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Both vestibular schwannoma (VS) and Meniere's disease (MD) patients underwent hydrops MRI to clarify the relationship between VS and endolymphatic hydrops (EH). Eighty patients with VS or MD underwent an inner ear test battery followed by hydrops MRI, and were then divided into 3 groups. Group A comprised 58 MD patients (62 ears) with positive EH but negative VS. Group B included 18 VS patients (18 ears) with negative EH, while Group C consisted of 4 patients (4 ears) who had VS concomitant with EH. Another 14 MD patients who tested negative for EH on hydrops MRI were initially excluded from this cohort, but were later included for comparison. The decreasing prevalence of EH at the cochlea, saccule and utricle in Group A was identified in 59 (95%), 42 (68%) and 40 (65%) ears, respectively, mimicking a declining sequence of abnormality rates running from audiometry (86%), cervical vestibular-evoked myogenic potential (cVEMP) test (55%) to the ocular (oVEMP) test (53%). However, such decreasing trend was not identified in Groups B and C. In Groups C and A combined, 4 (6%) of 62 EH patients had concomitant VS. Conversely, 4 (18%) of 22 VS patients in Groups C and B combined had concurrent EH. A very low (6%) rate of VS in EH patients indicates that VS in EH patients may be coincidental. In contrast, EH was identified in 18% prevalence of VS patients, mirroring the 22% prevalence of cochlear EH demonstrated in VS donors through histopathological studies. [ABSTRACT FROM AUTHOR]

Background: Neurosurgery performed in the semi-sitting position provides advantages for certain procedures. However, this approach is associated with potential complications, particularly venous air embolism. Due to typically negative venous pressure at the wound site, air can be drawn into the veins. This risk is especially high in patients presenting with an intra- or extracardiac right-to-left-shunt. Transoesophageal echocardiography can be used to detect a patent foramen ovale or other possible pulmonary-systemic shunt before placing the patient in the sitting position. Case presentation: In this report, we present two young patients undergoing scheduled microsurgical vestibular schwannoma removal in a semi-sitting position who were diagnosed with congenital heart defects during routine perioperative assessment to detect possible intracardiac right-to-left shunts, using pre- and intraoperative transesophageal echocardiography (TEE) and additionally conducting an agitated saline bubble study under Valsalva manoeuvre. Patient A was diagnosed with a persistent left superior vena cava and Patient B with an unroofed coronary sinus (UCS). These findings confronted the anesthesiological and surgical teams with difficult individual decisions regarding further perioperative management. Conclusions: Perioperative transesophageal echocardiography is a diagnostic tool to both detect intraoperative position-related air embolisms and to rule out intracardiac right-to-left shunts, e.g. a patent foramen ovale, in order to decide for or against a (semi-)sitting position. Depending on the surgical circumstances a semi-sitting positioning of patients presenting with an intracardiac right-to-left-shunt, e.g. a PFO, can be feasible in individual cases if there is an implemented therapeutic algorithm to immediately terminate significant venous air entry. However, since certain other intra- or extracardiac right-to-left-shunts, such as here presented PLSVC or UCS, are rare, there is no definitive way of estimating the amount of entered air through detected shunts or anomalous vessels. Therefore, it is recommended to avoid a (semi-)sitting position in favour of a lateral or prone position for a patient undergoing intracranial surgery, once the perioperative TEE shows air bubbles in the left atrium or ventricle whose origins cannot be defined solely through TEE for certain in order to ensure patient safety by minimizing the risk of intraoperative paradoxical air embolisms. [ABSTRACT FROM AUTHOR]

Introduction: The authors conducted meta-analyses regarding the association between cellular and mobile phone use and brain tumor development by applying various radiofrequency-electromagnetic radiation (RF-EMR) exposure subcategories. With changing patterns of mobile phone use and rapidly developing Wireless Personal Area Network (WPAN) technology (such as Bluetooth), this study will provide insight into the importance of more precise exposure subcategories for RF-EMR. Methods: The medical librarian searched MEDLINE (PubMed), EMBASE, and the Cochrane Library until 16 December 2020. Results: In these meta-analyses, 19 case-control studies and five cohort studies were included. Ipsilateral users reported a pooled odds ratio (OR) of 1.40 (95% CI 1.21–1.62) compared to non-regular users. Users with years of use over 10 years reported a pooled OR of 1.27 (95% CI 1.08–1.48). When stratified by each type of brain tumor, only meningioma (OR 1.20 (95% CI 1.04–1.39)), glioma (OR 1.45 (95% CI 1.16–1.82)), and malignant brain tumors (OR 1.93 (95% CI 1.55–2.39)) showed an increased OR with statistical significance for ipsilateral users. For users with years of use over 10 years, only glioma (OR 1.32 (95% CI 1.01–1.71)) showed an increased OR with statistical significance. When 11 studies with an OR with cumulative hours of use over 896 h were synthesized, the pooled OR was 1.59 (95% CI 1.25–2.02). When stratified by each type of brain tumor, glioma, meningioma, and acoustic neuroma reported the pooled OR of 1.66 (95% CI 1.13–2.44), 1.29 (95% CI 1.08–1.54), and 1.84 (95% CI 0.78–4.37), respectively. For each individual study that considered cumulative hours of use, the highest OR for glioma, meningioma, and acoustic neuroma was 2.89 (1.41–5.93) (both side use, > 896 h), 2.57 (1.02–6.44) (both side use, > 896 h), and 3.53 (1.59–7.82) (ipsilateral use, > 1640 h), respectively. For five cohort studies, the pooled risk ratios (RRs) for all CNS tumors, glioma, meningioma, and acoustic neuroma, were statistically equivocal, respectively. However, the point estimates for acoustic neuroma showed a rather increased pooled RR for ever-use (1.26) and over 10 years of use (1.61) compared to never-use, respectively. Discussion: In this meta-analysis, as the exposure subcategory used became more concrete, the pooled ORs demonstrated higher values with statistical significance. Although the meta-analysis of cohort studies yielded statistically inconclusive pooled effect estimates, (i) as the number of studies included grows and (ii) as the applied exposure subcategories become more concrete, the pooled RRs could show a different aspect in future research. Additionally, future studies should thoroughly account for changing patterns in mobile phone use and the growing use of earphones or headphones with WPAN technology. [ABSTRACT FROM AUTHOR]

Background: Surgical resection of vestibular schwannomas (VS) can be responsible for single-sided deafness (SSD). Hearing restoration can be a challenge both for the otolaryngologist and the patient. Patients and Methods: In a retrospective series, we analyzed the charts of SSD patients operated on for VS from 2005–2021, checking which type of hearing rehabilitation was chosen. All patients who wanted a hearing restoration underwent a hearing in noise test (HINT) in a stereo auditorium with and without a bone-anchored hearing device (BAHD) worn with a headband on the deaf side. Then, they had a preimplantation one-month trial with the BAHD at home vs. contralateral routing of signal (CROS) or BiCROS (with contralateral signal amplification) hearing aids (HAs). Results: Among 52 charts of the included adult SSD patients, only 29 (56%) eventually chose a hearing rehabilitation device (14 BAHD). Only one BAHD patient required a device explantation for skin complications, but then asked for reimplantation. Another one swapped the BAHD for HAs 2.5 years after. Two patients only occasionally used their BAHD with a headband. Nine patients preferred HAs, mainly BiCROS. Their contralateral hearing was significantly less than BAHD patients (p < 0.05), and only three used their HAs every day. Conclusions: Hearing rehabilitation in SSD patients after VS surgical resection is chosen in about 50% of cases. In complement of HINT, a real-life comparative hearing trial helps patients chose the best device, with good long-term results when a BAHD is chosen. HAs are preferred when contralateral hearing is altered but are not always worn. [ABSTRACT FROM AUTHOR]

Background/Objectives: Obstructive hydrocephalus associated with vestibular schwannoma (VS) is the most common in giant VS. Despite tumor removal, some patients may require ongoing ventriculo-peritoneal (VP) surgery. This investigation explores the factors contributing to the requirement for VP surgery following VS surgery in instances of persistent hydrocephalus (HCP). Methods: Volumetric MRI analyses of pre- and postoperative tumor volumes, cerebellum, cerebrum, ventricle system, fourth ventricle, brainstem, and peritumoral edema were conducted using Brainlab Smartbrush and 3D Slicer. The total brain volume was defined as the sum of the cerebrum, cerebellum, and brainstem. ROC analyses were performed to identify the optimum cut-off values of the volumetric data. Results: Permanent cerebrospinal fluid (CSF) diversion after surgery was indicated in 12 patients (12/71; 16.9%). The ratio of baseline volume fraction of brain ventricles to total brain ventricle volume (VTB ratio) was found to predict postoperative VP shunt dependency. The AUC was 0.71 (95% CI: 0.51–0.91), and the optimum threshold value (

