Acoustic neuroma (also sometimes called a neurinoma or schwannoma) is a benign (non-cancerous) tissue growth that arises on the eighth cranial nerve. This nerve transmits hearing and balance information from the inner ear to the brain. Most acoustic neuromas are diagnosed between the ages of thirty and sixty.
Definition: "A benign schwannoma of the eighth cranial nerve (vestibulocochlear nerve), mostly arising from the vestibular branch (vestibular nerve) during the fifth or sixth decade of life. Clinical manifestations include hearing loss; headache; vertigo; tinnitus; and facial pain. Bilateral acoustic neuromas are associated with Neurofibromatosis 2. (From Adams et al., Principles of Neurology, 6th ed, p673)" [Source: MeSH, 2013]
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MeSH term: Neuroma, Acoustic
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This list of publications is regularly updated (Source: PubMed).
Inter-observer variability between radiologists reporting on cerebellopontine angle tumours on magnetic resonance imaging.
J Laryngol Otol. 2017; 131(S1):S47-S49 [PubMed] Related Publications
OBJECTIVE: To determine intra- and inter-observer measurement variability of cerebellopontine angle tumours in a specialised institution.
METHODS: The magnetic resonance imaging maximal diameter of 12 randomly selected cerebellopontine angle tumours were independently measured by 4 neuroradiologists at a tertiary referral centre using a standard definition for maximal tumour diameter. Average deviation and intraclass correlation were subsequently calculated.
RESULTS: Inter-observer difference averaged 0.33 ± 0.04 mm (range, 0.0-0.8 mm). Intra-observer measurements were more consistent than inter-observer measurements, with differences averaging 0.17 mm (95 per cent confidence interval = 0.27-0.06, p = 0.002). Inter-observer reliability was 0.99 (95 per cent confidence interval = 0.97-0.99), suggesting high reliability between the readings.
CONCLUSION: The use of a standard definition for maximal tumour volume provided high reliability amongst radiologists' readings. To avoid oversizing tumours, it is recommended that conservative monitoring be conducted by the same institution with thin slice magnetic resonance imaging scans.
Functional outcome and complications after the microsurgical removal of giant vestibular schwannomas via the retrosigmoid approach: a retrospective review of 16-year experience in a single hospital.
BMC Neurol. 2017; 17(1):18 [PubMed] Free Access to Full Article Related Publications
METHODS: We consecutively enrolled 657 unilateral giant (>4 cm diameter) vestibular schwannoma patients treated in Huashan Hospital via the suboccipital retrosigmoid approach in the past 16 years. The extension of tumor removal, surgical mortality, facial nerve function, hearing, and the other main short and long-term complications were the studied parameters.
RESULTS: Gross total resection was performed in 556 patients (84.6%); near-total resection was achieved in 99 patients (15.1%). The mortality rate is 0.6%. The main short-term complications included 'new' deafness (47.6%), intracranial infection (7.6%), lower cranial nerve defects (7.5%) and pneumonia (6.2%). The facial nerve was preserved anatomically in 589 cases (89.7%). Good facial nerve functional outcome (House-Brackmann Grades I and II) postoperatively was achieved in 216 patients (32.9%). Other 308 cases (46.9%) were House-Brackmann grade III, and 133 patients (20.2%) were House-Brackmann grade IV-VI. Follow-up data were available for 566 of the 657 patients (86.1%). The common long-term complications were hearing loss (85.2%), facial paralysis (HB grade IV-VI, 24.4%) and facial numbness (15.7%).
CONCLUSIONS: Trends in the data lead the authors to suggest that the microsurgical technique, intraoperative nerve monitoring, and multidisciplinary cooperation, were the keys to improving prognostic outcomes in giant intracranial vestibular schwannoma patients.
Vertigo Perception and Quality of Life in Patients after Surgical Treatment of Vestibular Schwannoma with Pretreatment Prehabituation by Chemical Vestibular Ablation.
Biomed Res Int. 2016; 2016:6767216 [PubMed] Related Publications
miR-1 association with cell proliferation inhibition and apoptosis in vestibular schwannoma by targeting VEGFA.
Genet Mol Res. 2016; 15(4) [PubMed] Related Publications
Vestibular schwannomas treated with Cyberknife®: clinical outcomes.
Tumori. 2016; 102(6):569-573 [PubMed] Related Publications
METHODS: Patients treated with FSR for VS between 2007 and 2012 were retrospectively analyzed. Local control was determined using follow-up MRI. The hearing preservation (HP) rate was determined by analyzing pretreatment and posttreatment audiograms.
