Schwannoma Patients Research Articles

Preservation of facial nerve function after resection of vestibular schwannoma

The objective of this study was to provide a comprehensive assessment of reported outcomes for facial nerve preservation after vestibular schwannoma surgery. Most data regarding facial nerve function in patients undergoing microsurgical resection of vestibular schwannomas predominantly include series performed at a single institution. In an effort to minimise individual surgeon or institutional bias, we performed an analysis of the published literature on facial nerve outcomes following microsurgical resection of vestibular schwannomas. Materials and methods. We identified a total of 296 studies involving over 25,000 patients that included outcome data for facial nerve function of vestibular schwannoma patients treated surgically. Data regarding surgical approach, tumour size, patient age, and use of intra-operative monitoring were extracted and correlated with facial nerve function after surgery. Patients with preoperative facial nerve dysfunction (House-Brackmann score III or higher) were excluded and ‘facial nerve preservation’ was defined as grade I or II House-Brackmann function at last follow-up visit. Results. A total of 79 articles reporting on 11,873 patients met our inclusion criteria contributing to our analysis. Patients treated with the middle cranial fossa approach had a trend towards higher overall facial nerve preservation rate (85 %), compared to the translabyrinthine approach (81 %, p = 0.07), and did statistically better than the retrosigmoid approach (78 %, p < 0.0001). Patients with an average tumour size < 20 mm had significantly improved facial nerve preservation rates, compared to larger tumours (90 % vs. 67 %, p < 0.0001). Patients under 65 years of age had a lower facial nerve preservation rate (71 % vs. 84 %, p < 0.001). Finally, the use of intra-operative monitoring improved the facial nerve preservation rate (76 % vs. 71 %, p < 0.001). Conclusion. Factors that appear to be associated with facial nerve preservation after microsurgical resection of a vestibular schwannoma include tumour size <20 mm, use of the middle fossa approach and use of neuromonitoring during surgery. These data provide a summary assessment of the published literature regarding facial nerve preservation after microsurgical resection of vestibular schwannoma.

How many growing vestibular schwannomas tend to stop growing without any treatment?

To determine the long-term, spontaneous growth arrest rates in a large cohort of vestibular schwannoma patients. This paper describes a retrospective case series of 735 vestibular schwannoma patients organised into four groups: group A patients showed tumour growth which then stopped without any treatment; group B patients showed tumour growth which continued, but were managed conservatively; group C patients had a growing vestibular schwannoma and received active treatment; and group D patients had a stable, non-growing vestibular schwannoma. Demographics, tumour size and vestibular schwannoma growth rate (mm/month) were recorded. A total of 288 patients (39.2 per cent) had growing vestibular schwannomas. Of the patients, 103 (35.8 per cent) were managed conservatively, with 52 patients (50.5 per cent of the conservative management group, 18 per cent of the total growing vestibular schwannoma group) showing growth arrest, which occurred on average at four years following the diagnosis. Eighty-two per cent of vestibular schwannomas stopped growing within five years. Only differences between age (p = 0.016) and vestibular schwannoma size (p = 0.0008) were significant. Approximately 20 per cent of growing vestibular schwannomas spontaneously stop growing, predominantly within the first five years; this is important for long-term management.

Multiple Audiometric Analysis in the Screening of Vestibular Schwannoma.

IntroductionAsymmetric sensorineural hearing loss is the main early symptom of retrocochlear lesions, hence its importance in screening for vestibular schwannomas. Currently, there is no consensus regarding its definition. The objective was to identify the audiometric pattern that would serve as a predictor for vestibular schwannoma in patients with asymmetric hearing loss.Materials and methodsA cross-sectional study was conducted that included patients with asymmetric hearing loss attending a secondary care center and a tertiary care center. Clinical, audiometric and imaging (MRI with gadolinium) variables were collected. Asymmetric hearing loss was defined as a difference of 15 dB in one or more frequencies between both ears. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of different audiometric patterns were analyzed.ResultsA total of 107 patients were studied and divided into two groups: group 1 without vestibular schwannoma (n=98); and group 2 with vestibular schwannoma (n=9). No significant difference in demographic characteristics or audiometric patterns was found in patients with and without vestibular schwannoma. The audiometric pattern with the best sensitivity as a screening test was a difference >20 dB in the 4,000 Hz frequency, with a sensitivity of 77.78%, specificity of 30.61%, PPV of 8.33%, NPV of 93.75% and accuracy of 34.50%.ConclusionThe audiometric pattern with the best results was a difference >20 dB in the 4,000 Hz frequency range; however, patients with asymmetric hearing loss could not be differentiated from patients with retrocochlear lesions based only on audiometry. Asymmetrical hearing loss must be studied with MRI.

