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Stereotactic radiosurgery has been shown to be an effective method of managing vestibular schwannomas. The primary aim here is to establish the impact of pre-treatment fast-growing vestibular schwannomas on the efficacy of stereotactic radiosurgery.
Methods
PubMed, Medline and Embase databases were used. The ROBINS-I (‘Risk Of Bias In Non-randomised Studies - of Interventions’) tool was utilised to assess for risk of bias. Proportionate meta-analysis and sub-analysis for fast-growing tumours were performed to explore the success rate of stereotactic radiosurgery in stabilising or decreasing the tumour burden in vestibular schwannomas.
Results
Four moderate risk studies were included in the analysis. Overall, 91 per cent (95 per cent confidence interval = 0.83–0.97, p < 0.01, I2 = 80 per cent) of the tumours demonstrated successful size reduction or stabilisation following stereotactic radiosurgery. Nevertheless, the efficacy of stereotactic radiosurgery in reducing or stabilising fast-growing vestibular schwannomas decreased by 79 per cent (95 per cent confidence interval = 0.64–0.91, p = 0.11, I2 = 62 per cent).
Conclusion
Stereotactic radiosurgery has a statistically significant success rate in stabilising or decreasing the vestibular schwannoma size. This success rate is diminished in fast-growing vestibular schwannomas.
Imbalance and gait disturbances are common in patients with vestibular schwannoma (VS) and can result in significant morbidity. Current methods for quantitative gait analysis are cumbersome and difficult to implement. Here, we use custom-engineered instrumented insoles to evaluate the gait of patients diagnosed with VS.
Methods
Twenty patients with VS were recruited from otology, neurosurgery, and radiation oncology clinics at a tertiary referral center. Functional gait assessment (FGA), 2-minute walk test (2MWT), and uneven surface walk test (USWT) were performed. Custom-engineered instrumented insoles, equipped with an 8-cell force sensitive resistor (FSR) and a 9-degree-of-freedom inertial measurement unit (IMU), were used to collect stride-by-stride spatiotemporal gait parameters, from which mean values and coefficients of variation (CV) were determined for each patient.
Results
FGA scores were significantly correlated with gait metrics obtained from the 2MWT and USWT, including stride length, stride velocity, normalized stride length, normalized stride velocity, stride length CV, and stride velocity CV. Tumor diameter was negatively associated with stride time and swing time on the 2MWT; no such association existed between tumor diameter and FGA or DHI.
Conclusions
Instrumented insoles may unveil associations between VS tumor size and gait dysfunction that cannot be captured by standardized clinical assessments and self-reported questionnaires.
This study was aimed to evaluate the outcomes of patients with large (>2 cm in great diameter) vestibular schwannomas (VSs) treated with hypofractionated stereotactic radiotherapy (HFSRT) compared to small (<2 cm) ones and the impact of debulking surgery prior to radiation for large VSs.
Methods:
Fifty-nine patients with VSs treated with HFSRT (25 Gy in 5 fractions) were evaluated by tumour size and surgical status. Patients were divided based on tumour size: small VSs (n = 42) and large VSs (n = 17). The large group was further divided into the groups of pre-treatment debulking surgery (n = 8) and no surgery (n = 9). Rates of tumour control, brainstem necrosis and neurologic dysfunction were assessed following treatment. Pre-surgical magnetic resonance imaging (MRI) were used to generate hypothetical HFSRT plans to compare the effect of debulking surgery on dosimetry. Normal tissue complication probability (NTCP) modelling was performed to compare toxicity probabilities with and without surgical debulking in large VSs.
Results:
There was no statistical difference of tumour control rate between small and large VSs with 100% for small tumours and 94·1% for large tumours (p = 0·12), respectively. In large VSs patient, the tumour control rate of HFSRT was 100% (8/8) for surgically debulked patients and 89% (8/9) for non-surgically debulked patients (p = 0·35). There were no patients who experienced brainstem necrosis or progression of facial and trigeminal nerve symptoms after HFSRT in the entire groups of patients. Surgical debulking large VSs did not change the maximum point dose of brainstem (p = 0·98), but significantly decreased volumes of VSs and changed the minimum dose to the hottest 0·5 cc of tumour (p = 0·016) as well as the volume receiving at least 23 Gy (p = 0·023). NTCP modelling revealed very low rates (average < 1%) of brainstem toxicity with or without surgical debulking, but there was a significant difference favoring surgery (p < 0·05).
