Mobile phone use and brain tumour risk - COSMOS, a prospective cohort study.

BACKGROUND: Each new generation of mobile phone technology has triggered discussions about potential carcinogenicity from exposure to radiofrequency electromagnetic fields (RF-EMF). Available evidence has been insufficient to conclude about long-term and heavy mobile phone use, limited by differential recall and selection bias, or crude exposure assessment. The Cohort Study on Mobile Phones and Health (COSMOS) was specifically designed to overcome these shortcomings. METHODS: We recruited participants in Denmark, Finland, the Netherlands, Sweden, and the UK 2007-2012. The baseline questionnaire assessed lifetime history of mobile phone use. Participants were followed through population-based cancer registers to identify glioma, meningioma, and acoustic neuroma cases during follow-up. Non-differential exposure misclassification was reduced by adjusting estimates of mobile phone call-time through regression calibration methods based on self-reported data and objective operator-recorded information at baseline. Hazard ratios (HR) and 95% confidence intervals (CI) for glioma, meningioma, and acoustic neuroma in relation to lifetime history of mobile phone use were estimated with Cox regression models with attained age as the underlying time-scale, adjusted for country, sex, educational level, and marital status. RESULTS: 264,574 participants accrued 1,836,479 person-years. During a median follow-up of 7.12 years, 149 glioma, 89 meningioma, and 29 incident cases of acoustic neuroma were diagnosed. The adjusted HR per 100 regression-calibrated cumulative hours of mobile phone call-time was 1.00 (95 % CI 0.98-1.02) for glioma, 1.01 (95 % CI 0.96-1.06) for meningioma, and 1.02 (95 % CI 0.99-1.06) for acoustic neuroma. For glioma, the HR for ≥ 1908 regression-calibrated cumulative hours (90th percentile cut-point) was 1.07 (95 % CI 0.62-1.86). Over 15 years of mobile phone use was not associated with an increased tumour risk; for glioma the HR was 0.97 (95 % CI 0.62-1.52). CONCLUSIONS: Our findings suggest that the cumulative amount of mobile phone use is not associated with the risk of developing glioma, meningioma, or acoustic neuroma.

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

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

Superior petrosal vein sacrifice in translabyrinthine approach for resection of vestibule schwannoma.

PURPOSE: The aim of this study was to evaluate the safety and surgical outcome of superior petrosal vein (SPV, Dandy's vein) sacrifice in translabyrinthine approach (TLA) for resection of vestibule schwannoma (VS) as compared with SPV preservation, with further investigation of preoperational factors associated with the implement of SPV sacrifice. METHODS: The authors prospectively collected data from patients surgically treated for VS through TLA between June 2021 and April 2022 at the Gruppo Otologico. RESULTS: There were 30 and 49 patients in SPV sacrifice and preservation groups, respectively. SPV sacrifice group had significantly larger tumor size (2.46 vs. 1.40 cm), less percentage of solid tumor (26.7% vs. 83.7%), higher incidence of brainstem compression (80% vs. 26.5%), and higher percentage of facial numbness (20.0% vs. 4.1%) than SPV preservation group. Gross total resection (GTR) rates were 73.3% after SPV sacrifice and 87.8% after SPV preservation. Facial nerve preservation rates were similar. No complication related with SPV sacrifice was observed. Logistic regression analysis showed tumor size and complete solid consistency as significant risk factors associated with SPV sacrifice. ROC curve further demonstrated tumor size as a fair predictor (AUC = 0.833), with optimum cutoff value of 1.68 cm. CONCLUSION: SPV sacrifice via TLA as needed is a safe and effective maneuver for removal of relatively large VS. Tumor size and consistency can be used as a guidance in preoperational decision-making, with cutoff value of 1.68 cm and cystic formation as predictive indicators.

Stereotactic Radiosurgery for Vestibular Schwannoma With Radiographic Brainstem Compression.