Cancer-associated fibroblast (CAF) composition within the same organ varies across different cancer subtypes. Distinct CAF subtypes exhibit unique features due to interactions with immune cells and the tumor microenvironment. However, data on CAF subtypes in individuals with vestibular schwannoma (VS) are lacking. Therefore, we aimed to distinguish CAF subtypes at the single-cell level, investigate how stem-like CAF characteristics influence the tumor immune microenvironment, and identify CAF subtype-specific metabolic reprogramming pathways that contribute to tumor development. Data were analyzed from three patients with VS, encompassing 33,081 single cells, one bulk transcriptome cohort, and The Cancer Genome Atlas Pan-Cancer database (RNA sequencing and clinical data). Our findings revealed that antigen-presenting CAFs are linked to substantially heightened immune activity, supported by metabolic reprogramming, which differs from tumorigenesis. High expression of the stem-like CAF gene signature correlated with poor prognosis in low-grade gliomas within the pan-cancer database. This is the first study to classify CAF subtypes in VS patients and identify a therapeutic vulnerability biomarker by developing a stem-like CAF gene signature. Personalized treatments tailored to individual patients show promise in advancing precision medicine. [ABSTRACT FROM AUTHOR]

Background: Facial nerve paralysis is a severe dysfunction after vestibular schwannoma (VS) surgery. Methods: This monocentric study analyzed 61 patients who underwent sporadic VS surgery in a standardized manner. The primary endpoint was the facial nerve outcome (FNO) at 3 months after VS surgery. FNO was dichotomized into "good" (House–Brackmann (HB) score ≤ 2) and "poor" (HB > 2). Results: Poor FNO was observed in 11 patients (18.0%) at 3 months after VS surgery. Radiomic tumor shape features were analyzed, and the AUC of elongation in the prediction of a poor HB at 3 months was 0.70 (95% CI: 0.56–0.85, p = 0.03) and the optimum threshold value (≤/>0.35) yielded a sensitivity and specificity of 64.0% and 75.4%, respectively. Multivariable logistic regression analyses considering the extent of resection (0.35) revealed that more elongated VSs (≤0.35; OR: 5.8; 95%CI: 1.2–28.2; p = 0.03) and those with an increased EoR (≥93.4%; OR: 6.5; 95%CI: 1.0–42.5; p = 0.05) are independently associated with poorer FNO at 3 months after surgery. Conclusions: Highly elongated VS shape seems to be a risk factor for worsened facial nerve outcome at 3 months after surgery for Koos grade 3 and 4 tumors. [ABSTRACT FROM AUTHOR]

Background: A vestibular schwannoma (VS) is a benign tumor, causing audiological and vestibular symptoms. This study aimed to propose a comprehensive audio-vestibular test battery protocol for diagnosis and follow-up monitoring in patients with unilateral VSs undergoing surgical removal. Methods: The detailed interpretation of audiological and vestibular findings was presented in two example cases. The surgery was performed through the middle cranial fossa (#1) and translabyrinthine approach (#2). The participants were evaluated with tonal, speech, and impedance audiometry, ABR, caloric test, vHIT, cVEMP, oVEMP, SOT, and DHI. Patient and tumor characteristics were retrieved from the patient's history. Results: In the postoperative period, the reduction in gain of the lateral semicircular canal was observed in the vHITs of both patients. The DHI in case #1 increased after surgery, while it decreased in case #2. The improvement in postural performances compared to the preoperative SOT (CON 5, CON 6, composite score) and immediately after the procedure was observed. Conclusions: A specific diagnostic protocol is necessary to compare the results of different surgical techniques and approaches. Diagnostic tests performed before the surgery should be repeated within a specific time frame during postoperative follow-up to enable the comparison of results. The proposed protocol can help us better understand the processes ongoing during tumor growth and postoperative vestibular compensation. [ABSTRACT FROM AUTHOR]

Simple Summary: The mechanisms underlying the size progression of vestibular schwannomas remain poorly understood. Accordingly, this study was focused on investigating the potential role of messenger substances in regulating the growth of these tumors. These cytokines are produced by the tumor cells themselves or by other cells, such as immune cells. The influence of the cytokines on the size progression of tumors could facilitate the development and establishment of drug therapies for vestibular schwannoma patients, which are currently unavailable for sporadic vestibular schwannomas. Sporadic vestibular schwannomas (VSs) often exhibit slow or negligible growth. Nevertheless, some VSs increase significantly in volume within a few months or grow continuously. Recent evidence indicates a role of inflammation in promoting VS growth. Therefore, our study aimed to identify cytokines, which are associated with larger VSs. The expression of different cytokines in VS tumor samples and VS primary cultures was investigated. Additionally, the concentration of cytokines in cell culture supernatants of VS primary cultures and cerebrospinal fluid (CSF) of VS patients and healthy controls were determined. Correlation analysis of cytokine levels with tumor volume, growth rate, Koos grade, age, and hearing was examined with Spearman's-rank test. The mRNA expression of CC-chemokine ligand (CCL) 18, growth differentiation factor (GDF) 15, and interferon regulatory factor 4 correlated positively with tumor volume. Moreover, the amount of GDF15 in the cell culture supernatant of primary cells correlated positively with tumor volume. The concentrations of the cytokines CCL2, CCL5, and CCL18 and transforming growth factor beta (TGFB) 1 in the CSF of the patients were significantly different from those in the CSF controls. Inhibition of immune cell infiltration could be a putative approach to prevent and control VS growth. [ABSTRACT FROM AUTHOR]

Objective To investigate the imaging diagnosis and differential diagnosis methods for common tumors in the posterior fossa of children, and to propose a flow chart for the diagnosis of common tumors in the posterior fossa of children based on the limited diffusion of DWI according to the idea localization diagnosis followed by qualitative diagnosis. Methods A total of 118 pediatric patients with posterior fossa tumors who underwent surgical resection in Huashan Hospital, Fudan University from January 2021 to January 2024 were enrolled, and all of them underwent head CT and MRI examinations, and preoperative localization diagnosis and qualitative diagnosis were carried out accordingly. Then, based on whether DWI was diffusion limited, a flow chart for the diagnosis of common tumors in the posterior fossa of children was proposed. Results A total of 118 children with posterior fossa tumors were localization and qualitatively diagnosed, including 41 cases of fourth ventricular tumors, including medulloblastoma (27 cases), pilocytic astrocytoma (7 cases), ependymoma (5 cases), choroid plexus papilloma (one case), and rosette-forming glioneuronal tumor (RGNT, one case). There were 38 cases of brainstem tumors, including 24 cases of diffuse midline glioma, H3 K27 - altered, 5 cases of pilocytic astrocytoma, 3 cases of cavernous hemangioma, 2 cases of pediatric-type diffuse high-grade glioma (prone to diffuse midline glioma, H3 wild type), 2 cases of ganglio glioma, one case of atypical teratoid/rhabdoid tumor (AT/RT), and one case of pediatric - type diffuse low - grade glioma. There were 9 cases of cerebellopontine angle (CPA) tumors, including 3 cases of pilocytic astrocytoma, 2 cases of cholesteatoma, one case of medulloblastoma, one case of diffuse midline glioma, H3 K27-altered, one case of pilomyxoid astrocytoma, and one case of Ewing sarcoma. There were 30 cases of cerebellar tumors, including 15 cases of pilocytic astrocytoma, 7 cases of medulloblastoma, 2 cases of cavernous hemangioma, one case of ependymoma, one case of pediatric-type diffuse low-grade glioma, one case of pediatric-type diffuse high- grade glioma (prone to diffuse midline glioma, H3 wild type), one case of dysembryoplastic neuroepithelial tumor (DNT), one case of hamartoma, and one case of extrarenal rhabdomyomatoid tumor cerebellar metastases. Localization diagnosis, the accuracy of preoperative CT was 93.22% (110/118), while the accuracy of preoperative MRI was 100% (118/118). Qualitative diagnosis, 74 cases (62.71%) had accurate qualitative diagnosis, 23 cases (19.49%) had general diagnosis, and 21 cases (17.80%) had wrong diagnosis. The imaging differential diagnosis of posterior fossa tumors in children focuses on medulloblastoma, pilocytic astrocytoma, ependymoma, and diffuse midline glioma, H3 K27-altered. The incidence of limited diffusion of DWI in medulloblastoma, pilocytic astrocytoma and ependymoma outside the brainstem was 100% (35/35), 4% (1/25) and 5/6, and the difference among the three tumors was statistically significant (Z = - 5.601, P = 0.000). The incidence of limited diffusion of DWI in diffuse midline glioma, H3 K27- altered and pilocytic astrocytoma in the brainstem was 79.17% (19/24) and 1/5, and the difference between the two tumors was also statistically significant (Fisher's exact possibility: P = 0.038). Conclusions The early diagnosis of posterior fossa tumors in children is difficult, and DWI is an important basis for differential diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Background Vestibular schwannomas (VSs) are benign tumors of the vestibulocochlear nerve that often cause significant neurological and functional impairment. Patient-reported outcomes, including quality of life (QoL), are essential for understanding the overall impact of VS and its treatment. This study aimed to translate and culturally adapt the Vestibular Schwannoma Quality of Life (VSQOL) Index into German to expand its relevance to German-speaking populations. Methods We used a qualitative approach including translation and cognitive interviews with 10 patients who underwent VS surgery. The translation process followed the TRAPD protocol to ensure linguistic and conceptual accuracy. Cognitive interviews assessed the comprehensibility and relevance of the translated questionnaire. Results The translation showed remarkable consistency between translators, with minor discrepancies resolved by consensus. Cognitive interviews provided valuable insights that led to refinements in item wording. Participants emphasized the importance of an additional item on physician referrals, reflecting differences in health care systems between the United States and Germany. Conclusions The German VSQOL provides a comprehensive tool for assessing QoL in patients with VS that integrates patient-centered dimensions. A Validation study is underway to establish its reliability and validity.