Conclusions:
HFSRT is a safe and effective treatment for both small and large VSs and is a viable option for patients with large VSs who cannot undergo surgery, if NTCP of pre-debulking HFSRT dosimetry is lower.
Magnetic resonance imaging of the internal auditory meatus is a highly sensitive and specific way to diagnose vestibular schwannoma. However, the rate of incidental findings with this method is believed to be high and can lead to increased patient anxiety and health interventions with unclear benefit.
Method
A systematic review of the literature was performed using the Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines to identify incidental findings from magnetic resonance imaging of the internal auditory meatus; 12 studies were identified for inclusion within this review.
Results
A total of 10 666 patients were included within the review. The overall rate of diagnosis of vestibular schwannoma was 0.87 per cent; 21 per cent of the study population had incidental findings on magnetic resonance imaging of the internal auditory meatus, and 9.56 per cent had clinically significant incidental findings.
Conclusion
Standardised pre-scan counselling may mitigate the risks of overdiagnosis, but future work should be undertaken to assess the benefits of such a strategy as well as the exact significance of some incidental findings.
To determine the long-term, spontaneous growth arrest rates in a large cohort of vestibular schwannoma patients.
Methods
This paper describes a retrospective case series of 735 vestibular schwannoma patients organised into four groups: group A patients showed tumour growth which then stopped without any treatment; group B patients showed tumour growth which continued, but were managed conservatively; group C patients had a growing vestibular schwannoma and received active treatment; and group D patients had a stable, non-growing vestibular schwannoma. Demographics, tumour size and vestibular schwannoma growth rate (mm/month) were recorded.
Results
A total of 288 patients (39.2 per cent) had growing vestibular schwannomas. Of the patients, 103 (35.8 per cent) were managed conservatively, with 52 patients (50.5 per cent of the conservative management group, 18 per cent of the total growing vestibular schwannoma group) showing growth arrest, which occurred on average at four years following the diagnosis. Eighty-two per cent of vestibular schwannomas stopped growing within five years. Only differences between age (p = 0.016) and vestibular schwannoma size (p = 0.0008) were significant.
Conclusion
Approximately 20 per cent of growing vestibular schwannomas spontaneously stop growing, predominantly within the first five years; this is important for long-term management.
Optimal treatment for vestibular schwannomas has long been a debated topic in skull base surgery. Advancements in surgical technique and adjuncts, as well as radiation therapy, have further confounded what is considered the optimal treatment regimen. Goals of care have focused on maximal tumor resection and avoidance of cranial neuropathies. Treatment options continue to include surveillance imaging with close observation, microsurgical resection, and radiotherapy (either with stereotactic radiosurgery or hypofractionated treatments). This chapter reviews the current management options, with a focus on the development of hybrid strategies for the treatment of these challenging tumors.
Magnetic resonance imaging scans of the internal acoustic meatus are commonly requested in the investigation of audio-vestibular symptoms for potential vestibular schwannoma. There have been multiple studies into protocols for requesting magnetic resonance imaging for vestibular schwannoma, but none have been reported based on UK National Institute for Health and Care Excellence guidelines for investigating audio-vestibular symptoms. This study intended to identify the local magnetic resonance imaging detection rates and patterns of vestibular schwannoma, and to audit the conformity of scan requests with the National Institute for Health and Care Excellence guidelines, with a review of relevant literature.
Method
A retrospective analysis of 1300 magnetic resonance imaging scans of the internal acoustic meatus, compared against National Institute for Health and Care Excellence guidelines, was conducted over two years.
Results and conclusion
Sixteen scans were positive for vestibular schwannoma, with a detection rate of 1.23 per cent. All positive cases fit the guidelines; three of these could have been missed using other criteria. A total of 281 requests did not meet the guideline criteria but revealed no positive results, supporting the use of National Institute for Health and Care Excellence guidelines in planning magnetic resonance imaging scans for audio-vestibular symptoms.
Vestibular schwannomas can demonstrate great heterogeneity in their behaviour; approximately one-third will grow and two-thirds will not. This study aimed to determine whether there are factors present at diagnosis that can help predict outcomes.
Methods
This retrospective cohort study compared data from 735 patients from the past 20 years. Analysis of serial magnetic resonance imaging was carried out to place patients into growing and non-growing cohorts. Factors including size, age, follow-up time and presence of balance symptoms were compared.