Comparing Hearing Outcomes in Irradiated and Conservatively Managed Vestibular Schwannoma.

Compare hearing outcome for vestibular schwannoma patients following stereotactic radiosurgery (SRS) or conservative management. Retrospective review. University Hospital. Patients with small- or medium-sized sporadic vestibular schwannoma (intracanalicular or with CPA component <2 cm) who were managed conservatively or underwent SRS with available clinical, radiological, and audiometric data from the time of presentation (or just before radiotherapy for the SRS group) and most recent follow-up; with the two sets of data to be compared being at least 3 years apart (minimum follow-up period). SRS or observation. Pure-tone averages, speech discrimination scores, and corresponding hearing classifications. Two hundred forty-seven patients met our inclusion criteria; 140 were managed conservatively with a mean follow-up period of 5.9 ± 1.6 years and 107 underwent SRS with a mean follow-up period of 7.1 ± 1.9 years. There was significant deterioration of hearing measures for both groups; with the SRS group displaying consistently worse measures. SRS patients showed worse mean pure-tone averages and speech discrimination scores decline rates by 2.72 dB/yr and 2.98 %/yr, respectively, when compared with conservatively managed patients. Stratifying patients according to Tokyo's hearing classification revealed that 68.75% of conservatively managed patients who had baseline serviceable hearing preserved their hearing throughout the studied period compared with only 15.38% of the SRS patients. Based on our data we conclude that patients with small- and medium-sized tumors will have a better hearing outcome if managed via an initial conservative approach with radiotherapy reserved for those demonstrating disease progression.

Dynamic contrast-enhanced magnetic resonance imaging for evaluating early response to radiosurgery in patients with vestibular schwannoma.

This study aimed to use dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) to evaluate early treatment response in vestibular schwannoma (VS) patients after radiosurgery. Twenty-four VS patients who underwent gamma knife radiosurgery were prospectively followed up for at least four years. DCE-MRI sequences, in addition to standard MRI protocol, were obtained prior to radiosurgery, at 3 and 6months. Conventionally, treatment responses based on tumor volume changes were classified as regression or stable (RS), transient tumor enlargement (TTE), and continuous tumor enlargement (CTE). DCE-MRI parameters, such as Ktrans, Kep and Ve, were compared according to follow-up periods and between groups. The diagnostic performance was tested using receiver operating characteristic (ROC) curves. Changes in tumor volume were as follows at the last 48 months of follow-up: RS in 11 patients (45.8%), TTE in 10 patients (41.7%), and CTE in three patients (12.5%). The median time required to distinguish TTE from CTE using conventional MRI was 12months (range 9-18). The Ktrans and Ve were significantly decreased in patients with RS and TTE at 3 and 6months, but did not differ significantly in patients with CTE. There were no significant differences in Ktrans and Ve between patients with RS and TTE at 3 and 6months. Both Ktrans and Ve demonstrated high diagnostic performance in evaluating early treatment response to radiosurgery in patients with VS. DCE-MRI may aid in the monitoring and early prediction of treatment response in patients with VS following radiosurgery.

Clinical trials targeting neurofibromatoses-associated tumors: a systematic review.

