A neuroimaging measure to capture heterogeneous patterns of atrophy in Parkinson's disease and dementia with Lewy bodies.

INTRODUCTION: Parkinson's disease (PD) and Dementia with Lewy bodies (DLB) show heterogeneous brain atrophy patterns which group-average analyses fail to capture. Neuroanatomical normative modelling overcomes this by comparing individuals to a large reference cohort. Patient-specific atrophy patterns are measured objectively and summarised to index overall neurodegeneration (the 'total outlier count'). We aimed to quantify patterns of neurodegenerative dissimilarity in participants with PD and DLB and evaluate the potential clinical relevance of total outlier count by testing its association with key clinical measures in PD and DLB. MATERIALS AND METHODS: We included 108 participants with PD and 61 with DLB. PD participants were subclassified into high and low visual performers as this has previously been shown to stratify those at increased dementia risk. We generated z-scores from T1w-MRI scans for each participant relative to normative regional cortical thickness and subcortical volumes, modelled in a reference cohort (n = 58,836). Outliers (z < -1.96) were aggregated across 169 brain regions per participant. To measure dissimilarity, individuals' Hamming distance scores were calculated. We also examined total outlier counts between high versus low visual performance in PD; and PD versus DLB; and tested associations between these and cognition. RESULTS: There was significantly greater inter-individual dissimilarity in brain-outlier patterns in PD poor compared to high visual performers (W = 522.5; p < 0.01) and in DLB compared to PD (W = 5649; p < 0.01). PD poor visual performers had significantly greater total outlier counts compared to high (ß = -4.73 (SE = 1.30); t = -3.64; p < 0.01) whereas a conventional group-level GLM failed to identify differences. Higher total outlier counts were associated with poorer MoCA (ß = -0.55 (SE = 0.27), t = -2.04, p = 0.05) and composite cognitive scores (ß = -2.01 (SE = 0.79); t = -2.54; p = 0.02) in DLB, and visuoperception (ß = -0.67 (SE = 0.19); t = -3.59; p < 0.01), in PD. CONCLUSIONS: Neuroanatomical normative modelling shows promise as a clinically informative technique in PD and DLB, where patterns of atrophy are variable.

A challenging case of sporadic melanocytoma of the jugular foramen.

BACKGROUND: The jugular foramen (JF) can be the site of several tumours. Paragangliomas, schwannomas and meningiomas are the most commonly reported. We describe a case of melanocytoma originating from the JF and presenting with an accessory nerve palsy. ILLUSTRATIVE CASE: A 48-year-old woman presented with a 6-month history of cervical and left shoulder pain with wasting and weakness of the left trapezius. A Magnetic Resonance Imaging (MRI) showed a T1-hyperintense, T2-isointense, heterogeneously enhancing lesion involving the left JF and extending into the cerebello-medullary and cerebello-pontine cisterns. A retrosigmoid craniotomy was performed and a near-total removal achieved. The accessory nerve was involved by tumour and could not be preserved. Given the diagnostic uncertainty between melanotic schwannoma, metastatic melanoma and meningeal melanocytoma, next generation sequencing and genome-wide DNA methylation arrays were performed, documenting a mutation in GNA11 (c.6226A>T, p. Gln209Leu) and a methylation profile consistent with melanocytoma. The patient underwent adjuvant fractionated radiotherapy of the tumour remnant. A follow-up MRI 4 years after surgery did not show any tumour recurrence. CONCLUSIONS: The differential diagnosis of skull base pigmented tumours can be challenging, particularly when they occur in unusual locations such as the JF. They can be misdiagnosed given their similar clinical, neuroradiological and pathological features if anatomy of the site of origin is not carefully considered and molecular tests are not performed, leading to erroneous treatment and follow-up planning.

Combined neurosurgical and orbital intervention for spheno-orbital meningiomas - the Manchester experience.

