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Acoustic Neuroma

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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Latest Research Publications

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Teh SR, Ranguis S, Fagan P
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
BACKGROUND: Studies demonstrate the significance of intra- and inter-observer variability when measuring cerebellopontine angle tumours on magnetic resonance imaging, with measured differences as high as 2 mm.
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.

Huang X, Xu J, Xu M, et al.
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
BACKGROUND: Intracranial vestibular schwannoma still remain to be difficulty for its unique microsurgical technique and preservation of neuro-function, as well as reducing common complications that may arise in surgery.
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.

Čada Z, Balatková Z, Chovanec M, et al.
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
Surgical removal of vestibular schwannoma causes acute vestibular symptoms, including postoperative vertigo and oscillopsia due to nystagmus. In general, the dominant symptom postoperatively is vertigo. Preoperative chemical vestibular ablation can reduce vestibular symptoms postoperatively. We used 1.0 mL of 40 mg/mL nonbuffered gentamicin in three intratympanic installations over 2 days, 2 months preoperatively in 10 patients. Reduction of vestibular function was measured by the head impulse test and the caloric test. Reduction of vestibular function was found in all gentamicin patient groups. After gentamicin vestibular ablation, patients underwent home vestibular exercising for two months. The control group consisted of 10 patients who underwent only home vestibular training two months preoperatively. Postoperative rates of recovery and vertigo in both groups were evaluated with the Glasgow Benefit Inventory (GBI), the Glasgow Health Status Inventory (GHSI), and the Dizziness Handicap Inventory questionnaires, as well as survey of visual symptoms by specific questionnaire developed by us. There were no statistically significant differences between both groups with regard to the results of questionnaires. Patients who received preoperative gentamicin were more resilient to optokinetic and optic flow stimulation (p < 0.05). This trial is registered with clinical study registration number NCT02963896.

Li SL, Ma XH, Ji JF, et al.
miR-1 association with cell proliferation inhibition and apoptosis in vestibular schwannoma by targeting VEGFA.
Genet Mol Res. 2016; 15(4) [PubMed] Related Publications
A growing body of research has demonstrated the tumor suppressive function of microRNA (miR)-1 in many cancers. Our study aimed to investigate its role in vestibular schwannoma (VS). We examined miR-1 expression in 95 VS specimens and 79 normal vestibular nerves using quantitative real-time polymerase chain reaction. Moreover, miR-1 mimics, miR-1 inhibitors, and negative control oligonucleotides were transfected into HEI-193 human VS cells to investigate the functional significance of miR-1 expression in this condition at a cellular level. Finally, the role of vascular endothelial growth factor A (VEGFA) in miR-1-mediated HEI-193 cell growth was confirmed. miR-1 levels were significantly reduced in VS specimens compared with normal vestibular nerve tissues (P < 0.001). In addition, low levels of miR-1 were associated with larger tumor volumes. In functional assays, miR-1 suppressed HEI-193 cell proliferation and colony formation, and enhanced apoptosis. VEGFA was verified as a target gene of miR-1, and VEGFA overexpression partially negated the effects of miR-1 on HEI-193 cells. These findings suggest that miR-1 suppresses VS growth by targeting VEGFA, and should be considered as a potential therapeutic target for treatment of this condition.

Jumeau R, Bondiau PY, Parietti-Winkler C, et al.
Vestibular schwannomas treated with Cyberknife®: clinical outcomes.
Tumori. 2016; 102(6):569-573 [PubMed] Related Publications
PURPOSE: Fractionated stereotactic radiotherapy (FSR) is a recognized treatment for vestibular schwannomas (VS). This study's aim is to present clinical outcomes and local control (LC) results for patients with VS treated with FSR using the Cyberknife® (CK) in 2 French cancer centers.
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.

von Kirschbaum C, Gürkov R
Audiovestibular Function Deficits in Vestibular Schwannoma.
Biomed Res Int. 2016; 2016:4980562 [PubMed] Free Access to Full Article Related Publications
Introduction. Vestibular schwannomas (VS) are benign tumours of the vestibular nerve and can lead to hearing loss, tinnitus, vertigo, facial palsy, and brainstem compression. Audiovestibular diagnostic tests are essential for detection and treatment planning. Methods. Medline was used to perform a systematic literature review with regard to how audiovestibular test parameters correlate with symptoms, tumour size, and tumour location. Results. The auditory brainstem response can be used to diagnose retrocochlear lesions caused by VS. Since hearing loss correlates poorly with tumour size, a retrocochlear lesion is probably not the only cause for hearing loss. Also cochlear mechanisms seem to play a role. This can be revealed by abnormal otoacoustic emissions, despite normal ABR and new MRI techniques which have demonstrated endolymphatic hydrops of the inner ear. Caloric and head impulse tests show frequency specific dynamics and vestibular evoked myogenic potentials may help to identify the location of the tumour regarding the involved nerve parts. Conclusion. In order to preserve audiovestibular function in VS, it is important to stop the growth of the tumour and to avoid degenerative changes in the inner ear. A detailed neurotological workup helps to diagnose VS of all sizes and can also provide useful prognostic information.