BackgroundThere is a paucity of literature that comprehensively analyzes previous and current clinical trials targeting neurofibromatoses-related tumors. This article aims to provide readers with drug development efforts targeting these tumors by analyzing translational and clinical findings.MethodsThis systematic review was written according to the PRISMA guidelines. Inclusion criteria were clinical trials involving patients with neurofibromatosis type 1, type 2, or schwannomatosis that were treated with therapies targeting neurofibromatoses-associated tumors and that were registered on clinicaltrials.gov. In addition, a search was performed in PubMed, Web of Science, Google Scholar, and Embase European for articles fully describing these clinical trials.ResultsA total of 265 clinical trials were registered and screened for eligibility. Ninety-two were included in this systematic review involving approximately 4636 participants. The number of therapies analyzed was more than 50. Drugs under investigation mainly act on the MAPK/ERK and PI3K/AKT/mTOR pathways, tumor microenvironment, or aberrantly over-expressed cell surface receptors. Selumetinib was the most effective medication for treating a neurofibromatosis type 1-associated tumor with approximately 68%–71% partial response for inoperable or progressive plexiform neurofibromas in children 2 years of age and older and bevacizumab for a neurofibromatosis type 2-related tumor with approximately 36%–41% partial response for vestibular schwannomas in patients 12 years of age and older.ConclusionsThis systematic review presents the results of previous clinical investigations and those under development for neurofibromatoses-associated tumors. Clinicians may use this information to strategize patients to appropriate clinical trials.

Vestibular schwannoma – a retrospective study in the Republic of Moldova

Vestibular schwannoma is a benign, intracranial tumor of the 8th pair of cranial nerves. The incidence of vestibular schwannoma is 3-5.2 cases per 100000 people. It re­pre­sents 6% of all intracranial tumors and 80% of tumors located in the cerebellopontine angle. In this paper, the authors present the results of a retrospective study of vestibular schwannoma patients who underwent vestibular schwan­noma surgery between 2010 and 2019.

Activation of Raf signalling in NF2-null Schwann cells leads to sustained proliferation; an investigation of a new and inducible model for human schwannoma

Abstract Aims The NF2 gene encodes the tumour suppressor Merlin, which is deleted in 100% of patients with the familial tumour predisposition syndrome neurofibromatosis type 2 but also in 70% of those who develop sporadic schwannomas. The Raf-TR mouse model uses a tamoxifen-inducible Raf-kinase/ oestrogen receptor fusion protein (Raf-TR) expressed in myelinating Schwann cells to mimic a nerve injury response in Schwann cell by activating Raf/MEK/ERK signalling in the absence of peripheral nerve injury. We will assess whether Raf/MEK/ERK activation on an NF2 null background leads to tumourigenesis within the vestibular nerves and dorsal root ganglia (DRGs), two tumour sites identified in the Periostin-Cre mouse model in which schwannoma formation is spontaneous, with a view to generating an inducible NF2 null schwannoma mouse model. Method Mice with a Schwann cell specific loss of Merlin were crossed with mice carrying a tamoxifen-inducible Raf-TR gene to generate Raf-TR+/-; P0-Cre+/-; NF2fl/fl (Cre+) mice which were NF2 null and compared to Raf-TR+/-; P0-Cre-/-; NF2fl/fl (Cre-) littermate controls. Mice were injected with tamoxifen or vehicle for five consecutive days and their vestibular nerves and dorsal root ganglia (DRGs) were analysed at various timepoints . An EdU proliferation assay was used to quantify the proliferation in the vestibular ganglia, as well as the DRGs. Rates of proliferation were compared to Cre- age-matched littermate controls treated with tamoxifen or vehicle. Results In the Periostin-Cre NF2 null schwannoma model, tumours form spontaneously in the DRGs and vestibular ganglia. In our new model, we see a clear increase in proliferation at 21 d post-injection in the NF2 null (Cre+) tamoxifen-treated mice compared to control (Cre-) tamoxifen-treated controls in both DRGs and vestibular ganglia. Cre- tamoxifen-treated mice do not show increased proliferation compared to Cre- vehicle controls. Taken together, this shows that activation of the Raf/MEK/ERK pathway in Schwann cells only causes a sustained proliferation response on an NF2 null background in the DRGs and vestibular ganglia. We are assessing later timepoints to further characterise tumour development in these mice. Conclusion Combining the Raf-TR mouse model to create a demyelinating phenotype with an NF2 null background leads to vastly increased rates of proliferation at the sites of schwannoma tumourigenesis within the peripheral nervous system: the DRGs and the vestibular ganglia. The high proliferation in the vestibular ganglia in particular is similar to the development of vestibular schwannomas in patients with Neurofibromatosis type 2. The new mouse model used in this study shows potential to be very useful as an inducible schwannoma tumour model, in which we can study the early events of tumour formation.

