Structural connectivity changes in unilateral hearing loss.

Network connectivity, as mapped by the whole brain connectome, plays a crucial role in regulating auditory function. Auditory deprivation such as unilateral hearing loss might alter structural network connectivity; however, these potential alterations are poorly understood. Thirty-seven acoustic neuroma patients with unilateral hearing loss (19 left-sided and 18 right-sided) and 19 healthy controls underwent diffusion-weighted and T1-weighted imaging to assess edge strength, node strength, and global efficiency of the structural connectome. Edge strength was estimated by pair-wise normalized streamline density from tractography and connectomics. Node strength and global efficiency were calculated through graph theory analysis of the connectome. Pure-tone audiometry and word recognition scores were used to correlate the degree and duration of unilateral hearing loss with node strength and global efficiency. We demonstrate significantly stronger edge strength and node strength through the visual network, weaker edge strength and node strength in the somatomotor network, and stronger global efficiency in the unilateral hearing loss patients. No discernible correlations were observed between the degree and duration of unilateral hearing loss and the measures of node strength or global efficiency. These findings contribute to our understanding of the role of structural connectivity in hearing by facilitating visual network upregulation and somatomotor network downregulation after unilateral hearing loss.

Single-cell RNA sequencing analysis of vestibular schwannoma reveals functionally distinct macrophage subsets.

BACKGROUND: Vestibular schwannomas (VSs) remain a challenge due to their anatomical location and propensity to growth. Macrophages are present in VS but their roles in VS pathogenesis remains unknown. OBJECTIVES: The objective was to assess phenotypic and functional profile of macrophages in VS with single-cell RNA sequencing (scRNAseq). METHODS: scRNAseq was carried out in three VS samples to examine characteristics of macrophages in the tumour. RT-qPCR was carried out on 10 VS samples for CD14, CD68 and CD163 and a panel of macrophage-associated molecules. RESULTS: scRNAseq revealed macrophages to be a major constituent of VS microenvironment with three distinct subclusters based on gene expression. The subclusters were also defined by expression of CD163, CD68 and IL-1ß. AREG and PLAUR were expressed in the CD68+CD163+IL-1ß+ subcluster, PLCG2 and NCKAP5 were expressed in CD68+CD163+IL-1ß- subcluster and AUTS2 and SPP1 were expressed in the CD68+CD163-IL-1ß+ subcluster. RT-qPCR showed expression of several macrophage markers in VS of which CD14, ALOX15, Interleukin-1ß, INHBA and Colony Stimulating Factor-1R were found to have a high correlation with tumour volume. CONCLUSIONS: Macrophages form an important component of VS stroma. scRNAseq reveals three distinct subsets of macrophages in the VS tissue which may have differing roles in the pathogenesis of VS.

Preoperative diffusion tensor imaging: Fiber-trajectory-distribution-based tractography to identify facial nerve in vestibular schwannoma.

PURPOSE: Tractography of the facial nerve based on diffusion MRI is instrumental before surgery for the resection of vestibular schwannoma, but no excellent methods usable for the suppression of motion and image noise have been proposed. The aim of this study was to effectively suppress noise and provide accurate facial nerve reconstruction by extend a fiber trajectory distribution function based on the fourth-order streamline differential equations. METHODS: Preoperative MRI from 33 patients with vestibular schwannoma who underwent surgical resection were utilized in this study. First, T1WI and T2WI were used to obtain mask images and regions of interest. Second, probabilistic tractography was employed to obtain the fibers representing the approximate facial nerve pathway, and these fibers were subsequently translated into orientation information for each voxel. Last, the voxel orientation information and the peaks of the fiber orientation distribution were combined to generate a fiber trajectory distribution function, which was used to parameterize the anatomical information. The parameters were determined by minimizing the cost between the trajectory of fibers and the estimated directions. RESULTS: Qualitative and visual analyses were used to compare facial nerve reconstruction with intraoperative recordings. Compared with other methods (SD_Stream, iFOD1, iFOD2, unscented Kalman filter, parallel transport tractography), the fiber-trajectory-distribution-based tractography provided the most accurate facial nerve reconstructions. CONCLUSION: The fiber-trajectory-distribution-based tractography can effectively suppress the effect of noise. It is a more valuable aid for surgeons before vestibular schwannoma resection, which may ultimately improve the postsurgical patient's outcome.

