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Objective The study aimed to determine long-term outcomes in patients with intraoperative electrical conduction block in an anatomically intact facial nerve (FN). Methods Single center retrospective review of prospectively collected database of all vestibular schwannoma surgeries between January 1, 2008 and August 25, 2015. Operative notes were reviewed and patients with anatomically intact FNs, but complete conduction block at the end of surgery were included for analysis. Results In total, 371 patients had vestibular schwannoma surgery of which 18 met inclusion criteria. Mean follow-up was 34.28 months and average tumor size was 28.00 mm. Seventeen patients had House-Brackmann Grade VI facial palsy immediately postoperatively and one patient was grade V. At 1 year, three patients remained grade VI (17%), two improved to grade V (11%), seven to grade IV (39%), six to grade III (33%), and one patient to grade II (6%). On extended follow-up, five patients (28%) had additional 1 to 2 score improvement in facial function. Subset analysis revealed no correlation of tumor size, vascularity, adherence to nerve, operative approach, extent of resection, splaying of FN, and recurrent tumor or sporadic tumors to the extent of FN recovery. Conclusion Intraoperative conduction block does not condemn a patient to permanent FN palsy. There is potential for a degree of recovery comparable with those undergoing nerve grafting. Our data do not clearly support a policy of same-surgery or early-postoperative primary nerve grafting in the event of a complete conduction block, and instead we favor monitoring for recovery in an anatomically intact nerve.

Introduction Vestibular schwannomas (VS) are histologically benign tumors arising from cranial nerve VIII. Far from a homogenous proliferation of Schwann cells, mounting evidence has highlighted the complex nature of the inflammatory microenvironment in these tumors. Methods A review of the literature pertaining to inflammation, inflammatory molecular pathways, and immune-related therapeutic targets in VS was performed. Relevant studies published up to June 2020 were identified based on a literature search in the PubMed and MEDLINE databases and the findings were synthesized into a concise narrative review of the topic. Results The VS microenvironment is characterized by a dense infiltrate of inflammatory cells, particularly macrophages. Significantly higher levels of immune cell infiltration are observed in growing versus static tumors, and there is a demonstrable interplay between inflammation and angiogenesis in growing VS. While further mechanistic studies are required to ascertain the exact role of inflammation in angiogenesis, tumor growth, and Schwann cell control, we are beginning to understand the key molecular pathways driving this inflammatory microenvironment, and how these processes can be monitored and targeted in vivo. Conclusion Observational research has revealed a complex and heterogeneous tumor microenvironment in VS. The functional landscape and roles of macrophages and other immune cells in the VS inflammatory infiltrate are, however, yet to be established. The antiangiogenic drug bevacizumab has shown the efficacy of targeted molecular therapies in VS and there is hope that agents targeting another major component of the VS microenvironment, inflammation, will also find a place in their future management.

Testicular incidentalomas are non-palpable, asymptomatic lesions, most frequently detected on ultrasound examinations. Each incidentaloma should undergo a standardized diagnostic workup to exclude malignancy and recognize other potentially significant non-malignant conditions that may first present with an incidental finding on scrotal ultrasound. This position statement of the World Federation of Ultrasound in Medicine and Biology (WFUMB) summarizes the available evidence on management of testicular incidentalomas and describes efficient management strategies with particular reference to the role of ultrasound techniques. (C) 2021 World Federation for Ultrasound in Medicine & Biology. All rights reserved.

PURPOSE: To evaluate the incidence of mosaicism in de novo neurofibromatosis 2 (NF2).METHODS: Patients fulfilling NF2 criteria, but with no known affected family member from a previous generation (n = 1055), were tested for NF2 variants in lymphocyte DNA and where available tumor DNA. The proportion of individuals with a proven or presumed mosaic NF2 variant was assessed and allele frequencies of identified variants evaluated using next-generation sequencing.RESULTS: The rate of proven/presumed mosaicism was 232/1055 (22.0%). However, nonmosaic heterozygous pathogenic variants were only identified in 387/1055 (36.7%). When variant detection rates in second generation nonmosaics were applied to de novo cases, we assessed the overall probable mosaicism rate to be 59.7%. This rate differed by age from 21.7% in those presenting with bilateral vestibular schwannoma CONCLUSION: This study has identified a very high probable mosaicism rate in de novo NF2, probably making NF2 the condition with the highest expressed rate of mosaicism in de novo dominant disease that is nonlethal in heterozygote form. Risks to offspring are small and probably correlate with variant allele frequency detected in blood.

Purpose: We have evaluated deficiencies in existing diagnostic criteria for neurofibromatosis 2 (NF2). Methods: Two large databases of individuals fulfilling NF2 criteria (n=1361) and those tested for NF2 variants with criteria short of diagnosis (n=1416) were interrogated. We assessed the proportions meeting each diagnostic criterion with constitutional or mosaic NF2 variants and the Positive Predictive Value (PPV) with regard to definite diagnosis.Results: There was no evidence for usefulness of old criteria ‘glioma’ or ‘neurofibroma’. ‘Ependymoma’ had 100% PPV and high levels of confirmed NF2 diagnosis (67.7%). Those with bilateral vestibular schwannoma (VS) alone aged ≥60 years had the lowest confirmation rate (6.6%) and reduced PPV(80%). Siblings as a first-degree-relative, without an affected parent, had 0% PPV. All three individuals with unilateral VS and an affected sibling were proven not to have NF2. The biggest overlap was with LZTR1-associated schwannomatosis. In this category, seven individuals with unilateral VS plus ≥2 non-dermal schwannomas reduced PPV to 67%.Conclusion: The present study has confirmed important deficiencies in NF2 diagnostic criteria. The term ‘glioma’ should be dropped and replaced by ‘ependymoma’. Similarly ‘neurofibroma’ should be removed. Dropping ‘sibling’ from first-degree-relatives should be considered and testing of LZTR1 should be recommended for unilateral VS.