PURPOSE: Surgical resection of spheno-orbital meningioma (SOM) is challenging, requiring a multidisciplinary surgical approach. We present our experience of the surgical management of patients with SOM. METHODS: A retrospective analysis of patients with SOM who underwent joint neurosurgical and orbital surgical procedures between January 2000 and June 2017. Pre-operative clinical signs, indication for surgery, surgical complications and post-operative outcomes were recorded. RESULTS: Twenty-four operations were performed. Mean age was 49.5 years. Ninety-two percent of patients were female. Pre-operatively mean Snellen acuity vision was 6/12; 13 (54%) had an RAPD; 12 (50%) had reduced colour vision; 16 (67%) had a visual field defect. The majority (21 patients, 88%) had proptosis (average 4.5 mm ± 2.8 mm). The indication for surgery was evidence of visual dysfunction in 17 (71%), the remaining 7 (29%) had high risk of visual loss clinically or radiologically. Three-months post operatively, vision was stable in 13 (58%), improved in 6 (21%) and worse in 5 (17%). Average long-term follow-up was 82 months (1-220). Fourteen (58%) maintain improved or stable visual function. Four (17%) had reduced vision due to regrowth of the tumour at an average of 24 months. CONCLUSION: SOMs are very challenging to treat surgically. In this cohort the patients were predominantly young females with aggressive disease. Visual function was improved or stabilised in 79% of the patients.

Spinal ependymomas in NF2: a surgical disease?

The management of spinal cord ependymomas in Neurofibromatosis Type 2 (NF2) has traditionally been conservative, in contrast to the management of sporadic cases; the assumption being that, in the context of NF2, they did not cause morbidity. With modern management and improved outcome of other NF2 tumours, this assumption, and therefore the lack of role for surgery, has been questioned. To compare the outcome of conservative treatment of spinal ependymomas in NF2 with surgical intervention in selected patients. Retrospective review at two NF2 centers, Manchester, UK and Paris/Lille, France. In Manchester patients were managed conservatively. In France surgery was a treatment option. Inclusion in the study was based on tumor length of greater than 1.5 cm. The primary parameter assessed was acquired neurological deficit measured by the Modified McCormick Outcome Score. 24 patients from Manchester and 46 patients from France were analyzed. From Manchester, 27% of these patients deteriorated during the course of follow-up. This effectively represents the natural history of ependymomas in NF2. Of the surgical cases, 23% deteriorated postoperatively, but only 2/18 (11%) of those operated on in the NF2 specialist centers. Comparison of the two specialist centers Manchester/France showed a significantly improved outcome (P = 0.012, χ2 test) in the actively surgical center. Spinal ependymomas produce morbidity. Surgery can prevent or improve this in selected cases but can itself can produce morbidity. Surgery should be considered in growing/symptomatic ependymomas, particularly in the absence of overwhelming tumor load where bevacizumab is the preferred option.

Modified RS-LAMP assay and use of lateral flow devices for rapid detection of Leifsonia xyli subsp. xyli.

Ratoon stunt (RS) caused by bacterium Leifsonia xyli subsp. xyli (Lxx) results in substantial yield losses in sugarcane (Saccharum sp. L. hybrid). Since RS does not produce reliable symptoms in the field, laboratory-based techniques are necessary for detection. Loop-mediated isothermal amplification (LAMP) assay overcomes the limitations of laboratory-based techniques which are costly, time consuming and cannot be used for near-field detection. A sensitive LAMP assay was developed to detect Lxx at 65°C in 30 min. However, carry-over contamination affected the reliability of the assay. In the present study, contaminants were successfully eliminated by incorporation of uracil nucleoside glycosylase (1 U µl-1 ) into the LAMP assay and incubation for 10 min at 37°C. To avoid the use of colorimetric reagents, lateral flow devices were successfully used for the detection of LAMP products and were equally sensitive to detection by agarose gel electrophoresis. The use of exudate from leaf sheath discs as an alternate template for the LAMP assay was found to be less sensitive than xylem sap. The preprepared master mix could be stored for up to 4 months at -20°C without any reduction in performance. These changes make the assay suitable for near-field detection in laboratories with basic facilities. SIGNIFICANCE AND IMPACT OF THE STUDY: This study presents a modified loop-mediated isothermal amplification (LAMP) assay for the detection of Leifsonia xyli subsp. xyli. Modifications include incorporation of uracil nucleoside glycosylase to eliminate carry-over contamination and substitution of colorimetric detection for the use of lateral flow devices. LAMP master mix was preprepared and was stably stored up to 4 months at -20°C. Sugarcane leaf sheaths worked well as a substitute to xylem sap as template, although the sensitivity was lower. These modifications allow the assay to be conducted without contamination concerns and reduction in set up time, making it ideal for near-field diagnosis.