Kudose S, Kyriakos M, Awad MM
Gastric plexiform schwannoma in association with neurofibromatosis type 2.
Clin J Gastroenterol. 2016; 9(6):352-357 [PubMed] Related Publications
Plexiform schwannoma (PS) is an uncommon variant of schwannoma characterized by a multinodular (plexiform) growth pattern. It comprises up to 5 % of all schwannomas. The association between PS and neurofibromatosis type 1 or type 2 (NF1/NF2) is only rarely reported. Most cases of PS occur in the skin and subcutaneous soft tissue, with only a few reports of digestive tract involvement. We describe an 18-year-old male with NF2 who had bilateral vestibular schwannomas and multiple cutaneous PSs, and a 3-year history of abdominal pain. The patient ultimately underwent a distal gastrectomy for a partially obstructing submucosal antral mass, associated with an overlying ulcer. Histopathologic examination showed the mass to be a PS. The patient is alive and well, without symptoms, 12 months postoperatively. A review of the English language medical literature yielded only ten examples of PS arising in the digestive tract. Our patient is the first to be reported to have a gastric PS, and only the second patient to be reported with a digestive tract PS to have NF2, and the only patient reported to have both digestive tract and cutaneous PSs. Despite its rare occurrence with NF2, the finding of PS at any site should stimulate an examination for other manifestations of this disorder.

Faucett EA, Larsen BT, Khan R, et al.
A Diagnostic Dilemma: Multiple Primary Intracranial Tumors Without Vestibular Schwannomas.
Ann Otol Rhinol Laryngol. 2016; 125(11):938-942 [PubMed] Related Publications
Sinonasal schwannomas with intracranial extension are exceedingly rare, with only 7 cases reported in the literature. Schwannomas can be isolated or multiple and are commonly associated with familial disorders such as neurofibromatosis 2 (NF 2) or familial schwannomatosis or in sporadic cases seen in sporadic schwannomatosis. Nearly all people with NF2 older than 30 years of age will have the hallmark of bilateral vestibular schwannomas (VS). This case highlights a reported case of an adult with separate primary intracranial tumors. We review the diagnostic criteria of NF2 and schwannomatosis, a recently described third variant of neurofibromatosis. In this case, we incorporate family history, histopathology, and the pathophysiology of both disorders to help determine a diagnosis for this patient.

Bouček J, Vokřál J, Černý L, et al.
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
Skull base tumors and, in particular, vestibular schwannoma (VS) are among the etiological reasons for single-sided deafness (SSD). Patients with SSD have problems in understanding speech in a noisy environment and cannot localize the direction of sounds. For the majority, this is the handicap for which they try to find a solution. Apart from CROS hearing aids, Baha is one of the most frequently used systems for SSD compensation. 38 patients with single-sided deafness after retrosigmoid removal of a vestibular schwannoma underwent testing with a Baha softband from September 2010 to August 2014. Sixteen patients (42 %) finally decided to accept Baha implantation. Subjective experience with the Baha softband was evaluated by patients using the BBSS questionnaire immediately after testing. Objective evaluation of the effect was performed as a measurement of the sentence discrimination score in noise and side horizontal discrimination without a Baha and 6 weeks and 12 months after a sound processor fitting. There was a significant improvement in sentence discrimination in the 6 week (64.0 %) and 1 year (74.6 %) interval of follow-up in comparison with understanding without Baha (24.0 %, p = 0.001) in situations when sentences are coming from the side of the non-hearing ear and noise contralaterally with SNR -5 dB. Baha can significantly improve sentence discrimination in complex-listening situation in patients with SSD after the VS surgery.