Background Limited data exist on the disease course of neurofibromatosis type 2 (NF2) to guide clinical trial design. Methods A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990 and 2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred, and inheritance type. Interventions for NF2-related tumors were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death. Results Three hundred and fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring, 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65, respectively, per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting 40 years had poorer overall survival than those presenting at 26-39 years (P = .03 and P = .02, respectively) but those presenting between 16 and 39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P = .004). Conclusion Understanding disease course improves prognostication, allowing for better-informed decisions about care.

OBJECTIVE: Electrically evoked auditory brainstem responses (eABRs) can be recorded before cochlear implant (CI) surgery to verify auditory nerve function, and is particularly helpful in to assess the function of the auditory nerve in cases of auditory nerve hypoplasia. This is the first study to compare three preimplant eABRs recording techniques: 1) standard extracochlear, 2) novel intracochlear, and 3) conventional intracochlear with the CI. STUDY DESIGN: A within-participants design was used where eABRs were sequentially measured during CI surgery using three methods with stimulation from: 1) an extracochlear electrode placed at the round window niche, 2) two different electrodes on a recently developed Intracochlear Test Array (ITA), and 3) two different electrodes on a CI electrode array. SETTING: New adults implantees (n = 16) were recruited through the Manchester Auditory Implant Centre and eABR measurements were made in theater at the time of CI surgery. PATIENTS: All participants met the clinical criteria for cochlear implantation. Only participants with radiologically normal auditory nerves were recruited to the study. All participants were surgically listed for either a MED-EL Synchrony implant or a Cochlear Nucleus Profile implant, per standard practice in the implant centre. OUTCOME MEASURES: Primary outcome measures were: 1) charge (μC) required to elicit a threshold response, and 2) latencies (ms) in the threshold waveforms. Secondary outcome measures were: 1) morphologies of responses at suprathreshold stimulation levels and 2) wave V growth patterns. RESULTS: eABRs were successfully measured from 15 participants. In terms of primary outcome measures, the charge required to elicit a response using the extracochlear electrode (median = 0.075 μC) was approximately six times larger than all other electrodes and the latency of wave V was approximately 0.5 ms longer when using the extracochlear electrode (mean = 5.1 ms). In terms of secondary outcomes, there were some minor quantitative differences in responses between extracochlear and intracochlear stimulation; in particular, ITA responses were highly variable in quality. The ITA responses were rated poor quality in 33% of recordings and in two instances did not allow for data collection. When not disrupted by open circuits, the median ITA response contained one more waveform than the median extracochlear response. CONCLUSIONS: In this first study comparing intracochlear and extracochlear stimulation, the results show that both can be used to produce an eABR that is representative of the one elicited by the CI. In the majority of cases, extracochlear stimulation was the preferred approach for preimplant auditory nerve function testing because of consistency, recordings that could be analyzed, and because extracochlear placement of the electrode does not require a cochleostomy to insert an electrode.

ObjectivesCases of sporadic vestibular schwannoma (sVS) have a low rate of association with germline pathogenic variants. However, some individuals with sVS can represent undetected cases of neurofibromatosis type 2 (NF2) or schwannomatosis. Earlier identification of patients with these syndromes can facilitate more accurate familial risk prediction and prognosis.MethodsCases of sVS were ascertained from a local register at the Manchester Centre for Genomic Medicine. Genetic analysis was conducted in NF2 on blood samples for all patients, and tumour DNA samples when available. LZTR1 and SMARCB1 screening was also performed in patient subgroups.ResultsAge at genetic testing for vestibular schwannoma (VS) presentation was younger in comparison with previous literature, a bias resulting from updated genetic testing recommendations. Mosaic or constitutional germline NF2 variants were confirmed in 2% of patients. Pathogenic germline variants in LZTR1 were found in 3% of all tested patients, with a higher rate of 5% in patients SMARCB1 variants were identified within the cohort. Considering all individuals who received tumour DNA analysis, 69% of patients were found to possess two somatic pathogenic NF2 variants, including those with germline LZTR1 pathogenic variants.ConclusionsUndiagnosed schwannoma predisposition may account for a significant minority of apparently sVS cases, especially at lower presentation ages. Loss of NF2 function is a common event in VS tumours and may represent a targetable common pathway in VS tumourigenesis. These data also support the multi-hit mechanism of LZTR1-associated VS tumourigenesis.

We present the case of a 14 year old who underwent laparoscopic marsupialisation of a splenic cyst. Postoperative ultrasound demonstrated an echogenic collection in the surgical bed that was initially misdiagnosed as an infected collection/abscess due to its sonographic appearances but was finally correctly identified as haemostatic material that had been packed into the surgical cavity. We subsequently constructed a tissue phantom containing a compressed ball of SURGICEL (r) Absorbable Hemostat and demonstrated that it showed identical sonographic appearances. Haemostatic agents such as oxidised cellulose are often packed within the operative bed to control microvascular haemorrhage. These agents can be observed on a variety of postoperative imaging modalities, in particular ultrasound and computed tomography, and may cause diagnostic error. The critical importance of understanding the details of the surgical procedure and effective communication between the surgical team and ultrasound practitioner is emphasised to minimise the risk of misdiagnosis and unnecessary further imaging and radiological/surgical intervention.

Objectives/hypothesis Unilateral vestibular schwannoma (VS) occurs with a lifetime risk of around 1 in 1,000 and is due to inactivation of the NF2 gene, either somatically or from a constitutional mutation. It has been postulated that familial occurrence of unilateral VS occurs more frequently than by chance, but no causal mechanism has been confirmed. Study design Retrospective database analysis. Methods The likelihood of chance occurrence of unilateral VS, or occurring in the context of neurofibromatosis type 2 (NF2), was assessed using national UK audit data and data from the national NF2 database. Families with familial unilateral VS (occurrence in first- and second-degree relatives) were assessed for constitutional NF2 and LZTR1 genetic variants, and where possible the tumor was also analyzed. Results Approximately 1,000 cases of unilateral VS occurred annually in the United Kingdom between 2013 and 2016. Of these, 2.5 may be expected to have a first-degree relative who had previously developed a unilateral VS. The likelihood of this occurring in NF2 was considered to be as low as 0.05 annually. None of 28 families with familial unilateral VS had a constitutional NF2 intragenic variant, and in nine cases where the VS was analyzed, both mutational events in NF2 were identified and excluded from the germline. Only three variants of uncertain significance were found in LZTR1. Conclusions Familial occurrence of unilateral VS is very unlikely to be due to a constitutional NF2 or definitely pathogenic LZTR1 variant. The occurrence of unilateral VS in two or more first-degree relatives is likely due to chance. This phenomenon may well increase in clinical practice with increasing use of cranial magnetic resonance imaging in older patients. Level of evidence 2b Laryngoscope, 129:967-973, 2019.

