Impact of Latency Variations on the Predictive Value of Facial Nerve Proximal-to-Distal Amplitude Ratio during Vestibular Schwannoma Surgery.

Introduction This study highlights the relation between compound muscle action potential (CMAP) latency variations and the predictive value of facial nerve (FN) proximal-to-distal (P/D) amplitude ratio measured at the end of vestibular schwannoma resection. Methods Forty-eight patients underwent FN stimulation at the brainstem (proximal) and internal acoustic meatus (distal) using a current intensity of 2 mA. The proximal latency and the P/D amplitude ratio were assessed. House-Brackmann grades I & II indicated good FN function, and grades III to VI were considered fair/poor function. A P/D amplitude ratio > 0.6 was used as a cutoff to indicate a good FN function, while a ratio of ≤ 0.6 indicated a fair/poor FN function. Results The P/D amplitude ratio was measured for all patients, and the calculated sensitivity (SE), specificity (SP), positive predictive value (PPV), and negative predictive value (NPV) were 85.2, 85.7, 88.5, and 81.8%, respectively. The CMAPs from the mentalis muscle were then classified based on their proximal latency into group I (< 6 ms), group II (6-8 ms), and group III (> 8 ms). The SE, SP, PPV, and NPV became 90.5, 90.9, 95, and 83.3%, respectively, in group II. In group I, SE and NPV increased, whereas SP and PPV decreased. While in group III, SP and PPV increased, whereas SE and NPV decreased. Conclusion At a latency between 6 and 8 ms, the P/D amplitude ratio was predictive of outcomes with high SE and SP. When latency was < 6 ms or > 8 ms, the same predictive ability was not observed. Knowing the strengths and limitations is important for understanding the predictive value of the P/D amplitude ratio.

From bench to bedside: Advancing towards therapeutic treatment of vestibular schwannomas.

Vestibular schwannomas are rare intracranial tumors originating from Schwann cells of the vestibular nerve. Despite their benign nature, these tumors can exert significant mass effects and debilitating symptoms, including gradual hearing loss, vertigo, facial nerve dysfunction, and headaches. Current clinical management options encompass wait-and-scan, surgery, radiation therapy, and off-label medication. However, each approach exhibits its own challenges and harbors limitations that underscore the urgent need for therapeutic treatments. Over the past 2 decades, extensive elucidation of the molecular underpinnings of vestibular schwannomas has unraveled genetic anomalies, dysregulated signaling pathways, downstream of receptor tyrosine kinases, disrupted extracellular matrix, inflammatory tumor microenvironment, and altered cerebrospinal fluid composition as integral factors in driving the development and progression of the disease. Armed with this knowledge, novel therapeutic interventions tailored to the unique molecular characteristics of those conditions are actively being pursued. This review underscores the urgency of addressing the dearth of Food and Drug Administration-approved drugs for vestibular schwannoma, highlighting the key molecular discoveries and their potential translation into therapeutics. It provides an in-depth exploration of the evolving landscape of therapeutic development, which is currently advancing from bench to bedside. These ongoing efforts hold the promise of significantly transforming the lives of vestibular schwannoma patients in the future.

Likelihood-of-harm/help of microsurgery compared to radiosurgery in large vestibular schwannoma.