Growing versus non-growing vestibular schwannomas: assessment of natural history.

Vestibular schwannomas can demonstrate great heterogeneity in their behaviour; approximately one-third will grow and two-thirds will not. This study aimed to determine whether there are factors present at diagnosis that can help predict outcomes. This retrospective cohort study compared data from 735 patients from the past 20 years. Analysis of serial magnetic resonance imaging was carried out to place patients into growing and non-growing cohorts. Factors including size, age, follow-up time and presence of balance symptoms were compared. The median size of a growing vestibular schwannoma at diagnosis was 13 mm, whereas the non-growing median size was 10.65 mm (p < 0.001). Balance symptoms were present in 60.76 per cent of growing vestibular schwannoma patients but only in 38.75 per cent of patients with non-growing vestibular schwannomas (p < 0.001). This study highlights initial tumour size and balance symptoms as potential predictors of whether or not a vestibular schwannoma will grow; these results better facilitate our understanding of vestibular schwannoma natural history.

Outcome and Toxicity of Proton Therapy for Vestibular Schwannoma: A Cohort Study.

To assess the efficacy and toxicity of proton radiotherapy in vestibular schwannoma. Retrospective chart review and volumetric MRI-analyses. Tertiary referral center. Vestibular schwannoma patients treated with protons between 2003 and 2018. Proton radiotherapy. Tumor control was defined as not requiring salvage treatment. Progressive hearing loss was defined as a decrease in maximum speech discrimination score below the 95% critical difference in reference to the pretreatment score. Hearing assessment includes contralateral hearing and duration of follow-up. Dizziness and/or unsteadiness and facial and trigeminal nerve function were scored. Patients who had surgery prior to proton radiotherapy were separately assessed. Of 221 included patients, 136 received single fraction and 85 fractionated proton radiotherapy. Actuarial 5-year local control rate was 96% (95% CI 90-98%). The median radiological follow-up was 4.5 years. Progressive postirradiation speech discrimination score loss occurred in 42% of patients with audiometric follow-up within a year. Facial paresis was found in 5% (usually mild), severe dizziness in 5%, and trigeminal neuralgia in 5% of patients receiving protons as primary treatment. Proton radiotherapy achieves high tumor control with modest side effects aside from hearing loss in vestibular schwannoma patients. Limited and heterogeneous outcome reporting hamper comparisons to the literature. Potential sequelae of radiation therapy impacting vestibular function, cognitive function, and quality of life warrant further evaluation. Subgroups that benefit most from proton radiotherapy should be identified to optimize allocation and counterbalance its costs.

MRI T2-Weighted Cochlear Intensity as a Predictor of Hearing Loss with Vestibular Schwannoma Patients

Abstract Objective It is difficult to predict how hearing loss will progress with vestibular schwannomas (VSs) and to determine the optimal time for hearing preservation interventions. This study investigated the relationship between cochlear intensity on T2-weighted magnetic resonance imaging and hearing loss in VS patients over time. Design Retrospective cohort study. Setting Single major academic center. Participants Patients with a diagnosis of VS from 2007 to 2018. Main Outcome Measures Pure tone average (PTA) and cochlear-to-pontine relative signal intensity (RSI) measured at two time points. Results Fifty patients were included in the final analysis. For both affected and unaffected ears, the trend in PTA increased from baseline to follow-up. For affected ears, the trend in RSI increased from baseline to follow-up, while for the unaffected ears RSI decreased. There was a significant positive correlation between the initial RSI value in the ipsilateral ear when compared with the change in PTA from baseline to follow-up (r = 0.28, p = 0.048). Conclusion There is a trend between initial RSI and how hearing changes in the affected ears of patients with VS. Additional studies are needed to explore how this relationship may be better used to predict hearing loss.