OBJECTIVE: The safety of single-treatment stereotactic radiosurgery (SRS) for vestibular schwannoma (VS) with radiographic evidence of brainstem compression but without motor deficit is controversial. Data on linear accelerator (linac)-based SRS in this setting are scarce. We address this with an outcomes report from an unselected series of patients with VS with radiographic brainstem compression treated with linac SRS. METHODS: We included 139 patients with unilateral VS (any size) with radiographic brainstem compression (all without serious brainstem neurological deficits). The SRS prescription dose was 12.5 Gy (single fraction) using 6MV linac-produced photon beams, delivered with a multiple arc technique. Inclusion criteria required at least 1 year of radiographic follow-up with magnetic resonance imaging. The primary endpoint was freedom from serious brainstem toxicity (≥grade 3 Common Terminology Criteria for Adverse Events v5); the secondary was freedom from enlargement (tumor progression or any requiring intervention). We assessed serious cranial nerve complications, excluding hearing loss, defined as Common Terminology Criteria for Adverse Events v5 grade 3 toxicity. RESULTS: Median magnetic resonance imaging follow-up time was 5 years, and median tumor size was 2.5 cm in greatest axial dimension and 5 ml in volume. The median brainstem D0.03 ml=12.6 Gy and median brainstem V10 Gy=0.4 ml. At 5 years, the actuarial freedom from serious brainstem toxicity was 100%, and freedom from tumor enlargement (requiring surgery and/or due to progression) was 90%. Severe facial nerve damage in patients without tumor enlargement was 0.9%. CONCLUSION: Linac-based SRS, as delivered in our series for VS with radiographic brainstem compression, is safe and effective.

Management of sporadic intracanalicular vestibular schwannomas: A critical review and International Stereotactic Radiosurgery Society (ISRS) practice guidelines.

BACKGROUND: The choice of an appropriate strategy for intracanalicular vestibular schwannoma (ICVS) is still debated. We conducted a systematic review and meta-analysis with the aim to compare treatment outcomes amongst management strategies (conservative surveillance (CS), microsurgical resection (MR), or stereotactic radiosurgery (SRS)) aiming to inform guideline recommendations on behalf of the International Stereotactic Radiosurgery Society (ISRS). METHODS: Using PRISMA guidelines, we reviewed manuscripts published between January 1990 and October 2021 referenced in PubMed or Embase. Inclusion criteria were peer-reviewed clinical studies or case series reporting a cohort of ICVS managed with CS, MR, or SRS. Primary outcome measures included tumor control, the need for additional treatment, hearing outcomes, and posttreatment neurological deficits. These were pooled using meta-analytical techniques and compared using meta-regression with random effect. RESULTS: Forty studies were included (2371 patients). The weighted pooled estimates for tumor control were 96% and 65% in SRS and CS series, respectively (P < .001). Need for further treatment was reported in 1%, 2%, and 25% for SRS, MR, and CS, respectively (P = .001). Hearing preservation was reported in 67%, 68%, and 55% for SRS, MR, and CS, respectively (P = .21). Persistent facial nerve deficit was reported in 0.1% and 10% for SRS and MR series, respectively (P = .01). CONCLUSIONS: SRS is a noninvasive treatment with at least equivalent rates of tumor control and hearing preservation as compared to MR, with the caveat of better facial nerve preservation. As compared to CS, upfront SRS is an effective treatment in achieving tumor control with similar rates of hearing preservation.

Epidemiology and Risk Factors for Development of Sporadic Vestibular Schwannoma.

Vestibular schwannomas (VSs) are benign, slow-growing tumors of the eighth cranial nerve. Sporadic unilateral VSs constitute approximately 95% of all newly diagnosed tumors. There is little known about risk factors for developing sporadic unilateral VS. Potential risk factors that have been reported are familial or genetic risk, noise exposure, cell phone use, and ionizing radiation, whereas protective factors may include smoking and aspirin use. More research is needed to elucidate the risk factors for development of these rare tumors.

Noise exposure and the risk of cancer: a comprehensive systematic review.