Shuto T, Matsunaga S
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
Cyst formation is the most common long-term complication after gamma knife surgery (GKS) for cerebral arteriovenous malformations; however, this rarely occurs after GKS for vestibular schwannoma (VS). We describe two patients that developed aggressive, symptomatic large cysts at more than 4 and 12years after GKS for VS, although the tumor control for these patients had been acceptable at 3 and 11years, respectively. During the surgery, a reddish lesion, which was distinct from the surrounding tumor, was observed. The microscopic examination of the reddish lesion revealed dilated capillary vessels and hemorrhage, which was compatible with late radiation changes; these were usually seen in arteriovenous malformations following radiosurgery. The present patients indicated that cyst formation and enlargement may occur as a late complication following GKS even in extra-axial benign tumors such as VS.

Morris KA, Parry A, Pretorius PM
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
OBJECTIVE: To compare the sensitivity of linear and volumetric measurements on MRI in detecting schwannoma progression in patients with neurofibromatosis type 2 on bevacizumab treatment as well as the extent to which this depends on the size of the tumour.
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.

Paldor I, Chen AS, Kaye AH
Growth rate of vestibular schwannoma.
J Clin Neurosci. 2016; 32:1-8 [PubMed] Related Publications
Vestibular schwannoma (VS) is the most common tumor in the extra-axial posterior fossa compartment in adults. Growth rate is paramount to decision making regarding treatment and follow up of these tumors. We conducted a comprehensive review of the literature to answer four questions: What percentage of newly diagnosed VS will grow on follow-up? What factors correlate to tumor growth? What is the "normal" growth rate for sporadic VS? What factors characterize VS with rapid growth? Thirty-seven reports, with more than 4000 patients, fit our review criteria. One third of newly diagnosed VS will grow on follow-up of 1-3years. However, after 5years, up to one half will grow. Patient age and sex do not influence growth of VS. Hearing loss and vertigo at presentation do not predict tumor growth. It is unclear whether balance disturbance or tinnitus predict tumor growth. Tumor size and location do not predict tumor growth. Growth in the first year of observation is a strong predictor of tumor growth. The average growth rate of a VS is 0.99-1.11mm/year. However, the expected growth rate for VS that have been shown to grow at first follow-up is 3mm/year. Factors that may predict tumor growth of above 4mm/year are cystic and hemorrhagic features in the tumor, and hormonal treatment. VS grow at an average 1mm/year. VS that have been shown to grow at first follow-up should be considered for treatment, unless contraindicated. Long term follow-up is recommended for VS.

Deniz Bulut M, Yavuz A, Bora A, et al.
Imaging Findings for Bilateral Giant Vestibulocochlear Schwannoma.
Arch Iran Med. 2016; 19(7):518-20 [PubMed] Related Publications
Neurofibromatosis type 2 mostly develops with multiple neoplasms of the central and peripheral nervous system and is associated with ocular abnormalities. The presented case is a 19-year-old female patient with bilateral vestibulocochlear schwannomas in both pontocerebellar corners, intradural intra-extramedullary masses, and multiple neurofibromas in the spinal canal. The clinical picture for NF-2, also called central neurofibromatosis, is completely different from von Recklinghausen disease. Untreated bilateral vestibulocochlear schwannoma may cause hydrocephalus in NF-2, and lead to death. Therefore, it is recommended to carefully monitor and treat bilateral vestibulocochlear schwannoma in accordance with its stage.

Nash R, Majithia A, Singh A
The Agreement between Protocols for the Investigation of Asymmetrical Audiovestibular Symptoms.
J Int Adv Otol. 2016; 12(1):98-100 [PubMed] Related Publications
OBJECTIVE: There are a number of published criteria for the investigation of asymmetrical audiovestibular symptoms. Our aim was to determine the agreement between these protocols when determining whether to investigate a group of patients treated at our institution.
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.

Odagiri K, Hamada M, Iida M
Cholesterol granuloma presenting with facial nerve paralysis following translabyrinthine surgery: A case report.
Auris Nasus Larynx. 2017; 44(2):241-244 [PubMed] Related Publications
BACKGROUND: Cholesterol granuloma (CG) is a foreign body giant cell inflammatory reaction to blood degradation products, and clinically presents as a cyst with a thick fibrous lining filled with brownish-yellow fluid. We report an unusual case of CG with facial nerve paralysis 9 years after translabyrinthine (TL) surgery for vestibular schwannoma (VS) removal.
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.