Challenges of Cochlear Implantation in Intralabyrinthine Schwannoma Patients: Surgical Procedures and Auditory Outcome.

Intralabyrinthine schwannoma (ILS) is a rare benign tumor of the inner ear potentially causing unilateral sensorineural hearing loss and vertigo. This study evaluated the outcome of one surgical session comprising microsurgical ILS resection and cochlear implantation in terms of surgical feasibility, complications, and auditory outcome. Ten clinically and histologically confirmed ILS patients included in this study (three women and seven men; mean age 56.4 ± 8.6) underwent surgery between July 2015 and February 2020. Eight patients had intracochlear tumor location; the remaining two had vestibulocochlear and intravestibular ILS. One of the three following methods was used for tumor removal: an extended cochleostomy, subtotal cochleoectomy, or a translabyrinthine approach. Although negligible improvement was observed in two of the patients, two patients were lost to follow-up, and one opted out from using CI, the speech perception of the five remaining ILS patients improved as per the Freiburg Monosyllable Test (FMT) from 0% before surgery to 45– 50% after the implantation. Our study supports the presented surgical approach’s feasibility and safety, enabling tumor removal and hearing restoration shortly after surgery.

Is Cochlear Implantation Effective for Auditory Rehabilitation Following Vestibular Schwannoma Treatment?

Is Cochlear Implantation Effective for Auditory Rehabilitation Following Vestibular Schwannoma Treatment?

Microsurgical treatment of symptomatic vestibular schwannomas in patients under 40: different results before and after age of 30.

In 10-15% of cases of vestibular schwannoma (VS), age at diagnosis is 40years or less. Little is known about the differences in natural history, surgical findings, and postoperative outcomes of such younger patients as compared to those of greater age. To analyze clinical and surgical and imaging data of a consecutive series of n = 50 patients with unilateral sporadic VS, aged 40years or younger - separated in a very young group (15-30years) and a moderately young group (31-40years). Retrospective case series. Fifty consecutive patients under 40years of age underwent microsurgical resection of unilateral sporadic VS via the retrosigmoid approach. The study cohort was subdivided into two groups according to the age range: group A, age range 15-30years (n = 23 patients), and group B, age range 31-40years (n = 27 patients). The adherence of VS capsule to surrounding nervous structures and the tendency of the tumors to bleed were evaluated by reviewing video records; the course of the FN in relation to the tumor's surface was assessed in each case. Microsurgical removal of tumor was classified as total (T), near total (residual tumor volume < 5%), subtotal (residual tumor volume 5-10%), or partial (residual tumor volume > 10%). Mean tumor size of entire cohort was 2.53 (range: 0.6-5.8) cm: 2.84cm in group A and 2.36cm in group B (p = NS). Facial nerve course and position within the cerebellopontine angle did not differ significantly between the two groups. At 6-month follow-up, FN functional outcome was HBI-II in 69.5% in group A, versus 96.3% in group B (p < .001). Hearing preservation was achieved in 60.0% of patients of group A and in 58.3% of group B (p = NS). Total and near-total resection was feasible in 95.6% of cases of group A and in 88.9% of group B (p = NS). Tumor capsule was tightly adherent to nervous structures in 69.6% patients of group A and in 22.2% of group B (p < .05). Significant bleeding was encountered in 56.5% of group A tumors, and in 29.6% of group B tumors (p < .01). Microsurgery of VS in patients aged 40 or less is associated with good functional results, and with high rates of total and near total tumor removal. Patients < 30years of age have more adherent tumor capsules. Furthermore, their tumors exhibit a tendency to larger sizes, to hypervascularization, to profuse intraoperative bleeding and they present worse long-term functional FN results when compared to patients in their fourth decade of life. Our limited experience seems to suggest that a near total resection in very young VS patients with large tumors should be preferred in adherent and hypervascularized cases, in order to maximize resection and preserve function.