Background: Vestibular schwannomas (VS) are slowly growing tumor, deriving from the vestibular component of cranial nerve VIII. Primary treatment modalities include microsurgery, radiosurgery, a combination of microsurgery and radiosurgery, and conservative treatment. The management of VS is a matter of debate, particularly in case of small tumors (less than 2 cm in diameter size). Methods: We performed an institutional retrospective review of a consecutive series of 28 surgically treated patients suffering from VS, operated in our institution. Patients were assigned on the ground of the preoperative imaging in two groups: tumors with major diameter of extra-meatal portion measuring ≥ 3 cm (Group I, 16 patients) and tumors with major diameter < to 3 cm (Group II, 12 patients). Results: The total amount of 18/28 patients were females; the average age was 60.1 years. The average duration of the preoperative symptoms was 25.2 months, while the average interval between the diagnosis and surgical treatment was 8.68 months. The average preoperative volume was, respectively, for Group I and II tumors of 14.35 cm3 and 3.73 cm3. Age was associated to the duration of the preoperative symptoms (r = − 364; p = 0.044), with the probability to develop a complication (p = 0.031) and preoperative low HB score (r = − 324; p = 0.040). The presence of hypoacusis as preoperative symptom was strongly associated to a lesser postoperative maximum diameter and smaller residual disease (p = 0.014). Group I had a significantly longer hospitalization period in respect to Group II (p = 0.001) that impacted negatively to postoperative performance status (r = 0.368, p = 0.042). Conclusions: We confirmed the strong association between age and size of the lesion and the clinical results, where larger lesions in older patients are more prone to complications than smaller in young patients. We found that the patients presenting hypoacusis at onset were associate to lesser preoperative FN impairment outlining a possible differential involvement of the CN related to the internal meatus. We also introduce the strong association of Koos grade with postoperative performance status, hospitalization and facial nerve functionality. [ABSTRACT FROM AUTHOR]

Background: Vestibular schwannomas (VSs) are benign tumors of the vestibulocochlear nerve that often cause significant neurological and functional impairment. Patient-reported outcomes, including quality of life (QoL), are essential for understanding the overall impact of VS and its treatment. This study aimed to translate and culturally adapt the Vestibular Schwannoma Quality of Life (VSQOL) Index into German to expand its relevance to German-speaking populations. Methods: We used a qualitative approach including translation and cognitive interviews with 10 patients who underwent VS surgery. The translation process followed the TRAPD protocol to ensure linguistic and conceptual accuracy. Cognitive interviews assessed the comprehensibility and relevance of the translated questionnaire. Results: The translation showed remarkable consistency between translators, with minor discrepancies resolved by consensus. Cognitive interviews provided valuable insights that led to refinements in item wording. Participants emphasized the importance of an additional item on physician referrals, reflecting differences in health care systems between the United States and Germany. Conclusions: The German VSQOL provides a comprehensive tool for assessing QoL in patients with VS that integrates patient-centered dimensions. A Validation study is underway to establish its reliability and validity. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: The exclusive endoscopic transcanal transpromontorial approach (EETTA) has recently been developed for the removal of small‐sized acoustic neuromas in the labyrinth (intralabyrinthine schwannoma [ILS]) or internal auditory canal (IAC). Although small tumors that meet the indications for EETTA are also good candidates for cochlear implantation (CI), there are few reports on CI after schwannoma removal using EETTA. Here we present an outcome of patients who underwent simultaneous EETTA and CI. Methods: Five patients (two with IAC fundus tumors and three with ILS) who underwent simultaneous EETTA and CI between 2020 and 2022 were retrospectively enrolled. Their medical charts and test results were reviewed. Results: After at least 12 months of follow‐up, there were no severe surgical complications such as meningitis, infection, or skin necrosis. Four of the five patients responded to auditory stimulation. Three out of four auditory‐responsive patients scored >80% on sentence recognition. Conclusion: Simultaneous EETTA and CI are feasible for the treatment of ILS and IAC fundus tumors. Preservation of the cochlear nerve and modiolus is important for favorable CI outcomes. Therefore, ILS and IAC fundus tumors in patients with nonserviceable hearing should be surgically removed as early as possible to enable proper hearing rehabilitation with CI. Level of Evidence: Level 4. [ABSTRACT FROM AUTHOR]

Background: Treponema pallidum can invade the central nervous system (CNS) early in its infection, causing neurosyphilis. Neurosyphilis typically presents with meningovasculitis in the acute or subacute phase, while tabes dorsalis and dementia paralytica are classical conditions in the later stages. However, syphilis is often misdiagnosed as other conditions such as tumors or autoimmune diseases including vasculitis and encephalitis, which is why the condition is known as "The Great Mimicker." The increasing incidence of syphilis in recent years emphasizes the importance of early diagnosis and treatment; however, its multiple clinical manifestations impose diagnostic challenges for clinicians because it resembles other diseases. In this case series, we present the impressive manifestations of neurosyphilis through three unique radiological presentations. Case presentation: Case 1 details optic nerve involvement in an HIV-positive male, where MRI and fundoscopic findings confirmed syphilitic optic neuritis. Case 2 describes a patient in her pregnancy initially suspected of acoustic neuroma on MRI, later diagnosed with syphilitic gumma affecting the inner ear canal. Case 3 is a young male with clinical features mimicking temporal arteritis, ultimately identified as skull osteomyelitis secondarily causing inflammation of the musculus temporalis and meningitis. Conclusions: These cases underscore the necessity of considering syphilis in differential diagnoses, given the diversity of its clinical presentations. Radiology plays an important role in avoiding unnecessary interventions. The increasing prevalence of recurrent syphilis imposes diagnostic challenges, emphasizing the importance of the early diagnosis and treatment of neurosyphilis by clinicians. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Quick and appropriate diagnostics and the use of intraoperative monitoring (IM) of hearing during vestibular schwannoma (VS) resection increase the likelihood of hearing preservation. During surgery, various methods of IM can be used, i.e., auditory brainstem responses (ABRs), transtympanic electrocochleography (TT-ECochG), and direct cochlear nerve action potentials. The aim of the study was to evaluate the prognostic values of IM of hearing using ABR and TT-ECochG in predicting postoperative hearing preservation and to evaluate relationships between them during various stages of surgery. Methods: This retrospective study presents the pre- and postoperative audiological test results and IM of hearing records (TT-ECochG and ABR) in 75 (43 women, 32 men, aged 18–69) patients with diagnosed VS. Results: The preoperative pure tone average hearing threshold was 25.02 dB HL, while after VS resection, it worsened on average by 30.03 dB HL. According to the American Academy of Otolaryngology–Head and Neck Surgery (AAO—HNS) Hearing Classification, before and after (pre/post) surgery, there were 47/24 patients in hearing class A, 9/8 in B, 2/1 in C, and 17/42 in D. In speech audiometry, the average preoperative speech discrimination score at an intensity of 60 dB SPL was 70.93%, and after VS resection, it worsened to 38.93%. The analysis of electrophysiological tests showed that before the tumor removal the I–V ABR interlatencies was 5.06 ms, and after VS resection, it was 6.43 ms. Conclusions: The study revealed correlations between worse postoperative hearing and changes in intraoperatively measured ABR and TT-ECochG. IM of hearing is very useful in predicting postoperative hearing in VS patients and increases the chance of postoperative hearing preservation in these patients. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the effectiveness of intensive customized vestibular rehabilitation after vestibular schwannoma (VS) excision. Methods: 52 patients who underwent VS removal via a translabyrinthine approach from 2020 to 2022 were involved in this study. Bedside examination, video head impulse test (vHIT), functional head impulse test (fHIT), and the dizziness handicap inventory (DHI) were performed before and after the rehabilitation, which consisted of 10 sessions of specifically designed vestibular, visual, and physical integrated training. Results: After rehabilitation, the vHIT showed overall unchanged values on the affected and healthy side. In contrast, the scores of fHIT, which explores the higher connection of the vestibular system with visual and cerebellar pathways, improved on both the pathological and healthy sides after training (p-value 0.004 and 0.000, respectively). The effectiveness of the rehabilitation was reinforced by the DHI scores, which were considerably lower after training. Conclusions: To our knowledge, this is the first study to explore fHIT outcomes after removal of VS, estimating the impact of rehabilitation on the overall compensation process. The outcomes support the role of extensive postsurgical rehabilitation in the compensatory process, even just a few days after surgery. [ABSTRACT FROM AUTHOR]

