In decision making regarding the management of vestibular schwannomas, alongside clinical outcomes, an understanding of patient reported health-related quality of life measures is key. Therefore, the aim of this research is to compare health-related quality of life in vestibular schwannoma patients treated with active observation, stereotactic radiotherapy and microsurgical excision.

A cross-sectional study of patients diagnosed with unilateral sporadic vestibular schwannomas between 1995 and 2015 at a specialist tertiary centre was conducted. Patients completed the Penn Acoustic Neuroma Quality of Life questionnaire and handicap inventories for dizziness, hearing and tinnitus.

Of 234 patients, 136 responded (58.1 per cent). Management modality was: 86 observation, 23 stereotactic radiotherapy and 25 microsurgery. Females reported significantly worse dizziness; males reported significantly worse physical disability. Patients less than 65 years old reported significantly worse tinnitus and pain scores. Overall, quality of life was higher in the observation group.

Conservative management, where appropriate, is favourable with higher quality-of-life outcomes in this cohort. This must be weighed against the risks of a growing tumour.

This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma.

Audiovestibular handicap was assessed using the Hearing Handicap Inventory, Tinnitus Handicap Inventory and Dizziness Handicap Inventory. Patients completed questionnaires at presentation and at least one year following treatment with microsurgery, stereotactic radiosurgery or observation. Changes in audiovestibular handicap and factors affecting audiovestibular handicap were assessed.

All handicap scores increased at follow up, but not significantly. The Tinnitus Handicap Inventory and Dizziness Handicap Inventory scores predicted tinnitus and dizziness respectively. The Hearing Handicap Inventory was not predictive of hearing loss. Age predicted Tinnitus Handicap Inventory score and microsurgery was associated with a deterioration in Dizziness Handicap Inventory score.

Audiovestibular handicap is common in patients with vestibular schwannoma, with 75 per cent having some degree of handicap in at least one inventory. The overall burden of handicap was, however, low. The increased audiovestibular handicap over time was not statistically significant, irrespective of treatment modality.

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.

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.

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).

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.

To assess whether pre-habilitation with intratympanic gentamicin can accelerate vestibular compensation following vestibular schwannoma resection.

Seventeen studies were retrieved from the databases Medline, PubMed, Frontiers, Cochrane Library, Cambridge Core and ScienceDirect. Eight of the 17 studies met our criteria; the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were used. Heterogeneity, risk of bias and effect on post-operative recovery were assessed.

Four of the eight studies showed a statistically positive effect of pre-habilitation with gentamicin on the post-operative recovery process; the remainder also reported benefits, although not statistically significant. No study reported negative effects. Limitations were linked mostly to the limited number of enrolled patients and the outcome assessment methods.

Fifty per cent of the studies found a statistically positive effect of pre-habilitation with gentamicin prior to vestibular schwannoma resection. While the results are promising, due to the limited numbers further prospective studies are required to strengthen the evidence.

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.

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.

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).

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.

There are currently no guidelines for simultaneous vestibular schwannoma surgery and cochlear implantation. This paper therefore provides our experience and our results regarding predictive parameters of good hearing.

Morphological appearance of the cochlear nerve after tumour resection was used as the main criterion for implantation in the case series. Patients were then divided into responders and non-responders to cochlear implantation, and potential outcome predicting factors were evaluated in the two groups.

Nine of the 16 patients showed a response to cochlear implantation. Pre-surgery serviceable hearing was significantly more common in the responder group, while no difference was found in the two groups for other variables.

This study highlights how the morphological appearance of the cochlear nerve can be useful to predict the hearing outcome and indicates that satisfactory hearing results are closely related to pre-surgery serviceable hearing.

Abnormal gains in six-canal video head impulse test are attributed to semi-circular canal deficits. However, as video head impulse test responses are linked to the vestibulo-ocular reflex, it was hypothesised that abnormal gains can be caused by vestibulo-ocular reflex pathway deficits.

This study compared video head impulse test gains in 20 patients with superior semi-circular canal dehiscence (labyrinthine cause) and 20 side- and gender-matched patients with vestibular schwannomas (retrolabyrinthine cause), and investigated correlations between them (Mann–Kendall trend test).