ObjectivesSchwannomatosis is a dominantly inherited condition predisposing to schwannomas of mainly spinal and peripheral nerves with some diagnostic overlap with neurofibromatosis-2 (NF2), but the underlying epidemiology is poorly understood. We present the birth incidence and prevalence allowing for overlap with NF2.MethodsSchwannomatosis and NF2 cases were ascertained from the Manchester region of England (population=4.8 million) and from across the UK. Point prevalence and birth incidence were calculated from regional birth statistics. Genetic analysis was also performed on NF2, LZTR1 and SMARCB1 on blood and tumour DNA samples when available.ResultsRegional prevalence for schwannomatosis and NF2 were 1 in 126 315 and 50 500, respectively, with calculated birth incidences of 1 in 68 956 and 1 in 27 956. Mosaic NF2 causes a substantial overlap with schwannomatosis resulting in the misdiagnosis of at least 9% of schwannomatosis cases. LZTR1-associated schwannomatosis also causes a small number of cases that are misdiagnosed with NF2 (1%–2%), due to the occurrence of a unilateral vestibular schwannoma. Patients with schwannomatosis had lower numbers of non-vestibular cranial schwannomas, but more peripheral and spinal nerve schwannomas with pain as a predominant presenting symptom. Life expectancy was significantly better in schwannomatosis (mean age at death 76.9) compared with NF2 (mean age at death 66.2; p=0.004).ConclusionsWithin the highly ascertained North-West England population, schwannomatosis has less than half the birth incidence and prevalence of NF2.

The authors present the case of a 24-year-old female with neurofibromatosis type 2. Growth of the left vestibular schwannoma and progressive hearing loss prompted the decision to proceed to translabyrinthine resection with cochlear nerve preservation and cochlear implant insertion. Complete resection with preservation of the facial and cochlear nerves was achieved. The patient had grade 1 facial function and was discharged on postoperative day 4 following suturing of a minor CSF leak. This case highlights the feasibility of cochlear nerve preservation and cochlear implant insertion in appropriately selected patients, offering a combination of effective tumor control and hearing rehabilitation. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21122

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.

Turgut, Ahmet Tuncay (isu author) Varicoceles are relatively common particularly in asymptomatic men and are even more prevalent in subfertile men, representing the most common potentially correctable cause of male infertility. Ultrasound (US) is the imaging modality of choice for varicocele evaluation, but there is no widely accepted consensus on examination technique, diagnostic criteria, or classification. In view of this uncertainty, the guideline writing group (WG) of the European Society of Urogenital Radiology (ESUR) Scrotal and Penile Imaging Working Group (ESUR-SPIWG) undertook a literature review and assessment of the quality of relevant evidence. The group then produced evidence-based recommendations for varicocele US examination, interpretation, and classification by consensus agreement. The results are presented in the form of 15 clinical questions with a brief summary of the relevant evidence and the authorised recommendations from the SPIWG. This paper provides a short summary of the evidence evaluation and the complete recommendations. Key Points center dot Varicocele is a common clinical problem; it is highly prevalent amongst subfertile men and the most common potentially correctable cause of male infertility. center dot Ultrasound is the imaging modality of choice for varicocele assessment, but there is no generally agreed consensus on the US examination technique or the criteria that should be used for diagnosis, grading, and classification. center dot This paper summarises the recommendations of the ESUR-SPIWG for standardising the US assessment of varicoceles. This includes examination technique, image interpretation, classification, and reporting. WOS:000501139000002 31332561 Q1

OBJECTIVE Inflammation and angiogenesis may play a role in the growth of sporadic and neurofibromatosis type 2 (NF2)–related vestibular schwannoma (VS). The similarities in microvascular and inflammatory microenvironment have not been investigated. The authors sought to compare the tumor microenvironment (TME) in sporadic and NF2-related VSs using a combined imaging and tissue analysis approach. METHODS Diffusion MRI and high-temporal-resolution dynamic contrast-enhanced (DCE) MRI data sets were prospectively acquired in 20 NF2-related and 24 size-matched sporadic VSs. Diffusion metrics (mean diffusivity, fractional anisotropy) and DCE-MRI–derived microvascular biomarkers (transfer constant [Ktrans], fractional plasma volume, tissue extravascular-extracellular space [ve], longitudinal relaxation rate, tumoral blood flow) were compared across both VS groups, and regression analysis was used to evaluate the effect of tumor size, pretreatment tumor growth rate, and tumor NF2 status (sporadic vs NF2-related) on each imaging parameter. Tissues from 17 imaged sporadic VSs and a separate cohort of 12 NF2-related VSs were examined with immunohistochemistry markers for vessels (CD31), vessel permeability (fibrinogen), and macrophage density (Iba1). The expression of vascular endothelial growth factor (VEGF) and VEGF receptor 1 was evaluated using immunohistochemistry, Western blotting, and double immunofluorescence. RESULTS Imaging data demonstrated that DCE-MRI–derived microvascular characteristics were similar in sporadic and NF2-related VSs. Ktrans (p < 0.001), ve (p ≤ 0.004), and tumoral free water content (p ≤ 0.003) increased with increasing tumor size and pretreatment tumor growth rate. Regression analysis demonstrated that with the exception of mean diffusivity (p < 0.001), NF2 status had no statistically significant effect on any of the imaging parameters or the observed relationship between the imaging parameters and tumor size (p > 0.05). Tissue analysis confirmed the imaging metrics among resected sporadic VSs and demonstrated that across all VSs studied, there was a close association between vascularity and Iba1+ macrophage density (r = 0.55, p = 0.002). VEGF was expressed by Iba1+ macrophages. CONCLUSIONS The authors present the first in vivo comparative study of microvascular and inflammatory characteristics in sporadic and NF2-related VSs. The imaging and tissue analysis results indicate that inflammation is a key contributor to TME and should be viewed as a therapeutic target in both VS groups.