RESULTS: Forty patients were treated for VS with the CK in both centers. The mean maximal VS dimension was 18.3 mm (range 3-30). The median follow-up was 36 months and the LC was 97% at 3 years of follow-up and 89% after 5 years. The HP rate was 83% and no facial nerve impairment was reported.
CONCLUSIONS: Our results in terms of LC and HP rate are congruent with similar studies that use the CK to treat VS. It appears that the CK is safe and efficient in VS management even for large lesions. Further studies with larger cohorts are warranted.
Audiovestibular Function Deficits in Vestibular Schwannoma.
Biomed Res Int. 2016; 2016:4980562 [PubMed] Free Access to Full Article Related Publications
Gastric plexiform schwannoma in association with neurofibromatosis type 2.
Clin J Gastroenterol. 2016; 9(6):352-357 [PubMed] Related Publications
CLINICAL TRIAL REGISTRATION: None.
A Diagnostic Dilemma: Multiple Primary Intracranial Tumors Without Vestibular Schwannomas.
Ann Otol Rhinol Laryngol. 2016; 125(11):938-942 [PubMed] Related Publications
Baha implant as a hearing solution for single-sided deafness after retrosigmoid approach for the vestibular schwannoma: audiological results.
Eur Arch Otorhinolaryngol. 2017; 274(1):133-141 [PubMed] Related Publications
Two cases of cystic enlargement of vestibular schwannoma as a late complication following gamma knife surgery.
J Clin Neurosci. 2016; 33:239-241 [PubMed] Related Publications
Comparing the sensitivity of linear and volumetric MRI measurements to detect changes in the size of vestibular schwannomas in patients with neurofibromatosis type 2 on bevacizumab treatment.
Br J Radiol. 2016; 89(1065):20160110 [PubMed] Article available free on PMC after 01/09/2017 Related Publications
METHODS: We compared retrospectively, changes in linear tumour dimensions at a range of thresholds to volumetric tumour measurements performed using Brainlab iPlan(®) software (Feldkirchen, Germany) and classified for tumour progression according to the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) criteria.
RESULTS: Assessment of 61 schwannomas in 46 patients with a median follow-up of 20 months (range 3-43 months) was performed. There was a mean of 7 time points per tumour (range 2-12 time points). Using the volumetric REiNS criteria as the gold standard, a sensitivity of 86% was achieved for linear measurement using a 2-mm threshold to define progression.
CONCLUSION: We propose that a change in linear measurement by 2 mm (particularly in tumours with starting diameters 20-30 mm, the majority of this cohort) could be used as a filter to identify cases of possible progression requiring volumetric analysis. This pragmatic approach can be used if stabilization of a previously growing schwannoma is sufficient for a patient to continue treatment in such a circumstance.
ADVANCES IN KNOWLEDGE: We demonstrate the real-world limitations of linear vs volumetric measurement in tumour response assessment and identify limited circumstances where linear measurements can be used to determine which patients require the more resource-intensive volumetric measurements.
Growth rate of vestibular schwannoma.
J Clin Neurosci. 2016; 32:1-8 [PubMed] Related Publications
Imaging Findings for Bilateral Giant Vestibulocochlear Schwannoma.
Arch Iran Med. 2016; 19(7):518-20 [PubMed] Related Publications
The Agreement between Protocols for the Investigation of Asymmetrical Audiovestibular Symptoms.
J Int Adv Otol. 2016; 12(1):98-100 [PubMed] Related Publications
MATERIALS AND METHODS: Retrospective audit of the indications for arranging 854 consecutive magnetic resonance imaging scans of the internal auditory meatus. These indications were compared to the Oxford, Northern, Charing Cross, and Nashville guidelines on the investigation of asymmetrical audiovestibular symptoms.
RESULTS: The level of agreement was low, with kappa values ranging between 0.15 and 0.58 between the four selected protocols.
DISCUSSION: While these criteria seem very similar in nature, due to the number of patients with mild asymmetry and subtle distinctions such as the inclusion or exclusion of tinnitus, there are low levels of agreement between protocols. This study highlights another area of difficulty when determining which patients to investigate.
Cholesterol granuloma presenting with facial nerve paralysis following translabyrinthine surgery: A case report.
Auris Nasus Larynx. 2017; 44(2):241-244 [PubMed] Related Publications
CASE: A 70-year-old woman, who underwent TL surgery previously, presented with left facial palsy (FP). CT findings revealed devastated cochlea and uncovered facial nerve, and progression of FP up to House-Brackmann grade 6 was observed. Therefore, we suspected recurrence of cystic VS or facial schwannoma, although MRI was inconsistent with that of schwannoma. The cyst was completely surgically excised, and histopathology confirmed CG diagnosis. Postoperatively her facial movement improved to grade 4. Recurrence has not been encountered since 7 years.
