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European Archives of... Jan 2014The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to... (Review)
Review
The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.
Topics: Auditory Brain Stem Implants; Cochlear Implants; Contraindications; Deafness; Hearing Loss; Hearing Loss, Central; Humans; Neuroma, Acoustic; Ossification, Heterotopic; Otosclerosis; Treatment Outcome; Vestibulocochlear Nerve Diseases
PubMed: 23404468
DOI: 10.1007/s00405-013-2378-3 -
Journal of Neurosurgery. Pediatrics May 2020Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to... (Review)
Review
OBJECTIVE
Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to surgical treatment, and recurrence rates in these younger patients. The authors' goal was to describe their institutional experience with pediatric sporadic vestibular schwannomas and to conduct a meta-analysis of the existing literature to provide further insight into the presentation, tumor characteristics, and surgical outcomes for these rare tumors to help direct future treatment strategies.
METHODS
The authors performed a retrospective review of all patients 21 years of age or younger with unilateral vestibular schwannomas and without neurofibromatosis type 2 who underwent resection by the senior authors between 1997 and 2019. A systematic review of the literature and meta-analysis was also performed by entering the search terms "pediatric" and "vestibular schwannoma" or "acoustic neuroma," as well as "sporadic" into PubMed. Presentation, treatment, clinical outcomes, and follow-up were analyzed.
RESULTS
Fifteen patients were identified at the authors' institution, ranging in age from 12 to 21 years (mean 16.5 years). Common presenting symptoms included hearing loss (87%), headache (40%), vertigo (33%), ataxia (33%), and tinnitus (33%). At the time of surgery, the mean tumor size was 3.4 cm, with four 1-cm tumors. Four patients had residual tumor following their first surgery, 3 (75%) of whom had significant radiographic regrowth that required further treatment. The literature review identified an additional 81 patients from 26 studies with patient-specific clinical data available for analysis. This resulted in a total of 96 reported patients with an overall average age at diagnosis of 12.1 years (range 6-21 years) and an average tumor size of 4.1 cm.
CONCLUSIONS
Pediatric vestibular schwannomas present similarly to those in adults, although symptoms of mass effect are more common, as these tumors tend to be larger at diagnosis. Some children are found to have small tumors and can be successfully treated surgically. Residual tumors in pediatric patients were found to have a higher rate of regrowth than those in their adult counterparts.
PubMed: 32470932
DOI: 10.3171/2020.3.PEDS19514 -
La Clinica Terapeutica May 2021Over the process of establishing the causal relation-ship, medical and legal methodologies may be at variance over the definitional standards and terminologies applied,...
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.
Topics: Animals; Causality; Computers, Handheld; Humans; Mice; Neuroma, Acoustic; Occupational Exposure; Radiation, Nonionizing; Rats; Risk Factors
PubMed: 33956036
DOI: 10.7417/CT.2021.2313 -
Reviews on Environmental Health Dec 2023The 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... (Review)
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.
Topics: Male; Humans; Risk Factors; Environmental Exposure; Noise; Neuroma, Acoustic; Leisure Activities
PubMed: 36064622
DOI: 10.1515/reveh-2022-0021 -
Neurosurgical Review Jul 2023Proton beam therapy is considered, by some authors, as having the advantage of delivering dose distributions more conformal to target compared with stereotactic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Proton beam therapy is considered, by some authors, as having the advantage of delivering dose distributions more conformal to target compared with stereotactic radiosurgery (SRS). Here, we performed a systematic review and meta-analysis of proton beam for VSs, evaluating tumor control and cranial nerve preservation rates, particularly with regard to facial and hearing preservation.
METHODS
We reviewed, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) articles published between 1968 and September 30, 2022. We retained 8 studies reporting 587 patients.
RESULTS
Overall rate of tumor control (both stability and decrease in volume) was 95.4% (range 93.5-97.2%, p heterogeneity= 0.77, p<0.001). Overall rate of tumor progression was 4.6% (range 2.8-6.5%, p heterogeneity < 0.77, p<0.001). Overall rate of trigeminal nerve preservation (absence of numbness) was 95.6% (range 93.5-97.7%, I = 11.44%, p heterogeneity= 0.34, p<0.001). Overall rate of facial nerve preservation was 93.7% (range 89.6-97.7%, I = 76.27%, p heterogeneity<0.001, p<0.001). Overall rate of hearing preservation was 40.6% (range 29.4-51.8%, I = 43.36%, p heterogeneity= 0.1, p<0.001).
CONCLUSION
Proton beam therapy for VSs achieves high tumor control rates, as high as 95.4%. Facial rate preservation overall rates are 93%, which is lower compared to the most SRS series. Compared with most currently reported SRS techniques, proton beam radiation therapy for VSs does not offer an advantage for facial and hearing preservation compared to most of the currently reported SRS series.