Repeat stereotactic radiosurgery for progressive vestibular schwannomas after primary gamma knife radiosurgery.

PURPOSE: Limited data provides guidance on the management of vestibular schwannomas (VSs) that have progressed despite primary Gamma Knife radiosurgery (GKRS). The present article reports our long-term experience after repeat GKRS for VS with sustained progression after solely primary GKRS management. METHODS: A retrospective review of 1997 patients managed between 1987 and 2023 was conducted. Eighteen patients had sustained tumor progression after primary GKRS and underwent repeat GKRS. The median repeat GKRS margin dose was 11 Gy (IQR: 11-12), the median tumor volume was 2.0 cc (IQR: 1.3-6.3), and the median cochlear dose in patients with preserved hearing was 3.9 Gy (IQR: 3-4.1). The median time between initial and repeat GKRS was 65 months (IQR: 38-118). RESULTS: The median follow-up was 70 months (IQR: 23-101). After repeat GKRS, two patients had further tumor progression at 4 and 21 months and required partial resection of their tumors. The 10-year actuarial tumor control rate after repeat GKRS was 88%. Facial nerve function was preserved in 13 patients who had House-Brackmann grade 1 or 2 function at the time of repeat GKRS. Two patients with serviceable hearing preservation (Gardner-Robertson grade 1 or 2) at repeat GKRS retained that function afterwards. In patients with tinnitus, vestibular dysfunction, and trigeminal neuropathy, symptoms remained stable or improved for 16/16 patients, 12/15 patients, and 10/12 patients, respectively. One patient developed facial twitching in the absence of tumor growth 21 months after repeat GKRS. CONCLUSIONS: Repeat GKRS effectively controlled tumor growth and preserved cranial nerve outcomes in most patients whose VS had sustained progression after initial primary radiosurgery.

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.

Imaging as an early biomarker to predict sensitivity to everolimus for progressive NF2-related vestibular schwannoma.

PURPOSE: NF2-related schwannomatosis (NF2) is characterized by bilateral vestibular schwannomas (VS) often causing hearing and neurologic deficits, with currently no FDA-approved drug treatment. Pre-clinical studies highlighted the potential of mTORC1 inhibition in delaying schwannoma progression. We conducted a prospective open-label, phase II study of everolimus for progressive VS in NF2 patients and investigated imaging as a potential biomarker predicting effects on growth trajectory. METHODS: The trial enrolled 12 NF2 patients with progressive VS. Participants received oral everolimus daily for 52 weeks. Brain imaging was obtained quarterly. As primary endpoint, radiographic response (RR) was defined as ≥ 20% decrease in target VS volume. Secondary endpoints included other tumors RR, hearing outcomes, drug safety and quality of life (QOL). RESULTS: Eight participants completed the trial and four discontinued the drug early due to significant volumetric VS progression. After 52 weeks of treatment, the median annual VS growth rate decreased from 77.2% at baseline to 29.4%. There was no VS RR and 3 of 8 (37.5%) participants had stable disease. Decreased or unchanged VS volume after 3 months of treatment was predictive of stabilization at 12 months. Seven of eight participants had stable hearing during treatment except one with a decline in word recognition score. Ten of twelve participants reported only minimal changes to their QOL scores. CONCLUSIONS: Volumetric imaging at 3 months can serve as an early biomarker to predict long-term sensitivity to everolimus treatment. Everolimus may represent a safe treatment option to decrease the growth of NF2-related VS in patients who have stable hearing and neurological condition. TRN: NCT01345136 (April 29, 2011).

Automated, fast, robust brain extraction on contrast-enhanced T1-weighted MRI in presence of brain tumors: an optimized model based on multi-center datasets.