CONCLUSION: During follow-up after TL surgery for VS, care should be taken for possibility of CG occurring.
Expansive Extracranial Growth of Intracranial Meningioma in Neurofibromatosis Type 2.
Anticancer Res. 2016; 36(6):3161-7 [PubMed] Related Publications
Video head impulse in comparison to caloric testing in unilateral vestibular schwannoma.
Acta Otolaryngol. 2016; 136(11):1110-1114 [PubMed] Related Publications
OBJECTIVE: There is limited information that can be used to determine which of the video head impulse test (vHIT) and caloric test might be better used in the diagnosis and management of UVS. In this study, a group of participants with un-operated UVS was studied using both methods.
METHODS: The subjects' vestibular function was assessed using the vHIT and caloric testing. Tumour size was quantified using MRI and their balance disturbance assessed using the Jacobsen Dizziness Handicap Inventory (DHI).
RESULTS: Twenty of 30 subjects had an abnormal canal paresis according to the Jongkees' criterion (> 0.25); however, only 10/30 had an ipsilesional vHIT gain of <0.79. Canal paresis could be predicted from the ipsilesional and contralesional vHIT gains. Tumour size could also be predicted from the ipsilesional vHIT gain and canal paresis. However, DHI scores could not be predicted from the degree of canal paresis, vHIT gain, or the MRI measures.
Optimal outcomes for hearing preservation in the management of small vestibular schwannomas.
J Laryngol Otol. 2016; 130(7):606-10 [PubMed] Related Publications
METHODS: The Medline database was searched for publications that included the terms 'vestibular schwannoma' and/or 'acoustic neuroma', occurring in conjunction with 'hearing'. Articles were manually screened to identify those concerning vestibular schwannomas under 1.5 cm in greatest dimension. Thereafter, only publications discussing both pre-operative and post-operative hearing were considered.
RESULTS: Twenty-six papers were identified. Observation is an acceptable strategy for small tumours with slow growth where hearing preservation is not a consideration. In contrast, microsurgery, including the middle fossa approach, may provide excellent hearing outcomes, particularly when a small tumour has begun to cause hearing loss. Immediate post-operative hearing usually predicts long-term hearing. Recent data on stereotactic radiation suggest long-term deterioration of hearing following definitive therapy.
CONCLUSION: In patients under the age of 65 years with small vestibular schwannomas, microsurgery via the middle fossa approach offers durable preservation of hearing.
Hearing Restoration in Neurofibromatosis Type II Patients.
Yonsei Med J. 2016; 57(4):817-23 [PubMed] Article available free on PMC after 01/09/2017 Related Publications
Multifocal granulocytic sarcoma of the spine mimicking neurofibromatosis Type 2: case report.
J Neurosurg Spine. 2016; 25(4):523-527 [PubMed] Related Publications
Mechanism-based modeling of the clinical effects of bevacizumab and everolimus on vestibular schwannomas of patients with neurofibromatosis type 2.
Cancer Chemother Pharmacol. 2016; 77(6):1263-73 [PubMed] Related Publications
METHODS: Clinical data, including longitudinal tumor volumes in patients treated by bevacizumab (n = 13), everolimus (n = 7) or both (n = 2), were analyzed by means of mathematical modeling techniques. Together with clinical data, data from the literature were also integrated to account for drugs mechanisms of action.
RESULTS: We developed a model of vestibular schwannoma growth that takes into account the effect of vascular endothelial growth factors and mammalian target of rapamycin complex 1 on tumor growth. Behaviors, such as tumor growth rebound following everolimus treatment stops, was correctly described with the model. Preliminary results indicate that the model can be used to predict, based on early tumor volume dynamic, tumor response to variation in treatment dose and regimen.
CONCLUSION: The developed model successfully describes tumor volume growth before and during bevacizumab and/or everolimus treatment. It might constitute a rational tool to predict patients' response to these drugs, thus potentially improving management of this disease.
Abnormal Magnetic Resonance Imaging Findings in Patients With Sudden Sensorineural Hearing Loss: Vestibular Schwannoma as the Most Common Cause of MRI Abnormality.
Medicine (Baltimore). 2016; 95(17):e3557 [PubMed] Article available free on PMC after 01/09/2017 Related Publications
Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma.
Recent Results Cancer Res. 2016; 205:17-27 [PubMed] Related Publications
Auditory brainstem response - a valid and cost-effective screening tool for vestibular schwannoma?