Vestibular schwannoma but not superior semi-circular canal dehiscence was significantly associated with abnormal lateral (odds ratio = 9.00 (95 per cent confidence interval = 1.638–49.44), p = 0.011) and posterior (odds ratio = 9.00 (95 per cent confidence interval = 2.151–37.659), p = 0.003) canal status. In vestibular schwannoma patients, there was a statistically significant degree of dependence between all ipsilesional canal video head impulse test gains; such dependence was not observed in superior semi-circular canal dehiscence.

Vestibulo-ocular reflex gains differ in patients with labyrinthine and retrolabyrinthine disease; this suggests that abnormal gains can indicate deficits not only in the semi-circular canals but also elsewhere along the vestibulo-ocular reflex pathway.

To determine the long-term, spontaneous growth arrest rates in a large cohort of vestibular schwannoma patients.

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.

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.

Approximately 20 per cent of growing vestibular schwannomas spontaneously stop growing, predominantly within the first five years; this is important for long-term management.

Imaging detects acoustic neuroma, a rare pathology associated with asymmetric sensorineural hearing loss and tinnitus, that is mostly managed conservatively. Scanning indication is debatable, without evaluation in primary care, despite the high burden of audiovestibular symptoms and commissioning of general practitioner imaging.

Cohort evaluation of two years' internal auditory meatus magnetic resonance imaging in primary care.

Of 200 scans requested by 77 general practitioners, only 33 per cent conformed to guideline indications. Most were referred to specialists, regardless of result. Only 10.5 per cent were appropriately imaged to rule out neuroma without specialist referral. One neuroma was detected (diagnostic yield 0.5 per cent) in a patient already referred. Incidental findings were shown in 44.5 per cent, triggering low-value cascades in 18 per cent. Whilst fewer than 1 in a 1000 imaged patients may improve through surgery, 1 in 5 can suffer negative imaging cascades.

Considering the bi-directional relationship between distress and audio-vestibular symptoms, anxiety-provoking imaging overuse should be minimised. In low-prevalence primary care, retrocochlear imaging could be limited to those with asymmetric sensorineural hearing loss. Alternatively, assessment and imaging could be shifted to audiologist-led settings, with a wider therapeutic offer, likely more beneficial and cost-effective than conventional surgical pathways.

Neurofibromatosis type 2 (NF2) is a rare disorder associated with significant morbidity such as hearing loss that can lead to many psychiatric disorders.

Describe the psychiatric symptoms associated to NF2.

We report the case of a patient admitted to the locked unit of the psychiatric ward for agitation and persecutory delusion and diagnosed with NF2. The data was collected from the patient’s medical file. A review of the literature was performed by selecting articles from PubMed using ‘Psychosis acoustic neuromas’ and ‘Psychosis neurofibromatosis 2’ as key words.

This is the case of a 21-year-old patient who was admitted for behavioral disorders. Our patient had a medical history of a one-sided deafness treated with a hearing prosthesis. He was also followed irregularly by a free-lance psychiatrist. The start of trouble dated back to 3 years marked by behavioral disorders such as fugue, agitation, irritability and sleep disorder. The symptoms worsen in the last 3 months with appearance of hostility and delusion of persecution towards his mother. The patient declines to eat the food that his mother cooked for him and threatened her with a knife. The clinical overview includes delirium, clastic agitation strikes, emotional lability, cerebral ataxia and conjunctival hyperemia. Brain scanner showed an association of bilateral acoustic neuromas, cavernous and intraventricular meningioma. These clinical and radiological signs met the diagnosis for NF2 according to the consensus conference of the National Institute of Health in Bethesda (USA 1988).

The psychiatric symptoms reported in acoustic neuroma patients are usually described as transient.

Tinnitus is a common condition presenting to the ENT out-patient clinic. Vestibular schwannomas are benign cerebellopontine angle tumours that usually present with unilateral sensorineural hearing loss. Magnetic resonance imaging of the internal auditory meatus is the definitive investigation in their detection. The current recommendation is for unilateral tinnitus patients to undergo magnetic resonance imaging of the internal auditory meatus to exclude vestibular schwannoma.

To evaluate magnetic resonance imaging in the investigation of patients with unilateral non-pulsatile tinnitus without asymmetrical hearing loss.

A retrospective case series was conducted of all patients who underwent magnetic resonance imaging of the internal auditory meatus to investigate unilateral non-pulsatile tinnitus without asymmetrical hearing loss, from 1 January 2014 to 1 January 2019.

Of 2066 scans, 566 (27 per cent) were performed to investigate patients (335 female, 231 male) with unilateral non-pulsatile tinnitus without asymmetrical hearing loss. Three vestibular schwannomas were detected on imaging, and 134 incidental findings were discovered.