OBJECTIVES: Existing brain extraction models should be further optimized to provide more information for oncological analysis. We aimed to develop an nnU-Net-based deep learning model for automated brain extraction on contrast-enhanced T1-weighted (T1CE) images in presence of brain tumors. METHODS: This is a multi-center, retrospective study involving 920 patients. A total of 720 cases with four types of intracranial tumors from private institutions were collected and set as the training group and the internal test group. Mann-Whitney U test (U test) was used to investigate if the model performance was associated with pathological types and tumor characteristics. Then, the generalization of model was independently tested on public datasets consisting of 100 glioma and 100 vestibular schwannoma cases. RESULTS: In the internal test, the model achieved promising performance with median Dice similarity coefficient (DSC) of 0.989 (interquartile range (IQR), 0.988-0.991), and Hausdorff distance (HD) of 6.403 mm (IQR, 5.099-8.426 mm). U test suggested a slightly descending performance in meningioma and vestibular schwannoma group. The results of U test also suggested that there was a significant difference in peritumoral edema group, with median DSC of 0.990 (IQR, 0.989-0.991, p = 0.002), and median HD of 5.916 mm (IQR, 5.000-8.000 mm, p = 0.049). In the external test, our model also showed to be robust performance, with median DSC of 0.991 (IQR, 0.983-0.998) and HD of 8.972 mm (IQR, 6.164-13.710 mm). CONCLUSIONS: For automated processing of MRI neuroimaging data presence of brain tumors, the proposed model can perform brain extraction including important superficial structures for oncological analysis. CLINICAL RELEVANCE STATEMENT: The proposed model serves as a radiological tool for image preprocessing in tumor cases, focusing on superficial brain structures, which could streamline the workflow and enhance the efficiency of subsequent radiological assessments. KEY POINTS: • The nnU-Net-based model is capable of segmenting significant superficial structures in brain extraction. • The proposed model showed feasible performance, regardless of pathological types or tumor characteristics. • The model showed generalization in the public datasets.

Effects of intraoperative low-dose esketamine on postoperative pain after vestibular schwannoma resection: A prospective randomized, double-blind, placebo-controlled study.

AIMS: Esketamine may reduce acute postoperative pain in several settings. However, the effects of low-dose esketamine on postoperative pain after vestibular schwannoma (VS) resection with propofol/remifentanil total intravenous anaesthesia (TIVA) are unclear. The aim of this study is to observe the effects of intraoperative low-dose esketamine on postoperative pain after vestibular schwannoma resection. METHODS: This single-centre, randomized, placebo-controlled, double-blind trial included 90 adults undergoing VS resection via the retrosigmoid approach with TIVA. The patients were randomly allocated to two groups: esketamine or control (n = 45 in each group). Patients received low-dose esketamine (0.2 mg/kg) or a similar volume of normal saline after dural closure. The primary outcome was the pain score during movement (gentle head movement) at 24 h postoperatively. Secondary outcomes included recovery time, bispectral index (BIS) values and haemodynamic profiles during the first 30 min after esketamine administration, and adverse effects. RESULTS: Low-dose esketamine did not reduce pain scores at rest (P > .05) or with movement (P > .05) within the first 24 h after surgery. Esketamine moderately increased BIS values for at least 30 min after administration (P < .0001) but did not affect heart rate (P = .992) or mean arterial blood pressure (P = .994). Esketamine prolonged extubation time (P = .042, 95% confidence interval: 0.08 to 4.42) and decreased the effect-site concentration of remifentanil at extubation (P = .001, 95% confidence interval: -0.53 to -0.15) but did not affect the time to resumption of spatial orientation. Postoperative nausea and vomiting rates did not differ between groups, and no hallucinations or excessive sedation was observed. CONCLUSION: Intraoperative low-dose esketamine did not significantly reduce acute pain after VS resection with propofol/remifentanil TIVA. However, BIS values increased for at least 30 min after esketamine administration.

Incidence of vestibular schwannoma in Finland, 1990-2017.

BACKGROUND: An increasing trend in incidence of vestibular schwannomas (VS) has been reported, though not consistently, across populations.  Materials and methods: We obtained data from the Finnish Cancer Registry on 1,149 VS cases diagnosed in 1990-2017 with tabular data up to 2022. We calculated age-standardised incidence rates (ASR) overall, by sex, and for 10-year age groups. We analysed time trends using Poisson and joinpoint regression. RESULTS: The average ASR of VS in Finland during 1990-2017 was 8.6/1,000,000 person-years for women and 7.5/1,000,000 for men. A declining trend was found with an average annual percent change of -1.7% (95% confidence interval [CI]: -2.8%, -0.6%) for women, -2.2% (95% CI: -3.6%, -0.7%) for men, and -1.9% (95% CI: -2.9%, -1.0%) for both sexes combined. The ASR in women was 11.6/1,000,000 person-years in 1990 and it decreased to 8.2/1,000,000 by 2017. Correspondingly, the incidence in men was 7.1/1,000,000 in 1990 and decreased to 5.1/1,000,000 by 2017. Some decline in incidence over time was found in all age groups below 80 years, but the decline (2.3-3.1% per year) was statistically significant only in age groups 40-49, 50-59, and 60-69 years. In the oldest age group (80+ years), the incidence of VS increased by 16% per year. For 2018-2022, the ASR was 7.6/1,000,000 for both sexes combined, with a decline by -1.7% (95% CI: -2.3%, -1.2%) annually for the entire period 1990-2022. CONCLUSION: In contrast to the increasing incidence reported in some studies, we found a decreasing trend in VS incidence for both sexes in Finland.

