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Annals of Medicine and Surgery (2012) Dec 2023Vestibular schwannoma (VS) is a benign tumor that develops from Schwann cells of the eighth cranial pair, mainly in the cerebellopontine angle.
INTRODUCTION
Vestibular schwannoma (VS) is a benign tumor that develops from Schwann cells of the eighth cranial pair, mainly in the cerebellopontine angle.
CASE PRESENTATION
We report the case of a 30-year-old female patient who developed left otalgia associated with neglected tinnitus, the evolution of which was marked by the development of a static cerebellar syndrome and a behavioral disorder, whose brain MRI revealed a locally advanced process in the cerebellopontine angle at the expense of the vestibulocochlear nerve, in favor of a VS, complicated by involvement of the tonsils, which unfortunately led to the patient's death.
DISCUSSION
VS, formerly known as acoustic neuroma, is an extra-axial intracranial tumor that accounts for over 80% of pontocerebellar angle tumors, and is secondary in the majority of cases to inactivation of the neurofibromatosis type 2 (NF2) tumor suppressor gene, either by mutation of the NF2 gene or loss of chromosome 22q. In the majority of cases, it is unilateral and solitary, but in almost 8% of cases, it is associated with NF2. Cerebral MRI is the examination of choice for the detection, characterization, and diagnosis of VS without the need for biopsy, mainly with T1-weighted sequences before and after gadolinium injection. Treatment is based essentially on surgery or radiosurgery, depending on the size, impact, and expertise of the treatment team.
CONCLUSION
VS remains an important intracranial tumor entity, which can be life-threatening in cases of advanced local invasion.
PubMed: 38098578
DOI: 10.1097/MS9.0000000000001418 -
The Journal of International Advanced... Nov 2023The prevalence of sudden sensorineural hearing loss and facial palsy in patients with vestibular schwannoma and the association of sudden sensorineural hearing loss or...
BACKGROUND
The prevalence of sudden sensorineural hearing loss and facial palsy in patients with vestibular schwannoma and the association of sudden sensorineural hearing loss or facial palsy with vestibular schwannoma were investigated based on the population data of Korea.
METHODS
This retrospective study used the Korean National Health Insurance Service data. Patients with vestibular schwannoma and those with a previous history of sudden sensorineural hearing loss or facial palsy were identified based on diagnostic, medication, magnetic resonance imaging, or audiometric codes from 2005 to 2020. The control group was established with propensity score matching. The risk for vestibular schwannoma in patients with a previous history of sudden sensorineural hearing loss or facial palsy was analyzed.
RESULTS
There were 5751 patients in the vestibular schwannoma group and 23004 in the control group. The rate of patients with a previous history of sudden sensorineural hearing loss in the vestibular schwannoma group (25.8%) was significantly higher than in the control group (P -lt; .0001), as was the rate of patients with a previous history of facial palsy in the vestibular schwannoma group (4.7%) (P -lt; .0001). Previous history of sudden sensorineural hearing loss was a significant risk factor for vestibular schwannoma (hazard ratio=7.109, 95% confidence interval=6.696-7.547). Previous history of facial palsy was also a significant risk factor for vestibular schwannoma (hazard ratio=3.048, 95% confidence interval=2.695-3.447).
CONCLUSION
The prevalence of sudden sensorineural hearing loss or facial palsy was significantly higher in patients with vestibular schwannoma than in those without vestibular schwannoma. Based on the population data of Korea, sudden sensorineural hearing loss and facial palsy were significant risk factors for vestibular schwannoma.
Topics: Humans; Neuroma, Acoustic; Facial Paralysis; Retrospective Studies; Hearing Loss, Sensorineural; Hearing Loss, Sudden; Bell Palsy; Republic of Korea
PubMed: 38088318
DOI: 10.5152/iao.2023.231121 -
Otology & Neurotology : Official... Jan 2024Cystic vestibular schwannomas (cVSs) have more variable and less favorable clinical outcomes after microsurgical resection compared with solid VS (sVS). This study...