The association between noise exposure and increased risk of cancer has received little attention in the field of research. Therefore, the goal of this study was to conduct a systematic review on the relationship between noise exposure and the incidence of cancer in humans. In this study, four electronic bibliographic databases including Scopus, PubMed, Web of Science, and Embase were systematically searched up to 21 April 2022. All types of noise exposure were considered, including environmental noise, occupational noise, and leisure or recreational noise. Furthermore, all types of cancers were studied, regardless of the organs involved. In total, 1836 articles were excluded on the basis of containing exclusion criteria or lacking inclusion criteria, leaving 19 articles retained for this study. Five of nine case-control studies showed a significant relationship between occupational or leisure noise exposure and acoustic neuroma. Moreover, four of five case-control and cohort studies indicated statistically significant relationships between environmental noise exposure and breast cancer. Of other cancer types, two case-control studies highlighted the risk of Hodgkin and non-Hodgkin lymphoma and two cohort studies identified an increased risk of colon cancer associated with environmental noise exposure. No relationship between road traffic and railway noise and the risk of prostate cancer was observed. In total, results showed that noise exposure, particularly prolonged and continuous exposure to loud noise, can lead to the incidence of some cancers. However, confirmation of this requires further epidemiological studies and exploration of the exact biological mechanism and pathway for these effects.

Single-fraction stereotactic radiosurgery versus microsurgical resection for the treatment of vestibular schwannoma: a systematic review and meta-analysis.

BACKGROUND: Vestibular schwannomas are benign tumours for which various treatments are available. We performed a systematic review of prospective controlled trials comparing the patient-relevant benefits and harms of single-fraction stereotactic radiosurgery (sfSRS) with microsurgical resection (MR) in patients with vestibular schwannoma. METHODS: We searched for randomized controlled trials (RCTs) and non-randomized prospective controlled trials in MEDLINE, Embase, the Cochrane Central Register of Controlled Trials, and study registries (last search: 09/2021) and also screened reference lists of relevant systematic reviews. Manufacturers were asked to provide unpublished data. Eligible studies investigated at least one patient-relevant outcome. We assessed the risk of bias (high or low) at the study and outcome level. If feasible, meta-analyses were performed. We graded the results into different categories (hint, indication, or proof of greater benefit or harm). RESULTS: We identified three non-randomized prospective controlled trials of generally low quality with evaluable data on 339 patients with unilateral vestibular schwannoma. There was an indication of greater benefit of sfSRS compared with MR for facial palsy (OR 0.06, 95% CI 0.02-0.21, p < 0.001, 2 studies), hearing function (no pooled estimate available, 2 studies), and length of hospital stay (no pooled estimate available, 2 studies). We found no clinically relevant differences for mortality, vertigo, headaches, tinnitus, balance function, work disability, adverse events, and health-related quality of life. CONCLUSIONS: Our systematic review indicates that sfSRS has greater benefits than MR in patients with unilateral vestibular schwannoma. However, it is unclear whether this conclusion still holds after 2 years, as long-term studies are lacking. It is also unclear whether the effects of sfSRS are similar in patients with bilateral vestibular schwannomas. Long-term prospective studies including patients with this condition would therefore be useful. SYSTEMATIC REVIEW REGISTRATION: The full (German language) protocol and report (Commission No. N20-03) are available on the institute's website: www.iqwig.de/en/projects/n20-03.html.

Stereotactic radiosurgery for Koos grade IV vestibular schwannoma in young patients: a multi-institutional study.

PURPOSE: Surgery is the treatment of choice for large vestibular schwannomas (VS). Stereotactic radiosurgery (SRS) has been suggested as an alternative to resection in selected patients. However, the safety and efficacy of SRS in Koos grade IV patients ≤ 45 years old has not been evaluated. The aim of this study is to describe the clinical and radiological outcomes of Koos grade IV in young patient managed with a single-session SRS. METHODS: This retrospective, multicenter analysis included SRS-treated patients, ≤ 45 years old presenting with non-life threatening or incapacitating symptoms due to a Koos Grade IV VS and with follow-up ≥ 12 months. Tumor control and neurological outcomes were evaluated. RESULTS: 176 patients [median age of 36.0 (IQR 9) and median tumor volume of 9.3 cm3 (IQR 4.7)] were included. The median prescription dose was 12 Gy (IQR 0.5). Median follow-up period was 37.5 (IQR 53.5) months. The 5- and 10-year progression-free survival was 90.9% and 86.7%. Early tumor enlargement occurred in 10.9% of cases and was associated with tumor progression at the last follow-up. The probability of serviceable hearing preservation at 5- and 10-years was 56.8% and 45.2%, respectively. The probability of improvement or preservation of facial nerve function was 95.7% at 5 and 10-years. Adverse radiation effects were noted in 19.9%. New-onset hydrocephalus occurred in 4.0%. CONCLUSION: Single-session SRS is a safe and effective alternative to surgical resection in selected patients ≤ 45 years old particularly those with medical co-morbidities and those who decline resection. Longer term follow up is warranted.

