Carlson, Matthew L.

BACKGROUND: Surgical intervention remains an important option in the management of vestibular schwannoma (VSs). Development of a systematic approach to choose the most appropriate route for this intervention, based on existing published evidence, is an important goal. OBJECTIVE: To review the literature published since the 2018 Congress of Neurological Surgeons Guideline on surgical intervention for patients with sporadic VSs and use this information to update that set of recommendations. METHODS: The literature in the PubMed and MEDLINE databases from January 2015 through May 20, 2022, was searched for manuscripts pertaining to surgical intervention for VSs. Those manuscripts meeting inclusion criteria were then analyzed for creation of recommendations in response to a set of updated questions. RESULTS: The resultant findings included a considerable amount of data that did not alter the recommendations form the 2018 publication on this topic. Thus, recommendations stating hearing preservation surgery through the middle fossa or retrosigmoid approach may be considered in individuals with good preoperative hearing as an alternative to simple observation remain. In addition, if microsurgical resection is necessary after stereotactic radiosurgery, it is recommended that patients be counseled that there is an increased likelihood of a subtotal resection and decreased facial nerve function. In some questions, insufficient data were present to create an answer and that is stated. CONCLUSION: This guideline demonstrates surgical intervention for VSs and represents a range of options, and the choice of the intervention depends on the specific aspects of the lesion and the individual that harbors them. Objective refinement of those choices will require thoughtful research design by investigations that wish to address those items for which we still have insufficient information. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/7-surgical-resection-treatment-of-patients-with-ve-2

BACKGROUND: Given the increasing prevalence and shifting disease demographic of vestibular schwannoma toward smaller tumors in people with less advanced symptoms, increasing emphasis has been placed on functional hearing preservation. OBJECTIVE: To provide an update to the 2018 CNS Guideline on hearing preservation outcomes in patients with sporadic vestibular schwannoma. METHODS: Systematic review and clinical practice guideline summarizing data pertaining to hearing preservation outcomes within the first 10 years after contemporary stereotactic radiation, microsurgery, or observation with serial imaging. The analysis incorporates studies included in the 2018 guideline and additional literature published up to May 20, 2022, providing a comprehensive up-to-date review of published clinical outcome data over time. RESULTS: Pooled estimated rates of serviceable hearing preservation are presented for observation, radiosurgery, and microsurgery for adults with sporadic vestibular schwannoma who have documented serviceable hearing in the ipsilateral ear at the time of diagnosis. Overall estimated rates of maintaining serviceable hearing are 78% at 2 years, 59% at 5 years, and 47% at 10 years during observation; 71% at 2 years, 59% at 5 years, and 38% at 10 years after radiosurgery; and 48% at 2 years, 40% at 5 years, and 32% at 10 years after microsurgery. In addition, features portending hearing outcome among management modalities are reported to guide accurate patient counseling. CONCLUSION: Regardless of treatment modality, fewer than half of patients with sporadic vestibular schwannoma who present with serviceable hearing will maintain useful hearing by 10 years. Across all studies, microsurgery and radiosurgery seem to accelerate this decline over the natural history, although further research is needed given limitations of available evidence. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/4-hearing-preservation-outcomes-in-patients-with-s

BACKGROUND: Intraoperative neuromonitoring (IONM) has become vital in the management of vestibular schwannoma (VS) with the paradigm shift from tumor eradication to functional preservation. Several facial nerve (FN) monitoring strategies have been explored over the past few decades ranging from free-running electromyography, direct nerve stimulation, continuous nerve stimulation, facial motor evoked potentials, blink reflex, and others. Hearing preservation surgery is guided primarily by far-field auditory brainstem response and real-time cochlear nerve action potentials. Given the heterogeneity in tumor and patient factors, it remains very difficult to accurately predict cranial nerve outcomes, regardless of the monitoring strategy. OBJECTIVE: To critically appraise literature regarding IONM during VS surgery and update the previous evidence-based clinical practice guideline. METHODS: This is a systematic review of the literature, incorporating articles from March 2015 to May 2022. Literature published before 2015 that would have been included in the previous Congress of Neurological Surgeons guideline was not searched again in this update. RESULTS: FN monitoring provides better functional outcomes compared with anatomic dissection alone and may guide the extent of tumor resection. While facial motor evoked potentials and free-running electromyography can provide continuous noninvasive FN monitoring, there are insufficient data to determine which more strongly correlates with facial function outcomes. Both electrophysiological data and tumor size correlate with facial function outcomes. The ideal hearing monitoring strategy remains unclear as there are insufficient data comparing cochlear nerve action potentials with far-field auditory brainstem response. All studies were graded as Class III evidence. CONCLUSION: IONM should be used in all VS cases. While the optimal FN and hearing monitoring strategy remains elusive, available data support the use of a combination of strategies, including preoperative tumor size, to maximize sensitivity and specificity. There remains a significant need for high-quality comparative studies to determine which intraoperative monitoring scheme can provide intraoperative guidance and predict postoperative outcome. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/3-intraoperative-cranial-nerve-monitoring-in-manag