Hearing optimisation in neurofibromatosis type 2: A systematic review.

BACKGROUND: It is common for patients with neurofibromatosis type 2 to develop bilateral profound hearing loss hearing loss, and this is one of the main determinants of quality of life in this patient group. OBJECTIVES: The aim of this systematic review was to review the current literature regarding hearing outcomes of treatments for vestibular schwannomas in neurofibromatosis type 2 including conservative and medical management, radiotherapy, hearing preservation surgery and auditory implantation in order to determine the most effective way of preserving or rehabilitating hearing. SEARCH STRATEGY: A MESH search in PubMed using search terms (('Neurofibromatosis 2' [Mesh]) AND 'Neuroma, Acoustic'[Mesh]) AND 'Hearing Loss' [Mesh] was performed. A search using keywords was also performed. Studies with adequate hearing outcome data were included. With the exception of the cochlear implant studies (cohort size was very small), case studies were excluded. EVALUATION METHOD: The GRADE system was used to assess quality of publication. Formal statistical analysis of data was not performed because of very heterogenous data reporting. RESULTS: Conservative management offers the best chance of hearing preservation in stable tumours. The use of bevacizumab probably improves the likelihood of hearing preservation in growing tumours in the short term and is probably more effective than hearing preservation surgery and radiotherapy in preserving hearing. Of the hearing preservation interventions, hearing preservation surgery probably offers better hearing preservation rates than radiotherapy for small tumours but recurrence rates for hearing preservation surgery were high. For patients with profound hearing loss, cochlear implantation provides significantly better auditory outcomes than auditory brainstem implantation. Patients with untreated stable tumours are likely to achieve the best outcomes from cochlear implantation. Those who have had their tumours treated with surgery or radiotherapy do not gain as much benefit from cochlear implantation than those with untreated tumours. CONCLUSIONS: This review summarises the current literature related to hearing preservation/rehabilitation in patients with NF2. Whilst it provides indicative data, the quality of the data was low and should be interpreted with care. It is also important to consider that the management of vestibular schwannomas in NF2 is complex and decision-making is determined by many factors, not just the need to preserve hearing.

Source and risk factors of a cutaneous anthrax outbreak, Jiangsu, Eastern China, 2012.

Anthrax is still a severe public health problem and threat to human health. A cutaneous anthrax outbreak occurred in Jiangsu Province, a non-endemic anthrax region of eastern China, from July to August 2012. Epidemiological and laboratory investigation were initiated to trace the source of infection and identify the risk factors of the outbreak. On 25 July 2012, 17 persons were exposed to a sick cow, which had been imported from northeast China a few days previously. Of the 17 exposed, eight developed symptoms between 1 and 8 days and were diagnosed as cutaneous anthrax cases. Three main genes of Bacillus anthracis were detected from both human and cow meat samples, indicating that the outbreak was associated with this infected cow. A retrospective cohort study showed that contact with blood and presence of skin damage contributed to the case infection with B. anthracis. The outbreak highlights the need to enhance quarantine for imported livestock, which should have been vaccinated prior to importation, the significance of education for high-risk individuals, and training for primary healthcare workers even in anthrax-free areas.

