Citations

ObjectivesPrimary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL.Study DesignCase series and chart review.SettingTertiary referral center.Subjects and MethodsReview of 48 patients with PSBL treated between 1994 and 2016.ResultsThe median age at diagnosis was 60 years. Diffuse large B‐cell lymphoma comprised the most common subtype (56%). Presenting symptoms included diplopia (52%), trigeminal hypesthesia (38%), headache (29%), facial nerve weakness (25%), B‐symptoms (fevers, night sweats, and/or weight loss) (25%), and hearing loss (21%). Discrete lesions commonly mimicked meningioma, schwannoma, and nasopharyngeal carcinoma. Diffuse lesions imitated neurosarcoidosis, granulomatosis with polyangiitis, and carcinomatosis meningitis. Only 26% of all initial clinical evaluations suspected lymphoma. The combination of restricted diffusion on diffusion‐weighted imaging, an absence of flow voids or surrounding hyperostosis, bony erosion and/or marrow signal replacement, and heterogeneous contrast enhancement facilitated delineation between PSBL and common skull base pathology. Cerebrospinal fluid (CSF) analysis was diagnostic of lymphoma in 24% of cases. Lymphoma within CSF portended significantly worse overall survival (85% vs 18% at 3‐years; P <. 001).ConclusionA history significant for multiple cranial nerve palsies, B‐symptoms, and imaging findings inconsistent with common skull base pathology should raise suspicion for PSBL. CSF analysis in the setting of PSBL can provide diagnostic and prognostic value for patients.

Objective(1) Assess 3‐dimensional volumetric growth of untreated sporadic vestibular schwannomas (VSs) in a large cohort of patients treated with conservative observation. (2) Compare volumetric and conventional linear diameter measurements for detecting tumor growth.Study DesignCase series with chart review.SettingTertiary skull base referral center.Subjects and MethodsPatients with sporadic VS who elected initial conservative treatment with at least 2 serial magnetic resonance imaging (MRI) scans were included. Tumor volume was determined with 3‐dimensional segmentation of MRI sequences. The volumetric threshold for tumor growth was an increase ≥20% from baseline tumor volume. Tumor size based on linear diameter was assessed with the 1995 American Academy of Otolaryngology—Head and Neck Surgery Foundation guidelines for VS outcome reporting, with growth defined as an increase ≥2 mm.ResultsA total of 361 patients were included with a median radiologic follow‐up of 4.1 years (interquartile range [IQR], 2.5‐6.8). At diagnosis, 232 VSs (64%) were purely intracanalicular, and 129 (36%) extended into the cerebellopontine angle. The median baseline tumor volume was 0.161 cm3 (IQR, 0.054‐0.418). Overall, 69% of tumors demonstrated volumetric growth at a median of 1.1 years (IQR, 0.6‐2.1) after initial MRI. In contrast, based on linear measurement assessment, 48% of tumors demonstrated growth at a median of 1.8 years (IQR, 0.8‐3.1) from first MRI scan. Disequilibrium, facial hypoesthesia, aural fullness, initial tumor size, and nonincidental diagnosis were associated with tumor growth.ConclusionThree‐dimensional volumetric assessment of VS provides a more sensitive measure of tumor growth when compared with linear diameter assessment. Through volumetric analysis, the current study revealed that a significant proportion of VSs demonstrate growth during observation.

Introduction. Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. Methods. Case report and review of the English literature. Results. A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear. Audiometric testing confirmed profound SNHL in the left ear, while the right ear exhibited moderate-to-severe SNHL. Left-sided cochlear implantation was performed using a conventional length lateral wall electrode. Intraoperative device testing found normal impedance levels throughout the array; however, electrically evoked auditory potentials were absent on all electrodes. Upon initial activation 3 weeks after surgery, the patient reported excellent access to sound in the cochlear implant-only condition. He has made good progress at each subsequent visit; speech perception testing after seven months showed improvement from 0% to 32% on AzBio sentence and 53% on CNC phoneme testing in the cochlear implant-only condition. Conclusion. We report the third case of cochlear implantation in a patient with CMT. SNHL in CMT is hypothesized to result from disruption of synchronous activity of the cochlear nerve. In patients with CMT, cochlear implantation may reconstitute synchronous neural activity by way of supraphysiological electrical stimulation. Our results corroborate two earlier reports that cochlear implantation is a viable option for rehabilitation of SNHL in this unique subset of patients.

Abstract BACKGROUND Brain cavernous angiomas with symptomatic hemorrhage (CASH) are uncommon but exact a heavy burden of neurological disability from recurrent bleeding, for which there is no proven therapy. Candidate drugs to stabilize the CASH lesion and prevent rebleeding will ultimately require testing of safety and efficacy in multisite clinical trials. Much progress has been made in understanding the epidemiology of CASH, and novel biomarkers have been linked to the biological mechanisms and clinical activity in lesions. Yet, the ability to enroll and risk-stratify CASH subjects has never been assessed prospectively at multiple sites. Biomarkers and other outcomes have not been evaluated for their sensitivity and reliability, nor have they been harmonized across sites. OBJECTIVE To address knowledge gaps and establish a research network as infrastructure for future clinical trials, through the Trial Readiness grant mechanism, funded by National Institute of Neurological Disorders and Stroke/National Institutes of Health. METHODS This project includes an observational cohort study to assess (1) the feasibility of screening, enrollment rates, baseline disease categorization, and follow-up of CASH using common data elements at multiple sites, (2) the reliability of imaging biomarkers including quantitative susceptibility mapping and permeability measures that have been shown to correlate with lesion activity, and (3) the rates of recurrent hemorrhage and change in functional status and biomarker measurements during prospective follow-up. EXPECTED OUTCOMES We propose a harmonized multisite assessment of enrollment rates of CASH, baseline features relevant to stratification in clinical trials, and follow-up assessments of functional outcomes in relation to clinical bleeds. We introduce novel biomarkers of vascular leak and hemorrhage, with firm mechanistic foundations, which have been linked to clinical disease activity. We shall test their reliability and validity at multiple sites, and assess their changes over time, with and without clinical rebleeds, hence their fitness as outcome instruments in clinical trials. DISCUSSION The timing cannot be more opportune, with therapeutic targets identified, exceptional collaboration among researchers and the patient community, along with several drugs ready to benefit from development of a path to clinical testing using this network in the next 5 years.