Genome-wide association analysis identifies a susceptibility locus for sporadic vestibular schwannoma at 9p21.

Vestibular schwannomas are benign nerve sheath tumours that arise on the vestibulocochlear nerves. Vestibular schwannomas are known to occur in the context of tumour predisposition syndromes NF2-related and LZTR1-related schwannomatosis. However, the majority of vestibular schwannomas present sporadically without identification of germline pathogenic variants. To identify novel genetic associations with risk of vestibular schwannoma development, we conducted a genome-wide association study in a cohort of 911 sporadic vestibular schwannoma cases collated from the neurofibromatosis type 2 genetic testing service in the north-west of England, UK and 5500 control samples from the UK Biobank resource. One risk locus reached genome-wide significance in our association analysis (9p21.3, rs1556516, P = 1.47 × 10-13, odds ratio = 0.67, allele frequency = 0.52). 9p21.3 is a genome-wide association study association hotspot, and a number of genes are localized to this region, notably CDKN2B-AS1 and CDKN2A/B, also referred to as the INK4 locus. Dysregulation of gene products within the INK4 locus have been associated with multiple pathologies and the genes in this region have been observed to directly impact the expression of one another. Recurrent associations of the INK4 locus with components of well-described oncogenic pathways provides compelling evidence that the 9p21.3 region is truly associated with risk of vestibular schwannoma tumorigenesis.

Effect of deep learning-based reconstruction on high-resolution three-dimensional T2-weighted fast asymmetric spin-echo imaging in the preoperative evaluation of cerebellopontine angle tumors.

PURPOSE: We aimed to evaluate the effect of deep learning-based reconstruction (DLR) on high-spatial-resolution three-dimensional T2-weighted fast asymmetric spin-echo (HR-3D T2-FASE) imaging in the preoperative evaluation of cerebellopontine angle (CPA) tumors. METHODS: This study included 13 consecutive patients who underwent preoperative HR-3D T2-FASE imaging using a 3 T MRI scanner. The reconstruction voxel size of HR-3D T2-FASE imaging was 0.23 × 0.23 × 0.5 mm. The contrast-to-noise ratios (CNRs) of the structures were compared between HR-3D T2-FASE images with and without DLR. The observers' preferences based on four categories on the tumor side on HR-3D T2-FASE images were evaluated. The facial nerve in relation to the tumor on HR-3D T2-FASE images was assessed with reference to intraoperative findings. RESULTS: The mean CNR between the tumor and trigeminal nerve and between the cerebrospinal fluid and trigeminal nerve was significantly higher for DLR images than non-DLR-based images (14.3 ± 8.9 vs. 12.0 ± 7.6, and 66.4 ± 12.0 vs. 53.9 ± 8.5, P < 0.001, respectively). The observer's preference for the depiction and delineation of the tumor, cranial nerves, vessels, and location relation on DLR HR-3D T2FASE images was superior to that on non-DLR HR-3D T2FASE images in 7 (54%), 6 (46%), 6 (46%), and 6 (46%) of 13 cases, respectively. The facial nerves around the tumor on HR-3D T2-FASE images were visualized accurately in five (38%) cases with DLR and in four (31%) without DLR. CONCLUSION: DLR HR-3D T2-FASE imaging is useful for the preoperative assessment of CPA tumors.

Vestibular Decompensation Following COVID-19 Infection in a Person With Compensated Unilateral Vestibular Loss: A Rehabilitation Case Study.