To assess the compliance with cochlear implantation (CI) in children subsequently diagnosed with autism spectrum disorder (ASD).This was a retrospective case review and survey performed at a tertiary referral centre. Children meeting the criteria for CI who were implanted between 1989 and 2015 and who subsequently received a diagnosis of ASD were included. The primary outcome measure was to assess compliance with CI in children subsequently diagnosed with ASD. Secondary outcome measures included assessment of pre-CI risk factors that may have identified children at higher risk of a subsequent diagnosis of ASD, as well as the benefit obtained by these children following CI.1050 children were implanted between 1989 and 2015. Of these, 22 children were diagnosed with ASD after receiving their CI. The average age at implantation was 2.6 years (median 3, range 1-8 years). The average age for diagnosis of ASD was 5 years, approximately 2 years (median 22 months, range 2-85 months) following CI. Of these, 16/22 (712.7%) regularly use their CI. 6/22 (27.2%) children became non-users of their implant. Some degree of verbal communication was used by 13/22 (59%) of our studied group.There is a range of level of disabilities in ASD, with some relatively minor social communication difficulties through to severe language, cognitive, and behavioural difficulties. Compliance with CI is variable and appears to correlate with the severity of the ASD. Preoperative counselling should include information about the possible impact of later diagnosed disabilities such as ASD on performance.

Aim The aim of the study was to evaluate whether the twinkle artefact is a valuable feature in the sonographic diagnosis of superficial epidermoid cysts. Materials and methods A retrospective search was undertaken of our institution’s Radiology Information System and pathology database to identify cases of superficial masses showing the twinkle artefact that proceeded to surgical excision. Results Eighteen superficial masses demonstrating the twinkle artefact were identified that were submitted for pathological analysis. Of these, 17 were confirmed to represent epidermoid cysts and only 1 case had an alternative diagnosis (proliferating trichilemmal cyst). Conclusion The presence of the twinkle artefact appears to be a specific and valuable ancillary sonographic feature for the diagnosis of superficial epidermoid cysts.

Objective To present a review of all patients diagnosed with a facial nerve schwannoma (FNS) managed in our center over almost two decades, and suggest guidelines for their classification and management. Study design Retrospective case review Setting Tertiary referral center Patients Twenty-eight patients with a facial nerve schwannoma Intervention Conservative or surgical management depending on clinical and radiological features Main outcome measure Patient demographics, site of tumor, and clinical symptoms, including facial nerve function (House-Brackmann score) at baseline and follow-up. In those managed surgically, operative approach and surgical outcomes were also recorded. Results Of 28 patients, 16 were male. Mean age at presentation was 46 years. The majority presented with either facial weakness or hearing loss. The internal auditory canal segment of the facial nerve was the most commonly affected (19/28, 68%). Multi-segmental lesions were found in almost half (46%) of patients. Facial weakness was most commonly associated with involvement of the labyrinthine segment (89%). Overall, 16 (57%) patients were managed surgically. Conclusion FNS may be difficult to distinguish on both clinical and imaging grounds from other cerebellopontine pathologies on the basis of audiovestibular symptoms alone. The presence of facial weakness in combination with imaging findings suggestive of FNS is highly suggestive for FNS. In patients with brainstem compression, rapid tumor growth, or House-Brackmann greater than 4, we suggest a surgical approach based on preoperative audiovestibular status, helping optimize long-term facial function and minimize morbidity. Facial nerve reanimation at the time of primary surgery is preferred.

BACKGROUND: Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour syndrome with a phenotype which includes bilateral vestibular (eighth cranial nerve) schwannomas. Conventional thinking suggests that these tumours originate at a single point along the superior division of the eighth nerve. METHODS: High resolution MRI was performed in children genetically proven to have NF2. The superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) were visualised along their course with points of tumour origin calculated as a percentage relative to the length of the nerve. RESULTS: Out of 41 patients assessed, 7 patients had no identifiable eighth cranial nerve disease. In 16 patients there was complete filling of the internal auditory meatus by a tumour mass such that its specific neural origin could not be determined. In the remaining 18 cases, 86 discrete separate foci of tumour origin on the SVN or IVN could be identified including 23 tumours on the right SVN, 26 tumours on the right IVN, 18 tumours on the left SVN and 19 tumours on the left IVN. DISCUSSION: This study, examining the origins of vestibular schwannomas in NF2, refutes their origin as being from a single site on the transition zone of the superior division of the vestibular nerve. We hypothesise a relationship between the number of tumour foci, tumour biology and aggressiveness of disease. The development of targeted drug therapies in addition to bevacizumab are therefore essential to improve prognosis and quality of life in patients with NF2 given the shortcomings of surgery and radiation treatments when dealing with the multifocality of the disease.