PURPOSE: It has been shown that in large vestibular schwannomas (VS), radiosurgery (SRS) is inferior with respect to tumor control compared to microsurgical resection (SURGERY). However, SURGERY poses a significantly higher risk of facial-function deterioration (FFD). The aim of this study was to illustrate the effectiveness in terms of number-needed-to-treat/operate (NNO), number-needed-to-harm (NNH), and likelihood-of-harm/help (LHH) by comparing both treatment modalities in large VS. METHODS: This was a retrospective, dual-center cohort study. Tumor size was classified by Hannover Classification. Absolute risk reduction and risk increase were used to derive additional estimates of treatment effectiveness, namely NNO and NNH. LHH was then calculated by a quotient of NNH/NNO to illustrate the risk-benefit-ratio of SURGERY. RESULTS: Four hundred and forty-nine patients treated met the inclusion criteria. The incidence of tumor recurrence was significantly higher in SRS (14%), compared to SURGERY (3%) resulting in ARR of 11% and NNO of 10. At the same time, SURGERY was related to a significant risk of FFD resulting in an NNH of 12. Overall, the LHH calculated at 1.20 was favored SURGERY, especially in patients under the age of 40 years (LHH = 2.40), cystic VS (LHH = 4.33), and Hannover T3a (LHH = 1.83) and T3b (LHH = 1.80). CONCLUSIONS: Due to a poorer response of large VS to SRS, SURGERY is superior with respect to tumor control. One tumor recurrence can be prevented, when 10 patients are treated by SURGERY instead of SRS. Thus, LHH portrays the benefit of SURGERY in large VS even when taking raised FFD into account.

Evidence for inflammation in normal-appearing brain regions in patients with growing sporadic vestibular schwannoma: A PET study.

Background: Nonauditory symptoms can be a prominent feature in patients with sporadic vestibular schwannoma (VS), but the cause of these symptoms is unknown. Inflammation is hypothesized to play a key role in the growth and symptomatic presentation of sporadic VS, and in this study, we investigated through translocator protein (TSPO) positron emission tomography (PET) whether inflammation occurred within the "normal appearing" brain of such patients and its association with tumor growth. Methods: Dynamic PET datasets from 15 patients with sporadic VS (8 static and 7 growing) who had been previously imaged using the TSPO tracer [11C](R)-PK11195 were included. Parametric images of [11C](R)-PK11195 binding potential (BPND) and the distribution volume ratio (DVR) were derived and compared across VS growth groups within both contralateral and ipsilateral gray (GM) and white matter (WM) regions. Voxel-wise cluster analysis was additionally performed to identify anatomical regions of increased [11C](R)-PK11195 binding. Results: Compared with static tumors, growing VS demonstrated significantly higher cortical (GM, 1.070 vs. 1.031, P = .03) and whole brain (GM & WM, 1.045 vs. 1.006, P = .03) [11C](R)-PK11195 DVR values. The voxel-wise analysis supported the region-based analysis and revealed clusters of high TSPO binding within the precentral, postcentral, and prefrontal cortex in patients with growing VS. Conclusions: We present the first in vivo evidence of increased TSPO expression and inflammation within the brains of patients with growing sporadic VS. These results provide a potential mechanistic insight into the development of nonauditory symptoms in these patients and highlight the need for further studies interrogating the role of neuroinflammation in driving VS symptomatology.

Letter to Editor: Cystic vestibular schwannoma - a subgroup analysis from a comparative study between radiosurgery and microsurgery.

This letter evaluates the recent study on the management of cystic vestibular schwannomas (VS) compared to solid VS, focusing on the clinical outcomes of surgery (SURGERY) and radiosurgery (SRS). The study offers significant insights into the differences between these tumor types, emphasizing the challenges posed by cystic VS, including rapid growth, enhanced peritumoral adhesion, and worse post-operative facial nerve outcomes. Notably, cystic VS are associated with higher recurrence rates and poorer preoperative status. The study also highlights lower gross total resection (GTR) rates and poorer long-term tumor control in cystic VS. While SRS shows high rates of functional preservation, it is less effective in ensuring recurrence-free survival in cystic VS compared to solid VS, suggesting surgery may be preferable for achieving the best long-term outcomes, particularly when safe maximal resection is possible. However, the study's retrospective design and limited sample size, along with the lack of standardized follow-up protocols, may impact the generalizability of the findings. Future research should focus on prospective, multicenter studies with standardized protocols to develop evidence-based guidelines for managing cystic VS. Innovative techniques, such as advanced imaging and minimally invasive surgical approaches, may further improve diagnostic accuracy and treatment efficacy. This study underscores the complexities of managing cystic VS and the need for tailored treatment strategies.