BACKGROUND AND PURPOSE: Surgical removal of a vestibular schwannoma (vestibular schwannoma resection; VSR) results in a unilateral vestibular hypofunction with complaints of dizziness and imbalance. Although the anatomic lesion is permanent, recovery of balance and diminution of dizziness occurs through central neurophysiologic compensation. Compensation of the system is maintained through daily activity. Unfortunately, interruption of stimulus, such as decreased activities due to illness, can cause decompensation. Decompensation is described as the return of symptoms consistent with that experienced during the initial insult/injury (eg, dizziness, oscillopsia, balance difficulty). This case study describes a reoccurrence of vestibular dysfunction in a person with a history of VSR following hospitalization and protracted recovery from a COVID-19 infection. It further documents her recovery that may be a result of vestibular rehabilitation. CASE DESCRIPTION: A 49-year-old woman (M.W.) with a surgical history of VSR (10 years prior) and a medical history of significant COVID-19 infection, resulting in an intensive care unit stay and prolonged use of supplemental oxygen, presented to physical therapy with persistent dizziness and imbalance. The video head impulse test confirmed unilateral vestibular hypofunction. INTERVENTION: M.W. attended biweekly vestibular rehabilitation for 6 weeks and completed daily home exercises. OUTCOMES: At discharge, M.W. demonstrated improvements in patient-reported outcomes (Dizziness Handicap Inventory), functional testing (MiniBEST, 2-Minute Walk Test), and gaze stability measures (video head impulse testing, dynamic visual acuity). DISCUSSION: Vestibular decompensation preluded by a COVID-19 infection caused a significant decrease in functional mobility. Vestibular rehabilitation targeted at gaze and postural stability effectively reduced symptoms and facilitated recovery to M.W.'s pre-COVID-19 level of function. Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1 available at: http://links.lww.com/JNPT/A458 ).

Contrast-enhanced CT rim sign may predict vestibular schwannoma adhesion and postoperative complications.

AIM: To investigate the clinical and radiological features to predict adhesion between vestibular schwannoma (VS) and brain tissue which is a critical risk factor for postoperative infarction and residual tumour. MATERIAL AND METHODS: One hundred and seven consecutive VS surgeries were analysed. After excluding cases without contrast-enhanced (CE) computed tomography (CT), Koos grades 1 and 2, and cases with incomplete clinical data, 44 patients were finally included in the study. Enhancement of the tumour capsule on the brainstem side on CE-CT was defined as the CE-CT rim sign, which was analysed along with clinical characteristics, including tumour adhesion and postoperative complications. RESULTS: Eight patients exhibited CE-CT rim signs; 17 had tumour adhesions. Four patients had postoperative infarction at the ipsilateral middle cerebellar peduncle; 18 exhibited postoperative infarction and/or residual tumour at the middle cerebellar peduncle. The CE-CT rim sign significantly correlated with tumour adhesion, postoperative infarction, and postoperative infarction and/or residual tumour in the cerebellar peduncle. Univariate regression analysis revealed that the CE-CT rim sign significantly correlated with tumour adhesion (odds ratio [OR] 6.81, 95% confidence interval [CI] 1.18-39.25, p=0.032) and postoperative infarction and/or residual tumour at the cerebellar peduncle (OR 6.00, 95% CI 1.04-34.31, p=0.044). CONCLUSION: The CE-CT rim sign was identified in 18.2% of patients with VS and significantly correlated with tumour adhesion and postoperative complications, such as postoperative infarction and residual tumour. This study highlights the importance of the preoperative CE-CT rim sign in VS, which is predictive of tumour adhesion and postoperative complications.

Fully Endoscopic Retrosigmoid Approach for Cerebellopontine Angle Tumors.

BACKGROUND: Fully endoscopic or endoscope-controlled approaches are essentially keyhole approaches in which rigid endoscopes are the sole visualization tools used during the whole procedure. At the early attempts of endoscope-assisted cranial surgery, it was noted that rigid endoscopes enabled overcoming the problem of suboptimal visualization when small exposures are used. The technical specifications and design of the currently available rigid endoscopes are associated with a group of unique features that define the endoscopic view and lay the basis for its superiority over the microscopic view during brain surgery. Fully endoscopic retrosigmoid approach for cerebellopontine angle tumors is a minimally invasive approach that is not routinely practiced by neurosurgeons, with few series published so far. Unfamiliarity with the technique, steep learning curve, and concerns about inadequate exposure, neurovascular injury, and decreased visibility may explain this fact. In this chapter we elaborate on the surgical technique and nuances of the fully endoscopic retrosigmoid approach and present an overview of the published series. METHODS: From a prospective database of endoscopic procedures maintained by the senior author, clinical data, imaging studies, operative charts, and videos of cases undergoing fully endoscopic retrosigmoid approach for cerebellopontine angle tumors were retrieved and analyzed. The pertinent literature was also reviewed. RESULTS: The surgical technique of the fully endoscopic retrosigmoid approach was formulated. CONCLUSION: The endoscopic technique has many advantages over the conventional procedures. In our hands, the technique has proven to be feasible, efficient, and minimally invasive with excellent results.

