Pure tone average thresholds (33 vs. 39 dB) and speech scores were also comparable between both groups. Conclusion: Ipsilateral CI in patients with VS that have not been surgically resected can provide beneficial hearing rehabilitation outcomes.