Friedrich RE, Hagel C
Expansive Extracranial Growth of Intracranial Meningioma in Neurofibromatosis Type 2.
Anticancer Res. 2016; 36(6):3161-7 [PubMed] Related Publications
The purpose of this report is to detail three rare cases of neurofibromatosis type 2 (NF2) with symptomatic extracranial extension of intracranial meningioma. We present ocular findings, imaging techniques applied, pathological findings of the space-occupying lesions, and therapy. One of these patients, the daughter of one of the other individuals, presented with a large neck mass, but no surgically treatable findings associated with the external growth of the meningioma. The patients complained of symptoms associated with the extracranial portion of the intracranial meningioma, rather than of the intracranial primaries. However, facial and neck surgical care is very limited in patients with such advanced-stage tumours. The prolongation of life was unquestionably predominantly determined by the behaviour of the intracranial tumour. Head and neck surgeons should be aware of the rare possibility that solid tumours of this region could be extracranial-extending meningioma in an inherited disease.

Tranter-Entwistle I, Dawes P, Darlington CL, et al.
Video head impulse in comparison to caloric testing in unilateral vestibular schwannoma.
Acta Otolaryngol. 2016; 136(11):1110-1114 [PubMed] Related Publications
CONCLUSIONS: Although there was a statistically significant relationship between the results of the vHIT and the caloric test, the limited strength of this relationship suggests that, for unilateral vestibular schwannoma (UVS), caloric testing and vHIT may provide complementary information on vestibular function.
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.

Peng KA, Wilkinson EP
Optimal outcomes for hearing preservation in the management of small vestibular schwannomas.
J Laryngol Otol. 2016; 130(7):606-10 [PubMed] Related Publications
OBJECTIVE: To undertake a systematic review of the role of microsurgery, in relation to observation and stereotactic radiation, in the management of small vestibular schwannomas with serviceable hearing.
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.

Lee JM, Chang JW, Choi JY, et al.
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
Patients with neurofibromatosis type II will eventually succumb to bilateral deafness. For patients with hearing loss, modern medical science technology can provide efficient hearing restoration through a number of various methods. In this article, several hearing restoration methods for patients with neurofibromatosis type II are introduced.

Lekovic GP, Schwartz MS, Go JL
Multifocal granulocytic sarcoma of the spine mimicking neurofibromatosis Type 2: case report.
J Neurosurg Spine. 2016; 25(4):523-527 [PubMed] Related Publications
In this report the authors report on a patient with a very indolent course of granulocytic sarcoma, characterized by steroid-induced remission of spinal and cranial tumors and recurrence over a period of several years. This 24-year-old man with history of leukemia presented with rapid-onset quadriparesis secondary to multiple extraaxial masses of the cervicothoracic spine, from C-5 to T-3, and lumbosacral spine, from L-5 to the coccyx. Although the imaging features were highly suggestive of neurofibromatosis Type 2, the patient's history and clinical course were consistent with granulocytic sarcoma; repeat imaging and, later, needle biopsy definitively established the diagnosis of granulocytic sarcoma. Laminectomy and surgical decompression of the spine were not required and, arguably, could have posed unnecessary risk to the patient. This case illustrates that the successful management of a patient presenting with profound neurological deficits due to intradural spinal cord tumors may sometimes be nonsurgical.

Ouerdani A, Goutagny S, Kalamarides M, et al.
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
PURPOSE: To describe the natural growth of vestibular schwannoma in patients with neurofibromatosis type 2 and to predict tumor volume evolution in patients treated with bevacizumab and everolimus.
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.

Jeong KH, Choi JW, Shin JE, Kim CH
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
The etiology of sudden sensorineural hearing loss (SSNHL) remains unclear in most cases. This study aimed to assess abnormal magnetic resonance imaging (MRI) findings in patients with SSNHL and evaluate the value of MRI in identifying the cause of SSNHL.A retrospective analysis of the charts and MRI findings of 291 patients with SSNHL was performed.In 291 patients, MRI abnormality, which was considered a cause of SSNHL, was detected in 13 patients. Vestibular schwannoma involving the internal auditory canal (IAC) and/or cerebellopontine angle was observed in 9 patients. All 9 patients had intrameatal tumors, and 6 of the 9 patients displayed extrameatal extension of their tumors. The tumor was small (<1 cm) or medium-sized (1.1-2.9 cm) in these 6 patients. Intralabyrinthine schwannoma, labyrinthine hemorrhage, IAC metastasis, and a ruptured dermoid cyst were each observed in 1 patient.The most commonly observed MRI abnormality in patients with SSNHL was vestibular schwannoma, and all of the lesions were small or medium-sized tumors involving the IAC.

