OBJECTIVE:To evaluate the diagnostic accuracy of non-imaging screening tools used to guide magnetic resonance imaging (MRI) referral in adults with asymmetric sensorineural hearing loss undergoing investigation for suspected vestibular schwannoma (VS). DATABASES REVIEWED:Medline (via Ovid; 1946 to August 19, 2025), PubMed, Embase, Cochrane Library, and Scopus. MATERIALS AND METHODS:A PRISMA-compliant systematic review (PROSPERO CRD420251131909) included prospective and retrospective cohort and case-control studies assessing clinical or audiological screening criteria against MRI in adults (above 16 y) investigated for possible VS. Primary outcomes were sensitivity and specificity. Two reviewers independently screened studies, extracted data, and assessed study quality using the Newcastle-Ottawa Scale. Due to clinical and methodological heterogeneity, results were synthesized narratively without meta-analysis. RESULTS:Twenty studies comprising 9119 patients (1169 VS cases) were included. Pure tone audiometry thresholds were most frequently evaluated (10 studies), followed by auditory brainstem response (ABR; 7 studies), vestibular testing (2 studies), and audiometric configuration (1 study). Audiometric threshold demonstrated an inverse relationship between sensitivity and specificity: lower thresholds achieved sensitivities approaching 100% but markedly reduced specificity. Stricter criteria improved specificity but often reduced sensitivity below the 90% threshold considered acceptable for screening. ABR showed high sensitivity in some cohorts but reduced detection of tumors ≤10 mm. Caloric and hyperventilation testing demonstrated variable sensitivity, and audiometric configuration showed poor sensitivity. Most studies were retrospective, with heterogeneity in thresholds and risk-of-bias assessments limiting comparability. CONCLUSIONS:No screening test consistently achieved high sensitivity and specificity for VS detection. Audiometric protocols remain the best first-line screening tools, though prospective validation is required to maximize diagnostic yield while minimizing unnecessary MRI utilization.

The auditory brainstem implant (ABI) is a surgically implanted neuroprosthetic that may be used in the management of profound hearing loss in paediatric patients that are ineligible for cochlear implantation, primarily due to anatomical constraints. We describe our technique for the implantation of a paediatric ABI. Surgical steps include post auricular incision, the creation of a periosteal flap, subperiosteal pocket and bony recess for the device, retrosigmoid craniotomy, placement of the electrodes adjacent to the cochlear nucleus and multi-layer closure. We highlight novel technical modifications to reduce the risk of complications and optimise outcomes. Paediatric auditory brainstem implants offer the possibility of sound awareness to patients with profound hearing loss that would not benefit from cochlear implantation or other hearing aid solutions.

Aims: Macroscopic "gross total resection" was the traditional goal for patients undergoing resection of vestibular schwannoma. There has been a shift toward "subtotal" resection in an attempt to mitigate the risk of cranial nerve injury, reserving radiotherapy and/or revision surgery for growing residual tumors. The decision for subtotal versus total resection may be made preoperatively. Additionally, "near-total" resection has been acknowledged as microscopic residual disease, the decision of which is often made intraoperatively when cranial nerve risk is encountered. In recent years, some centers have advocated for planned subtotal resection, citing overall lower risks of facial palsy; however, to date, no robust comparison of facial nerve outcomes has been performed. This study reviews a United Kingdom national, multicenter registry to explore the demographic and disease features of extent of resection and compare cranial nerve outcomes.

Importance:In the literature, there is a lack of data reporting tumor control rates after radiotherapy in actively growing vestibular schwannomas (VS). Data for this rarely studied population are needed. Objective:To estimate tumor control rates in radiologically growing VS treated with first-line radiotherapy. Design, Setting, and Participants:This international, multicenter cohort study used prospectively collected data from patients with growing unilateral VS treated first-line with radiotherapy between January 2000 and December 2023 from 8 tertiary referral skull base units. The data were analyzed in June 2025. Exposures:Radiotherapy as an initial treatment for VS. Main Outcomes and Measures:The primary outcome was treatment failure, ie VS growth postradiotherapy, which was predefined as an increase in maximum intracranial tumor diameter (ICTD) of 3 mm or greater within the first 2 years after radiotherapy or 2 mm or greater thereafter. Secondary outcomes were treatment failure based on different definitions of VS growth: (1) an increase in ICTD of 2 mm or greater, (2) an increase in ICTD of 3 mm or greater, and (3) conversion to surgery. Results:A total of 1883 patients (975 female individuals [51.8%]; median age at diagnosis, 63 years [IQR, 53-71 years]) were included in the study. Using the primary definition of treatment failure (an increase in ICTD of ≥3 mm within the first 2 years postradiotherapy or ≥2 mm thereafter), the Kaplan-Meier estimate yielded a 10-year tumor control rate of 76.1% (95% CI, 72.7%-79.2%). For secondary outcome definitions, 10-year tumor control rates were 60.1% (95% CI, 57.5%-64.3%) for an ICTD increase of 2 mm or greater, 78.3% (95% CI, 75.0%-81.2%) for an increase of 3 mm or greater, and 92.6% (95% CI, 90.4%-94.3%) for conversion to surgery. Neither pretreatment tumor size nor tumor location (intracanalicular vs extracanalicular) were significantly associated with treatment failure. Conclusions and Relevance:The results of this cohort study provide tumor control outcomes for radiologically growing VS treated with radiotherapy using several clinically relevant definitions of growth. By focusing exclusively on this rarely isolated subgroup, the findings offer targeted data to potentially inform treatment expectations and future research.