Topics: Humans; Neuroma, Acoustic; Proton Therapy; Hearing; Cranial Nerves; Facial Nerve; Radiosurgery; Treatment Outcome; Follow-Up Studies; Retrospective Studies
PubMed: 37402894
DOI: 10.1007/s10143-023-02060-x -
Neurosurgical Review Aug 2021Most studies reported that cystic vestibular schwannoma (CVS) surgery has a less favourable prognosis than solid vestibular schwannoma (SVS) surgery. However, some... (Meta-Analysis)
Meta-Analysis Review
Most studies reported that cystic vestibular schwannoma (CVS) surgery has a less favourable prognosis than solid vestibular schwannoma (SVS) surgery. However, some studies report that it is unclear whether surgical outcomes for these conditions exhibit significant differences. The aim of this meta-analysis was to pool the current literature and describe and analyse any differences in the clinical symptoms and surgical outcomes among CVS and SVS. PubMed, Embase, and Cochrane databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines through April 22, 2020. Outcomes were analysed using a meta-analysis of the proportions. The results of the search yielded 17 studies that met the criteria for inclusion and analysis, involving 3074 participants (including 821 patients with CVSs and 2253 patients with SVSs). No significant differences in the extent of tumour resection (gross-total resection versus non-gross-total resection; RR, 0.93; 95% CI, 0.86-1.01; p = 0.096) and most other outcomes were noted between CVS and SVS cohorts. However, facial nerve function (House-Brackmann Grade I or II versus III or more) of CVS patients was worse (RR, 0.86; 95% CI, 0.78-0.93; p < 0.001) compared with SVS patients with more than 1 year of follow-up after surgery. The criteria of cystic acoustic neuroma needs to be further unified, and prospective cohort studies with larger sample sizes should be performed for further verification of these results in the future.
Topics: Facial Nerve; Humans; Neuroma, Acoustic; Retrospective Studies; Treatment Outcome
PubMed: 33009643
DOI: 10.1007/s10143-020-01400-5 -
The Laryngoscope Mar 2017To report two patients with a history of microvascular decompression (MVD) for hemifacial spasm who presented with Teflon granulomas (TG) mimicking cerebellopontine... (Review)
Review
OBJECTIVES/HYPOTHESIS
To report two patients with a history of microvascular decompression (MVD) for hemifacial spasm who presented with Teflon granulomas (TG) mimicking cerebellopontine angle (CPA) tumors and to perform a systematic review of the English-language literature.
STUDY DESIGN
Case series at a single tertiary academic referral center and systematic review.
METHODS
Retrospective chart review with analysis of clinical, radiological, and histopathological findings. Systematic review using PubMed, Embase, MEDLINE, and Web of Science databases.
RESULTS
Two patients with large skull base TGs mimicking CPA tumors clinically and radiographically were managed at the authors' institution. The first presented 4 years after MVD with asymmetrical sensorineural hearing loss, multiple progressive cranial neuropathies, and brainstem edema due to a growing TG. Reoperation with resection of the granuloma confirmed a foreign-body reaction consisting of multinucleated giant cells containing intracytoplasmic Teflon particles. The second patient presented 11 years after MVD with asymmetrical sensorineural hearing loss and recurrent hemifacial spasm. No growth was noted over 2 years, and the patient has been managed expectantly. Only one prior case of TG after MVD for hemifacial spasm has been reported in the English literature.
CONCLUSIONS
TG is a rare complication of MVD for hemifacial spasm. The diagnosis should be suspected in patients presenting with a new-onset enhancing mass of the CPA after MVD, even when performed decades earlier. A thorough clinical and surgical history is critical toward establishing an accurate diagnosis to guide management and prevent unnecessary morbidity. Surgical intervention is not required unless progressive neurologic complications ensue.
LEVEL OF EVIDENCE
4 Laryngoscope, 127:715-719, 2017.
Topics: Academic Medical Centers; Adult; Biopsy, Needle; Cerebellar Neoplasms; Cerebellopontine Angle; Diagnosis, Differential; Female; Follow-Up Studies; Granuloma, Foreign-Body; Hemifacial Spasm; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Microvascular Decompression Surgery; Middle Aged; Polytetrafluoroethylene; Postoperative Complications; Reoperation; Retrospective Studies; Sampling Studies; Treatment Outcome
PubMed: 27320780
DOI: 10.1002/lary.26126 -
Bioelectromagnetics Apr 2012We conducted a systematic review of scientific studies to evaluate whether the use of wireless phones is linked to an increased incidence of the brain cancer glioma or... (Meta-Analysis)
Meta-Analysis Review
We conducted a systematic review of scientific studies to evaluate whether the use of wireless phones is linked to an increased incidence of the brain cancer glioma or other tumors of the head (meningioma, acoustic neuroma, and parotid gland), originating in the areas of the head that most absorb radiofrequency (RF) energy from wireless phones. Epidemiology and in vivo studies were evaluated according to an agreed protocol; quality criteria were used to evaluate the studies for narrative synthesis but not for meta-analyses or pooling of results. The epidemiology study results were heterogeneous, with sparse data on long-term use (≥ 10 years). Meta-analyses of the epidemiology studies showed no statistically significant increase in risk (defined as P < 0.05) for adult brain cancer or other head tumors from wireless phone use. Analyses of the in vivo oncogenicity, tumor promotion, and genotoxicity studies also showed no statistically significant relationship between exposure to RF fields and genotoxic damage to brain cells, or the incidence of brain cancers or other tumors of the head. Assessment of the review results using the Hill criteria did not support a causal relationship between wireless phone use and the incidence of adult cancers in the areas of the head that most absorb RF energy from the use of wireless phones. There are insufficient data to make any determinations about longer-term use (≥ 10 years).