Background/Objectives: Surgical resection of medium to large vestibular schwannomas (VSs, Koos grade 3 and 4) is a widely used approach, although stereotactic radiosurgery (SRS) is increasingly proposed as initial treatment. The quality of life-centered approach is challenged in cases where tumor growth control cannot be achieved with SRS, thus necessitating salvage surgery. We present a series of eight consecutive patients who required surgery due to continued tumor growth after SRS. Methods: Of the 146 patients with VS grades 3 and 4 initially treated with SRS, only eight patients (mean age, 54 ± 7.2 years; range, 42–63 years) required subsequent surgery. Their mean tumor volume was 9.9 ± 3.2 cm3. The mean time from SRS to first tumor progression and planned subtotal resection was 23 ± 5.9 months and 45 ± 17.5 months, respectively. SRS was not performed after the surgery in favor of a "wait and rescan" approach. Tumor residue was monitored on follow-up magnetic resonance imaging. In all patients, tumor growth control after planned subtotal resection was maintained at 63 ± 19.8 months. Results: None of the 146 patients had serious complications after SRS. In the eight patients who required surgery, tumor growth between 22% and 212% (mean, 4 cm3) was reported within 26 to 84 months after SRS. Before salvage surgery, they scored 1 point on the House–Brackmann scale. Subtotal excision was performed, and VIIth nerve function was preserved in all patients. At 63 ± 19.8 months, 3 patients had a House–Brackmann score of 1, four patients had a score of 2, and one patient had a score of 3. Conclusions: Surgical excision of medium to large VS after SRS can be relatively safe, provided that a quality of life-centered approach of subtotal resection is used. [ABSTRACT FROM AUTHOR]

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 I2 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. [ABSTRACT FROM AUTHOR]

This study examined the association between hearing loss in sporadic vestibular schwannoma patients and the proteome of perilymph (PL), cerebrospinal fluid (CSF), and vestibular schwannoma. Intraoperative sampling of PL and of CSF, and biopsy of vestibular schwannoma tissue, was performed in 32, 32, and 20 patients with vestibular schwannoma, respectively. Perilymph and CSF in three patients with meningioma and normal hearing were also sampled. The proteomes were identified by liquid chromatography coupled to high-resolution tandem mass spectrometry. Preoperative hearing function of the patients was evaluated with pure tone audiometry, with mean values at frequencies of 500, 1000, 2000, and 4000 Hz (PTA4) in the tumor-affected ear used to delineate three hearing groups. Analysis of the PL samples revealed significant upregulation of complement factor H-related protein 2 (CFHR2) in patients with severe to profound hearing loss after false discovery rate correction. Pathway analysis of biofunctions revealed higher activation scores in the severe/profound hearing loss group of leukocyte migration, viral infection, and migration of cells in PL. Upregulation of CFHR2 and activation of these pathways indicate chronic inflammation in the cochlea of vestibular schwannoma patients with severe to profound hearing loss compared with patients with normal hearing or mild hearing loss. [ABSTRACT FROM AUTHOR]

The authors present the case of a young boy who experienced progressive unilateral hearing loss initially believed to be unrelated to any other medical condition. Methods: The patient received a thorough evaluation, which included a comprehensive battery of audiological tests, a CT scan, and a gadolinium-enhanced MRI. Results: A repeated imaging investigation revealed the presence of a mass that mimicked a vestibular schwannoma (VS), but despite this, the boy was ultimately diagnosed with cerebral manifestations of B-cell acute lymphoblastic leukemia (B-ALL). Conclusions: Cerebral lesions originating from the internal auditory canal are rare in cases of B-ALL. In this case, the initial signs and symptoms of the disease were solely related to the audiovestibular system, making the diagnostic process particularly complicated. Unilateral hearing loss cases may indicate the presence of potentially life-threatening conditions, even if the hearing loss appears to be clinically non-syndromic. For these reasons, unilateral hearing losses necessitate a comprehensive interdisciplinary diagnostic approach from the very start of auditory manifestation and, in particular, if the hearing impairment demonstrates threshold progression. [ABSTRACT FROM AUTHOR]

Preservation of function is an important goal during surgical management of cochleovestibular schwannomas. We here demonstrate the relief of vertigo and the preservation of function of all five vestibular receptors after removal of an intracochlear schwannoma with extension to the fundus of the internal auditory canal. A 61-year-old male with a five-year history of left-sided deafness, tinnitus, vertigo attacks, and an MRI consistent with an intracochlear schwannoma with limited extension through the modiolus to the fundus of the internal auditory canal (IAC) underwent transcanal, transcochlear total tumor removal and—due to a cerebrospinal fluid leak from the fundus of the IAC—revision surgery with lateral petrosectomy and blind sac closure of the external auditory canal. Despite complete removal of the cochlear partition of the inner ear (total cochlectomy), the patient's vestibular receptors remained functional, and the vertigo symptoms disappeared. These results show that vestibular labyrinthine function may not only be preserved after partial or subtotal cochlectomy but also after complete cochlear removal. This further confirms the vestibular labyrinth's robustness and encourages surgical management of transmodiolar schwannomas with limited extension to the fundus of the IAC. [ABSTRACT FROM AUTHOR]

Vestibular schwannoma can cause vestibular dysfunction; however, conflicting evidence exists regarding whether this affects the incidence of fall-related injuries in this patient population. This matched cross-sectional and cohort study assess the risk of fall-related injuries in patients with vestibular schwannoma. The study included patients with vestibular schwannoma treated at a tertiary referral hospital in Sweden between 1988 and 2014. Information on fall-related injuries was obtained from the National Patient Register, and matched population comparisons were randomly selected in a 1:25 ratio. Fall-related injuries occurring pre- (within 5 years before the diagnosis of vestibular schwannoma) and post-diagnostically (up to 3 years after diagnosis or intervention) were registered. The association between vestibular schwannoma and fall-related injuries was estimated using logistic regression and Cox proportional hazards analyses. We identified 1153 patients with vestibular schwannoma (569 [49%] women and 584 [51%] men), and 28815 population comparisons. Among the patients, 9% and 7% had pre- and post-diagnostic fall-related injuries, respectively, and among the comparisons, 8% and 6% had pre- and post-diagnostic fall-related injuries, respectively. There was no increased risk of pre- (OR 1.14; CI 0.92–1.41) or post-diagnostic 1 year (HR 1.16; CI 0.87–1.54) or 3 years (HR 1.11; CI 0.89–1.29) fall-related injury among the total patient cohort. In age-stratified analyses, we found an increased risk of pre-diagnostic fall-related injury among patients aged 50–69 years (OR 1.42; CI 1.10–1.88). Patients who underwent middle fossa surgery, regardless of age, had an increased risk of post-surgery fall-related injury within 3 years of follow-up (HR 2.68; CI 1.06–6.81). We conclude that patients with vestibular schwannoma have a low risk of enduring fall-related injuries. Middle-aged patients with dizziness and fall-related injuries should be considered for a vestibular clinical evaluation. Our results highlight the importance of rehabilitation in avoiding future fall-related injuries among patients undergoing middle fossa surgery. [ABSTRACT FROM AUTHOR]