Effect of bending direction on the mechanical behaviour of 3.5 mm String-of-Pearls and Limited Contact Dynamic Compression Plate constructs.

OBJECTIVE: To compare the bending properties of String-of-Pearls® (SOP) and Limited Contact Dynamic Compression Plate® (LC-DCP) constructs in orthogonal bending directions. METHODS: 3.5 mm SOP and LC-DCP plates were fixed to a bone model simulating a comminuted tibial fracture. Specimens were non-destructively tested in both mediolateral and craniocaudal bending for 10 cycles. Bending stiffness and total angular deformation were compared using parametric analyses (p <0.05). RESULTS: For both constructs, stiffness was significantly less when bending moments were applied against the thickness of the plates (mediolateral bending) than against the width (craniocaudal bending). When compared to the mediolateral plane, bending constructs in the craniocaudal plane resulted in a 49% (SOP group) and 370% (LC-DCP group) increase in stiffness (p <0.001). Mediolateral bending stiffness was significantly greater in the SOP than the LC-DCP constructs. Conversely, in craniocaudal bending, SOP constructs stiffness was significantly less than that of the LC-DCP constructs. The differences between the two constructs in total angular deformation had an identical pattern of significance. CLINICAL SIGNIFICANCE: This study found that SOP showed less variability between the orthogonal bending directions than LC-DCP in a comminuted fracture model, and also described the bi-planar bending behaviour of both constructs. Although not exhibiting identical bending properties in both planes, SOP constructs had a more homogenous bending behaviour in orthogonal loading directions. The difference between the SOP with a circular cross sectional shape compared to the rectangular shape of standard plates is probably responsible for this difference.

Rapid Increase in Neural Conduction Time in the Adult Human Auditory Brainstem Following Sudden Unilateral Deafness.

Individuals with sudden unilateral deafness offer a unique opportunity to study plasticity of the binaural auditory system in adult humans. Stimulation of the intact ear results in increased activity in the auditory cortex. However, there are no reports of changes at sub-cortical levels in humans. Therefore, the aim of the present study was to investigate changes in sub-cortical activity immediately before and after the onset of surgically induced unilateral deafness in adult humans. Click-evoked auditory brainstem responses (ABRs) to stimulation of the healthy ear were recorded from ten adults during the course of translabyrinthine surgery for the removal of a unilateral acoustic neuroma. This surgical technique always results in abrupt deafferentation of the affected ear. The results revealed a rapid (within minutes) reduction in latency of wave V (mean pre = 6.55 ms; mean post = 6.15 ms; p < 0.001). A latency reduction was also observed for wave III (mean pre = 4.40 ms; mean post = 4.13 ms; p < 0.001). These reductions in response latency are consistent with functional changes including disinhibition or/and more rapid intra-cellular signalling affecting binaurally sensitive neurons in the central auditory system. The results are highly relevant for improved understanding of putative physiological mechanisms underlying perceptual disorders such as tinnitus and hyperacusis.

Current practice and factors influencing the provision of periodontal healthcare in primary dental care in Scotland: an explorative study.

AIMS: To understand current practice and the beliefs of dentists and hygienists towards factors which may influence the management of patients with periodontal diseases in primary dental care in Scotland. To inform the scope of a guidance publication and the future development of diagnostic analyses tools. METHODS: A qualitative approach of semi-structured telephone interviews with a randomly selected sample of dentists and hygienists was used. Interviews continued until saturation was reached and were audio-recorded and transcribed verbatim. The data were analysed using the method of framework analysis. To increase the participation of hygienists the topic guide was adapted into a self-reporting questionnaire and posted to all hygienists in Scotland. RESULTS: Eighteen dentists and three hygienists were interviewed. Key themes identified were extent of control over working environment, capabilities to treat appropriately and changing patients' oral hygiene behaviour. Factors which facilitated the management of patients with periodontal diseases were 'access to a hygienist' and 'beliefs about capabilities', while 'routine and habit', 'motivation of patients' and 'time and money' were perceived as barriers. Some evidence of variation in treatment provision and of varying levels of confidence among practitioners about treating more advanced periodontal conditions was identified. Two hundred and thirty-seven hygienists returned the postal questionnaire. Agreement was found with the views of the interviewed hygienists on beliefs about capabilities and routine record-keeping. CONCLUSION: To improve the periodontal health of patients requires consideration of both extrinsic and intrinsic factors present in primary dental care. Practitioners would find having guidance on managing patients with basic periodontal examinations of three and four, referral criteria to secondary care, record-keeping and techniques to change patients' oral hygiene behaviour particularly useful. Applying the evaluative framework pre-publication indicated where knowledge translation interventions may be required in the future.