Shaw A
Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma.
Recent Results Cancer Res. 2016; 205:17-27 [PubMed] Related Publications
Bilateral vestibular schwannomata and meningiomata are the tumours most commonly associated with neurofibromatosis type II (NF2). These tumours may also be seen in patients with schwannomatosis and familial meningioma, but these phenotypes are usually easy to distinguish. The main diagnostic challenge when managing these tumours is distinguishing between sporadic disease which carries low risk of subsequent tumours or NF2 with its associated morbidities and reduced life expectancy. This chapter outlines some of the diagnostic and management considerations along with associated evidence.

Rafique I, Wennervaldt K, Melchiors J, Caye-Thomasen P
Auditory brainstem response - a valid and cost-effective screening tool for vestibular schwannoma?
Acta Otolaryngol. 2016; 136(7):660-2 [PubMed] Related Publications
CONCLUSION: Contemporary auditory brainstem response (ABR) is not valid as a screening tool for VS, when considering the sensitivity of 80%, the specificity of 77%, and the positive predictive value of 3.4%, MRI screening is superior to ABR in Denmark when considering cost-effectiveness.
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.

Rashid A, Karam SD, Rashid B, et al.
Multisession Radiosurgery for Hearing Preservation.
Semin Radiat Oncol. 2016; 26(2):105-11 [PubMed] Related Publications
Clinically relevant dose-tolerance limits with reliable estimates of risk in 1-5 fractions for cochlea are still unknown. Timmerman׳s limits from the October 2008 issue of Seminars in Radiation Oncology have served as the basis for clinical practice, augmented by updated constraints in TG-101 and QUANTEC, but the corresponding estimates of risk have not yet been well-reported. A total of 37 acoustic neuroma CyberKnife cases from Medstar Georgetown University Hospital treated in 3 or 5 fractions were combined with single-fraction Gamma Knife data from the 69 cases in Timmer 2009 to form an aggregate dataset of 106 cochlea cases treated in 1-5 fractions. Probit dose-response modeling was performed in the DVH Evaluator software to estimate normal tissue complication probability. QUANTEC recommends keeping single-fraction maximum dose to the cochlea less than 14Gy to maintain less than 25% risk of serviceable hearing loss, and our 17.9% risk estimate for 14Gy in 1 fraction is within their predicted range. In 5 fractions, our estimate of the Timmerman 27.5Gy maximum cochlea dose limit was 17.4%. For cases in which lower risk is required, the Timmerman 12Gy in 1 fraction and the TG-101 limit of 25Gy in 5 fractions had an estimated risk level of 11.8% and 13.8%, respectively. High-risk and low-risk dose tolerance with risk estimates in 1-5 fractions are all presented.

Chen M, Fan Z, Zheng X, et al.
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
PURPOSE: Many epidemiological studies have investigated environmental risk factors for the development of acoustic neuroma. However, these results are controversial. We conducted a meta-analysis of case-control studies to identify any potential relationship between history of noise exposure, smoking, allergic diseases, and risk of acoustic neuroma.
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.

He YB, Yu CJ, Ji HM, et al.
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
BACKGROUND: Determining the nerve of origin for vestibular schwannoma (VS), as a method for predicting hearing prognosis, has not been systematically considered. The vestibular test can be used to investigate the function of the superior vestibular nerve (SVN) and the inferior vestibular nerve (IVN). This study aimed to preoperatively distinguish the nerve of origin for VS patients using the vestibular test, and determine if this correlated with hearing preservation.
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.

Kunert P, Dziedzic T, Czernicki T, et al.
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
INTRODUCTION: The aim of this study was to analyze the frequency and consequences of postoperative complications (PC) after surgery for sporadic vestibular schwannoma and to find factors that increase the risk of PC occurrence.
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.

Kunert P, Dziedzic T, Nowak A, et al.
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
BACKGROUND: Vestibular schwannomas are slow growing, benign tumors. There are three possible management options: surgery, radiation treatment or active surveillance. The aim of this study was to assess the general outcome and risk of tumor recurrence.
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.

Shah PV, Kozin ED, Kaplan AB, Lee DJ
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
INTRODUCTION: The auditory brainstem implant (ABI) is a neuroprosthetic device that provides sound sensations to individuals with profound hearing loss who are not candidates for a cochlear implant (CI) because of anatomic constraints. Herein we describe the ABI for family physicians.
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.

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