Approximately 2% of congenital profound deafness cases are due to cochlear nerve (CN) deficiency. MRI is essential to confirm if the nerve is deficient, but because of limitations with resolution, especially when the internal auditory canal is narrowed, it is often unable to distinguish between hypoplasia and aplasia. A full audiometric test battery should always be performed, even if the MRI suggests CN aplasia, as there will sometimes be evidence of audition. Electrically evoked auditory brainstem response testing can be carried out transtympanically via the round window or using an intracochlear test electrode to help determine the status of the CN. If any test suggests the presence of a CN, then cochlear implantation (CI) should be considered. Children should be followed up closely with audiometric, electrophysiological and language assessments to determine the benefits. They may initially show benefit but fail to progress. CI results are variable and often result in poor outcomes with Categories of Auditory Perception scores of

Objective:To determine the specificity of the current clinical diagnostic criteria for neurofibromatosis type 2 (NF2) relative to the requirement for unilateral vestibular schwannoma (VS) and at least 2 other NF2-related tumors.Methods:We interrogated our Manchester NF2 database, which contained 205 individuals meeting NF2 criteria who initially presented with a unilateral VS. Of these, 83 (40.7%) went on to develop a contralateral VS. We concentrated our genetic analysis on a group of 70 who initially fulfilled NF2 criteria with a unilateral vestibular schwannoma and at least 2 additional nonintradermal schwannomas.Results:Overall, 5/70 (7%) individuals with unilateral VS and at least 2 other schwannomas had a pathogenic or likely pathogenic LZTR1 mutation. Twenty of the 70 subsequently developed bilateral disease. Of the remaining 50, 5 (10%) had a germline LZTR1 mutation, equivalent to the number (n = 5) with a germline NF2 mutation.Conclusions:The most common etiology for unilateral VS and 2 additional NF2-associated tumors in this cohort was mosaic NF2. Germline LZTR1 and germline NF2 mutations were equally common in our cohort. This indicates that LZTR1 must be considered when making a diagnosis of NF2 in the presence of unilateral VS in individuals without a germline NF2 mutation.

Transverse testicular ectopia is a rare congenital anomaly in which both testes migrate along the same inguinal canal toward the scrotum. This report describes the case of the rarest form of transverse testicular ectopia in a 23-year-old man with coexisting testicular microlithiasis in one of the two testes as diagnosed on sonography and confirmed on MRI. We are unaware of any previous published reports of this association. Although the etiology of both conditions is debatable, each is believed to be associated with an increased risk of testicular malignancy, and this poses a dilemma for the future management of this patient.

The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

The increasing detection of small testicular lesions by ultrasound (US) in adults can lead to unnecessary orchiectomies. This article describes their nature, reviews the available literature on this subject and illustrates some classical lesions. We also suggest recommendations to help characterization and management. The ESUR scrotal imaging subcommittee searched for original and review articles published before May 2015 using the Pubmed and Medline databases. Key words used were ‘testicular ultrasound’, ‘contrast-enhanced sonography’, ‘sonoelastography’, ‘magnetic resonance imaging’, ‘testis-sparing surgery’, ‘testis imaging’, ‘Leydig cell tumour’, ‘testicular cyst’. Consensus was obtained amongst the members of the subcommittee, urologist and medical oncologist. Simple cysts are frequent and benign, and do not require follow up or surgery. Incidentally discovered small solid testicular lesions detected are benign in up to 80 %, with Leydig cell tumours being the most frequent. However, the presence of microliths, macrocalcifications and hypoechoic areas surrounding the nodule are findings suggestive of malignant disease. Asymptomatic small testicular lesions found on ultrasound are mainly benign, but findings such as microliths or hypoechoic regions surrounding the nodules may indicate malignancy. Colour Doppler US remains the basic examination for characterization. The role of newer imaging modalities in characterization is evolving. • Characterization of testicular lesions is primarily based on US examination. • The role of MRI, sonoelastography, contrast-enhanced ultrasound is evolving. • Most small non-palpable testicular lesions seen on ultrasound are benign simple cysts. • Leydig cell tumours are the most frequent benign lesions. • Associated findings like microliths or hypoechoic regions may indicate malignancy.

Pooled data from 16 radiology centers were retrospectively analyzed to seek patients with pathologically proven testicular lymphoma and grayscale and color Doppler images available for review. Forty-three cases were found: 36 (84%) primary and 7 (16%) secondary testicular lymphoma. With unilateral primary lymphoma, involvement was unifocal (n = 10), multifocal (n = 11), or diffuse (n = 11). Synchronous bilateral involvement occurred in 6 patients. Color Doppler sonography showed normal testicular vessels within the tumor in 31 of 43 lymphomas (72%). Testicular lymphoma infiltrates through the tubules, preserving the normal vascular architecture of the testis. Depiction of normal testicular vessels crossing the lesion is a useful adjunctive diagnostic criterion.

The subcommittee on scrotal imaging, appointed by the board of the European Society of Urogenital Radiology (ESUR), have produced guidelines on imaging and follow-up in testicular microlithiasis (TML). The authors and a superintendent university librarian independently performed a computer-assisted literature search of medical databases: MEDLINE and EMBASE. A further parallel literature search was made for the genetic conditions Klinefelter’s syndrome and McCune-Albright syndrome. Proposed guidelines are: follow-up is not advised in patients with isolated TML in the absence of risk factors (see Key Points below); annual ultrasound (US) is advised for patients with risk factors, up to the age of 55; if TML is found with a testicular mass, urgent referral to a specialist centre is advised. Consensus opinion of the scrotal subcommittee of the ESUR is that the presence of TML alone in the absence of other risk factors is not an indication for regular scrotal US, further US screening or biopsy. US is recommended in the follow-up of patients at risk, where risk factors other than microlithiasis are present. Risk factors are discussed and the literature and recommended guidelines are presented in this article. • Follow up advised only in patients with TML and additional risk factors. • Annual US advised for patients with risk factors up to age 55. • If TML is found with testicular mass, urgent specialist referral advised. • Risk factors – personal/ family history of GCT, maldescent, orchidopexy, testicular atrophy.

Background The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. Objective To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. Methods The prospective database consists of 1253 patients with NF2. One thousand and nine are known to be alive at last follow-up. The presence and laterality/pathology of vestibular schwannoma at diagnosis and last follow-up was sought. Results There were no cases of spontaneous MPNST with 2114 proven (n = 1150) and presumed benign (n = 964) vestibular schwannomas found. Two patients had developed MPNST (1 presumed) after having previously undergone stereotactic radiosurgery for a vestibular schwannoma. Conclusion In this series, and from the literature, malignant transformation of a vestibular schwannoma was not a feature of NF2 in the unirradiated patient. NF2 patients should not be told that they have an increased risk of malignant change in a vestibular schwannoma unless they undergo radiation treatment. However, very much larger datasets are required before it can be determined whether there is any association between NF2 and MPNST in the unirradiated patient.