Results
The median size of a growing vestibular schwannoma at diagnosis was 13 mm, whereas the non-growing median size was 10.65 mm (p < 0.001). Balance symptoms were present in 60.76 per cent of growing vestibular schwannoma patients but only in 38.75 per cent of patients with non-growing vestibular schwannomas (p < 0.001).
Conclusion
This study highlights initial tumour size and balance symptoms as potential predictors of whether or not a vestibular schwannoma will grow; these results better facilitate our understanding of vestibular schwannoma natural history.
To compare the measured bone conduction threshold at 3 kHz with the calculated threshold in newly diagnosed sudden sensorineural hearing loss.
Methods
A retrospective chart review was conducted of pure tone audiograms in confirmed sudden sensorineural hearing loss cases.
Results
Of 157 patients with sudden sensorineural hearing loss, 144 had idiopathic hearing loss, 8 had vestibular schwannoma and 5 had Ménière's disease. The r value for the correlation between the two methods of 3 kHz assessment for all patients was 0.887 (p < 0.001). The mean difference between the measured and calculated 3 kHz thresholds was 0.76 ± 7.96 dB, 0.4 ± 8.08 dB and 1.5 ± 1.8 dB in the sudden sensorineural hearing loss, idiopathic and Ménière's disease groups, respectively. The mean difference between the measured and calculated 3 kHz thresholds was significantly greater in the vestibular schwannoma group (6.86 ± 4.38 dB) than in the idiopathic group (p = 0.013).
Conclusion
The 3 kHz frequency may encompass important audiometric information. A discrepancy between the measured and calculated bone conduction 3 kHz thresholds raises suspicion of an underlying vestibular schwannoma as an aetiology for sudden sensorineural hearing loss, and these thresholds should therefore be measured independently and routinely.
Vestibular schwannomas (VS) are benign slow-growing tumours treated either with microsurgery or stereotactic radiosurgery (SRS) or both. The aim of this study was to correlate the outcome factors—tumour control and adverse factors—facial nerve function and hearing loss with patient and treatment factors.
Materials and methods:
A retrospective review of the records of 98 patients with 99 VS treated from June 2007 to June 2014, all patients receiving Linear Accelerator (LINAC)-based SRS.
Results:
Median follow-up period was 5·6 years (range: 1–12 years). The response to treatment was stable disease in 37 (37·4%), regression in 46 (46·5%), asymptomatic minimal progression in 9 (9·1%) and symptomatic progression in 5 (5%) and unknown in 2 (2%) patients. There was no evidence of SRS induced tissue damage on magnetic resonance scans for any. Hearing preservation rate after SRS was 92%. The patients who developed worsening of facial function were predominantly in the cohort that had prior surgery.
Findings:
SRS is an effective modality to treat VS lesser than 3 cm in size. Tumour control rate was 95% with a median follow-up period of 5·6 years. The complication rates were 8% each for facial function worsening and worsening of hearing. Prior surgery was a statistically significant factor that affected facial nerve function deterioration.
To review the literature regarding screening for vestibular schwannoma in the context of demographic changes leading to increasing numbers of elderly patients presenting with asymmetric auditory symptoms.
Methods
A systematic review of the literature was performed, with narrative synthesis and statistical analysis of data where appropriate.
Results
Vestibular schwannomas diagnosed in patients aged over 70 years exhibit slower growth patterns and tend to be of smaller size compared to those tumours in younger age groups. This fact, combined with reduced life expectancy, renders the probability of these tumours in the elderly requiring active treatment with surgery or stereotactic radiotherapy to be extremely low. Vestibular schwannomas in the elderly are much more likely to be managed by serial monitoring with magnetic resonance imaging. The weighted yield of magnetic resonance imaging in the diagnosis of vestibular schwannoma in all age groups is 1.18 per cent, with almost 85 scans required to diagnose 1 tumour.
Conclusion
An evidence-based approach to the investigation of asymmetric hearing loss and tinnitus in the elderly patient can be used to formulate guidelines for the rational use of magnetic resonance imaging in this population.
To determine the impact of pre-operative intratympanic gentamicin injection on the recovery of patients undergoing translabyrinthine resection of vestibular schwannomas.