Mammalian inner ear hair cell loss leads to permanent hearing and balance dysfunction. In contrast to the cochlea, vestibular hair cells of the murine utricle have some regenerative capacity. Whether human utricular hair cells regenerate in vivo remains unknown. Here we procured live, mature utricles from organ donors and vestibular schwannoma patients, and present a validated single-cell transcriptomic atlas at unprecedented resolution. We describe markers of 13 sensory and non-sensory cell types, with partial overlap and correlation between transcriptomes of human and mouse hair cells and supporting cells. We further uncover transcriptomes unique to hair cell precursors, which are unexpectedly 14-fold more abundant in vestibular schwannoma utricles, demonstrating the existence of ongoing regeneration in humans. Lastly, supporting cell-to-hair cell trajectory analysis revealed 5 distinct patterns of dynamic gene expression and associated pathways, including Wnt and IGF-1 signaling. Our dataset constitutes a foundational resource, accessible via a web-based interface, serving to advance knowledge of the normal and diseased human inner ear. Loss of inner ear hair cells leads to permanent hearing loss and balance dysfunction. Whether human utricular cells regenerate is unknown. Here, the authors present a single-cell resource of utricular cells from organ donors and schwannoma patients and describe transcriptional changes during homeostasis and in response to damage. [ABSTRACT FROM AUTHOR]

Background: Vestibular schwannoma (VS), also known as acoustic neuroma, is a benign, well-encapsulated, and slow-growing tumor that originates from Schwann cells, which form the myelin sheath around the vestibulocochlear nerve (VIII cranial nerve). The surgical treatment of this condition presents a challenging task for surgeons, as the tumor's location and size make it difficult to remove without causing damage to the surrounding structures. In recent years, fluorescein sodium (FS) has been proposed as a tool to enhance surgical outcomes in VS surgery. This essay will provide an analytical comparison of the use of FS in VS surgery, evaluating its benefits and limitations and comparing surgical outcomes with and without FS-assisted surgery. Methods: In a retrospective study conducted at San Filippo Neri Hospital, we examined VS cases that were operated on between January 2017 and December 2023. The patients were divided into two groups: group A, which consisted of patients who underwent surgery without the use of FS until January 2020 (102 cases), and group B, which included patients who underwent surgery with FS after January 2020 (55 cases). All operations were performed using the retrosigmoid approach, and tumor size was classified according to the Koos, et al. classification system. The extent of surgical removal was evaluated using both the intraoperative surgeon's opinion and postoperative MRI imaging. Preoperatively and postoperatively, facial nerve function and hearing were assessed. In group B, FS was used to assist the surgical procedures, which were performed using a surgical microscope equipped with an integrated fluorescein filter. Postoperative clinical and MRI controls were performed at six months and annually, with no patients lost to follow-up. Results: This study investigated the impact of intraoperative fluorescein exposure on tumor resection and clinical outcomes in patients with VS. The study found a statistically significant difference in the tumor resection rates between patients who received fluorescein intraoperatively (p = 0.037). Further analyses using the Koos classification system revealed a significant effect of fluorescein exposure, particularly in the Koos 3 subgroup (p = 0.001). Notably, no significant differences were observed in hearing loss or facial nerve function between the two groups. A Spearman correlation analysis revealed a positive correlation between tumor size and Koos, age, and size, but no significant correlation was found between facial nerve function tests. Conclusions: FS-assisted surgery for VS may potentially enhance tumor resection, allowing for more comprehensive tumor removal. [ABSTRACT FROM AUTHOR]

Background: Vestibular schwannoma (VS) is a benign tumor of the eighth cranial nerve formed from neoplastic Schwann cells. Although VS can cause a variety of symptoms, tinnitus is one of the most distressing symptoms for patients and can greatly impact quality of life. The objective of this systematic review is to comprehensively examine and compare the outcomes related to tinnitus in patients undergoing treatment for VS. Specifically, it evaluates patient experiences with tinnitus following the removal of VS using the various surgical approaches of traditional surgical resection and gamma knife radiosurgery (GKS). By delving into various aspects such as the severity of tinnitus post-treatment, the duration of symptom relief, patient quality of life, new onset of tinnitus after VS treatment, and any potential complications or side effects, this review aims to provide a detailed analysis of VS treatment on tinnitus outcomes. Methods: Following PRISMA guidelines, articles were included from PubMed, Science Direct, Scopus, and EMBASE. Quality assessment and risk of bias analysis were performed using a ROBINS-I tool. Results: Although VS-associated tinnitus is variable in its intensity and persistence post-resection, there was a trend towards a decreased tinnitus burden in patients. Irrespective of the surgical approach or the treatment with GKS, there were cases of persistent or worsened tinnitus within the studied cohorts. Conclusion: The findings of this systematic review highlight the complex relationship between VS resection and tinnitus outcomes. These findings underscore the need for individualized patient counseling and tailored treatment approaches in managing VS-associated tinnitus. The findings of this systematic review may help in guiding clinicians towards making more informed and personalized healthcare decisions. Further studies must be completed to fill gaps in the current literature. [ABSTRACT FROM AUTHOR]

Simple Summary: The hallmark of NF2 is bilateral vestibular schwannomas, which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. To better understand disease progression and characterize treatment response, we developed a panel of five tests suitable for the mouse vestibular schwannoma model and investigated how tumor growth and treatment affect gait, coordination, and motor function. These methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. NF2-related Schwannomatosis (NF2-SWN) is a disease that needs new solutions. The hallmark of NF2-SWN, a dominantly inherited neoplasia syndrome, is bilateral vestibular schwannomas (VSs), which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. Standard treatments for growing VSs include surgery and radiation therapy (RT); however, both carry the risk of further nerve damage that can result in deafness and facial palsy. The resultant suffering and debility, in combination with the paucity of therapeutic options, make the effective treatment of NF2-SWN a major unmet medical need. A better understanding of these mechanisms is essential to developing novel therapeutic targets to control tumor growth and improve patients' quality of life. Previously, we developed the first orthotopic cerebellopontine angle mouse model of VSs, which faithfully mimics tumor-induced hearing loss. In this model, we observed that mice exhibit symptoms of ataxia and vestibular dysfunction. Therefore, we further developed a panel of five tests suitable for the mouse VS model and investigated how tumor growth and treatment affect gait, coordination, and motor function. Using this panel of ataxia tests, we demonstrated that both ataxia and motor function deteriorated concomitantly with tumor progression. We further demonstrated that (i) treatment with anti-VEGF resulted in tumor size reduction, mitigated ataxia, and improved rotarod performance; (ii) treatment with crizotinib stabilized tumor growth and led to improvements in both ataxia and rotarod performance; and (iii) treatment with losartan did not impact tumor growth nor ameliorate ataxia or motor function. Our studies demonstrated that these methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type 2 (NF2)-related schwannomatosis is a rare autosomal dominant monogenic disorder caused by mutations in the NF2 gene. The hallmarks of NF2-related schwannomatosis are bilateral vestibular schwannomas (VS). The current treatment options for NF2-related schwannomatosis, such as observation with serial imaging, surgery, radiotherapy, and pharmacotherapies, have shown limited effectiveness and serious complications. Therefore, there is a critical demand for novel effective treatments. Gene therapy, which has made significant advancements in treating genetic diseases, holds promise for the treatment of this disease. This review covers the genetic pathogenesis of NF2-related schwannomatosis, the latest progress in gene therapy strategies, current challenges, and future directions of gene therapy for NF2-related schwannomatosis. [ABSTRACT FROM AUTHOR]

Abstract Background Tinnitus is very common in patients with vestibular schwannoma (VS). We analyzed the related factors of tinnitus after surgery. Methods One hundred seventy-three patients diagnosed with unilateral VS operated via the retrosigmoid approach were included in the study. All patients underwent relevant examinations and completed the THI scale before surgery and 6 months after surgery. The prognosis of tinnitus was evaluated according to the changes in THI. Results Of the 129 preoperative tinnitus patients, postoperative tinnitus resolved in 12.4%, improved in 29.5%, remained unchanged in 28.6%, and worsened in 29.5%. 18.2% of 44 patients without preoperative tinnitus appeared new-onset tinnitus postoperatively. Thirty-six patients never had tinnitus. Patients with smaller tumor sizes (≤ 3 cm) were more likely to experience preoperative tinnitus. Younger patients and those with serviceable hearing preoperatively were more likely to report their tinnitus unchanged or worsened. A new onset of postoperative tinnitus in the preoperative non-tinnitus group was found in better preoperative hearing function. Conclusions In this study, 70% of patients had persistent tinnitus after vestibular schwannoma resection. The prognosis of tinnitus was influenced by age and preoperative hearing function. Tinnitus is a bothersome symptom and is often underestimated by doctors. Assessment of tinnitus is mandatory during the management of vestibular schwannoma.