Ultrasound-guided lesser occipital nerve combined with great auricular nerve block for vestibular schwannoma craniotomy via a suboccipital retrosigmoid approach: a prospective, double-blind randomized controlled trial.

PURPOSE: This aim of this study was to investigate the analgesic efficacy and safety of lesser occipital nerve combined with great auricular nerve block (LOGAB) for craniotomy via a suboccipital retrosigmoid approach. METHODS: Patients underwent vestibular schwannoma resection via a suboccipital retrosigmoid approach were randomly assigned to receive ultrasound-guided unilateral LOGAB with 5 ml of 0.5% ropivacaine (LOGAB group) or normal saline (NSB group). Numeric rating scale (NRS) scores at rest and motion were recorded within 48 h after surgery. Mean arterial pressure (MAP), heart rate (HR), opioid consumption and other variables were measured secondly. RESULTS: Among 59 patients who were randomized, 30 patients received ropivacaine, and 29 patients received saline. NRS scores at rest (1.8 ± 0.5 vs. 3.2 ± 0.8, P = 0.002) and at motion (2.2 ± 0.7 vs. 3.2 ± 0.6, P = 0.013) of LOGAB group were lower than those of NSB group within 48 h after surgery. NRS scores of motion were comparable except for 6th and 12th hour (P < 0.05) in the LOGAB group. In LOGAB group, MAP decreased significantly during incision of skin and dura (P < 0.05) and intraoperative opoid consumption was remarkably reduced (P < 0.01). Postoperative remedial analgesia was earlier in the NSB group (P < 0.001). No patients reported any adverse events. CONCLUSION: Among patients undergoing craniotomy for vestibular schwannoma via a suboccipital retrosigmoid approach, LOGAB may be a promising treatment for perioperative analgesia and has the potential to maintain intraoperative hemodynamic stability. CLINICAL TRIAL REGISTRATION NUMBER: Chictr.org.cn ChiCTR2000038798.

Sporadic pediatric vestibular schwannoma: a case report in a 4-year-old boy.

Sporadic vestibular schwannomas (VSs) are rare in children. When occurred in the pediatric population, they usually appear bilaterally and are related to neurofibromatosis type 2 (NF2). The current study reports a 4-year-old boy without family history of VS or NF2 who presented with a large (5.7-cm) VS involving the right cerebellopontine angle and internal auditory canal. Through seven-staged surgical interventions and two stereotactic γ­knife radiosurgery, the disease was stabilized. At 2-year follow-up, the child had right ear hearing loss, grade IV facial palsy, and normal motor function and gait. No definite evidence of gene mutation regarding NF2 can be identified after sequence analysis and deletion/duplication testing. This case highlights the significance of considering the possibility of sporadic VSs, even in very young children. It emphasizes the importance of not overlooking initial symptoms, as they may indicate the presence of a large tumor and could potentially result in delayed diagnosis.

Sporadic vestibular schwannoma in a pediatric population: a case series.

PURPOSE: To describe the characteristics, management, and outcomes of pediatric patients with sporadic vestibular schwannoma (sVS). METHODS: This was a case series at a tertiary care center. Patients were identified through a research repository and chart review. Interventions were microsurgery, stereotactic radiosurgery (SRS), and observation. Outcome measures were tumor control, facial nerve function, and hearing. RESULTS: Eight patients over 2006-2022 fulfilled inclusion criteria (unilateral VS without genetic or clinical evidence of neurofibromatosis type 2 (NF2); age ≤ 21) with a mean age of 17 years (14-20). Average greatest tumor length in the internal auditory canal was 9.7 mm (4.0-16.1). Average greatest tumor dimension (4/8 tumors) in the cerebellopontine angle was 19.1 mm (11.3-26.8). Primary treatment was microsurgery in five (62.5%) patients, observation in two (25%), and SRS in one (12.5%). Four (80%) surgical patients had gross total resections, and one (20%) had regrowth post-near total resection and underwent SRS. One observed patient and the primary SRS patient have remained radiographically stable for 3.5 and 7 years, respectively. The other observed patient required surgery for tumor growth after 12 months of observation. Two surgical patients had poor facial nerve outcomes. All post-procedural patients developed anacusis. Mean follow-up was 3 years (0.5-7). CONCLUSIONS: We describe one of the largest reported cohorts of pediatric sVS in the USA. Diligent exclusion of NF2 is critical. Given the high likelihood of eventually requiring intervention and known adverse effects of SRS, microsurgery remains the preferred treatment. However, observation can be considered in select situations.