OBJECTIVE
Cystic vestibular schwannomas (cVSs) have more variable and less favorable clinical outcomes after microsurgical resection compared with solid VS (sVS). This study compares the preoperative presentation and postoperative outcomes between cVS and sVS.
STUDY DESIGN
Retrospective cohort study.
SETTING
Two tertiary skull base referral centers.
METHODS
Consecutive adult patients who underwent VS resection from 2016 to 2021 were included. Univariate and multivariate analyses compared differences in baseline symptoms and postoperative outcomes between cVS and sVS.
RESULTS
There were a total of 315 patients (64% female; mean age, 54 yrs) and 46 (15%) were cystic. cVS were significantly larger than sVS (maximum diameter, 28 vs. 18 mm, p < 0.001) and had higher rates of dysphagia and dysphonia preoperatively (p < 0.02). cVSs were more likely to undergo translabyrinthine resection (76 vs. 50%, p = 0.001) and have a higher rate of subtotal resection (STR) compared with sVS (30 vs. 13%, p = 0.003). At latest follow-up, fewer cVS achieved good facial nerve (FN) outcome (House-Brackmann [HB] I/II) (80 vs. 90%, p = 0.048). Subanalysis of cVS and sVS matched in tumor size, and surgical approach did not show differences in the rate of STR or FN outcomes (HB I/II, 82 vs. 78%, p = 0.79).
CONCLUSION
In this large multi-institutional series, cVSs represent a distinct entity and are characterized by larger tumor size and higher incidence of atypical symptoms. Although cVSs were more likely to undergo STR and portend worse FN outcomes than sVSs, this may be due to their larger tumor size rather than the presence of the cystic component.
Topics: Adult; Humans; Female; Middle Aged; Male; Neuroma, Acoustic; Retrospective Studies; Treatment Outcome; Follow-Up Studies; Neurosurgical Procedures; Facial Nerve; Postoperative Complications
PubMed: 38085765
DOI: 10.1097/MAO.0000000000004062 -
Scientific Reports Dec 2023It is difficult to distinguish other pathologies mimicking Ménière's disease (MD) clinically. This study aims to investigate the differences of imaging findings and...
It is difficult to distinguish other pathologies mimicking Ménière's disease (MD) clinically. This study aims to investigate the differences of imaging findings and features between MD and other menieriform diseases via intravenous gadolinium-enhanced magnetic resonance imaging (MRI). 426 patients with menieriform symptoms, including MD, vestibular migraine (VM), and vestibular schwannoma (VS), underwent 3D-FLAIR and 3D-T2WI MRI 6 h after the intravenous gadolinium injection. MR images were analyzed for inner ear morphology, perilymphatic enhancement (PE), EH and other abnormalities. EH was observed at a higher rate in MD patients (85.71%) than patients with other menieriform diseases (VM group = 14.75%, VS group = 37.50%). The prevalence of unilateral EH as well as both cochlear and vestibular EH showed significant differences between MD and VM groups. The prevalence of cochlear EH (I and II) and vestibular EH (II and III) was different between MD and VM groups. The prevalence of PE was higher in MD than VM group. The degrees of cochlear and vestibular hydrops were higher in the definite than probable MD group (P < 0.05). Using these imaging features, MRI can be used to help differentiate MD from other menieriform diseases.
Topics: Humans; Meniere Disease; Endolymphatic Hydrops; Gadolinium; Vestibule, Labyrinth; Vertigo; Magnetic Resonance Imaging; Neuroma, Acoustic; Migraine Disorders
PubMed: 38057393
DOI: 10.1038/s41598-023-49066-5 -
Neuro-oncology Advances 2023Both stereotactic radiosurgery () and microsurgical resection () are available as treatment options for sporadic vestibular schwannoma (VS). There are very few direct...
BACKGROUND
Both stereotactic radiosurgery () and microsurgical resection () are available as treatment options for sporadic vestibular schwannoma (VS). There are very few direct comparative studies comparing both treatment modalities in large cohorts allowing detailed subgroup analysis. This present study aimed to compare the nuances in the treatment of VS by and in 2 highly specialized neurosurgical centers.