Temporal and spatial patterns of diarrhoea in the Mekong Delta area, Vietnam.

This study examined the temporal and spatial patterns of diarrhoea in relation to hydro-meteorological factors in the Mekong Delta area in Vietnam. A time-series design was applied to examine the temporal pattern of the climate-diarrhoea relationship using Poisson regression models. Spatial analysis was applied to examine the spatial clusters of diarrhoea using Global Moran's I and local indicators of spatial autocorrelation (LISA). The temporal pattern showed that the highest peak of diarrhoea was from weeks 30-42 corresponding to August-October annually. A 1 cm increase in river water level at a lag of 1 week was associated with a small [0·07%, 95% confidence interval (CI) 0·01-0·1] increase in the diarrhoeal rate. A 1 °C increase in temperature at lag of 2 and 4 weeks was associated with a 1·5% (95% CI 0·3-2·7) and 1·1% (95% CI 0·1-2·3) increase in diarrhoeal risk, respectively. Relative humidity and diarrhoeal risk were in nonlinear relationship. The spatial analysis showed significant clustering of diarrhoea, and the LISA map shows three multi-centred diarrhoeal clusters and three single-centred clusters in the research location. The findings suggest that climatic conditions projected to be associated with climate change have important implication for human health impact in the Mekong Delta region.

Rare giant frontal sinus osteoma mimicking fibrous dysplasia.

OBJECTIVE: To present the first report of a giant frontal sinus osteoma treated by excision and single-stage reconstruction with custom-made titanium cranioplasty and left orbital roof prostheses. CASE REPORT: A 31-year-old man with a history of chronic frontal sinusitis presented with a deforming, painless, midline forehead swelling of 11 years' duration, which had been treated unsuccessfully in Nigeria. Differential diagnosis included both benign and malignant bony tumours. Computerised tomography revealed a giant bony frontal sinus tumour extending beyond the sinus roof and breaching the left orbit, consistent with fibrous dysplasia. Given the extent of the tumour, open craniectomy was performed for surgical extirpation. Histological analysis identified multiple osteomas. This surgical approach achieved excellent cosmesis, with no evidence of recurrence at 12-month follow up. CONCLUSION: Forehead swelling may pose diagnostic and management dilemmas for the ENT surgeon; however, effective management is facilitated by a multidisciplinary approach.

Bilateral vestibular schwannomas in older patients: NF2 or chance?

BACKGROUND: Neurofibromatosis type 2 (NF2) is an autosomal dominant condition with high spontaneous mutation rate which predisposes to the development of multiple nerve sheath tumours (schwannomas), meningiomas and ependymoma. The cardinal feature and main diagnostic criterion for the diagnosis of NF2 remains the development of bilateral vestibular schwannoma (BVS). With increasing use of MRI screening the possibility of a 'chance' diagnosis of BVS has been mooted with a potential frequency of one in two million people in their lifetime. Until now, however, no evidence for such an event has been published. We aimed to demonstrate that chance occurrence can occur and to estimate its frequency among those with just BVS late in life. METHODS: Two vestibular schwannomas from the same patient were DNA sequenced and underwent loss of heterozygosity analysis. RESULTS: We show that a man who developed BVS, at ages 52 and 67 years developed these tumours sporadically by demonstrating that there were no molecular events in common between the two tumours. Furthermore from a database of over 1200 patients with NF2, we have estimated that ~25% of cases of BVS over 50 years and 50% over 70 years of age where no other features of NF2 are present represent a chance occurrence rather than due to an underlying mosaic or constitutional NF2 mutation. CONCLUSIONS: Patients presenting with BVS later in life should be appraised of the potential likelihood they may not have NF2 and the resultant further reduction in risks of transmission to offspring.