Methods
This prospective, case–control pilot study included eight patients undergoing surgical labyrinthectomy, divided into two groups: four patients who received pre-operative intratympanic gentamicin and four patients who did not. The post-operative six-canal video head impulse test responses and length of in-patient stay were assessed.
Results
The average length of stay was shorter for patients who received intratympanic gentamicin (6.75 days; range, 6–7 days) than for those who did not (9.5 days; range, 8–11 days) (p = 0.0073). Additionally, the gentamicin group had normal post-operative video head impulse test responses in the contralateral ear, while the non-gentamicin group did not.
Conclusion
Pre-operative intratympanic gentamicin improves the recovery following vestibular schwannoma resection, eliminating, as per the video head impulse test, the impact of labyrinthectomy on the contralateral labyrinth.
To evaluate the effects of CyberKnife stereotactic radiotherapy for the treatment of vestibular schwannoma on hearing, as evaluated by audiological tests.
Methods
Patients with vestibular schwannoma were evaluated before and after CyberKnife radiosurgery. Evaluation included pure tone thresholds, speech discrimination scores, auditory brainstem responses and radiological signs.
Results
The study comprised 26 patients diagnosed with vestibular schwannoma and subsequently treated with CyberKnife radiosurgery. The mean follow-up time was 16.4 months. The mean post-treatment hearing preservation rate was 69.23 per cent. There was no significant relationship between hearing loss after treatment and patient age, radiation dosage during treatment, or size of tumour. With regard to auditory brainstem responses, patients with hearing loss following treatment had a significantly higher inter-peak latency between waves I–III than patients with preserved hearing.
Conclusion
Stereotactic CyberKnife radiosurgery is an excellent alternative treatment modality for patients with vestibular schwannoma, and results in acceptable preservation of hearing. Residual hearing following CyberKnife therapy is not significantly affected by factors such as age, size of tumour or dosage of treatment.
To assess the feasibility of non-contrast T2-weighted magnetic resonance imaging as compared to T1-weighted post-contrast magnetic resonance imaging for detecting acoustic neuroma growth.
Methods
Adult patients with acoustic neuroma who underwent at least three magnetic resonance imaging scans of the internal auditory canals with and without contrast in the past nine years were identified. T1- and T2-weighted images were reviewed by three neuroradiologists, and tumour size was measured. Accuracy of the measurements on T2-weighted images was defined as a difference of less than or equal to 2 mm from the measurement on T1-weighted images.
Results
A total of 107 magnetic resonance imaging scans of 26 patients were reviewed. Measurements on T2-weighted magnetic resonance imaging scans were 88 per cent accurate. Measurements on T2-weighted images differed from measurements on T1-weighted images by an average of 1.27 mm, or 10.4 per cent of the total size. The specificity of T2-weighted images was 88.2 per cent and the sensitivity was 77.8 per cent.
Conclusion
The T2-weighted sequences are fairly accurate in measuring acoustic neuroma size and identifying growth if one keeps in mind the caveats associated with the tumour characteristics or location.
Fractionated stereotactic radiotherapy (FSRT) is an alternative treatment for large vestibular schwannomas (VS), if patients are not fit for or refuse surgery. In this study, we compared long-term clinical and radiological outcome in both small–medium sized and larger tumours.
Material and methods
A retrospective study was performed of patients with sporadic VS who underwent primarily conventional FSRT. In total, 50 consecutive patients were divided into two groups by volume. Clinical and volumetric parameters were analysed.
Results
In all, 41 patients (82%) had large tumours affecting the 4th ventricle (modified Koos stage 4). Definitive expansion of VS occurred in eight out of 50 patients (16%). After 7·2 years (median) the overall freedom from clinical failure was 100% in smaller and 92% in larger schwannomas (arbitrarily sized >7·4 cc). Useful hearing was preserved in only 35% of the patients. The facial nerve remained intact in all cases, while new deficit of the trigeminal nerve occurred in 20% of the cases. Of the larger tumours 20% needed a cerebrospinal fluid (CSF) shunt.
Conclusions
FSRT is a treatment in its own right as it is highly effective in both smaller and larger VS without causing permanent disabling complications. The outcome is beneficial also in larger tumours that affect the 4th ventricle.
To determine the frequency of incidental findings found on magnetic resonance imaging scans of the internal auditory meatus performed to investigate audiovestibular symptoms, and to determine how to best manage these when found.