OBJECTIVE:Translabyrinthine excision of a vestibular schwannoma is associated with acute vestibular failure. Preoperative intratympanic gentamicin (ITG) injections can improve objective balance function after surgery but its clinical benefits remain to be established. METHODS:Adult patients undergoing translabyrinthine removal of a vestibular schwannoma between January 2014 and February 2018 underwent preoperative vestibular function testing. Patients were divided in to 3 groups, those with vestibular function (VF) who received ITG injections, those with VF but did not receive ITG and those with no VF. Groups were compared according to degree of vertigo, length of stay, time to unassisted mobilization, and postoperative anti-emetic consumption. RESULTS:Forty six patients had ITG injections (Group 1), 7 had residual VF but refused treatment (Group 2), 21 had no VF (Group 3). Group 1 had a significant improvement in vertigo over time whereas groups 2 and 3 did not. There was a statistically significant 70% decrease in time to independent mobilization between Group 1 and other groups and a 19% decrease in length of stay in Group 1 compared to other groups although this did not reach statistical significance. Two patients had injection-related complications. Group 1 used less anti-emetics than other groups but this was not statistically significant. CONCLUSION:Preoperative intratympanic gentamicin injection with vestibular rehabilitation exercises is associated with less postoperative vertigo and earlier postoperative mobilization. There was reduced duration of hospitalization and decreased consumption of anti-emetic but not significantly so possibly because of low numbers of patients in the no treatment group. LEVEL OF EVIDENCE:2 Laryngoscope, 134:3316-3322, 2024.

Background Evidence on hearing outcome measures when assessing hearing preservation following stereotactic radiosurgery (SRS) for adults with vestibular schwannoma (VS) has not previously been collated in a structured review.Objective The objective of the present study was to perform a scoping review of the evidence regarding the choice of hearing outcomes and other methodological characteristics following SRS for adults with VS.Methods The protocol was registered in the International Platform of Registered Systematic Review and Meta-Analysis Protocols (INPLASY) and reported according to the Preferred Reporting Items for Systematic Review and Meta-Analyses extension guidelines for scoping reviews. A systematic search of five online databases revealed 1,591 studies, 247 of which met the inclusion criteria.Results The majority of studies ( n = 213, 86%) were retrospective cohort or case series with the remainder ( n = 34, 14%) prospective cohort. Pure-tone audiometry and speech intelligibility were included in 222 (90%) and 158 (64%) studies, respectively, often summarized within a classification scheme and lacking procedural details. Fifty-nine (24%) studies included self-report measures. The median duration of follow-up, when reported, was 43 months (interquartile range: 29, 4-150).Conclusion Evidence on hearing disability after SRS for VS is based on low-quality studies which are inherently susceptible to bias. This review has highlighted an urgent need for a randomized controlled trial assessing hearing outcomes in patients with VS managed with radiosurgery or radiological observation. Similarly, consensus and coproduction of a core outcome set to determine relevant hearing and communication outcome domains is required. This will ensure that patient priorities, including communication abilities in the presence of background noise and reduced participation restrictions, are addressed.