OBJECTIVE: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2).STUDY DESIGN: Retrospective case review.SETTING: Tertiary referral centre.PATIENTS: Implanted with a Cochlear ABI22 or ABI24M between 1994 and 2009 because of NF2 disease.INTERVENTION(S): Rehabilitative.MAIN OUTCOME MEASURE(S): Surgical complication rate; audiological outcomes.RESULTS: There were 50 primary ABI insertions in 49 patients, including 16 inserted at the time of first side tumor removal as a sleeper, and two revision repositionings which failed to improve outcome. Postoperatively three patients had cerebrospinal fluid leaks which did not require reoperation, one patient had meningitis, and eleven patients suffered either temporary or permanent lower cranial nerve dysfunction. Twenty-nine patients became full time users; a further 12 patients became non-users. Three patients died while their device was inactive. Five patients retain serviceable contralateral hearing. Audiological open set testing of users showed means of: environmental sounds discrimination 51%; phoneme discrimination: with ABI alone 22%/lip reading (LR) 45%/ABI with LR 65%; sentence testing: with ABI alone 13%/LR 19%/ABI with LR 54%.CONCLUSIONS: The majority of patients with NF2 implanted with an ABI find the device a useful aid to communication in conjunction with LR and in recognizing common environmental sounds. A small proportion gain open set discrimination. Almost a third of patients may end up as non-users. There is probably an increased risk of postoperative lower cranial nerve dysfunction so careful preoperative assessment is advised.

Prostate cancer is the most common malignancy in men, in general. Most patients diagnosed with prostate cancer have localized disease confined to the prostate. A small percentage of patients with aggressive tumors will progress to develop local, extracapsular tumor extension and distant metastases. The aim of prostate cancer management is to identify and treat those patients with aggressive disease before they develop locally advanced or metastatic disease, and to avoid overtreating indolent tumors, which are unlikely to be life threatening. Imaging has been shown to be valuable in local staging of prostate cancer and as an aid to the management of clinically significant disease. In this article, we discuss the different established imaging modalities and emerging techniques for prostate cancer imaging in patients with clinically localized disease who may be suitable for radical treatment.

The pathological and radiological hallmarks of multiple sclerosis (MS) include multiple demyelinated lesions disseminated throughout the white matter of the central nervous system (CNS). More recently, the cerebral cortex has been shown to be affected in MS, but the elucidation of events causing cortical demyelination has been hampered by the lack of animal models reflecting such human cortical pathology. In this report, we have described the presence of cortical gray matter and callosal white matter demyelinating lesions in the chronic experimental autoimmune encephalomyelitis (EAE) mouse model of MS. Similar to the pathological lesions of MS patients, EAE lesions have been classified as type I-leukocortical, type II-intracortical and type III-subpial. All of these lesions had varying degrees of demyelination, inflammatory cells and reactive astrocytes. Similar to MS, cortical layers during EAE showed demyelination, microglia activation, synaptic protein alterations and apoptotic cells. In addition, the callosal white matter during EAE had many inflammatory demyelinating lesions and axon degeneration. Functional electrophysiological conduction analysis showed deficits in both myelinated and unmyelinated callosal axons during early and late EAE. The chronic EAE mouse model has features that mimic cortical and callosal pathology of MS, and can be potentially used to screen agents to prevent these features of disease.

Received March 2, 2010, from the Department of Ultrasound, Derriford Hospital, Plymouth, England. Revision requested April 2, 2010. Revised manuscript accepted for publication April 26, 2010. Address correspondence to Irving T. Wells, MBBS, BSc, MRCP, Peninsula Radiology Academy, Plymouth International Business Park, Plymouth, Devon PL6 5WR, England. E-mail: tobywells@doctors.net.uk Abbreviations CT, computed tomography e present a case in which contrastenhanced sonography was used to differentiate a benign splenic lesion from a possible isolated splenic metastasis in a patient with known malignancy. A 57-year-old patient with prostate cancer was referred for imaging before resection. His prostatic tumor was thought to be locally confined on magnetic resonance imaging, but staging computed tomography (CT) showed a focal splenic mass (Figure 1) without evidence of metastatic disease elsewhere. A sonographic examination with a sulfur hexafluoride microbubble contrast agent (SonoVue, Bracco SpA, Milan, Italy) was performed for further evaluation of the splenic lesion. Gray scale sonography showed a 7-cm well-defined slightly hyperechoic mass containing a few small cystic areas occupying most of the spleen (Figure 2A). Power Doppler analysis showed predominantly peripheral blood flow but also some central flow (Figure 2B). On contrast-enhanced sonography, the lesion showed enhancement in the arterial (Figure 2C) and portal venous (Figure 2D) phases and retained contrast on delayed images (Figure 2E). Its enhancement profile was similar to that of adjacent splenic parenchyma. The diagnoses based on the sonographic appearances were of a giant splenic hemangioma or hamartoma. A radiolabeled red cell scan was performed, which showed homogeneous uptake within the spleen, further supporting the diagnosis of a nonmalignant lesion.

A review of sporadic and NF2-related vestibular schwannoma surgery in children (under 18 years of age) with a specific interest in resection rates, recurrence, facial nerve outcomes, hearing preservation, hearing rehabilitation and genetic analysis.A retrospective analysis of prospectively collected data of 35 consecutively operated vestibular schwannomas in 29 paediatric patients that underwent 38 operations between 1992 and 2007. Pre- and post-operative radiology, facial nerve function, pure tone audiogram and speech discrimination tests were performed with a mean follow-up of 4.5 years. Tumour and blood mutations were analysed in 86% of patients.Total resection was achieved in all sporadic cases and 68% of NF2 cases. Near total resection led to tumour recurrence in 5 out of 10 cases. The facial nerve was anatomically preserved in 92%. Facial nerve function was excellent to good (Grades 1-3) in 88% with outcome related to tumour size. Hearing preservation was successful in 3 of 11 cases.Surgery with complete resection results in excellent tumour control, but it is more difficult to attain total resection in NF2 with a relatively high recurrence rate of persistently growing tumours. A better facial outcome is associated with smaller tumours, near-total resection and first time surgery. Hearing preservation is possible in a minority. Hearing rehabilitation can be successful by utilising cochlear implants and auditory brain stem implants (ABI) as appropriate. Overall there is a low complication rate and results are comparable with adult series.