Stereotactic radiosurgery for vestibular schwannomas in neurofibromatosis type 2 patients: a systematic review and meta-analysis.

INTRODUCTION: Neurofibromatosis type 2 (NF2) is characterized by often bilateral vestibular schwannomas (VS) that result in progressive hearing loss and compression of nearby brainstem structures causing cranial nerve palsies. Treatment of these tumors remains challenging, as both surgical removal and expectant management can result in symptom progression. Stereotactic radiosurgery (SRS) has been investigated for the management of NF2-associated VS; however, the role, promises, and pitfalls of this treatment modality remain unclear. METHODS: Ovid MEDLINE, EMBASE, Web of Science, and Cochrane Reviews were searched for studies assessing SRS outcome in NF2-associated VS only. Primary endpoints included tumor control, serviceable hearing, presence of tinnitus, and cranial nerve V and VII symptoms. RESULTS: A total of 16 studies (589 patients harboring 750 tumors) were analyzed. Clinical tumor control was achieved in 88% of cases (95% CI 80-95%); salvage surgery was needed in 8% (95% CI 4-13%) of cases. Treatment resulted in a worsening of pre-treatment serviceable hearing (OR = 0.26, p < 0.01), increased facial nerve (OR = 1.62, p < 0.01) and trigeminal nerve (OR = 1.42, p = 0.07) impairment. The incidence of vestibular symptoms and hydrocephalus were not consistently reported and thus could not be assessed. CONCLUSIONS: The treatment of NF2-associated VS continues to pose a challenge, as current SRS regimens result in impaired hearing and worse cranial nerve comorbidities, despite achieving high tumor control. It remains unclear if these findings have to be regarded as treatment complications or, rather, continued disease progression.

Transcriptomic Profile Reveals Deregulation of Hearing-Loss Related Genes in Vestibular Schwannoma Cells Following Electromagnetic Field Exposure.

Hearing loss (HL) is the most common sensory disorder in the world population. One common cause of HL is the presence of vestibular schwannoma (VS), a benign tumor of the VIII cranial nerve, arising from Schwann cell (SC) transformation. In the last decade, the increasing incidence of VS has been correlated to electromagnetic field (EMF) exposure, which might be considered a pathogenic cause of VS development and HL. Here, we explore the molecular mechanisms underlying the biologic changes of human SCs and/or their oncogenic transformation following EMF exposure. Through NGS technology and RNA-Seq transcriptomic analysis, we investigated the genomic profile and the differential display of HL-related genes after chronic EMF. We found that chronic EMF exposure modified the cell proliferation, in parallel with intracellular signaling and metabolic pathways changes, mostly related to translation and mitochondrial activities. Importantly, the expression of HL-related genes such as NEFL, TPRN, OTOGL, GJB2, and REST appeared to be deregulated in chronic EMF exposure. In conclusion, we suggest that, at a preclinical stage, EMF exposure might promote the transformation of VS cells and contribute to HL.

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

BACKGROUND: To better counsel vestibular schwannoma patients, it is necessary to understand the tumor control rates of stereotactic radiosurgery (SRS). OBJECTIVES: To determine tumor control rates, factors determining control and complication rates following SRS. METHODS: Tertiary hospital retrospective cohort. RESULTS: 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). CONCLUSIONS: 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.

Can a causal relationship be established between acoustic neuroma and occupational exposure to non-ionizing radiations from mobile devices? Comparison between scientific literature data and medico-legal methodology.