Acta Otolaryngol. 2016; 136(7):660-2 [PubMed] Related Publications
OBJECTIVES: The aim of this study is to assess the sensitivity, specificity, and cost-effectiveness of ABR vs MRI, when screening for VS in a clinical setting.
METHOD: Data was collected on all patients subjected to an ABR during a 3-year period (2005-2008) at a tertiary referral center. The data were compared to results of MRI and with data from The Danish National VS Database. In total, 1447 adults underwent ABR, of whom 349 were referred for a subsequent MRI. Sensitivity and specificity were calculated. To assess the cost-effectiveness of ABR vs MRI, procedure costs for ABR and MRI were retrieved from the National Board of Health.
RESULTS: Twelve (3.4%) of 349 patients were diagnosed with a VS. Of the 1098 patients not referred for MRI, three (0.3%) were later diagnosed with a VS. The sensitivity and specificity of ABR in diagnosing a VS were 80% and 77%, respectively. Procedure costs were 326 EURO for the ABR and 319 EURO for the MRI.
Multisession Radiosurgery for Hearing Preservation.
Semin Radiat Oncol. 2016; 26(2):105-11 [PubMed] Related Publications
Risk Factors of Acoustic Neuroma: Systematic Review and Meta-Analysis.
Yonsei Med J. 2016; 57(3):776-83 [PubMed] Article available free on PMC after 01/09/2017 Related Publications
MATERIALS AND METHODS: We searched PubMed to identify relevant articles. Two researchers evaluated the eligibility and extracted the data independently.
RESULTS: Eleven case-control studies were included in our meta-analysis. Acoustic neuroma was found to be associated with leisure noise exposure [odds ratio (OR)=1.33, 95% confidence interval (CI): 1.05-1.68], but not with occupational noise exposure and ever noise exposure (OR=1.20, 95% CI: 0.84-1.72 and OR=1.15, 95% CI: 0.80-1.65). The OR of acoustic neuroma for ever (versus never) smoking was 0.53 (95% CI: 0.30-0.94), while the subgroup analysis indicated ORs of 0.95 (95% CI: 0.81-1.10) and 0.49 (95% CI: 0.41-0.59) for ex-smoker and current smoker respectively. The ORs for asthma, eczema, and seasonal rhinitis were 0.98 (95% CI: 0.80-1.18), 0.91 (95% CI: 0.76-1.09), and 1.52 (95% CI: 0.90-2.54), respectively.
CONCLUSION: Our meta-analysis is suggestive of an elevated risk of acoustic neuroma among individuals who were ever exposed to leisure noise, but not to occupational noise. Our study also indicated a lower acoustic neuroma risk among ever and current cigarette smokers than never smokers, while there was no significant relationship for ex-smokers. No significant associations were found between acoustic neuroma and history of any allergic diseases, such as asthma, eczema, and seasonal rhinitis.
Significance of Vestibular Testing on Distinguishing the Nerve of Origin for Vestibular Schwannoma and Predicting the Preservation of Hearing.
Chin Med J (Engl). 2016; 129(7):799-803 [PubMed] Article available free on PMC after 01/09/2017 Related Publications
METHODS: A total of 106 patients with unilateral VS were enrolled in this study prospectively. Each patient received a caloric test, vestibular-evoked myogenic potential (VEMP) test, and cochlear nerve function test (hearing) before the operation and 1 week, 3, and 6 months, postoperatively. All patients underwent surgical removal of the VS using the suboccipital approach. During the operation, the nerve of tumor origin (SVN or IVN) was identified by the surgeon. Tumor size was measured by preoperative magnetic resonance imaging.
RESULTS: The nerve of tumor origin could not be unequivocally identified in 38 patients (38/106, 35.80%). These patients were not subsequently evaluated. In 26 patients (nine females, seventeen males), tumors arose from the SVN and in 42 patients (18 females, 24 males), tumors arose from the IVN. Comparing with the nerve of origins (SVN and IVN) of tumors, the results of the caloric tests and VEMP tests were significantly different in tumors originating from the SVN and the IVN in our study. Hearing was preserved in 16 of 26 patients (61.54%) with SVN-originating tumors, whereas hearing was preserved in only seven of 42 patients (16.67%) with IVN-originating tumors.
CONCLUSIONS: Our data suggest that caloric and VEMP tests might help to identify whether VS tumors originate from the SVN or IVN. These tests could also be used to evaluate the residual function of the nerves after surgery. Using this information, we might better predict the preservation of hearing for patients.
Surgery for sporadic vestibular schwannoma. Part II. Complications (not related to facial and auditory nerves).