Abnormalities in the spleen are less common than in most other abdominal organs. However, they will be regularly encountered by ultrasound practitioners, who carefully evaluate the spleen in their abdominal ultrasound studies. Conventional grey scale and Doppler ultrasound are frequently unable to characterise focal splenic abnormalities; even when clinical and laboratory information is added to the ultrasound findings, it is often not possible to make a definite diagnosis. Contrast-enhanced ultrasound (CEUS) is easy to perform, inexpensive, safe and will usually provide valuable additional information about splenic abnormalities, allowing a definitive or short differential diagnosis to be made. It also identifies those lesions that may require further imaging or biopsy, from those that can be safely dismissed or followed with interval ultrasound imaging. CEUS is also indicated in confirming the nature of suspected accessory splenic tissue and in selected patients with abdominal trauma. This article describes the CEUS examination technique, summarises the indications for CEUS and provides guidance on interpretation of the CEUS findings in splenic ultrasound.

Renal artery stenosis (RAS) is an important and potentially reversible cause of both hypertension and renal failure. Ultrasound offers an attractive, non-invasive, method to examine the renal arteries and confirm or exclude the presence of RAS. This article will describe the techniques and diagnostic criteria employed in the ultrasound diagnosis of this condition and will also evaluate the usefulness of ultrasound in comparison with other non-invasive imaging techniques.

Cochlear implantation is the principal treatment for restoring hearing and speech understanding in individuals with sensorineural hearing loss who gain limited benefit from hearing aids. Cochlear i...

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.

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

Objective To review the postoperative surgical outcomes of cystic vestibular schwannomas (CVSs), especially facial nerve outcomes, and compare these results with those from matched solid vestibular schwannomas (SVS) resected during the same period at a tertiary referral center. Study design Retrospective case series. Methods One hundred thirty-one surgically managed patients with cystic vestibular schwannomas (CVSs) were age, sex, and tumor size matched to 131 surgically managed patients with solid vestibular schwannomas (SVSs). Demographics, tumor morphology, surgical approach, extent of resection, facial and nonfacial complications, and recurrence rates were compared between the 2 groups. Subtotal removal was defined as removal of at least 95% of the tumor. Results The mean maximal tumor diameter was 2.8 cm for both groups. For CVS, gross total tumor resection (GTR) was achieved in 92 patients (70.2%), and subtotal tumor resection (STR) was achieved in 39 patients (29.8%). Postoperative facial nerve outcomes at 1-year follow-up were good (HB Grade I-III) in 116 (88.5%) of 131 CVS patients. Twenty-three patients developed nonfacial nerve-related complications (17.6%). For SVS, GTR was achieved in 102 patients (77.9%), and STR was achieved in 29 patients (22.1%). Postoperative facial nerve outcomes at 1-year follow-up were good (HB Grade I-III) in 118 (90.1%) of 131 SVS patients. Nonfacial nerve related complications occurred in 14 patients (10.7%). None of the differences in outcome between the 2 groups were statistically significant. Conclusion The difference in surgical outcomes is minimal between patients with CVS and those with SVS, not reaching statistical significance. We think, with judicious surgical management, similar outcomes can be achieved in cystic tumors and solid tumors.

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.

OBJECTIVE To date, only a very limited number of lipomas of the cerebellopontine angle (CPA) have been reported. Our objective was to examine clinical and radiologic features of CPA lipomas and determine the most appropriate management plan. STUDY DESIGN Retrospective case series. SETTING Tertiary referral center. PATIENTS Patients with CPA lipomas were identified through the skull base multidisciplinary meeting database. INTERVENTIONS Radiologic surveillance and clinical assessment. MAIN OUTCOME MEASURES Tumor growth, assessed through radiologic measurements on serial magnetic resonance imaging, demographics, presenting symptoms, and any correlation between weight gain and lipoma growth were among the examined factors. RESULTS Of the 15 patients with CPA lipomas, six were female and nine were male, with an average age at presentation of 50.2 years (range, 31.7-76.4 yr) and an average follow-up time of 51.7 months (range, 6-216 mo). The lipomas were unilateral in all cases, nine on the right (60%) and six on the left (40%) side. None of the lipomas increased in size. All patients were treated conservatively. Sensorineural hearing loss was the main presenting symptom (80%) followed by tinnitus (46.7%) and vertigo (20%). None of the patients suffered from facial nerve dysfunction. There was no correlation between weight gain and tumor growth. CONCLUSION CPA lipomas can be diagnosed accurately with appropriate magnetic resonance imaging techniques and be managed conservatively with safety. Cochleovestibular are the most common presenting symptoms, whereas facial nerve involvement is rare. CPA lipomas do not tend to grow and can be monitored on a less regular basis.

The assessment process is critical in deciding whether a profoundly deaf child with cochlear nerve deficiency (CND) will be suitable for a cochlear or auditory brainstem implant (ABI). Magnetic resonance imaging (MRI) using submillimetric T2 weighted gradient echo or turbo spin echo sequences is mandatory for all profoundly deaf children to diagnose CND. Evidence of audition on behavioural or electrophysiological tests following both auditory and electrical stimulation sometimes allows identification of significant auditory tissue not visible on MRI. In particular electric auditory brainstem response (EABR) testing may allow some quantification of auditory tissue and help decide whether a cochlear implant will be beneficial. Age and cognitive development are the most critical factors in determining ABI benefit. Hearing outcomes from both cochlear implants and ABIs are variable and likely to be limited in children with CND. A proportion of children will get no benefit. Usually the implants would be expected to provide recognition of environmental sounds and understanding of simple phonetics. Most children will not develop normal speech and they will often need to learn to communicate with sign language. The ABI involves a major neurosurgical procedure and at present the long term outcomes are unknown. It is therefore essential that parents who are considering this intervention have plenty of time to consider all aspects and the opportunity for in depth discussion.