Delayed Facial Nerve Dysfunction Following CyberKnife® Radiosurgery for Vestibular Schwannoma.

OBJECTIVE: The incidence and risk factors for facial nerve dysfunction (FND) following CyberKnife® therapy for vestibular schwannoma (VS) remain poorly understood. This study investigates whether differential radiation doses to vulnerable segments of the facial nerve may be associated with FND outcomes. METHODS: Patients were identified who underwent CyberKnife® radiosurgery for VS at a single institution. Basic demographics, tumor characteristics, and facial nerve function were collected. Total radiation doses to tumor, internal auditory canal (IAC), and labyrinthine segment of facial nerve (LSFN) were evaluated. RESULTS: Six out of 64 patients experienced FND following CyberKnife® treatment for VS (9.38%, 6/64). Patients with FND were compared to those without FND (control). Of the 64 patients, complete radiation records were obtained for 30 patients (6 FND vs. 24 control). There were no significant differences in demographic or tumor characteristics between control and FND cohorts. More severe FND (HB ≥ 4) had significantly larger tumors (3.74 vs. 1.27 cm3, p = 0.037) with directionally decreased time to FND (3.50 vs. 33.5 months, p = 0.106) than patients with HB < 4, respectively. There were directionally, nonsignificant differences between maximum radiation doses to the LSFN (2492.4 vs. 2557.0 cGy, p = 0.121) and IAC (2877.3 vs. 2895.5 cGy, p = 0.824) between the control and FND cohorts, respectively. CONCLUSIONS: FND may represent an underrecognized sequelae of CyberKnife® radiosurgery for VS that can occur many months following treatment. Further studies are needed to elucidate the effect of differential radiation exposure to the facial nerve with FND following treatment. LEVEL OF EVIDENCE: III (Retrospective Cohort Study) Laryngoscope, 2024.

Association of audiovestibular findings with fluid-attenuated inversion recovery magnetic resonance imaging in patients with vestibular schwannoma.

BACKGROUND: Vestibular schwannoma (VS) is a benign tumor of the vestibular nerve. Flair-attenuated inversion recovery (FLAIR) of magnetic resonance imaging (MRI) images are sensitive in detecting high protein contents of fluids. OBJECTIVES: To investigate the association between signal intensity (SI) on FLAIR images and audiovestibular findings in patients with VS. METHODS: Medical records of twenty-five patients with VS were retrospectively analyzed. RESULTS: Larger tumors were associated with increased FLAIR SI of the cochlea, vestibule, and semicircular canal (SCC) on the affected side compared to those of the unaffected side. Pure-tone audiometry (PTA), and speech audiometry were associated with the SI of the affected cochlea. There was no significant correlation between the SI of the vestibule and vestibular evoked myogenic potential, SI of the SCC, and caloric test or video head impulse test results. CONCLUSION: Our study suggests that tumor size was significantly associated with high SI on FLAIR imaging, and audiological findings were associated with the SI of the affected cochlea. Further studies with larger cohorts are required to confirm the association between vestibular function and FLAIR imaging in VS.

Vestibular Rehabilitation: Improving Symptomatic and Functional Outcomes of Persons with Vestibular Schwannoma: A Systematic Review.