Vestibular Schwannoma (VS) is a benign nerve sheath tumors comprised of Schwann cells. This tumor is encapsulated, slow-growing, and originates from the internal auditory canal, extending into the cerebellopontine angle (CPA). The incidence in individuals aged 20-44 is 0.75 per 100,000 cases, with bilateral VS incidence of 0.8 per 50,000 cases. Tumors in CPA are the most common type in the posterior fossa and cause serious neurological symptoms or become life-threatening when tumors enlarge and compress the brainstem. The majority of tumors are VS (acoustic neuromas), accounting for 80%-90% of cases. Common clinical symptoms include hearing loss, tinnitus, and vertigo. Additionally, these tumors cause compression of the trigeminal and facial nerves. Advances in rapidly evolving imaging technology and surgical methods have improved diagnosis and management. A 24-year-old male complained of hearing impairment for the past 3 years alongside headaches, and dizziness leading to a feeling of imbalance, double, and blurry vision, as well as a sensation of facial thickness on the left side. Neurological examination showed cranial nerve abnormalities, including bilateral paresis of cranial nerves III, IV, VI, left cranial nerves V and VII, bilateral cranial nerve VIII, right cranial nerve XII, and cerebellar abnormalities such as intention tremor, dysmetria, dysdiadokokinesia, wide-based gait, and falling to the right during Romberg testing with both eyes open and closed. The patient underwent a contrast-enhanced MRI of the head, followed by a right CPA tumors excision through craniotomy. A detailed understanding of the medical history, physical examination, and radiological proved to be crucial in establishing an accurate diagnosis and appropriate management. This was considered essential to minimize a worse prognosis.

ObjectiveTo determine the usefulness of a personalized tool and its effect on the decision-making process for those with vestibular schwannoma (VS).Study designProspective study.SettingSingle institution, academic tertiary care lateral skull base surgery program.PatientsPatients diagnosed with VS.InterventionsA comprehensive clinical decision support (CDS) tool was constructed from a previously published retrospective patient-reported data obtained from members of the Acoustic Neuroma Association from January to March 2017. Demographic, tumor, and treatment modality data, including associated side effects, were collected for 775 patients and integrated in an interactive and personalized web-based tool.Main outcome measuresPre- and posttool questionnaires assessing the process of deciding treatment for VS using a decisional conflict scale (DCS) and satisfaction with decision (SWD) scale were compared.ResultsA pilot study of 33 patients evaluated at a single institution tertiary care center with mean ± SD age of 63.9 ± 13.5 years and with average tumor size of 7.11 ± 4.75 mm were surveyed. CDS implementation resulted in a mean ± SD total DCS score decrease from 43.6 ± 15.5 to 37.6 ± 16.4 ( p < 0.01) and total SWD score increase from 82.8 ± 16.1 to 86.2 ± 14.4 ( p = 0.04), indicating a significant decrease in decisional conflict and increase in satisfaction.ConclusionsImplementing a decision-making tool after diagnosis of VS reduced decisional conflict and improved satisfaction with decision. Patients considered the tool to be an aid to their medical knowledge, further improving their comfort and understanding of their treatment options. These findings provide a basis for developing predictive tools that will assist patients in making informed medical decisions in the future.

Automatic segmentation of vestibular schwannoma (VS) from routine clinical MRI has potential to improve clinical workflow, facilitate treatment decisions, and assist patient management. Previous work demonstrated reliable automatic segmentation performance on datasets of standardized MRI images acquired for stereotactic surgery planning. However, diagnostic clinical datasets are generally more diverse and pose a larger challenge to automatic segmentation algorithms, especially when post-operative images are included. In this work, we show for the first time that automatic segmentation of VS on routine MRI datasets is also possible with high accuracy. We acquired and publicly release a curated multi-center routine clinical (MC-RC) dataset of 160 patients with a single sporadic VS. For each patient up to three longitudinal MRI exams with contrast-enhanced T1-weighted (ceT1w) (n = 124) and T2-weighted (T2w) (n = 363) images were included and the VS manually annotated. Segmentations were produced and verified in an iterative process: (1) initial segmentations by a specialized company; (2) review by one of three trained radiologists; and (3) validation by an expert team. Inter- and intra-observer reliability experiments were performed on a subset of the dataset. A state-of-the-art deep learning framework was used to train segmentation models for VS. Model performance was evaluated on a MC-RC hold-out testing set, another public VS datasets, and a partially public dataset. The generalizability and robustness of the VS deep learning segmentation models increased significantly when trained on the MC-RC dataset. Dice similarity coefficients (DSC) achieved by our model are comparable to those achieved by trained radiologists in the inter-observer experiment. On the MC-RC testing set, median DSCs were 86.2(9.5) for ceT1w, 89.4(7.0) for T2w, and 86.4(8.6) for combined ceT1w+T2w input images. On another public dataset acquired for Gamma Knife stereotactic radiosurgery our model achieved median DSCs of 95.3(2.9), 92.8(3.8), and 95.5(3.3), respectively. In contrast, models trained on the Gamma Knife dataset did not generalize well as illustrated by significant underperformance on theMC-RC routineMRI dataset, highlighting the importance of data variability in the development of robust VS segmentation models. The MC-RC dataset and all trained deep learning models were made available online. [ABSTRACT FROM AUTHOR]

Background: Aging is a significant risk factor for many neurodegenerative diseases and neurological tumors. Previous studies indicate that the frailty index, facial aging, telomere length (TL), and epigenetic aging clock acceleration are commonly used biological aging proxy indicators. This study aims to comprehensively explore potential relationships between biological aging and neurodegenerative diseases and neurological tumors by integrating various biological aging proxy indicators, employing Mendelian randomization (MR) analysis. Methods: Two-sample bidirectional MR analyses were conducted using genome-wide association study (GWAS) data. Summary statistics for various neurodegenerative diseases and neurological tumors, along with biological aging proxy indicators, were obtained from extensive meta-analyses of GWAS. Genetic single-nucleotide polymorphisms (SNPs) associated with the exposures were used as instrumental variables, assessing causal relationships between three neurodegenerative diseases (Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis), two benign neurological tumors (vestibular schwannoma and meningioma), one malignant neurological tumor (glioma), and four biological aging indicators (frailty index, facial aging, TL, and epigenetic aging clock acceleration). Sensitivity analyses were also performed. Results: Our analysis revealed that genetically predicted longer TL reduces the risk of Alzheimer's disease but increases the risk of vestibular schwannoma and glioma (All Glioma, GBM, non-GBM). In addition, there is a suggestive causal relationship between some diseases (PD and GBM) and DNA methylation GrimAge acceleration. Causal relationships between biological aging proxy indicators and other neurodegenerative diseases and neurological tumors were not observed. Conclusion: Building upon prior investigations into the causal relationships between telomeres and neurodegenerative diseases and neurological tumors, our study validates these findings using larger GWAS data and demonstrates, for the first time, that Parkinson's disease and GBM may promote epigenetic age acceleration. Our research provides new insights and evidence into the causal relationships between biological aging and the risk of neurodegenerative diseases and neurological tumors. [ABSTRACT FROM AUTHOR]

Background: Our primary objective was to monitor nonprogressive unilateral vestibular schwannomas (VSs) to assess the efficiency of rapid bedside examinations, such as the video head impulse test (vHIT) and skull vibration-induced nystagmus test (SVINT), in identifying vestibular damage. Methods: An observational study was conducted from March 2021 to March 2022 on all adult patients (>18 years old) with a confirmed nonprogressive VS (no active treatment). The SVINT (using a 100 Hz vibrator with two (SVINT2) or three (SVINT3) stimulation locations) and vHIT (for the six semicircular canals (SCCs)) were performed on all patients. The asymmetry of function between the vestibules was considered significant when the gain asymmetry was greater than 0.1. Rapid and repeatable assessment of VSs using two- and three-stimulation SVINT plus vHIT was performed to quantify intervestibular asymmetry. Results: SVINT3 and SVINT2 triggered VIN in 40% (24/60) and 65% (39/60) of patients, respectively. There was significant asymmetry in the vestibulo-ocular reflex (VOR), as shown by a VS-side gain < healthy-side gain in 58% (35/60) of the patients. Among the patients with significant gain asymmetry between the two vestibules according to the vHIT (VS-side gain < healthy-side gain), the proportion of patients expressing vestibular symptomatology was significantly greater than that of patients without any symptoms [67% (29/43) vs. 35% (6/17), respectively; p = 0.047]. Conclusions: The SVINT2 can be combined with the vHIT to form an interesting screening tool for revealing vestibular asymmetry. This work revealed the superiority of mastoid stimulation over vertex stimulation for SVINT in patients with unilateral vestibular loss. [ABSTRACT FROM AUTHOR]