Purpose. To determine the frequency of use of the term “superficial femoral vein” (SFV) in the radiologic reports from a sample of sonographic investigations for suspected deep vein thrombosis and to assess the potential for clinical error in their interpretation. Methods. Retrospective review of 425 consecutive reports from medical patients attending the Imaging Department over a 6-month period for the presence of the term “superficial femoral vein” and for the presence of thrombus. A questionnaire was sent to a sample of referring clinicians to assess their understanding of the anatomy of the deep venous system of the leg and indications for anticoagulant treatment. Results. Of the 425 sonographic investigations reviewed, 90 (21.2%) used the term “superficial femoral vein,” and 12 (13.3%) were positive for SFV thrombus. Among 87 clinicians, 74.7% believed the SFV to be part of the superficial venous system and that its thrombosis did not require anticoagulant treatment, although anticoagulation is now indicated in selected cases of superficial venous thrombosis. Conclusion. Seventy-five percent of clinicians do not recognize the SFV as being part of the deep venous system and that its thrombosis requires anticoagulant treatment. In this study, 13% of SFV examined were positive for thrombus, and four patients (4.4%) had an isolated SFV thrombus that could have been left untreated due to this misunderstanding. Use of the term “superficial femoral vein” is prone to misinterpretation by clinicians and potentially hazardous to patients. It should be replaced by “common femoral vein” and “femoral vein” in reports. © Wiley Periodicals, Inc. J Clin Ultrasound, 2011.

OBJECTIVE: To investigate the outcomes from ipsilateral simultaneous or sequential cochlear implantation in patients with neurofibromatosis type 2 (NF2) after vestibular schwannoma removal with cochlear nerve preservation. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary referral NF2 center. PATIENTS: Six patients with NF2. INTERVENTION: Removal of vestibular schwannoma (VS) with preservation of the cochlear nerve and cochlear implantation. Four patients had their surgery via a translabyrinthine approach. Two patients had a retrosigmoid approach. A cochlear implant was inserted at the same time as tumor removal in 4 cases and sequentially in 2 cases. MAIN OUTCOME MEASURES: Surgical and audiometric outcomes using Bamford-Kowal-Bench (BKB) and City of New York University (CUNY) sentence scores. RESULTS: The average age at implantation was 24 years (range, 15-36 yr). Follow-up ranged from 5 to 93 months, with an average of 38 months. All patients had useful hearing in the contralateral ear before surgery. One patient gained no benefit from cochlear implantation and proceeded to have an auditory brainstem implant. Of those that had functional cochlear nerves, the average BKB score in quiet was 64%, BKB score in noise was 42%, and CUNY score with lipreading was 97%. Results varied within the group, but all patients gained significant benefit and continue to use their CI at least intermittantly. CONCLUSION: The present series demonstrates that in selected cases, cochlear implantation can be successful after a translabyrinthine approach for VS removal and for restoring hearing after failed retrosigmoid hearing preservation surgery. All patients found the cochlear implant offered useful hearing even in the presence of contralateral hearing.

OBJECTIVE: Hearing loss resulting from bilateral vestibular schwannomas (VSs) has a significant effect on the quality of life of patients with neurofibromatosis Type 2 (NF2). A national consensus protocol was produced in England as a guide for cochlear implantation (CI) and auditory brainstem implantation (ABI) in these patients. STUDY DESIGN: Consensus statement. SETTING: English NF2 Service. PARTICIPANTS: Clinicians from all 4 lead NF2 units in England. MAIN OUTCOME MEASURES: A protocol for the assessment, insertion and rehabilitation of CI and ABI in NF2 patients. RESULTS: Patients should undergo more detailed hearing assessment once their maximum aided speech discrimination score falls below 50% in the better hearing ear. Bamford-Kowal-Bench sentence testing scores below 50% should trigger assessment for auditory implantation, as recommended by the National Institute for Clinical Excellence guidelines on CI. Where this occurs in patients with bilateral stable VS or a unilateral stable VS where the contralateral cochlear nerve was lost at previous surgery, CI should be considered. Where VS surgery is planned, CI should be considered where cochlear nerve preservation is thought possible, otherwise an ABI should be considered. Intraoperative testing using electrically evoked auditory brainstem responses or cochlear nerve action potentials may be used to determine whether a CI or ABI is inserted. CONCLUSION: The NF2 centers in England agreed on this protocol. Multisite, prospective assessments of standardized protocols for auditory implantation in NF2 provide an essential model for evaluating candidacy and outcomes in this challenging patient population.

The legal environment is one factor that influences injection drug users’ (IDUs) risk for HIV and other bloodborne pathogens such as hepatitis C virus (HCV). We examined the association between law enforcement encounters (i.e., arrests and citations) and receptive syringe sharing among IDUs in the context of an intensified policing effort. We conducted a mixed methods analysis of 30 qualitative and 187 quantitative interviews with IDUs accessing services at a Los Angeles, CA syringe exchange program from 2008 to 2009. Qualitative findings illustrate concerns related to visibility, drug withdrawal, and previous history of arrest/incarceration. In quantitative analysis, the number of citations received, current homelessness, and perceiving that being arrested would be a “big problem” were independently associated with recent syringe sharing. Findings illustrate some of the unintended public health consequences associated with intensified street-level policing, including risk for HIV and HCV transmission.

To analyse the long-term outcome of translabyrinthine surgery for vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2). RESEARCH TYPE: Retrospective cohort study.Two tertiary referral NF2 units.One hundred and forty eight translabyrinthine operations for patients with VS were performed. Preoperative stereotactic radiotherapy had been performed on 12(9.4%) patients.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.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.