Continuous Head Motion is a Greater Motor Control Challenge than Transient Head Motion in Patients with Loss of Vestibular Function

Background. The vestibular system is vital for gaze stability via the vestibulo-ocular reflex, which generates compensatory eye motion in the direction opposite to head motion. Consequently, individuals with peripheral vestibular loss demonstrate impaired gaze stability that reduces functional capacity and quality of life. To facilitate patients’ compensatory strategies, two classes of gaze stabilization exercises are often prescribed: (i) transient (eg, ballistic) and (ii) continuous. However, the relative benefits of these two classes of exercises are not well understood. Objective. To quantify head motion kinematics in patients with vestibular loss while they performed both classes of exercises. Methods. Using inertial measurement units, head movements of 18 vestibular schwannoma patients were measured before and after surgical deafferentation and compared with age-matched controls. Results. We found that the head movement during both classes of exercises paralleled those of natural head movement recorded during daily activities. However, head movement patterns were more informative for continuous than transient exercises in distinguishing patients from healthy controls. Specifically, we observed coupling between kinematic measures in control subjects that was absent in patients for continuous but not transient head motion exercises. In addition, kinematic measures (eg, cycle duration) were predictive of standard clinical measures for continuous but not transient head motion exercises. Conclusions. Our data suggest that performing continuous head motion is a greater motor control challenge than transient head motion in patients with less reliable vestibular feedback during the sub-acute stage of recovery, which may also prove to be a reliable measure of progression in vestibular rehabilitation protocols.

Tumor Control Following Stereotactic Radiosurgery in Patients with Vestibular Schwannomas - A Retrospective Cohort Study.

To better counsel vestibular schwannoma patients, it is necessary to understand the tumor control rates of stereotactic radiosurgery (SRS). To determine tumor control rates, factors determining control and complication rates following SRS. Tertiary hospital retrospective cohort. 579 tumors (576 patients) were treated with SRS. 477 tumors (474 patients, 82%) had ≥1 year follow up and 60% (344) ≥3 years follow up. 88% of tumors had primary SRS and 6.7% salvage SRS. Median follow up time was 4.6 years. At 3 years, the tumor control rate of primary SRS was 89% (258 of 290) in sporadic tumors compared to 43% in Neurofibromatosis type II (3 of 17) (p < 0.01). Our bivariable survival data analysis showed that Neurofibromatosis type II, documented pre-SRS growth, tumor measured by maximum dimension, SRS given as nonprimary treatment increased hazard of failure to control. There was one case of malignancy and another of rapid change following intra-tumoral hemorrhage. For tumors undergoing surgical salvage (25 of 59), 56% had a total or near-total resection, 16% had postoperative CSF leak, with 12% new facial paralysis (House-Brackmann grade VI) and worsening of facial nerve outcomes (House-Brackmann grade worse in 59% at 12 mo). Control of vestibular schwannoma after primary SRS occurs in the large majority. Salvage surgical treatment was notable for higher rates of postoperative complications compared to primary surgery reported in the literature.

Excision of Greater Superficial Petrosal Nerve Schwannoma Via a Pure Endoscopic Endonasal Approach.

Greater superficial petrosal nerve (GSPN) schwannomas are an exceedingly rare nerve sheath tumor. The current literature search was conducted using Medline and Embase database by key search terms. Only 31 cases have been reported in the literature so far. Facial palsy, hearing loss, and xerophthalmia accounted for 48.4% (15), 41.9% (13), and 29% (9) of all cases, respectively. The middle cranial fossa approach was used in all previous reports. A retrospective review of 2 GSPN schwannomas patients treated by endoscopic endonasal approach (EEA) in our center was collected. Clinical records, including clinical features, pre- and postoperative images, surgery, and follow-up information, were reviewed. In all cases, clinical features including facial numbness and headache were found, with tinnitus in case 1, hearing loss, xerophthalmia in case 2. Imaging studies showed a solid mass that originated in the anterior of the petrous bone. Two patients were treated by EEA. Furthermore, no recurrence was found during the follow-up period (15-29 months) in both of the 2 cases after the operation. Complete resection of GSPN schwannomas can be achieved via the pure EEA. Endoscopic endonasal approach for radical removal of tumors is safe and feasible.

The impact of vestibular schwannoma and its management on employment.