METHODS
This is a retrospective bicentric cohort study. Data from patients treated between 2005 and 2011 were collected retrospectively. Recurrence-free survival (RFS) was assessed radiographically by contrast-enhanced magnetic resonance imaging.
RESULTS
The study population included = 901 patients with a mean follow-up of 7 years. Overall, the incidence of recurrence was 7% after , and 11% after with superior tumor control in in the Kaplan-Meier-analysis ( = 0.031). In small tumors (Koos I and II), tumor control was equivalent in both treatment arms. In large VS (Koos III and IV), however, RFS was superior in . The extent of resection correlated with RFS ( < .001). Facial and hearing deterioration was similar in both treatment arms in small VS, but more pronounced in of large VS. Tinnitus, vertigo, imbalance, and trigeminal symptoms were more often improved by than .
CONCLUSIONS
can achieve similar tumor control compared to in smaller VS (Koos I and II)-with similar postinterventional morbidities. In large VS (Koos III and IV), long-term tumor control of is inferior to . Based on these results, we suggest that if combination therapy is chosen, the residual tumor should not exceed the size of Koos II.
PubMed: 38024239
DOI: 10.1093/noajnl/vdad146 -
The Journal of Laryngology and Otology Jun 2024This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma.
OBJECTIVE
This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma.
METHODS
Audiovestibular handicap was assessed using the Hearing Handicap Inventory, Tinnitus Handicap Inventory and Dizziness Handicap Inventory. Patients completed questionnaires at presentation and at least one year following treatment with microsurgery, stereotactic radiosurgery or observation. Changes in audiovestibular handicap and factors affecting audiovestibular handicap were assessed.
RESULTS
All handicap scores increased at follow up, but not significantly. The Tinnitus Handicap Inventory and Dizziness Handicap Inventory scores predicted tinnitus and dizziness respectively. The Hearing Handicap Inventory was not predictive of hearing loss. Age predicted Tinnitus Handicap Inventory score and microsurgery was associated with a deterioration in Dizziness Handicap Inventory score.
CONCLUSION
Audiovestibular handicap is common in patients with vestibular schwannoma, with 75 per cent having some degree of handicap in at least one inventory. The overall burden of handicap was, however, low. The increased audiovestibular handicap over time was not statistically significant, irrespective of treatment modality.
Topics: Humans; Neuroma, Acoustic; Female; Male; Middle Aged; Tinnitus; Adult; Dizziness; Aged; Surveys and Questionnaires; Disability Evaluation; Microsurgery; Radiosurgery; Hearing Loss; Treatment Outcome
PubMed: 38017610
DOI: 10.1017/S002221512300213X -
Journal of Clinical Medicine Nov 2023(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such...
(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such cases. Moreover, the presence of middle and inner ear pathologies combined may represent a surgical challenge. No studies have described a combined surgical approach for these coexisting conditions (VS and cholesteatoma), nor the hearing rehabilitation outcomes of using cochlear implants for these patients. (2) Case Report: This paper is on a female patient who underwent simultaneous surgical treatments for VS and middle ear cholesteatoma in the right ear followed by a cochlear implant, describing the technique and the audiological results. (3) Conclusions: The surgical approach was successful and enabled the resection of lesions with the auditory nerve and cochlea preservation. Cochlear implantation in the right ear showed positive postoperative results, with an improvement in the results with the CI in silent and noisy environments.
PubMed: 38002751
DOI: 10.3390/jcm12227139 -
CNS Neuroscience & Therapeutics Apr 2024The purpose of this study was to identify significant prognostic factors associated with facial paralysis after vestibular schwannoma (VS) surgery and develop a novel...
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.
Topics: Humans; Neuroma, Acoustic; Facial Nerve; Retrospective Studies; Facial Paralysis; Nomograms
PubMed: 37990346
DOI: 10.1111/cns.14526 -
Clinical Trials (London, England) Feb 2024Individuals with neurofibromatosis, including neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2)-related schwannomatosis (SWN), and other forms of SWN, often...