OBJECTIVE: Persons with vestibular schwannoma suffer from dizziness, imbalance, and decreased function leading to reduced quality of life. Other forms of peripheral vestibular hypofunction show improvements in these signs and symptoms with vestibular rehabilitation; however, the efficacy of this intervention for those with vestibular schwannoma is unknown. Therefore, the aim of this systematic review was to determine the effect of vestibular physical therapy on subjective and objective measures of vestibular symptoms and function in people with vestibular schwannoma. METHODS: Four electronic databases were searched: PubMed, CINAHL, EMBASE, and Cochrane. Included studies were experimental or observational in design and featured patients with vestibular schwannoma who had undergone vestibular physical therapy. Screening and quality assessment was completed independently by 2 researchers. Risk of bias was assessed with a tool appropriate for study design (eg, Cochrane Risk of Bias 2.0 tool for randomized trials). The Grading of Recommendations Assessment, Development and Evaluation approach was used to synthesize findings. RESULTS: Twenty-three studies were included. Overall, the effect of vestibular physical therapy for patients with vestibular schwannoma was uncertain. Outcomes of dizziness, static and dynamic balance, and vestibular function all showed very low certainty on the Grading of Recommendations Assessment, Development and Evaluation assessment. Multimodal physical therapist interventions consistent with clinical practice guidelines (eg, gaze stability, habituation, balance training, gait training) demonstrated potential for improvement in dizziness, balance, and vestibular function, respectively. Results were mostly insignificant when a single modality was used. CONCLUSION: There may be benefit in multimodal vestibular physical therapy for people with vestibular schwannoma to improve symptoms and function. More high-quality studies specific to vestibular schwannoma prehabilitation and rehabilitation are needed to increase the certainty in the evidence. IMPACT: Physical therapists are encouraged to use multimodal vestibular rehabilitation for vestibular schwannoma in clinical practice in line with clinical guidelines for peripheral vestibular hypofunction.

Defining tumor growth in vestibular schwannomas: a volumetric inter-observer variability study in contrast-enhanced T1-weighted MRI.

PURPOSE: For patients with vestibular schwannomas (VS), a conservative observational approach is increasingly used. Therefore, the need for accurate and reliable volumetric tumor monitoring is important. Currently, a volumetric cutoff of 20% increase in tumor volume is widely used to define tumor growth in VS. The study investigates the tumor volume dependency on the limits of agreement (LoA) for volumetric measurements of VS by means of an inter-observer study. METHODS: This retrospective study included 100 VS patients who underwent contrast-enhanced T1-weighted MRI. Five observers volumetrically annotated the images. Observer agreement and reliability was measured using the LoA, estimated using the limits of agreement with the mean (LOAM) method, and the intraclass correlation coefficient (ICC). RESULTS: The 100 patients had a median average tumor volume of 903 mm3 (IQR: 193-3101). Patients were divided into four volumetric size categories based on tumor volume quartile. The smallest tumor volume quartile showed a LOAM relative to the mean of 26.8% (95% CI: 23.7-33.6), whereas for the largest tumor volume quartile this figure was found to be 7.3% (95% CI: 6.5-9.7) and when excluding peritumoral cysts: 4.8% (95% CI: 4.2-6.2). CONCLUSION: Agreement limits within volumetric annotation of VS are affected by tumor volume, since the LoA improves with increasing tumor volume. As a result, for tumors larger than 200 mm3, growth can reliably be detected at an earlier stage, compared to the currently widely used cutoff of 20%. However, for very small tumors, growth should be assessed with higher agreement limits than previously thought.

Ruptured distal anterior inferior cerebellar artery aneurysm years after stereotactic radiosurgery for vestibular schwannoma: A case report and literature review.

Background: Aneurysmal formation after stereotactic radiosurgery (SRS) for vestibular schwannoma (VS) is a rare complication. Its characteristics and the best treatment strategies remain controversial, and the clinical course is especially unknown because reported aneurysms are first incidentally detected, or aneurysmal rupture occurs suddenly, and they are treated immediately. Case Description: A 68-year-old man who underwent SRS for VS 20 years ago presented with subarachnoid hemorrhage (SAH) due to rupture of a radiation-induced fusiform anterior inferior cerebellar artery aneurysm. He was treated with parent artery occlusion, resulting in a modified Rankin scale grade 2. This report illustrates the first case of detected aneurysm formation before rupture with retrospective magnetic resonance imaging evaluation. Conclusion: We describe the possible risk of rapid progression and rupture of aneurysms, focusing on the interval from SRS to aneurysmal formation. The period of formation of SRS-induced aneurysms is suspected to vary from years to decades regardless of radiation doses; however, aneurysms estimated as pseudoaneurysms have an extremely high risk of rupture within a few years, even when small in size. If aneurysms are discovered unruptured, there are some advantages in not only the prevention of poor prognosis due to SAH but also in the availability of optional therapeutic strategies using revascularization. Long-term annual follow-up, including vessel examination, is warranted not only to assess tumor status but also for early detection of any vascular lesions.