Intralabyrinthine schwannomas are a rare subgroup of vestibular schwannomas located within the membranous labyrinth of the inner ear and are known for their variable clinical presentations and symptoms. In the present study, we report on a patient with a persistent history of dizziness and positional vertigo, who was misdiagnosed with posterior canalithiasis. As hearing loss was not developed until late in the disease course, the patient was not properly diagnosed until magnetic resonance imaging revealed an intralabyrinthine schwannoma, which was not discovered on earlier imaging. In addition to the unusual clinical presentation, we describe the audio-vestibular profile of our patient. We suggest that a thorough vestibular evaluation, including caloric testing and a careful examination of the inner ear on imaging, is warranted in cases of treatment of refractory vertigo, even in patients where a diagnosis seems certain. [ABSTRACT FROM AUTHOR]

Purpose: India is rapidly adopting advanced treatments like Stereotactic Radiosurgery (SRS). However, there is a paucity of data on SRS practice. The aim of study is to assess the current status of technology and practices of machine quality assurance (QA) and patient specific quality assurance for SRS in India. Materials and Methods: A survey questionnaire was designed using Google Forms and sent to chief/senior medical physicists across 220 radiotherapy centers in India on July 15, 2022. It contained questions on infrastructure availability, treatment planning, and QA. Results: SRS was found to be extensively used for the treatment of brain metastases (99.3%), followed by meningioma (50.3%), acoustic neuroma (45.5%), and pituitary tumours (33.1%). The most commonly used photon energy and treatment technique were 6MV FFF and VMAT, respectively. A prescription isodose line ranging from 70% to 100% was selected by linac users. Most linac institutes verify pretreatment doses. There was a lack of uniformity in the analysis metrics such as Low Dose Threshold, Dose Difference, and Distance to Agreement. A survey revealed that the variety of SRS QA programs being followed at Indian radiotherapy centers. Conclusion: This is the first study to report the physics practice of SRS in India. The survey shows a need to carry out a postal dose audit for small static photon fields in India. [ABSTRACT FROM AUTHOR]

Background: Gamma Knife Radiosurgery (GKRS) has established a role in treating various benign brain pathologies. The radiosurgery planning necessitates a proper understanding of radiation dose distribution in relation to the target lesion and surrounding eloquent area. The quality of a radiosurgery plan is determined by various planning parameters. Here, we have reviewed various GKRS planning parameters and analyzed their correlation with the morphology of treated brain lesions. Method: A total of 430 treatment plans (71 meningioma, 133 vestibular schwannoma/VS, 150 arteriovenous malformation/AVM, 76 pituitary adenoma/PA treated with GKRS between December 2013 and May 2023) were analyzed for target coverage (TC), conformity index (CI), homogeneity index (HI), and gradient index (GI). Result: The values of CIPaddick and CILomax for PA were lower and differed significantly from meningioma, VS, and AVM. The value of HI for PA was higher and differed significantly when compared with meningioma, VS, and AVM. The values of HI for AVM were also significantly higher than VS and meningioma. The mean GI was 3.02, 2.92, 3.03, and 2.88 for meningioma, VS, AVM, and PA, respectively. The value of GI for meningioma and AVM was significantly higher when compared with the values for VS and PA. The mean TC was 0.94 for meningioma, 0.96 for VS, 0.95 for AVM, and 0.90 for PA. The value TC of PA was lower and differed significantly when compared with VS, AVM, and meningioma. Lesions with a volume of ≤1 cc had poor planning metrics as the spillage of radiation may be higher. Conclusion: The GKRS planning parameters depend on the size, shape, nature, and location of intracranial lesions. Therefore, each treatment plan needs to be evaluated thoroughly and a long-term follow-up is needed to establish their relation with clinical outcome. [ABSTRACT FROM AUTHOR]

Simple Summary: In this study, we explored the use of bevacizumab as a treatment for NF2-related schwannomatosis, a condition characterized by the development of schwannomas on both vestibulocochlear nerves. This study revealed that the majority of patients experienced either improved or preserved hearing and effective control of the targeted vestibular schwannoma with bevacizumab. However, common adverse events included hypertension and fatigue, and severe adverse events led to treatment discontinuation in a quarter of the patients. Thus, while bevacizumab demonstrates positive effects on hearing, tumor control, and symptomatology in NF2-related schwannomatosis, careful consideration of potential side effects is crucial. (1) Background: NF2-related schwannomatosis, characterized by the development of bilateral vestibular schwannomas, often necessitates varied treatment approaches. Bevacizumab, though widely utilized, demonstrates variable effectiveness on hearing and tumor growth. At the same time, (serious) adverse events have been frequently reported. (2) Methods: A single center retrospective study was conducted, on NF2-related schwannomatosis patients treated with bevacizumab from 2013 to 2023, with the aim to assess treatment-related and clinical outcomes. Outcomes of interest comprised hearing, radiologic response, symptoms, and adverse events. (3) Results: Seventeen patients received 7.5 mg/kg bevacizumab for 7.1 months. Following treatment, 40% of the patients experienced hearing improvement, 53%, stable hearing, and 7%, hearing loss. Vestibular schwannoma regression occurred in 31%, and 69% remained stable. Further symptomatic improvement was reported by 41%, stable symptoms by 47%, and worsened symptoms by 12%. Treatment discontinuation due to adverse events was observed in 29% of cases. Hypertension (82%) and fatigue (29%) were most frequently reported, with no occurrences of grade 4/5 toxicities. (4) Conclusion: Supporting previous studies, bevacizumab demonstrated positive effects on hearing, tumor control, and symptoms in NF2-related schwannomatosis, albeit with common adverse events. Therefore, careful consideration of an appropriate management strategy is warranted. [ABSTRACT FROM AUTHOR]

Objective. Anticoagulant therapy is a risk factor for repeated intratumoral hemorrhage and acute enlargement of a vestibular schwannoma (VS) with neurological deficits. Therefore, we describe two cases of patients on oral anticoagulant therapy with intratumoral hemorrhage in which anticoagulant therapy prior to surgical resection was discontinued. We also discuss other similar cases from the literature since this is a rare event. Case Reports. We described the two cases of intratumoral hemorrhage in acoustic neurinoma and conducted a literature review of similar cases of patients with intratumoral hemorrhage in acoustic neurinoma who were also on oral anticoagulants. Both patients presented with CN-VII palsy prior to surgery; both also fully recovered after surgery except for hearing loss on the tumor side. Our literature review found 50 cases of VS (reported as vestibular schwannomas in the literature) with intratumoral hemorrhage. From this total, 11 patients used oral anticoagulant therapy with reported poor outcomes and high mortality; 9 of these 11 cases were reported in the past 20 years. The incidence is expected to rise due to increased use of anticoagulant therapy due to onset of atrial fibrillation, atherosclerosis, and thromboembolism from longer human lifespan. Conclusion. Anticoagulant therapy represents a risk factor for intratumoral hemorrhage and acute enlargement of VS tumor mass with neurological deficits. [ABSTRACT FROM AUTHOR]

Background: In order to better understand the pathophysiology of surgically induced hearing loss after vestibular schwannoma (VS) surgery, we postoperatively analyzed the hearing status in a series of patients where hearing was at least partially preserved. Methods: Hearing was assessed through tonal audiometry, speech discrimination score, maximum word recognition score (dissyllabic word lists—MaxIS), otoacoustic emissions (OAEs), and auditory brainstem response (ABR). The magnetic resonance imaging (MRI) tumor characterization was also noted. Results: In a series of 24 patients operated on for VS over 5 years, depending on the results of this triple hearing exploration, we could identify, after surgery, patients with either a myelin alteration or partial damage to the acoustic fibers, others with a likely partial cochlear ischemia, and some with partial cochlear nerve ischemia. One case with persisting OAEs and no preoperative ABR recovered hearing and ABR after surgery. Long follow-up (73 ± 57 months) revealed a mean hearing loss of 30 ± 20 dB with a drastic drop of MaxIS. MRI revealed only 25% of fundus invasion. Conclusion: a precise analysis of hearing function, not only with classic audiometry but also with ABR and OEAs, allows for a better understanding of hearing damage in VS surgery. [ABSTRACT FROM AUTHOR]