Objectives: This study aims to synthesize current knowledge and outcomes related to pediatric auditory brainstem implantation (ABI) in children with severe inner ear malformations (IEMs). It highlights the clinical management practices, challenges, and potential future directions for consensus development in this field. Methods: A systematic review of findings presented at the Third International Pediatric ABI Symposium organized by the Hacettepe Cochlear Implant team between 3 and 5 September 2020 was conducted, incorporating data from 41 departments across 19 countries. Relevant clinical outcomes, imaging techniques, surgical approaches, and rehabilitation strategies were analyzed to identify key trends and variability in practices. Results: The review indicates that children receiving ABIs exhibit diverse auditory outcomes influenced by individual anatomical variations and developmental factors. Early implantation, particularly before the age of three, positively correlates with better auditory and language development. Multicenter experiences underscore the necessity of tailored decision-making, which considers both surgical candidacy and comprehensive rehabilitation resources. Discussion:: The variability in outcomes emphasizes the need for improved consensus and guidelines regarding eligibility, surgical techniques, and multidisciplinary rehabilitation approaches. Notable complications and the necessity for thorough imaging assessments were also identified as critical components affecting clinical decisions. Conclusion: A formal consensus statement is warranted to standardize best practices in ABI management. This will not only enhance patient outcomes but also guide future research efforts to address the remaining challenges in the treatment of children with severe IEMs. Enhanced collaboration among team members will be pivotal in achieving these objectives.

The objective of this consensus is to provide otolaryngologists with appropriate strategies in the management of external auditory canal (EAC) carcinoma. In the absence of randomized controlled trials, the consensus is based on expert opinions utilizing the Rand/UCLA appropriateness method [Fitch and Aguilar in The RAND/UCLA appropriateness method user's manual, RAND Corporation, Santa Monica, CA, 2001], drawing from existing literature and clinical experience. The management recommendations are structured around 12 key areas, including: definition and pathology, pathogenesis, clinical manifestations, work-up, tumor staging system, surgical management of primary tumor, surgical management of the parotid gland and the temporomandibular joint, lymph node metastasis, radiotherapy, chemotherapy, reconstruction, and follow-up. Management strategies for EAC carcinoma rely on tumor extension and histopathological features. Surgical removal with free surgical margins or combination with radiotherapy, chemotherapy are most often the best options. Given the rarity of the disease, prospective, randomized, multi-institutional clinical trials should be designed to enable reliable comparisons of the outcomes of EAC carcinoma treatments, thereby providing evidence-based clinical data to establish widely accepted guidelines. It emphasizes the need for a multidisciplinary team to be involved in the management of EAC carcinoma, and regular follow-up should be implemented postoperatively.

ObjectiveTo compare cochlear implant (CI) speech perception outcomes in patients with sporadic vestibular schwannoma (VS) managed with observation, radiosurgery, or microsurgery.Study DesignRetrospective review.SettingEleven tertiary academic medical centers.PatientsOne hundred patients with sporadic VS who received an ipsilateral CI.InterventionsIpsilateral cochlear implantation.Main Outcome MeasuresPure-tone thresholds, monosyllabic speech perception testing scores, and rates of open-set speech acquisition.ResultsOf the 100 patients studied, 54 underwent microsurgery, 26 underwent radiosurgery, 19 continued observation, and 1 underwent multimodal therapy. Among all patients, the median post-implantation pure-tone average was 31 dB (interquartile range [IQR] 25-39 dB) and the median monosyllabic speech perception score was 30% (IQR 0-60%) at a median of 12 months (IQR 5-25 months) post-implantation. Patients who were managed with microsurgery (median speech perception score 11%, IQR 0-52%) exhibited poorer implant outcomes overall compared with those managed with observation (median speech perception score 52%, IQR 40-72%) or radiosurgery (median speech perception score 30%, IQR 16-60%). Open-set speech perception was achieved in 61% of patients managed with microsurgery, 100% with observation, and 80% with radiosurgery. In a multivariable setting, those managed with observation (p = 0.02) or who underwent radiosurgery (p = 0.04) were significantly more likely to achieve open-set speech perception compared with patients who underwent microsurgery.ConclusionsCochlear implants offer benefit in selected patients with sporadic VS. Although achieved in over half of people after microsurgery, open-set speech perception is more reliably attained in patients who are treated with observation or radiosurgery compared with microsurgical resection. These data may inform patient counseling and VS tumor management in people who may benefit from implantation.