Hearing Function after CyberKnife for Vestibular Schwannoma: A Systematic Review.

Introduction CyberKnife (CK) radiosurgery is a treatment strategy for vestibular schwannoma (VS). Objectives To evaluate hearing preservation (HP) after CK for VS. Data Synthesis The study was conducted following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) statement, and it was registered at the International Prospective Register of Systematic Reviews (PROSPERO, under number CRD42021250300). The inclusion criteria were based on the population, intervention, comparison, outcome, timing and study design (PICOTS) strategy: population - patients with VS; intervention - CK; Comparison - none; Outcome - serviceable HP defined by Gardner and Robertson as grades I or II, or by the American Academy of Otolaryngology and Head and Neck Surgery as classes A or B; timing - mean follow-up longer than 1 year; and study design - retrospective or prospective studies. The exclusion criteria were: studies not published in English; studies published before January 2000 and after October 2021; and studies only including patients with neurofibromatosis type 2 or submitted to a previous treatment. The PubMed/MEDLINE, EMBASE, Web of Science, Cochrane Library, LILACS, and IBECS databases were used and last searched on October 27th, 2021. Statistical heterogeneity was assessed using I 2 statistics. The appraisal checklist was used to assess the risk of bias in the included studies. A total of 222 studies were analyzed, and 13 were included in the synthesis, which represents 493 participants with serviceable hearing before intervention. The mean HP rate after CK using a random effects model was of 68% (95% confidence interval [95%CI]: 59-76%) at a mean follow-up of 42.96 months. Conclusion The longer follow-up period was associated with a lower HP rate after CK radiosurgery for VS in the qualitative synthesis.

Surgical Outcomes and Predictive Factor Analysis for Facial Nerve Preservation in Patients With Cerebellopontine Angle (CPA) Tumors: A Ten-Year Single Institutional Study.

Objective To analyze the surgical outcome and predictive factors for facial nerve preservation in patients with surgically operated cerebellopontine angle (CPA) tumors. Methodology Methodology Data were retrospectively retrieved from inpatient medical records of patients admitted with CPA tumors from January 1, 2011, to December 31, 2020, at our institute. Epidemiological, clinical and radiological findings, histopathological types, surgical outcomes, and facial nerve function of these patients were recorded using a data-gathering tool. Results Out of 230 patients, 188 (81.7%) were diagnosed histopathologically with vestibular schwannoma (VS), 20 (8.7%) with meningioma, 15 (6.5%) with epidermoid, and 7 (3.1%) with other conditions. The most common clinical features were hearing loss in VS and headaches in meningioma and epidermoid. Preoperatively, 103 (44.8%) had grade 2, 68 (29.6%) had grades 3 or 4, and 8 (3.5%) had grade 5 facial nerve palsy, while post-operatively, 93 (40.9%) patients had grade 2, 83 (36.6%) had grades 3 or 4, and 6 (2.6%) had grade 5 facial palsy. Greater facial nerve preservation was observed in patients with tumor sizes <4 cm (p=0.0041) and in those who underwent near-total (NTR) or subtotal resection (STR) (p=0.0442). Excellent facial nerve outcomes (HB grades 1 or 2) were noted in patients who underwent intraoperative facial nerve monitoring (p<0.0001). CSF leak and meningitis were present in 3.5% and 2.2% of patients, respectively. The mortality rate was 6.1%, with a recurrence rate of 4.8%. Conclusion Intraoperative facial nerve monitoring, tumor size less than 4 cm, and extent of resection (NTR/STR) are predictive factors that significantly affect facial nerve outcomes.

Long-term hearing prognosis after vestibular schwannoma surgery with retrolabyrinthine approach.