Neurol Neurochir Pol. 2016; 50(2):90-7 [PubMed] Related Publications
MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; age ranged from 18 to 74) operated on with the retrosigmoid (217) or translabyrinthine (3) approach. Complicated postoperative period was defined as an occurrence of at least one of: cerebrospinal fluid (CSF) leakage, hematoma in the tumor bed, intracerebellar hematoma, cerebellar swelling, brainstem stroke, hydrocephalus (HCP), healing problems, meningitis and cranial nerves (excluding VII-VIII) palsies or cerebellar symptoms. Correlation studies and multivariate regression analysis were performed.
RESULTS: PC occurred in 55 patients (25%). PC included lower cranial nerve (LCN) palsy (8.2%), cerebellar symptoms (7.3%), CSF leakage (5.9%), HCP (5%), CNVI palsy (3.1%), meningitis (1.8%), cerebellar swelling (1.4%), CNV dysfunction (0.9%), intracerebellar hematoma (0.5%) and lethal brainstem stroke (0.5%). In long term follow-up, LCN deficit was present in 2 patients (0.9%), cerebellar syndrome in 4(1.8%) and facial hypoesthesia in 2(0.9%). One patient (0.5%) developed bilateral blindness, secondary to preoperative optic nerve atrophy. As a result of PC, 10 patients (4.5%) required 11 additional surgical procedures. In statistical analysis, PC were independently related to preoperative cerebellar syndrome (p=0.002) and tumor size (>30 mm vs.<30 mm, p<0.05). The risk of PC diminished significantly with the increased number of performed procedures from 40% at the beginning to 16.4% in the last 55 cases.
CONCLUSIONS: Tumor size, cerebellar syndrome at presentation and experience of the team were the three most important risk factors for PC occurrence. Permanent deficit secondary to PC remained in only 4% of the patients.
Surgery for sporadic vestibular schwannoma. Part I: General outcome and risk of tumor recurrence.
Neurol Neurochir Pol. 2016; 50(2):83-9 [PubMed] Related Publications
MATERIALS AND METHODS: The study included 220 consecutive patients (134 women, 86 men; the age ranged from 18 to 74) operated with the retrosigmoid transmeatal approach. The largest extrameatal diameter of the tumor ranged from 8 to 72mm (mean 30mm). According to the Samii grading scale, the tumors were classified as follows: T2-12 (6%), T3-51 (23%) and T4-157 (71%). Gross total resection was performed in 217 patients and neartotal in 3.
RESULTS: Two hundred and eighteen (99.1%) patients were discharged home in a satisfactory neurological condition (GR or MD in GOS). One (0.5%) patient died due to brainstem infarction. One (0.5%) patient had unchanged severe cerebellar syndrome in comparison to the preoperative period (SD in GOS). In long-term follow-up, one patient went blind within a few months after surgery. Including the results of further neurosurgical procedures for CSF leak, shunt implantation, tumor regrowth and facial nerve reanimation, 98.6% of the patients were fully independent but with different neurological deficits. Tumor recurrence was observed in 5 (2.3%) patients during the follow-up period (mean term: 6.4 years). The average time to recurrence diagnosis was 8.8 years. All those patients were operated on again without any adjuvant therapy and there was no further re-growth at mean follow-up of 5.2 years.
CONCLUSIONS: Complete removal of VS is usually curative and poses very low risks of severe disability (if audio-facial sequels are not included), mortality and long-term recurrence. For recurrent tumors, carefully tailored revision surgery without irradiation offers a high efficacy with low risk of complications.
Pediatric Auditory Brainstem Implant Surgery: A New Option for Auditory Habilitation in Congenital Deafness?
J Am Board Fam Med. 2016 Mar-Apr; 29(2):286-8 [PubMed] Related Publications
METHODS: PubMed was searched to identify articles relevant to the ABI, as well as articles that contain outcomes data for pediatric patients (age <18 years) who have undergone ABI surgery.
RESULTS: The ABI was originally developed for patients with neurofibromatosis type 2 (NF2) who become deaf from bilateral vestibular schwannomas. Over the past decade, indications for an ABI have expanded to adult patients without tumors (without NF2) who cannot receive a CI and children with no cochlea or cochlear nerve. Outcomes among NF2 ABI users are modest compared to cochlear implant patients, but recent studies from Europe suggest that some non-tumor adult and pediatric ABI users achieve speech perception.
CONCLUSION: The ABI is a reasonable surgical option for children with profound hearing loss due to severe cochlear or cochlear nerve deformities. Continued prospective data collection from several clinical trials in the U.S. will provide greater understanding on long term outcomes that focus on speech intelligibility.