Objectives To assess how otological surgery resumed following the UK’s first wave of the COVID-19 pandemic and what challenges clinicians faced when operating. Design A multi-centre prospective audit of elective and emergency otological surgery Participants 1130 cases from 79 hospital sites across the UK (excluding Northern Ireland). Data was collected over three, 4-week audit periods from 15th June to 6th September 2020. Main outcome measures Which operations were being performed in England, Scotland and Wales? What was the use of personal protective equipment (PPE)? Were there a greater number of complications? What was the level of trainee participation? Did any patients or patients contract COVID-19? Results 85.8% of operations took place in England. 69.1% of operations were middle ear procedures and 58% of patients were adults. 83.2% were Caucasian and 93.9% of patients had minimal co-morbidities (ASA 1 or 2). 91.1% were for tested SARS-CoV-2 pre-operatively, none of whom tested positive. 70.4% isolated for 7-14 days pre-operatively. 28.2% of surgeons wore full personal protective equipment (PPE), compared with 66.6% of anaesthetists and 68.2% of scrub staff. Trainees were present in 80.3% of cases. Complications were reported in 4% of cases; surgical site infection was the most common. No patients or staff contracted SARS-CoV-2 during the audit. Conclusions Restarting otology surgery after the first wave of the SARS-CoV-2 pandemic was performed safely across the UK, with no increase in complication rates or SARS-CoV-2 transmission. However, there were challenges to operating with PPE and trainees have been affected by reduced exposure to surgical cases.

There is no guidance surrounding postoperative venous thromboembolism (VTE) prophylaxis using pharmacological agents (chemoprophylaxis) in patients undergoing skull base surgery. The aim of this study was to compare VTE and intracranial haematoma rates after skull base surgery in patients treated with/without chemoprophylaxis. Review of prospective quaternary centre database including adults undergoing first-time skull base surgery (2009–2020). VTE was defined as deep vein thrombosis (DVT) and pulmonary embolism (PE) within 6 months of surgery. Multivariate logistic regression was used to determine factors predictive of postoperative intracranial haematoma/VTE. Propensity score matching (PSM) was used in group comparisons. One thousand five hundred fifty-one patients were included with a median age of 52 years (range 16–89 years) and female predominance (62

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.

ObjectivesNew diagnostic criteria for NF2-related schwannomatosis (NF2) were published in 2022. An updated UK prevalence was generated in accordance with these, with an emphasis on the rate of de novo NF2 (a 50% frequency is widely quoted in genetic counselling). The distribution of variant types among de novo and familial NF2 cases was also assessed.MethodsThe UK National NF2 database identifies patients meeting updated NF2 criteria from a highly ascertained population cared for by England’s specialised service. Diagnostic prevalence was assessed on 1 February 2023. Molecular analysis of blood and, where possible, tumour specimens forNF2, LZTR1andSMARCB1was performed.Results1084 living NF2 patients were identified on prevalence day (equivalent to 1 in 61 332). The proportion with NF2 inherited from an affected parent was only 23% in England. If people without a confirmed molecular diagnosis or bilateral vestibular schwannoma are excluded, the frequency of de novo NF2 remains high (72%). Of the identified de novo cases, almost half were mosaic. The most common variant type was nonsense variants, accounting for 173/697 (24.8%) of people with an established variant, but only 18/235 (7.7%) with an inheritedNF2pathogenic variant (p<0.0001). Missense variants had the highest proportion of familial association (56%). The prevalence ofLZTR1-related schwannomatosis andSMARCB1-related schwannomatosis was 1 in 527 000 and 1 in 1.1M, respectively, 8.4–18.4 times lower than NF2.ConclusionsThis work confirms a much higher rate of de novo NF2 than previously reported and highlights the benefits of maintaining patient databases for accurate counselling.

Objective:Surveillance plays a crucial role in managing patients with vestibular schwannomas (VSs). Consensus is lacking on the optimal duration, frequency, and modality of imaging. Standardizing this approach would ensure safe and effective care, reduce patient distress, and promote consistency in management decisions among clinicians. Methods:In July 2022, a multi-disciplinary Delphi consensus was conducted at the British Skull Base Society Meeting. Expert United Kingdom-based skull-base surgeons and neuroradiologists were presented semi-systematic literature reviews summarizing current evidence on VS management. Anonymized opinions were collated and discussed to reach a majority vote, which was deemed the final consensus opinion. Results:Recommendations for VS managed by surveillance are: (1) surveillance frequency should decrease over time; (2) surveillance may be discontinued when the remaining lifetime risk of VS growth is <0.5% (∼8.5 years); (3) factors such as age, VS size, VS location, and cystic components should be considered. Surveillance after surgery or radiotherapy has limited evidence but recommendations are: (4) surveillance should be adjusted based on residual tumor size or nodular enhancement. Imaging modality and sequences are recommended to be (5) high-resolution magnetic resonance imaging with T1, T2, FIESTA/CISS multiplanar sequences, contrast is not required in untreated cases, and linear reporting of measurements is pragmatically sufficient. Conclusion:This consensus and literature review provides an evidence and expert opinion-based guide to help clinicians with the surveillance of patients with VS. Further research should focus on better understanding the dynamic nature of growth risks and the growth characteristics of postintervention VS to enable personalized growth risk stratification.