Geniculate neuralgia: a systematic review.

OBJECTIVE: To systematically summarise the peer-reviewed literature relating to the aetiology, clinical presentation, investigation and treatment of geniculate neuralgia. DATA SOURCES: Articles published in English between 1932 and 2012, identified using Medline, Embase and Cochrane databases. METHODS: The search terms 'geniculate neuralgia', 'nervus intermedius neuralgia', 'facial pain', 'otalgia' and 'neuralgia' were used to identify relevant papers. RESULTS: Fewer than 150 reported cases were published in English between 1932 and 2012. The aetiology of the condition remains unknown, and clinical presentation varies. Non-neuralgic causes of otalgia should always be excluded by a thorough clinical examination, audiological assessment and radiological investigations before making a diagnosis of geniculate neuralgia. Conservative medical treatment is always the first-line therapy. Surgical treatment should be offered if medical treatment fails. The two commonest surgical options are transection of the nervus intermedius, and microvascular decompression of the nerve at the nerve root entry zone of the brainstem. However, extracranial intratemporal division of the cutaneous branches of the facial nerve may offer a safer and similarly effective treatment. CONCLUSION: The response to medical treatment for this condition varies between individuals. The long-term outcomes of surgery remain unknown because of limited data.

Sources of Streptococcus dysgalactiae in English and Welsh sheep flocks affected by infectious arthritis (joint ill).

In order to investigate sheep sources of Streptococcus dysgalactiae in flocks affected with joint ill, 10 sheep flocks in England and Wales with laboratory-confirmed cases of infectious arthritis (joint ill) caused by S dysgalactiae were visited during a disease outbreak while a further four flocks were visited during the lambing period in the year following an outbreak. A total of 5239 samples were collected for bacterial culture from 797 ewes and their 1314 lambs. S dysgalactiae was isolated from nine of 894 samples (1 per cent) on farms visited during an outbreak, and from 7 of 4462 samples (0.2 per cent) collected in the year following an outbreak. The 16 samples from which S dysgalactiae was isolated came from the vaginas of eight ewes, milk of one ewe, navels of four lambs, mouths of two lambs and noses of one lamb. In vitro testing of the survival of S dysgalactiae on straw, hay and in water at different temperatures was performed, and it was isolated from these substrates for up to 42, 35 and 0 days, respectively.

Drinking water contributes to excessive iodine intake among children in Hebei, China.

BACKGROUND/OBJECTIVES: In Hebei province, China, over six million people are potentially exposed to excessive iodine through consumption of high iodine underground drinking water and consumption of iodized salt. The aim of this study is to evaluate the contributions of drinking water and iodized salt on children's iodine nutrition in one area of Hebei province in order to refine strategies to correct the excessive iodine intake in these areas. SUBJECTS/METHODS: To investigate the relationships between iodine content in water, iodized salt and urinary iodine content (UIC) in children (8-10 years), we randomly sampled three towns with a known median water iodine (MWI) of 150-300 µg/l in Hengshui City, Hebei province and collected water, salt and urine samples. RESULTS: The median UIC was 518.1 µg/l, the overall MWI was 247.0 µg/l, and 83% of children sampled were found to have urinary iodine concentrations higher than the WHO criterion of 300 µg/l. There was a significant and positive correlation between the median UIC of the children and the MWI in the 12 villages where the children lived (Spearman R=0.79, P=0.002), but the UIC was not significantly correlated with the median salt intake (MSI) (Spearman R=-0.17, P=0.6). A multiple linear regression analysis indicated that 68.7% of the variability in median UIC is associated with variability in MWI in the 12 villages. CONCLUSIONS: Iodine in drinking water was identified to be the key contributor to this excessive iodine in children indicating that in these areas, intervention should focus on providing alternative drinking water supplies.