Comparative analysis of upfront stereotactic radiosurgery and watchful waiting in the management of newly diagnosed vestibular schwannomas: a systematic review and meta-analysis.

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.

Age, preoperative tumor volume and widening of the internal acoustic meatus are independent factors associated with poor preoperative hearing in vestibular schwannoma patients - results of a single-center retrospective analysis.

Preoperative hearing function shows wide variations among patients diagnosed with vestibular schwannoma. Besides the preoperative tumor size there are other factors that influence the preoperative hearing function that are frequently discussed. A comprehensive analysis of a large cohort of vestibular schwannomas has the potential to describe new insights and influence the preoperative management. We analyzed clinical factors, imaging data and the expression of the proliferation marker MIB1 as potential influencing factors on the preoperative hearing function in a retrospective cohort of 523 primary sporadic vestibular schwannomas. The results of the preoperative audiometry were quantified using the Gardner-Robertson Score. Uni- and multivariate analyses were performed. Serviceable hearing (Gardner-Robertson class 1 or 2) was documented in 391 patients (74.8%). Factors associated with non-serviceable hearing (Gardner-Robertson class 3-5) were patients of older age (p < 0.0001), larger preoperative tumor volume (p = 0.0013) and widening of the internal acoustic meatus compared to the healthy side (p = 0.0353). Gender and differences in the expression of the proliferation marker MIB1 had no influence on preoperative hearing. In the multivariate nominal logistic regression older age (OR 27.60 (CI 9.17-87.18), p < 0.0001), larger preoperative tumor volume (OR 20.20 (CI 3.43-128.58), p = 0.0011) and widening of the internal acoustic canal (OR 7.86 (CI 1.77-35.46), p = 0.0079) remained independent factors associated with non-serviceable hearing. Widening of the internal acoustic canal is an independent factor for non-serviceable preoperative hearing in vestibular schwannoma patients together with older age and larger preoperative tumor volume.

Vestibular schwannoma microneurosurgery in patients over 70: a single institution experience and proposal of a treatment algorithm.

BACKGROUND: Elderly patients with vestibular schwannoma (VS) are commonly observed. OBJECT: Retrospective analysis of 25 patients aging ≥ 70 operated on in our neurosurgical department for unilateral VS. The purpose of our study is to propose an algorithm for the treatment of VS in elderly patients. METHODS: American Society of Anesthesiology (ASA) Grade I-II patients and Grade III with life-threatening tumors were enrolled. Karnofsky Performance Status Scale (KPS) was used for evalutation of the quality of life. The House-Brackmann (HB) scale for facial nerve (FN) outcome was used. Tumor size was categorized according to Koos' classification. A retrosigmoid approach was used in all cases, except one in which a translabyrinthine approach was performed. Surgical removal graduation: total (GTR), near total (NTR > 95%), subtotal (STR > 90%). The clinical and radiological follow-up period was set first at six months and then at one year after surgery. FN results evaluation was performed at one year, categorized according to House-Brackmann grades I-VI. RESULTS: Mean age: 74,4 years (70-83); 28% ASA I, 56% ASA II, 16% ASA III. Mean tumor size: 2,7 cm (1,5-4,2 cm). GTR/NTR: 68%, STR 32%. Mortality was zero. At last follow-up (one year after surgery) FN results were: HBI 81%, HBII 9.5%, HBIII 9.5%; HB IV 0%. Only 4 patients had preoperative HB IV, of whom one improved from HB IV to HB III. Transient complications occurred only in large VS. Re-growth of residue after STR was observed in 3 cases, treated with SRS in 2 cases and observed in 1. CONCLUSIONS: An algorithm of treatment of vestibular schwannoma in the elderly is proposed. In particular, in patients in general good conditions, age does not appear to be a major contraindication for microsurgery of VS. FN results at last follow-up are satisfactory and the complication rates are acceptable.