PURPOSE: The retrolabyrinthine approach is a surgical method designed to preserve hearing after surgery. When paired with intraoperative monitoring and an endoscope, this approach has demonstrated high rates of postoperative hearing preservation. However, the long-term prognosis of hearing preservation after utilizing this approach for vestibular schwannomas remains unexplored. This study aimed to examine the long-term outcomes of preserved hearing, providing insights into the suitability of the retrolabyrinthine approach for hearing preservation surgery. METHODS: This study included 34 patients with preserved hearing after vestibular schwannoma surgery using the retrolabyrinthine approach at a single center. Long-term hearing prognosis and requirement for additional interventions were retrospectively examined. RESULTS: Immediate after post-operative hearing preservation rate was 71.7%. Among the 34 patients with preserved hearing post-vestibular schwannoma surgery, four (11.8%) required additional interventions. Other patients experienced a gradual deterioration in their hearing status, with an approximate 10 dB decline during the 5-year follow-up; however, a serviceable hearing level persisted long after the surgery in these individuals. CONCLUSIONS: This study indicated the rationale for the retrolabyrinthine approach as a hearing preservation surgery for vestibular schwannomas, emphasizing its long-term hearing prognosis.

Cochlear implantation in patients with inner ear schwannomas: a systematic review and meta-analysis of audiological outcomes.

PURPOSE: In patients with inner ear schwannomas (IES), reports on hearing rehabilitation with cochlear implants (CI) have increased over the past decade, most of which are case reports or small case series. The aim of this study is to systematically review the reported hearing results with CI in patients with IES considering the different audiologic outcome measures used in different countries. METHODS: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline, a search of published literature was conducted. We included patients with IES (primary or with secondary extension from the internal auditory canal (IAC) to the inner ear, sporadic or NF2 related) undergoing cochlear implantation with or without tumour removal. The audiological results were divided into the categories "monosyllables", "disyllables", "multisyllabic words or numbers", and "sentences". RESULTS: Predefined audiological outcome measures were available from 110 patients and 111 ears in 27 reports. The mean recognition scores for monosyllabic words with CI were 55% (SD: 24), for bisyllabic words 61% (SD: 36), for multisyllabic words and numbers 87% (SD: 25), and 71% (SD: 30) for sentences. Results from for multisyllabic words and numbers in general showed a tendency towards a ceiling effect. Possible risk factors for performance below average were higher complexity tumours (inner ear plus IAC/CPA), NF2, CI without tumour removal ("CI through tumour"), and sequential cochlear implantation after tumour removal (staged surgery). CONCLUSION: Hearing loss in patients with inner ear schwannomas can be successfully rehabilitated with CI with above average speech performance in most cases. Cochlear implantation thus represents a valuable option for hearing rehabilitation also in patients with IES while at the same time maintaining the possibility of MRI follow-up. Further studies should investigate possible risk factors for poor performance. Audiological tests and outcome parameters should be reported in detail and ideally be harmonized to allow better comparison between languages.

Bilateral vestibular schwannoma with a cooccurring meningioma in a child: a case report and review of literature.

Introduction: Meningioma and vestibular schwannoma (VS) are the first and second most common benign central nervous system tumors. The coexistence of VS and meningioma presents a rare clinical scenario, particularly in pediatric patients. This report presents a case of bilateral VS with a cooccurring meningioma in a Nepali child and provides an overview of the literature on this condition. Case report: A 15-year-old male presented with bilateral sensorineural hearing loss, seizures, and neurological deficits and was ultimately diagnosed with concomitant bilateral acoustic neuroma and meningioma. The patient underwent radiosurgery for bilateral VS and nonoperative management of the meningioma. Long-term follow-up revealed symptomatic improvement, emphasizing the importance of a multidisciplinary approach in managing such complex cases. The management of these tumors requires tailored treatment strategies guided by tumor characteristics and associated risks. Discussion: Meningioma and VS are common tumors of the central nervous system. Their coexistence is possible in neurofibromatosis type 2 but is exceedingly rare in pediatric age group. The tumors, often coexisting, pose diagnostic challenges. Diagnosis relies on clinical and genetic features, with multidisciplinary management involving various specialists. Treatment aims to preserve function and quality of life, utilizing approaches such as bevacizumab and surgical intervention. The role of radiation therapy remains uncertain. Genetic testing and regular monitoring are vital for early detection and intervention. Conclusion: The cooccurrence of acoustic neuromas and meningiomas is poorly understood, with limited reported cases and unclear pathophysiological mechanisms. Further research into the genetic and molecular mechanisms underlying the coexistence of these tumors is needed to optimize patient outcomes in this rare clinical entity.