BackgroundEmployment is an important factor in quality of life. For vestibular schwannoma (VS) patients, employment is not self-evident, because of the sequelae of the disease or its treatment and their effects on daily life.ObjectivesThis study assessed employment status, sick leave (absenteeism) and being less productive at work (presenteeism) in the long-term follow-up of VS patients, and evaluated the impact of treatment strategy (active surveillance, surgery or radiotherapy).MethodsA cross-sectional survey study was performed in a tertiary university hospital in the Netherlands. Patients completed the iMTA-post productivity questionnaire (iPCQ). Employment status was compared to that of the general Dutch population. Employment, absenteeism and presenteeism were compared between patients under active surveillance, patients after radiotherapy and post-surgical patients.ResultIn total 239 patients participated, of which 67% were employed at the time of the study. Only 14% had a disability pension, which was comparable to the age-matched general Dutch population. The proportion of patients with absenteeism was 8%, resulting in a 4% reduction of working hours. Presenteeism was reported by 14% of patients, resulting in a 2% reduction of working hours. The median number of working hours per week was 36, and since the diagnosis, these hours had been reduced by 6%. There were no significant differences between treatment modalities.ConclusionOn average, long-term employment status and working hours of VS patients are comparable to the age-matched general population. Treatment strategies do not seem to differentially impact on long-term employment of VS patients.

The SH3PXD2A-HTRA1 fusion transcript is extremely rare in Norwegian sporadic vestibular schwannoma patients

IntroductionVestibular schwannoma (VS) is a benign intracranial tumor in which the underlying genetics is largely uncertain, apart from mutations in the tumor suppressor gene NF2. Alternative tumorigenic mechanisms have been proposed, including a recurrent in-frame fusion transcript of the HTRA1 and SH3PXD2A genes. The gene product of the SH3PXD2A-HTRA1 fusion has been shown to promote proliferation, invasion and resistance to cell death in vitro and tumor growth in vivo. The aim of this study was to replicate the findings and to investigate the frequency of this fusion gene in another cohort of vestibular schwannoma patients.MethodsThe SH3PXD2A-HTRA1 transcript was synthesized in vitro using PCR and used as a positive control to assess the sensitivity of a real-time PCR assay. This real-time PCR assay was used to search for the presence of the fusion transcript in 121 Norwegian sporadic VS patients.ResultsThe real-time PCR assay showed a high sensitivity and was able to detect as low as ~ 5 copies of the fusion transcript. Out of the 121 investigated tumors, only 1 harbored the SH3PXD2A-HTRA1 fusion.ConclusionEven though the SH3PXD2A-HTRA1 fusion has been shown to be a driver of tumorigenesis, our results suggest that it is a rare event in our VS patients. Further investigation is warranted in order to elucidate whether our results represent an extreme, and if the fusion is present also in other neoplasms.

Surgical Outcomes After Conservative Resection of Vestibular Schwannoma in the Elderly.

To assess outcomes after surgery for vestibular schwannoma in patients over 70 years of age. Retrospective chart review. Two tertiary otology and neurotology centers. Patients undergoing primary surgery for vestibular schwannoma between 2007 and 2018. Postoperative complications and surgical outcomes. A total of 452 patients met inclusion criteria, 31 of whom (6.9%) were over 70 years of age. Age ranged from 18 to 90 years with a mean of 53 years. Elderly patients were more likely to have pre-existing hypertension (58.1% versus 34.0%, p = 0.007) and diabetes mellitus (19.4% versus 7.4%, p = 0.02). Elderly patients were less likely to undergo gross total resections of their tumors (35.5% versus 60.6%, p = 0.05) although they were not statistically significantly more likely to undergo subtotal (<95%) resections (25.8% versus 14.7%, p > 0.05). Elderly patients were also less likely to undergo second stage procedures (0% versus 9.5%, p = 0.04). There were no significant differences between elderly and non-elderly patients in the rates of any complications, ultimate facial nerve function, or duration of surgery. No patients over 70 years of age expired within 1 year of surgery. Conservative surgery for vestibular schwannoma in appropriately selected elderly patients is appropriate and safe, given adequate consideration to risk-benefit analysis and goals of care.