Method:
A retrospective review was conducted of internal auditory meatus magnetic resonance imaging scans during a three-month period in the radiology department at a UK district general hospital.
Results:
A total of 109 scans were reviewed. Of these, 92.7 per cent showed no retrocochlear pathology, 0.9 per cent showed vestibular schwannoma, 6.4 per cent revealed vascular loops, and 2.8 per cent showed incidental findings that warranted further action and investigation. Of the scans, 40.4 per cent showed other incidental pathologies such as age-related ischaemic changes, and sinus disease that required no further intervention. Of the magnetic resonance imaging scans reviewed, 49.5 per cent were entirely normal.
Conclusion:
Almost half of the scans investigating audiovestibular symptoms showed incidental findings. Otolaryngologists should have an understanding of the significance of the most commonly encountered incidental findings, and should counsel patients appropriately and refer them onward when necessary.
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.
Vestibular schwannomas are a rare cause of asymmetrical hearing loss, and routine screening with magnetic resonance imaging can be costly. This paper reports results on vestibular schwannoma screening at our institution and compares the cost of screening to a utility of hearing benefit.
Method:
All screening examinations with magnetic resonance imaging performed for asymmetrical hearing loss between 2006 and 2011 were retrospectively reviewed. The cost per new vestibular schwannoma diagnosis was calculated. The cost per patient for those who benefitted from intervention was estimated based on rates of hearing preservation reported in the literature.
Results:
Forty-five (4.3 per cent) of 1050 screening examinations with magnetic resonance imaging performed for asymmetrical hearing loss were positive for vestibular schwannoma, and the cost per new diagnosis was $11 436. The estimated screening cost per patient for those who benefitted from surgery or radiation was $147 030, while US federal compensation for unilateral hearing loss was $44 888.
Conclusion:
Although we achieved a lower screening cost per new diagnosis than reported in the current literature, there remains disparity between the screening cost per benefitted patient and the ‘benefit’ of hearing.
To report a case of meningioma arising from the nervus intermedius.
Methods:
This paper comprises a case report, literature review, and discussion regarding the presentation of a nervus intermedius meningioma, comparing and contrasting this to other relevant neoplasms of the internal auditory canal and cerebellopontine angle.
Results:
Tumours of the cerebellopontine angle include vestibular schwannomas, facial schwannomas and, more rarely, nervus intermedius schwannomas. The nervus intermedius is a division of the facial nerve at the cerebellopontine angle, with parasympathetic and afferent somatic components. Our patient presented with progressive hearing loss. An ipsilateral internal auditory canal mass at the fundus, as indicated by magnetic resonance imaging and electroneuronography, was suggestive of vestibular schwannoma. Intra-operative dissection revealed a nervus intermedius tumour. Histological evaluation indicated a meningioma rather than a schwannoma.
Conclusion:
This is the first reported case of meningioma involving the nervus intermedius. The implications this pathology may have on surgical approach, facial nerve outcomes, and the need for improved pre-operative imaging and intra-operative monitoring are discussed. A review of the current literature on nervus intermedius tumour is provided.
To determine factors affecting facial nerve outcome of vestibular schwannoma surgery.
Methods:
This retrospective cohort study comprised 652 patients. The outcome measure was House–Brackmann classification at two years post-operatively. Univariate and multivariate analyses were carried out to determine the factors affecting facial nerve outcome. The incidence rates of hemifacial spasm, metallic taste and crocodile tear syndrome were recorded.
Results:
For tumours less than 1.5 cm, 95 per cent of outcomes were normal, 100 per cent were satisfactory (House–Brackmann grades I–III) and 0 per cent were unsatisfactory (grades IV–VI). For tumours 1.5–2.4 cm, 83 per cent of outcomes were normal, 99 per cent were satisfactory and 1 per cent were unsatisfactory. For tumours 2.5–3.4 cm, 68 per cent of outcomes were normal, 96 per cent were satisfactory and 4 per cent were unsatisfactory. For tumours 3.5–4.4 cm, 52 per cent of outcomes were normal, 80 per cent were satisfactory and 20 per cent were unsatisfactory. For tumours larger than 4.4 cm, 50 per cent of outcomes were normal, 72 per cent were satisfactory and 28 per cent were unsatisfactory.
Conclusion:
Tumour size and operation year were significant predictors of facial nerve outcome. The surgical learning curve was steepest for the first 50 patients.