Objective To describe the potential applications of awake cochlear implantation (CI). Patients Adult patients with sensorineural hearing loss who qualify for CI. Interventions CI under local anesthesia, without any form of systemic analgesia or anesthesia. Main Outcome Measures Acoustic hearing preservation, subjective patient report of cochlear implant pitch matching, and subjective patient report of cochlear implant sound quality. Results Four illustrative cases are presented, highlighting the potential uses of CI performed under local anesthesia for optimizing outcomes in hearing preservation, implant pitch matching, and implant sound quality. Conclusions CI under local anesthesia allows surgeons to use real-time patient feedback during electrode array insertion to minimize intracochlear trauma, particularly in cases where hearing preservation is a goal of CI, and to potentially improve implant performance by optimizing pitch matching and sound quality.

Objective To assess integration of a single-use robotic-assisted cochlear implant (RA-CI) electrode array insertion system into surgical workflows, identify potential challenges, and inform best practices. Study Design Survey. Setting Survey data from 16 surgeons across 10 US hospitals. Patients CI candidates 12 years and older with radiographically normal cochleae. Intervention CI surgery with single-use RA-CI insertion system during electrode array insertion. Main Outcome Measures Number of RA-CI to achieve proficiency, estimated additional time for RA-CI, and best practices to successfully incorporate the RA insertion system into CI surgery. Results A total of 121 RA-CI cases were performed. All surgeons completed the survey. Most surgeons (62.5%) reported they would feel comfortable using the device within five cases. Eight (50%) reported use of the RA system added 5 to 10 minutes to the case, and seven (44%) reported an added 10–15 minutes. Providing adequate incision size to secure the unit base superior to the temporal line and maximizing exposure of the facial recess were the most recommended best practices. Additional recommendations included pulling the receiver/stimulator forward within the pocket to facilitate a full electrode array insertion and considering drive head placement and mastoidectomy size to maximize overall visibility during electrode array insertion. Conclusions Proficiency with an RA electrode array insertion system during CI surgery can be achieved with minor adjustments to the surgical approach, within relatively few use cases (generally <5) and with minimal time addition (between 5 and 15 min). Training to specific best practices is important before initial clinical use.

Objective Present the rationale for the development of a new, patient-facing vital sign for adult hearing. Study Design Structured A3 process. Setting Fourteen virtual meetings and two in-person meetings held between May 2021 and June 2022. Main Outcome Measure(s) Identification and refinement of a countermeasure to develop and imbed a hearing health vital sign for adult hearing. Results Through a rigorous, structured A3 process, the Hearing Health Collaborative identified and refined the rationale for and the qualities associated with a public-facing vital sign for adult hearing loss. Specifically, such a vital sign must embody five qualities: simple, accessible, stable, inclusive, and meaningful. These qualities together would ensure that a hearing health vital sign would be useful not only as a screening tool but also as an instrument to guide next steps in workup and potentially treatment. Conclusions Successful identification and implementation of a hearing metric or “vital sign” for the presence of adult hearing loss, using precedent convention of other medical diseases, may help reframe public perceptions surrounding hearing loss, improve awareness and literacy regarding the presence of the disease, and prompt action to seek diagnostic assessment.