Topics: Adult; Brain Neoplasms; Case-Control Studies; Cell Phone; Cohort Studies; Glioma; Humans; Meningeal Neoplasms; Meningioma; Neoplasms, Radiation-Induced; Neuroma, Acoustic; Parotid Neoplasms; Radio Waves; Risk Assessment; Risk Factors
PubMed: 22021071
DOI: 10.1002/bem.20716 -
Neurological Research Oct 2003Conservative management, gamma-knife (GK) radiosurgery, and microsurgery are therapeutic options for acoustic neurinomas (ANs). To determine the outcomes and risks of... (Comparative Study)
Comparative Study Review
Conservative management, gamma-knife (GK) radiosurgery, and microsurgery are therapeutic options for acoustic neurinomas (ANs). To determine the outcomes and risks of these methods this systematic review analyzed data from 903 patients with conservative management, 1475 with GK radiosurgery, and 5005 with microsurgery from 38 studies identified in MEDLINE searches. Conservative management over a 3.1-year period showed that 51% of ANs showed a tumor growth, an average tumor growth rate was 1.87 mm year-1, 20% of ANs ultimately required surgical intervention, and a third of the patients lost useful hearing. GK radiosurgery significantly reduced the percentage of ANs that enlarged, to 8%, and reduced the percentage that underwent microsurgery to 4.6% over a 3.8-year period. Microsurgery removed 96% of ANs totally, with tumor recurrence, mortality, and major disability rates of 1.8%, 0.63%, and 2.9%, respectively. The majority of ANs grow slowly, but ultimately require intervention. Carrying the risk of hearing loss, conservative management should be supplemented with close follow-up. With a low rate of morbidity, GK radiosurgery suppresses tumor growth and provides good tumor control. Microsurgery provides the best tumor control, although mortality and morbidity are not completely eliminated.
Topics: Chi-Square Distribution; Disease Management; Humans; Microsurgery; Neuroma, Acoustic; Radiosurgery; Risk Factors; Treatment Outcome
PubMed: 14579782
DOI: 10.1179/016164103101202075 -
Pediatric Neurology Sep 2022The neurofibromatoses comprise three different genetic conditions causing considerable morbidity and mortality: neurofibromatosis type 1 (NF1), neurofibromatosis type 2... (Review)
Review
INTRODUCTION
The neurofibromatoses comprise three different genetic conditions causing considerable morbidity and mortality: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). This review summarizes recent and ongoing clinical trials involving patients with neurofibromatoses to better understand the current state of clinical trial research centered around these conditions and inform areas of need.
METHODS
A search was conducted using the Cochrane Central Register of Controlled Trials and clinicaltrials.gov databases. Inclusion and exclusion criteria were designed to identify clinical trials focused on patients with NF1, NF2, or SWN completed in or after 2010 and in process as of December 31, 2021. Information was collected using standardized guidelines.
RESULTS
A total of 134 clinical trials were included, with 75 (56%) completed and 59 (44%) in process. For completed trials, 74% (n = 56) involved patients with NF1, and of those based on specific tumors (n = 26, 46%), the majority focused on plexiform neurofibromas (PNs) (n = 12, 46%). For ongoing trials, 79% (n = 47) involve patients with NF1, and of those based on specific tumors (n = 29, 61%), the majority are focused on PNs (n = 13, 45%).
CONCLUSION
Both recent and ongoing clinical trials have primarily focused on patients with NF1 and the treatment of PNs. This research has led to the first FDA-approved drug for NF1-PN and has changed management of these tumors, allowing for systemic therapy rather than reliance on only a surgical modality. Trials evaluating comorbid psychiatric conditions and quality of life among patients with any of the neurofibromatoses appear less common. These areas may warrant focus in future studies to improve clinical management.
Topics: Humans; Neurilemmoma; Neurofibroma, Plexiform; Neurofibromatoses; Neurofibromatosis 1; Neurofibromatosis 2; Quality of Life; Skin Neoplasms
PubMed: 35759947
DOI: 10.1016/j.pediatrneurol.2022.06.003