The detection rate of vestibular schwannoma in this group was just 0.3 per cent. This paper questions the utility of magnetic resonance imaging evaluation in these patients.

To present our data evaluating the feasibility of simultaneous cochlear implantation with resection of acoustic neuroma.

This paper describes a case series of eight adult patients with a radiologically suspected acoustic neuroma, treated at a tertiary referral centre in Newcastle, Australia, between 2012 and 2015. Patients underwent cochlear implantation concurrently with removal of an acoustic neuroma. The approach was translabyrinthine, with facial nerve monitoring and electrically evoked auditory brainstem response testing. Standard post-implant rehabilitation was employed, with three and six months’ follow-up data collected. The main outcome measures were: hearing, subjective benefit of implant, operative complications and tumour recurrence.

Eight patients underwent simultaneous cochlear implantation with resection of acoustic neuroma over a 3-year period, and had 25–63 months’ follow up. There were no major complications. All patients except one gained usable hearing and were daily implant users.

Simultaneous cochlear implantation with resection of acoustic neuroma has been shown to be a safe treatment option, which will be applicable in a wide range of clinical scenarios as the indications for cochlear implantation continue to expand.

To evaluate VIIth–XIIth cranial nerve (hypoglossal–facial nerve) anastomosis results by age.

A total of 34 patients who attended a follow-up visit in 2016, aged 20–63 years, were enrolled. The House–Brackmann facial nerve function grading system and the Facial Clinimetric Evaluation scale were applied.

Regarding post-anastomosis facial nerve function, in the group aged 40 years or less, 14 patients (78 per cent) had House–Brackmann grade III and 4 patients (22 per cent) had House–Brackmann grade IV facial nerve function post-anastomosis. In the group aged over 40 years, nine patients (56 per cent) had House–Brackmann grade III and seven patients (44 per cent) had House–Brackmann grade IV facial nerve function post-anastomosis. There was a statistically significant difference between the two groups in mean facial movement domain scores (p = 0.02). Analysis between age and facial movement score in all 34 patients demonstrated a moderate negative correlation (Pearson correlation coefficient: −0.38) and statistical significance (p = 0.02).

Facial reanimation yielded better results in younger than in older patients.

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.

A systematic review of the literature was performed, with narrative synthesis and statistical analysis of data where appropriate.

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.

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 examine when cochlear fibrosis occurs following a translabyrinthine approach for vestibular schwannoma resection, and to determine the safest time window for potential cochlear implantation in cases with a preserved cochlear nerve.

This study retrospectively reviewed the post-operative magnetic resonance imaging scans of patients undergoing a translabyrinthine approach for vestibular schwannoma resection, assessing the fluid signal within the cochlea. Cochleae were graded based on the Isaacson et al. system (from grade 0 – no obstruction, to grade 4 – complete obliteration).

Thirty-nine patients fulfilled the inclusion criteria. The cochleae showed no evidence of obliteration in: 75 per cent of patients at six months, 38.5 per cent at one year and 27 per cent beyond one year. Most changes happened between 6 and 12 months after vestibular schwannoma resection, with cases of an unobstructed cochlear decreasing dramatically, from 75 per cent to 38.5 per cent, within this time.

The progress of cochlear obliteration that occurred between 6 and 12 months following vestibular schwannoma resection indicates that the first 6 months provides a safer time window for cochlear patency.

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.

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.

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.

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.

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.

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.

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 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.

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.

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.

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 in younger patients have been observed to be larger in size and grow more quickly.

This study aimed to evaluate the expression of three important cell cycle proteins, cyclin D1, cyclin D3 and Ki-67, in vestibular schwannoma patients separated into two age groups: ≤40 years or >40 years.

Immunohistochemical detection of cyclin D1, cyclin D3 and Ki-67 was undertaken in 180 surgically resected vestibular schwannomas.

The proliferation index of vestibular schwannomas was statistically higher in the ≤40 years age group compared to that in the >40 years age group (mean of 4.52 vs 3.27, respectively; p = 0.01). Overexpression of cyclin D1 and cyclin D3 was found in 68 per cent and 44 per cent of tumours, respectively.

There was an increased Ki-67 proliferation index in the younger age group that appears to correlate with clinical behaviour. Vestibular schwannomas in both age groups show increased expression of cyclin D1 and cyclin D3.