AbstractObjectiveTo examine associations with changes in quality‐of‐life (QOL) outcomes following treatment of vestibular schwannoma (VS) using stereotactic radiosurgery (SRS).Study DesignProspective longitudinal study.SettingTertiary academic center.MethodsPatients who were treated for sporadic VS using SRS from 2015 to 2022 were included. QOL outcomes were measured using the disease‐specific Penn Acoustic Neuroma QOL (PANQOL) scale.ResultsSeventy‐nine patients with pre‐SRS and at least one post‐SRS PANQOL assessments were available for analysis. The mean change in total PANQOL scores was −2 (SD 13) on a 100‐point scale. The mean duration between assessments was 4.6 years (SD 2.0). Age at SRS, sex, and SRS treatment doses were not significantly associated with changes in total PANQOL scores. Total PANQOL scores improved a mean of 4 points for patients with tumors confined to the internal auditory canal but declined a mean of 5 points for patients with tumors extending into the cerebellopontine angle (P = .01); however, these changes did not exceed the minimum clinically significant threshold of 11 points. The correlation coefficient between treated tumor volume at SRS and change in total PANQOL scores was −0.30 (P = .007). Changes in PANQOL total (P = .5) and hearing domain (P = .3) scores for patients who maintained serviceable hearing or progressed to nonserviceable hearing did not significantly differ.ConclusionTumor extent and treated volume at SRS had a statistically significant but likely not clinically important impact on total PANQOL scores. Progression to nonserviceable hearing did not have a significant impact on PANQOL total or hearing domain scores.

To address the limitations of existing instruments, the authors developed the Vestibular Schwannoma Quality of Life (VSQOL) Index. The Index captures the multifaceted impacts of vestibular schwannoma (VS) on daily life across eight categories: hearing problems; dizziness and imbalance; pain, discomfort, and tinnitus; problems with face or eyes; impact on physical, emotional, and social well-being; difficulty with thinking and memory; satisfaction or regret; and impact on employment. Initially developed in English, this digital supplement of the Journal of Neurosurgery presents translations of the VSQOL Index to Simplified Chinese, Spanish, Japanese, Arabic, French, and Norwegian. These translations aim to improve access to VS quality of life (QOL) research globally with enhanced racial, ethnic, and geographic representation. The goal is to comprehensively evaluate the impact of VS diagnosis and management on well-being and QOL by offering the VSQOL Index in patients’ native, or first-learned, languages. The English version and these translations may be used for noncommercial research, clinical, or educational purposes without written permission from the authors. Investigators interested in translating the VSQOL Index to other languages should contact the corresponding author for translation methodology instructions.

The authors developed a new disease-specific patient-reported outcome measure to capture the multifaceted impacts of vestibular schwannoma on daily life, the Vestibular Schwannoma Quality of Life (VSQOL) Index, which contains 40 items grouped into eight categories: hearing problems; dizziness and imbalance; pain, discomfort, and tinnitus; problems with face or eyes; impact on physical, emotional, and social well-being; difficulty with thinking and memory; satisfaction or regret; and impact on employment. 1 The VSQOL Index was developed in English, although the availability of this instrument in other languages can improve access to quality of life (QOL) research worldwide with enhanced racial, ethnic, and geographic representation. The authors partnered with FACITtrans, a component of the Functional Assessment of Chronic Illness Therapy (FACIT) group with more than 25 years of experience in translation, cultural adaptation, and linguistic validation of patient-reported outcome measures, to translate the VSQOL Index to Norwegian using a rigorous translation methodology. 2–5 The translation of the VSQOL Index presented at the end of this article may be used for noncommercial research, clinical, or educational purposes without seeking written permission from the authors. By providing a suite of translations in this digital supplement of the Journal of Neurosurgery , the authors hope to increase the reach and breadth of vestibular schwannoma QOL research and facilitate international collaborations.

The authors developed a new disease-specific patient-reported outcome measure to capture the multifaceted impacts of vestibular schwannoma on daily life, the Vestibular Schwannoma Quality of Life (VSQOL) Index, which contains 40 items grouped into eight categories: hearing problems; dizziness and imbalance; pain, discomfort, and tinnitus; problems with face or eyes; impact on physical, emotional, and social well-being; difficulty with thinking and memory; satisfaction or regret; and impact on employment. 1 The VSQOL Index was developed in English, although the availability of this instrument in other languages can improve access to quality of life (QOL) research worldwide with enhanced racial, ethnic, and geographic representation. The authors partnered with FACITtrans, a component of the Functional Assessment of Chronic Illness Therapy (FACIT) group with more than 25 years of experience in translation, cultural adaptation, and linguistic validation of patient-reported outcome measures, to translate the VSQOL Index to French using a rigorous translation methodology. 2–5 The translation of the VSQOL Index presented at the end of this article may be used for noncommercial research, clinical, or educational purposes without seeking written permission from the authors. By providing a suite of translations in this digital supplement of the Journal of Neurosurgery , the authors hope to increase the reach and breadth of QOL research and facilitate international collaborations.