Background: Gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) (T1WI) is the conventional imaging technique of choice to detect vestibular schwannoma (VS) recurrence or regrowth, despite suboptimal specificity secondary to enhancing postoperative changes. Furthermore, recent concerns regarding the accumulation of gadolinium in body tissues have led for a call to reduce the number of contrast-enhanced examinations. The objective of the current study is to evaluate the diagnostic accuracy of high-resolution noncontrast (three-dimensional [3D] T2) MRI relative to gadolinium-enhanced T1WI in the detection of VS recurrence after resection. Methods: Following Institutional Review Board approval, 13 consecutive postoperative patients with VS recurrence or regrowth were identified from a prospectively maintained clinical database in which recurrence was determined by progression on serial postoperative MRI examination. Three blinded neuroradiologists retrospectively evaluated a total of 41 postoperative MRI examinations from these patients using only gadolinium-enhanced T1WI and 3D T2 MRI for recurrence or regrowth. Interobserver agreement, differences in detection between the two sequences, and the sensitivity and specificity of 3D T2 MRI were assessed. Results: Fifteen of the 41 postoperative MRIs demonstrated progression, as determined by examiner consensus on the gadolinium-enhanced T1WI. Agreement, measured using Krippendorff's alpha, was 0.82 for the 3D T2 images and 0.83 for the contrast-enhanced T1WI. All the three examiners demonstrated no difference in the detection of progression between the two sequences (McNemar's test p values 0.69 for examiner 1, 0.63 for examiner 2, and 0.99 for examiner 3). The sensitivity of 3D T2 was 0.78 (CI 0.60–0.96), while the specificity was 0.94 (CI 0.86–1.00). Conclusion: Noncontrast high-resolution 3D T2 MRI seems sufficient to assess for recurrence or regrowth after VS resection. The results of this study have implications for reducing cost, time, and adverse events associated with gadolinium administration in this population requiring serial follow-up examinations. These promising, yet preliminary findings warrant confirmation with a larger prospective cohort of patients.

Objective: To report the presentation, diagnosis, management, and convalescence of labyrinthine sequestrum (LS) and summarize all previously published cases. Patient(s): Eleven-year-old female with LS. Intervention(s): Multidisciplinary diagnostic evaluation and treatment. Main Outcome Measures: Imaging and laboratory findings, medical and surgical treatment. Results: We describe a case of LS secondary to medically recalcitrant suppurative otitis media in an 11-year-old female and review all eight previously reported cases. The index patient presented after 6 months of otitis media, profound unilateral hearing loss, with symptoms suggesting meningitis. Temporal bone CT demonstrated marked bony destruction of the left otic capsule. Gadolinium-enhanced MRI showed an enhancing process with evidence of meningitis and subdural empyema. The patient was treated with surgical debridement and culture directed antibiotic therapy. Posttreatment imaging showed resolution of intracranial infection with fibrous bony healing of the otic capsule resembling fibrous dysplasia. Conclusion: LS is a rare form of labyrinthitis characterized by centrifugal destruction of the otic capsule. The current index case highlights the importance of combined medical and surgical treatment and describes for the first time in the literature the fibrous ossification of the otic capsule following disease resolution.

Background: Hamstring autograft size <8 mm has been shown to be a predictor for failure after anterior cruciate ligament (ACL) reconstruction. The ability to predict graft size preoperatively is helpful in counseling patients about the possible need for graft augmentation. Purpose: To determine whether preoperative ultrasound (US) measurements of hamstring tendons can predict intraoperative graft diameter during ACL reconstruction. Study Design: Cohort study (diagnosis); Level of evidence, 2. Methods: Twenty patients undergoing unilateral isolated ACL reconstruction were prospectively enrolled in the study (10 males, 10 females; mean ± SD age, 22.8 ± 6.6 years; height, 175.1 ± 7.1 cm; weight, 81.4 ± 14.2 kg; body mass index, 26.5 ± 4.1 kg/m 2 ). Hamstrings were assessed by US, and double-looped semitendinosus-gracilis hamstring size was independently calculated with a freehand selection method on a nonmagnified US image by 2 orthopaedic surgeons. Intraoperative autograft size was determined with a standard graft-sizing tool. Intra- and interrater reliability was measured with intraclass correlation coefficients (ICCs) and standard error of the measure (SEM). A receiver operating characteristic curve was calculated to assess the ability of the US measurement to predict intraoperative measurements. Results: The mean autograft diameter by US was 8.9 ± 0.98 mm, while the mean intraoperative hamstring graft size was 8.1 ± 0.89 mm. There was excellent intrarater (ICC 2,1 = 0.95, SEM = 0.32 mm) and interrater (ICC 2,1 = 0.88, SEM = 0.55 mm) reliability for US measurements. Receiver operating characteristic analysis showed that US did not consistently quantify graft size. Graft size did not significantly correlate with height, weight, or body mass index in our sample ( P > .05). Conclusion: These results suggest that preoperative US imaging of the hamstring tendons is unreliable in predicting intraoperative graft diameter.