BACKGROUND: Over the process of establishing the causal relation-ship, medical and legal methodologies may be at variance over the definitional standards and terminologies applied, which can hinder the activities of expert witnesses. OBJECTIVES: The article's authors have set out to assess whether, and under what conditions, a causal relationship can be established between acoustic neuroma and exposure to non-ionizing radiation from mobile communication devices. METHODS: The study design is a Systematic Review. The authors have drawn upon a 2020 Turin Court of Appeals ruling which found such a causal relationship in a somewhat peculiar case: rare tumor and exposure to non-ionizing radiation of unusually long and regular dura-tion. The case presents several peculiarities, herein analyzed in light of a) scientific evidence relative to the etiopathogenesis of the neuroma; b) available medico-legal literature defining causality evaluation criteria, and lastly c) court filings in regard to the probability standards applied to prove causal relationship. RESULTS: A direct tie cannot be proven, not even in cases of sub-stantially intense and lengthy exposure, if the medico-legal standards applied are not consistent enough to prove that nexus is more likely than not. DISCUSSION: Several elements suggest a causal relationship is unlikely: a) a dearth of evidence on humans; b) rats exposed to such radiation have developed cardiac tumors, not in their ears; c) exposure has caused no tumors in mice; d) the length of exposure is incompat-ible with tumor size and type. That fourth point only concerns the case herein explored, whereas the first three have a general scope of validity. The main limitation of the present study design is the heterogeneity among the included studies. Retrospective and prospective studies have been included, which may be a source of bias.

Early life-threating enlargement of a vestibular schwannoma after gamma knife radiosurgery.

Stereotactic gamma knife radiosurgery (GKS) may induce a transient enlargement of vestibular schwannomas (VS). This phenomenon, known as pseudoprogression or swelling, starts at about 3 months following GKS, peaks at about 6 months, and typically subsides thereafter, usually without significant neurological deterioration. We describe a 34-year-old female who developed an aggressive enlargement of a VS 1 month after GKS. The patient was treated with an immediate external ventricular drainage and surgical resection via retrosigmoid approach for an acute neurological deterioration due to hydrocephalus and brainstem compression. Histopathological examination revealed a VS with abundant intratumoral thrombosis and necrosis, suggesting that its rapid expansion could be related to massive radiation-induced tumor necrosis. The present case indicated that rapid life-threating enlargement of a VS may occur as an early complication following GKS.

Case-control study on occupational exposure to extremely low-frequency electromagnetic fields and the association with acoustic neuroma.

Exposure to extremely low-frequency electromagnetic fields (ELF-EMF) was in 2002 classified as a possible human carcinogen, Group 2B, by the International Agency for Research on Cancer at WHO based on an increased risk for childhood leukemia. In case-control studies on brain and head tumours during 1997-2003 and 2007-2009 we assessed life-time occupations in addition to exposure to different agents. The INTEROCC ELF-EMF Job-Exposure Matrix was used for associating occupations with ELF-EMF exposure (µT) with acoustic neuroma. Cumulative exposure (µT-years), average exposure (µT) and maximum exposed job (µT) were calculated. No increased risk for acoustic neuroma was found in any category. For cumulative exposure in the highest exposure category 8.52+ µT years odds ratio (OR) = 1.2, 95% confidence interval (CI) = 0.8-2.0, p linear trend = 0.37 was calculated. No statistically significant risks were found in the time windows 1-14 years, and 15+ years, respectively. In conclusion occupational ELF-EMF was not associated with an increased risk for acoustic neuroma.

Occupational Noise Exposure and Vestibular Schwannoma: A Case-Control Study in Sweden.