Prestin in Human Perilymph, Cerebrospinal Fluid, and Blood as a Biomarker for Hearing Loss.

OBJECTIVE: Determining the concentration of prestin in human blood, cerebrospinal fluid (CSF), and perilymph (PL), and evaluating its suitability as a clinical biomarker for sensori-neural hearing loss (SNHL). STUDY DESIGN: Human blood, CSF, and PL samples were intraoperatively collected from 42 patients with tumors of the internal auditory canal or with intracochlear tumors undergoing translabyrinthine or middle fossa tumor removal. Prestin concentration was measured using enzyme-linked immunosorbent assay and linear regression analyses were performed to investigate its associations with audiological as well as vestibular test results. SETTING: Tertiary referral center. RESULTS: The median prestin concentration in blood samples of the 42 study participants (26 women, mean ± standard deviation age, 52.7 ± 12.5 years) was 1.32 (interquartile range, IQR, 0.71-1.99) ng/mL. CSF prestin levels were significantly higher with 4.73 (IQR, 2.45-14.03) ng/mL (P = .005). With 84.74 (IQR, 38.95-122.00) ng/mL, PL prestin concentration was significantly higher compared to blood (P = .01) and CSF (P = .03) levels. Linear regression analyses showed significant associations of CSF prestin concentration with preoperative hearing levels (pure-tone average and word recognition; P = .008, R2 = 0.1894; P = .03, R2 = 0.1857), but no correlations with blood or PL levels. CONCLUSION AND RELEVANCE: This study's findings highlight the volatile nature of prestin levels and provide the first insights into this potential biomarker's concentrations in body fluids apart from blood. Future investigations should comprehensively assess human prestin levels with different etiologies of SNHL, prestin's natural homeostasis and systemic circulation, and its temporal dynamics after cochlear trauma. Finally, clinically approved detection kits for prestin are urgently required prior to considering a potential translational implementation of this diagnostic technique.

Matrix metalloproteinase 9: An emerging biomarker for classification of adherent vestibular schwannoma.

Background: The progression of vestibular schwannoma (VS) is intricately linked with interactions between schwannoma cells and the extracellular matrix. Surgical resection of VS is associated with substantial risks as tumors are adherent to the brainstem and cranial nerves. We evaluate the role of matrix metalloproteinase 9 (MMP9) in VS and explore its potential as a biomarker to classify adherent VS. Methods: Transcriptomic analysis of a murine schwannoma allograft model and immunohistochemical analysis of 17 human VS were performed. MMP9 abundance was assessed in mouse and human schwannoma cell lines. Transwell studies were performed to evaluate the effect of MMP9 on schwannoma invasion in vitro. Plasma biomarkers were identified from a multiplexed proteomic analysis in 45 prospective VS patients and validated in primary culture. The therapeutic efficacy of MMP9 inhibition was evaluated in a mouse schwannoma model. Results: MMP9 was the most highly upregulated protease in mouse schwannomas and was significantly enriched in adherent VS, particularly around tumor vasculature. High levels of MMP9 were found in plasma of patients with adherent VS. MMP9 outperformed clinical and radiographic variables to classify adherent VS with outstanding discriminatory ability. Human schwannoma cells secreted MMP9 in response to TNF-α which promoted cellular invasion and adhesion protein expression in vitro. Lastly, MMP9 inhibition decreased mouse schwannoma growth in vivo. Conclusions: We identify MMP9 as a preoperative biomarker to classify adherent VS. MMP9 may represent a new therapeutic target in adherent VS associated with poor surgical outcomes that lack other viable treatment options.