This multicenter retrospective cohort study aimed to evaluate the effectiveness of the retrosigmoid surgical approach in decompressing vestibular schwannomas, focusing on tumor decompression, neurological function preservation, and postoperative complications. A cohort of 60 patients, operated between 2016 and 2019, was analyzed for age, sex, symptoms, tumor size, surgery duration, complications, mortality, and facial/auditory functions using established criteria. Hearing loss was observed in 80% of patients, mainly progressive, with tumor size emerging as a critical prognostic factor. Facial weakness affected 10% of patients preoperatively; postoperatively, 35% of patients had affected facial nerve function, with 10% exhibiting poor or no facial nerve function, linked to resection extent rather than tumor size. Tinnitus was more prevalent with larger tumors, whereas headaches were common irrespective of size. Balance disorders improved after surgery, especially in case of larger tumors. Functional recovery varied, with 41.67% of patients returning to their previous activity within 4 months, 25% within 4-12 months, and 33.33% remaining inactive. The mortality rate was low at 3.3 %, with two deaths out of 60 patients after surgery. This analysis highlights surgery risks for vestibular schwannomas (e.g., facial nerve decline, tinnitus, headaches), but also emphasizes benefits like improved balance and low mortality. Many patients regain professional activity, stressing the importance of informed treatment decisions for this condition. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to identify significant prognostic factors associated with facial paralysis after vestibular schwannoma (VS) surgery and develop a novel nomogram for predicting facial nerve (FN) outcomes. Methods: Retrospective data were retrieved from 355 patients who underwent microsurgery via the retrosigmoid approach for VS between December 2017 and December 2022. Univariate and multivariate logistic regression analysis were used to construct a radiographic features‐based nomogram to predict the risk of facial paralysis after surgery. Results: Following a thorough screening process, a total of 185 participants were included. The univariate and multivariate logistic regression analysis revealed that tumor size (p = 0.005), fundal fluid cap (FFC) sign (p = 0.014), cerebrospinal fluid cleft (CSFC) sign (p < 0.001), and expansion of affected side of internal auditory canal (IAC) (p = 0.033) were independent factors. A nomogram model was constructed based on these indicators. When applied to the validation cohort, the nomogram demonstrated good discrimination and favorable calibration. Then we generated a web‐based calculator to facilitate clinical application. Conclusion: Tumor size, FFC and CSFC sign, and the expansion of the IAC, serve as good predictors of postoperative FN outcomes. Based on these factors, the nomogram model demonstrates good predictive performance. [ABSTRACT FROM AUTHOR]

Background: Tinnitus is very common in patients with vestibular schwannoma (VS). We analyzed the related factors of tinnitus after surgery. Methods: One hundred seventy-three patients diagnosed with unilateral VS operated via the retrosigmoid approach were included in the study. All patients underwent relevant examinations and completed the THI scale before surgery and 6 months after surgery. The prognosis of tinnitus was evaluated according to the changes in THI. Results: Of the 129 preoperative tinnitus patients, postoperative tinnitus resolved in 12.4%, improved in 29.5%, remained unchanged in 28.6%, and worsened in 29.5%. 18.2% of 44 patients without preoperative tinnitus appeared new-onset tinnitus postoperatively. Thirty-six patients never had tinnitus. Patients with smaller tumor sizes (≤ 3 cm) were more likely to experience preoperative tinnitus. Younger patients and those with serviceable hearing preoperatively were more likely to report their tinnitus unchanged or worsened. A new onset of postoperative tinnitus in the preoperative non-tinnitus group was found in better preoperative hearing function. Conclusions: In this study, 70% of patients had persistent tinnitus after vestibular schwannoma resection. The prognosis of tinnitus was influenced by age and preoperative hearing function. Tinnitus is a bothersome symptom and is often underestimated by doctors. Assessment of tinnitus is mandatory during the management of vestibular schwannoma. [ABSTRACT FROM AUTHOR]

Neurilemmoma, also known as Schwannoma, is a benign, slow-growing, encapsulated neoplasm that arises from Schwann cells of the peripheral nerve sheath. Although it is extremely rare, it affects the head and neck region in roughly 25-45% of all cases. Intraorally, the tongue is the most common site and is rarely involves vestibular mucosa. Here, we report a rare case of Schwannoma of vestibular mucosa in a 13-year-old girl and contribute a review to the current literature. Clinically, based on age, site, and appearance, a diagnosis of a benign tumour such as lipoma, fibroma, neurofibroma, and benign lesions of salivary glands was made. An excisional biopsy showed solely Antoni type A tissue with central acellular eosinophilic Verocay bodies surrounded by spindle-shaped neurilemma cells arranged compactly with wavy, twisted nuclei arranged in a palisaded manner. The patient was recurrence-free after 6 months. Hence, this case is of interest due to its rarity in terms of age, site, and histopathology (Antoni type A neurilemmoma) for a presumed initial diagnosis. Here, we also hypothesize regarding the type of growth pattern in the earlier diagnosed cases. [ABSTRACT FROM AUTHOR]

Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select patients with progressing unilateral VS and good hearing (AAO-HNS class A) treated with stereotactic gamma knife surgery (GKS). Their audiograms were analyzed along with treatment metrics and patient data. Results: Hearing outcomes with a median follow-up of 5 years post-treatment showed statistically significant loss of serviceable hearing: 34.1% of patients maintained good hearing (AAO-HNS class A), and 56.1% maintained serviceable hearing (AAO-HNS class A and B). Non-hearing outcomes are favorable with excellent tumor control and low facial nerve morbidity. Conclusions: Hearing declines over time in intracanalicular VS treated with GKS, with a significant loss of serviceable hearing after 5 years. The mean cochlear dose and the presence of cochlear aperture obliteration by the tumor are the main statistically significant factors involved in the hearing outcomes. [ABSTRACT FROM AUTHOR]

Background: Radiotherapy (RT) plays an important role in the therapeutic management of vestibular schwannoma (VS). Fractionated stereotactic radiotherapy (FSRT) or radiosurgery (SRS) are the two modalities available. The purpose of this article is to review the results of VS RT studies carried out over the last ten years. Materials and Methods: A literature search was performed with PubMed and Medline by using the words vestibular schwannoma, acoustic neuroma, radiotherapy, and radiosurgery. Results: In small (<3 cm) VS, SRS offers a local control rate of >90%, which seems similar to microsurgery, with a favorable tolerance profile. Hypofractionated FSRT (three to five fractions) is a relatively recent modality and has shown similar outcomes to normofractionated FSRT. Hearing preservation may highly differ between studies, but it is around 65% at 5 years. Conclusions: SRS and FRST are non-invasive treatment options for VS. SRS is often preferred for small lesions less than 3 cm, and FSRT for larger lesions. However, no randomized study has compared these modalities. [ABSTRACT FROM AUTHOR]

Introduction: The middle cranial fossa (MCF) approach is a well-established procedure in surgery of the internal auditory canal, as well as with the retrosigmoid and translabyrinthine approaches. It is commonly used in the hearing-preserving microsurgery of small vestibular schwannomas (VS). The debate about the "best" approach for the microsurgery of small VS without contact to the brainstem is controversial. It has been stated that the MCF approach leads to irreversible damage to the temporal lobe, which may be evident in follow-up magnet resonance imaging (MRI) as gliosis in up to 70% of patients. Materials and Methods: This study represents a retrospective chart analysis conducted at a tertiary university hospital. Here, 76 postoperative MRIs were re-evaluated by an experienced neuroradiologist and compared with the preoperative images. Temporal lobe gliosis was classified on an ordinal scale as absent, slight, moderate or severe. Occurrence of gliosis was matched to the clinical predictors (tumor stage, tumor volume, sex, age, and side). Results: No case of severe or moderate gliosis was found in the patient group. Slight gliosis of the temporal lobe was rare and was only detected in four patients (5%). There was no relation between clinical predictors and the incidence of gliosis. Conclusions: In our cohort, postoperative MR imaging did not reveal relevant damage to the temporal lobe parenchyma. This confirms the safe concept of microsurgery of small tumors via the middle fossa approach. The severe glioses described in other studies may be caused by a forced insertion of the retractor or by more extended approaches. However, further prospective neurocognitive studies seem to be necessary in order to assess functional changes in the temporal lobe. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to investigate the perilymph metabolism by analyzing the 3D real IR MR findings in acoustic neuroma (AN) after intravenous administration of gadolinium (Gd). Methods: Eleven patients (6 men and 5 women) diagnosed with AN were included, and 3D real IR MRI was performed 4 hours after intravenous Gd injection. The signal intensity and details of inner ear, tumor, and internal auditory canal (IAC) by MRI were analyzed. Results: Four patients had tumors confined to the IAC, and 5 had tumors that extended to the cerebellopontine angle cistern. The signal intensity of the cochlea, vestibule, and IAC fundus was conspicuously enhanced in 3D real IR images than the control side. One patient had a tumor in the cochlea, in which the signal intensity of the semicircular canal and vestibule was increased. One patient had an intravestibular tumor in which the signal intensity of the semicircular canal was increased and the cochlea had endolymphatic hydrops in the affected ear. Conclusions: The synchronously increased signal intensity in the inner ear and IAC may indicate that IAC may serve as a channel for removal of the perilymph in the inner ear; the blockage by the tumor may have changed the hydrodynamics of the perilymph to cause a longer retention of Gd in the inner ear. [ABSTRACT FROM AUTHOR]