It has been suggested that the association between self-reported occupational noise exposure and vestibular schwannoma (VS), found in several studies, represents recall bias. Therefore, we aimed to study the relationship in a large case-control study using occupational noise measurements. We performed a case-control study using data from Sweden for 1,913 VS cases diagnosed in 1961-2009 and 9,566 age- and sex-matched population controls. We defined occupational history by linkage to national censuses from 1960, 1970, 1980, and 1990. We estimated occupational noise exposure for each case and control using a job-exposure matrix. There was no association between occupational noise exposure and VS. Among subjects assessed as ever exposed to occupational noise levels of ≥85 dB (214 cases and 1,142 controls), the odds ratio for VS per 5 years of exposure was 1.02 (95% confidence interval: 0.90, 1.17). Workers with noise levels of ≥85 dB for at least 15 years (5-year latency period), showed no increased risk of VS (odds ratio = 0.98, 95% confidence interval: 0.73, 1.31) compared with those who had never been exposed to noise levels of 75 dB or higher. In summary, our large study does not support an association between occupational noise exposure and VS.

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation.

OBJECTIVE: Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. INTERVENTIONS: Periodic radiologic follow-up, tumor resection, and hearing rehabilitation. MAIN OUTCOME MEASURES: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. RESULTS: Three NF2 patients (mean age at diagnosis, 26.3 ±â€Š3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ±â€Š1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. CONCLUSIONS: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

Psychiatric disorders in the acoustic neuroma: about a case.

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder characterized by formation of central nervous system tumors. They are associated to significant morbidity due to multiple problems such as hearing loss that can lead to many psychiatric disorders.

Efficacy and safety of bevacizumab for vestibular schwannoma in neurofibromatosis type 2: a systematic review and meta-analysis of treatment outcomes.

BACKGROUND: Individual evidence suggests that the anti-angiogenic agent bevacizumab may control vestibular schwannoma (VS) growth and promote hearing preservation in patients with neurofibromatosis type 2 (NF2). However, such metadata has yet to be consolidated, as well as its side-effect profile yet to be fully understood. Our aim was to pool systematically-identified metadata in the literature and substantiate the clinical efficacy and safety of bevacizumab with respect to radiographic tumor response, hearing, and treatment outcomes. METHODS: Searches of seven electronic databases from inception to March 2019 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. The incidence of outcomes was then extracted and pooled by random-effects meta-analysis of proportions. RESULTS: Eight articles reporting 161 NF2 patients with 196 assessable VS met satisfied all criteria. Radiographic response to bevacizumab was partial regression in 41% (95% CI 31-51%), no change in 47% (95% CI 39-55%), and tumor progression in 7% (95% CI 1-15%). In patients with assessable audiometric data, bevacizumab treatment resulted in hearing improvement in 20% (95% CI 9-33%), stability in 69% (95% CI 51-85%) and additional loss in 6% (95% CI 1-15%) Serious bevacizumab toxicity was observed in 17% (95% CI 10-26%). Subsequent surgical intervention was required in 11% (95% CI 2-20%). CONCLUSIONS: Bevacizumab may arrest both tumor progression and hearing loss in select NF2 patients presenting with VS lesions. However, a considerable proportion of patients are anticipated to experience serious adverse events; correspondingly, judicious use of bevacizumab for symptomatic management of VS in NF2 is recommended.

Noise exposure as a risk factor for acoustic neuroma: a systematic review and meta-analysis.

Objective: To investigate whether acoustic neuroma is associated with noise. Design: PubMed, Cochrane, Embase and CINAHL databases were searched. A meta-analysis was performed to calculate odds ratio (OR) and 95% confidence interval (CI) using quality-effect models. Study sample: A total of eight studies with moderate or high quality involving 75,571 participants met the inclusion criteria. Results: There was no significant relationship between overall noise exposure and acoustic neuroma (OR:1.02, 95% CI: 0.64-1.63). However, further subgroup analysis showed that leisure noise exposure (OR: 1.73, 95% CI: 1.10-2.73), above five years' exposure (OR: 1.81, 95% CI: 1.14-2.85) and continuous exposure (OR:2.77, 95% CI: 1.70-4.49) were associated with an increased risk of acoustic neuroma. Conclusions: These results suggest an elevated risk of acoustic neuroma among individuals who have been exposed to occupational noise when some subgroup analysis are conducted. Leisure noise in particular seems to play a significant role in the development of acoustic neuroma. However, due to the heterogeneity among the included studies, this conclusion should be interpreted with cautions, even though the continuous long-term consequences should not be ignored.