Body mass index and risk of pneumonia: a systematic review and meta-analysis.

The aims of our meta-analysis were to examine the pattern and gender's influence on body mass index (BMI) - pneumonia relationship. Published studies were searched from PubMed, Web of Science, Cochrane Library databases using keywords of pneumonia, BMI and epidemiologic studies. Random-effects analysis was applied to estimate pooled effect sizes from individual studies. The Cochrane Q-test and index of heterogeneity (I(2) ) were used to evaluate heterogeneity, and Egger's test was used to evaluate publication bias. Random-effects meta-regression was applied to examine the pattern and gender's influence on BMI-pneumonia relationship. A total of 1,531 studies were initially identified, and 25 studies finally were included. The pooled relative risk (RR) and meta-regression model revealed a J-shaped relationship between BMI and risk of community-acquired pneumonia (underweight, RR 1.8, 95% confidence interval [CI], 1.4-2.2, P < 0.01; overweight, 0.89, 95%CI, 0.8-1.03, P, 0.1; obesity, 1.03, 95% CI, 0.8-1.3, p. 8) and U-shaped relationship between BMI and risk of influenza-related pneumonia (underweight, RR 1.9, 95% CI, 1.2-3, P < 0.01; overweight, 0.89, 95% CI, 0.79-0.99, P, 0.03; obesity, 1.3, 95% CI, 1.05-1.63, p. 2; morbidity obesity, 4.6, 95% CI, 2.2-9.8, P < 0.01); whereas, no difference in risk of nosocomial pneumonia was found across the BMI groups. Gender difference did not make significant contribution in modifying BMI-pneumonia risk relationship.

Outcome of translabyrinthine surgery for vestibular schwannoma in neurofibromatosis type 2.

OBJECTIVES: To analyse the long-term outcome of translabyrinthine surgery for vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2). RESEARCH TYPE: Retrospective cohort study. SETTING: Two tertiary referral NF2 units. PATIENTS: One hundred and forty eight translabyrinthine operations for patients with VS were performed. Preoperative stereotactic radiotherapy had been performed on 12(9.4%) patients. RESULTS: Mean tumour size was 3.1 cm. Total tumour excision was achieved in 66% of cases, capsular remnants were left in 24% of cases, and subtotal excision was achieved in 5% and partial removal was achieved in 5%. The radiological residual/recurrence rate was 13.9%. The perioperative mortality was 1.6%. At 2 years postoperatively, facial function was expressed in terms of House-Brackmann score (HB): HB 1 in 53.4%, HB 1/2 in 61.3%, HB 1-3 in 83.2% and HB 4-6 in 16.8%. All nine patients who underwent surgery following failed stereotactic radiotherapy had HB 3 function or better. Among 9.5% of the cases, 14 facial nerves were lost during surgery and repaired using direct anastomosis or grafting. There was no tinnitus present preoperatively in 27% of the cases, and 22% of patients developed tinnitus postoperatively. In patients with preoperative tinnitus, 61% remained the same, 17% got it resolved and only in 21% it worsened. The preoperative hydrocephalus rate was 26%, and among 15% of the cases five ventriculo-peritoneal (VP) shunts were performed. The cerebrospinal fluid leak rate was 2.5%. Fifty-six patients underwent auditory brainstem implantation (ABI) and two patients had cochlear implant (CI) sleepers inserted. CONCLUSIONS: The management of patients with NF2 presents the clinician with a formidable challenge with many patients still presenting themselves late with the neurological compromise and a large tumour load. There is still an argument for the management by observation until the neurological compromise dictates interventional treatment particularly with the option of hearing rehabilitation with ABI or CI. The translabyrinthine approach provides a very satisfactory means of reducing the overall tumour volume.