BACKGROUND/AIMS
Individuals with neurofibromatosis, including neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2)-related schwannomatosis (SWN), and other forms of SWN, often experience disease manifestations and mental health difficulties for which psychosocial interventions may help. An anonymous online survey of adults with neurofibromatosis assessed their physical, social, and emotional well-being and preferences about psychosocial interventions to inform clinical trial design.
METHODS
Neurofibromatosis clinical researchers and patient representatives from the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration developed the survey. Eligibility criteria included age ≥ 18 years, self-reported diagnosis of NF1, NF2, or SWN, and ability to read and understand English. The online survey was distributed internationally by the Neurofibromatosis Registry and other neurofibromatosis foundations from June to August 2020.
RESULTS
Surveys were completed by 630 adults (18-81 years of age; M = 45.5) with NF1 (78%), NF2 (14%), and SWN (8%) who were mostly White, not Hispanic/Latino, female, and from the United States. The majority (91%) reported that their neurofibromatosis symptoms had at least some impact on daily life. In the total sample, 51% endorsed a mental health diagnosis, and 27% without a diagnosis believed they had an undiagnosed mental health condition. Participants indicated that neurofibromatosis affected their emotional (44%), physical (38%), and social (35%) functioning to a high degree. Few reported ever having participated in a drug (6%) or psychosocial (7%) clinical trial, yet 68% reported they "probably" or "definitely" would want to participate in a psychosocial trial if it targeted a relevant concern. Top treatment targets were anxiety, healthier lifestyle, and daily stress. Top barriers to participating in psychosocial trials were distance to clinic, costs, and time commitment. Respondents preferred interventions delivered by clinicians via individual sessions or a combination of group and individual sessions, with limited in-person and mostly remote participation. There were no significant group differences by neurofibromatosis type in willingness to participate in psychosocial trials ( = 0.27). Regarding interest in intervention targets, adults with SWN were more likely to prefer psychosocial trials for pain support compared to those with NF1 ( < 0.001) and NF2 ( < 0.001).
CONCLUSION
This study conducted the largest survey assessing physical symptoms, mental health needs, and preferences for psychosocial trials in adults with neurofibromatosis. Results indicate a high prevalence of disease manifestations, psychosocial difficulties, and untreated mental health problems in adults with neurofibromatosis and a high degree of willingness to participate in psychosocial clinical trials. Patient preferences should be considered when designing and implementing psychosocial interventions to develop the most feasible and meaningful studies.
Topics: Adult; Female; Humans; United States; Adolescent; Neurofibromatoses; Neurilemmoma; Skin Neoplasms; Neurofibromatosis 2; Neurofibromatosis 1; Surveys and Questionnaires
PubMed: 37962219
DOI: 10.1177/17407745231209224 -
Journal of Clinical Medicine Nov 2023The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved...
The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved using intraoperative EMG monitoring, hearing preservation remains challenging, with the recent use of cochlear nerve action potential (CNAP) monitoring. This prospective longitudinal series of VS with useful hearing operated on using a retrosigmoid approach included 37 patients with a mean largest extrameatal VS. diameter of 25 ± 8.7 mm (81% of Koos stage 4). CNAP was detected in 51% of patients, while auditory brainstem responses (ABR) were present in 22%. Patients were divided into two groups based on the initial intraoperative CNAP status, whether it was present or absent. FN function was preserved (grade I-II) in 95% of cases at 6 months. Serviceable hearing (class A + B) was preserved in 16% of the cases, while 27% retained hearing with intelligibility (class A-C). Hearing with intelligibility (class A-C) was preserved in 42% of cases when CNAP could be monitored in the early stages of VS resection versus 11% when it was initially absent. Changes in both the approach to the cochlear nerve and VS resection are mandatory in preserving CNAP and improve the rate of hearing preservation.
PubMed: 37959371
DOI: 10.3390/jcm12216906