Binaural hearing aid intervention for a patient with nonserviceable hearing loss after surgery for vestibular schwannoma: A case report.

A vestibular schwannoma is a benign tumor; however, the schwannoma itself and interventions can cause sensorineural hearing loss. Most vestibular schwannomas are unilateral tumors that affect hearing only on one side. Attention has focused on improving the quality of life for patients with unilateral hearing loss and therapeutic interventions to address this issue have been emphasized. Herein, we encountered a patient who was a candidate for hearing preservation surgery based on preoperative findings and had nonserviceable hearing after the surgery, according to the Gardner-Robertson classification. Postoperatively, the patient had decreased listening comprehension and ability to localize sound sources. He was fitted with bilateral hearing aids, and his ability to localize sound sources improved. Although the patient had postoperative nonserviceable hearing on the affected side and age-related hearing loss on the unaffected side, hearing aids in both ears were useful for his daily life. Therefore, the patient was able to maintain a binaural hearing effect and the ability to localize the sound source improved. This report emphasizes the importance of hearing preservation with vestibular schwannomas, and the demand for hearing loss rehabilitation as a postoperative complication can increase, even if hearing loss is nonserviceable.

Surgical results for one-stage VII/VIII schwannoma resection and hemihypoglossal-facial neurorrhaphy.

BACKGROUND: This retrospective study evaluated the outcomes of patients undergoing one-stage resection of VII/VIII schwannomas and hemihypoglossal-facial neurorrhaphy via the translabyrinthine approach (TLA). METHODS: The study encompassed ten consecutive patients with unilateral hearing loss (six women, four men, mean age: 49.5 ± 12.1 years) who underwent surgery. The cohort included two patients with vestibular schwannomas (VSs), four with facial nerve schwannomas (FNSs) (two originating from the geniculate ganglion of the facial nerve and two from the cerebellopontine angle), one with VS regrowth, and three with residual VSs. Preoperative facial nerve function, assessed using the House-Brackmann (HB) scale, was Grade V in one and Grade VI in nine patients. The mean preoperative duration of facial paralysis was 7.5 ± 6.9 months. RESULTS: All patients underwent gross total resection. Postoperatively, one patient experienced cerebrospinal fluid leaks, which were successfully managed with lumbar drains and surgical revisions. At follow-up, facial nerve function improved in all patients: HB Grade V to III in one, HB Grade VI to III in one, HB Grade VI to IV in seven, and Grade VI to V in one. No tumor recurrence was observed during the follow-up period (mean duration: 16.6 ± 9.3 months), and no patient had hemilingual atrophy. CONCLUSION: The TLA for one-stage resection of VII/VIII schwannomas and facial nerve reconstruction is effective in treating both regrowth and residual VSs and FNSs in the cerebellopontine angle or petrosal bone in patients with severe preoperative facial palsy. This technique facilitates simultaneous tumor removal and nerve anastomosis, thereby reducing the need for multiple surgical interventions in patients with hearing loss and compromised facial nerve function.

Palliative Gamma Knife Radiosurgery for a Small Part of a Large Vestibular Schwannoma in an Elderly Patient.

We report a case of a large vestibular schwannoma in an 80-year-old female patient that shrank after palliative Gamma Knife radiosurgery (GKS). Neurological symptoms included hearing deterioration and facial palsy. The tumor volume was 21.9 mL. Craniotomy was considered high-risk, and conventional GKS was risky, owing to the risk of transient enlargement. Therefore, GKS was performed on only a portion of the tumor. The marginal dose (12 Gy) volume was 3.8 mL (17.4%). The tumor began to shrink after transient enlargement. Sixty months later, the tumor volume was only 3.1 mL, and the patient was able to maintain independent activities of daily living without salvage treatment.