Use of a dedicated open transcarotid access system for neurovascular disease.

BACKGROUND: Transcarotid artery revascularization (TCAR) is an increasingly popular technique for the management of extracranial carotid stenosis. Its off-label use in the treatment of intracranial neurovascular disease is poorly described. Our objective is to describe the use of a dedicated open transcarotid access system for the treatment of neurovascular pathologies other than extracranial carotid stenosis. METHODS: We conducted a retrospective review of a prospectively maintained database of consecutive patients who underwent treatment of neurovascular disease at a single academic center using the ENROUTE Transcarotid Arterial Sheath. Demographics, procedural characteristics, and patient outcomes were reported. RESULTS: Twenty patients were included in the study between September 2017 and March 2023. The following pathologies were treated: intracranial atherosclerotic disease (ICAD, nine patients), complex cervico-petrous carotid disease (five patients), intracranial aneurysms (three patients), and large vessel occlusion-acute ischemic stroke (three patients). Eighteen of the 20 cases were performed with active carotid flow reversal. All cases were successfully completed. There were no access-related complications. One periprocedural complication was incurred: a microguidewire perforation during an exchange maneuver for the treatment of ICAD. CONCLUSION: An open transcarotid approach using a dedicated transcarotid system may offer a safe alternative access strategy for the endovascular treatment of complex neurovascular pathologies when a traditional transfemoral or transradial approach is contraindicated or failed.

TLR agonists polarize interferon responses in conjunction with dendritic cell vaccination in malignant glioma: a randomized phase II Trial.

In this randomized phase II clinical trial, we evaluated the effectiveness of adding the TLR agonists, poly-ICLC or resiquimod, to autologous tumor lysate-pulsed dendritic cell (ATL-DC) vaccination in patients with newly-diagnosed or recurrent WHO Grade III-IV malignant gliomas. The primary endpoints were to assess the most effective combination of vaccine and adjuvant in order to enhance the immune potency, along with safety. The combination of ATL-DC vaccination and TLR agonist was safe and found to enhance systemic immune responses, as indicated by increased interferon gene expression and changes in immune cell activation. Specifically, PD-1 expression increases on CD4+ T-cells, while CD38 and CD39 expression are reduced on CD8+ T cells, alongside an increase in monocytes. Poly-ICLC treatment amplifies the induction of interferon-induced genes in monocytes and T lymphocytes. Patients that exhibit higher interferon response gene expression demonstrate prolonged survival and delayed disease progression. These findings suggest that combining ATL-DC with poly-ICLC can induce a polarized interferon response in circulating monocytes and CD8+ T cells, which may represent an important blood biomarker for immunotherapy in this patient population.Trial Registration: ClinicalTrials.gov Identifier: NCT01204684.

Angiographic evidence of an inadvertent cannulation of the marginal sinus following central line migration: illustrative case.

BACKGROUND: Central venous catheters (CVCs) play an indispensable role in clinical practice. Catheter malposition and tip migration can lead to severe complications. The authors present a case illustrating the endovascular management of inadvertent marginal sinus cannulation after an internal jugular vein (IJV) catheter tip migration. OBSERVATIONS: A triple-lumen CVC was inserted without complications into the right IJV of a patient undergoing a repeat sternotomy for aortic valve replacement. Two weeks postinsertion, it was discovered that the tip had migrated superiorly, terminating below the torcula in the posterior fossa. In the interventional suite, a three-dimensional venogram confirmed the inadvertent marginal sinus cannulation. The catheter was carefully retracted to the sigmoid sinus to preserve the option of catheter exchange if embolization became necessary. After a subsequent venogram, which displayed an absence of contrast extravasation, the entire catheter was safely removed. The patient tolerated the procedure well. LESSONS: Clinicians must be vigilant of catheter tip migration and malposition risks. Relying solely on postinsertion radiographs is insufficient. Once identified, prompt management of the malpositioned catheter is paramount in reducing morbidity and mortality and improving patient outcomes. Removing a malpositioned catheter constitutes a critical step, best performed by a specialized team under angiographic visualization.

Dendritic Cell Vaccination in Conjunction with a TLR Agonist Polarizes Interferon Immune Responses in Malignant Glioma Patients.

Autologous tumor lysate-pulsed dendritic cell (ATL-DC) vaccination is a promising immunotherapy for patients with high grade gliomas, but responses have not been demonstrated in all patients. To determine the most effective combination of autologous tumor lysate-pulsed DC vaccination, with or without the adjuvant toll-like receptor (TLR) agonists poly-ICLC or resiquimod, we conducted a Phase 2 clinical trial in 23 patients with newly diagnosed or recurrent WHO Grade III-IV malignant gliomas. We then performed deep, high-dimensional immune profiling of these patients to better understand how TLR agonists may influence the systemic immune responses induced by ATL-DC vaccination. Bulk RNAseq data demonstrated highly significant upregulation of type 1 and type 2 interferon gene expression selectively in patients who received adjuvant a TLR agonist together with ATL-DC. CyTOF analysis of patient peripheral blood mononuclear cells (PBMCs) showed increased expression of PD-1 on CD4+ T-cells, decreases in CD38 and CD39 on CD8+ T cells and elevated proportion of monocytes after ATL-DC + TLR agonist administration. In addition, scRNA-seq demonstrated a higher expression fold change of IFN-induced genes with poly-ICLC treatment in both peripheral blood monocytes and T lymphocytes. Patients who had higher expression of interferon response genes lived significantly longer and had longer time to progression compared to those with lower expression. The results suggest that ATL-DC in conjunction with adjuvant poly-ICLC induces a polarized interferon response in circulating monocytes and specific activation of a CD8+ T cell population, which may represent an important blood biomarker for immunotherapy in this patient population. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01204684.

The Fate of the Inconclusive Periprosthetic Joint Infection Workup and Reliability of Data Points.

In 2018, periprosthetic joint infection (PJI) criteria were revised to include a new category labeled "inconclusive." The purpose of this study was to characterize and describe the fate of the inconclusive PJI workup and to analyze preoperative factors associated with outcomes. We reviewed all PJI workups at our institution during a 3-year period (426 patients). Patients were labeled "infected," "not infected," or "inconclusive" according to 2018 PJI preoperative criteria. In addition to standard diagnostic variables, the presence or absence of clinical elements that increase the pretest probability of infection were collected. Patients with any missing preoperative diagnostic test results and those with clinical follow-up less than 30 days were excluded. Logistic regression was used to identify the factors associated with infection. Two hundred ninety-six workups remained after exclusion criteria were applied, consisting of 66 (22.2%) with a preoperative score of 6 or greater defined as infected, 52 (17.6%) inconclusive (score 2-5), and 178 (60.1%) not infected (score 0-1). Postoperative re-scoring of the inconclusive group based on intraoperative findings as per the 2018 criteria identified 6 of 52 (11.5%) as infected, 12 (23.1%) inconclusive, and 34 (65.4%) not infected. Among those preoperatively scored as inconclusive, variables statistically correlated with the presence of infection included history of PJI, factors that increase skin barrier penetration (eg, psoriasis and venous stasis), and presence of comorbidities predisposing to infection. For patients labeled inconclusive, clinical elements of the pretest probability for infection (eg, history of prior PJI) were as reliable as any diagnostic test, including alpha-defensin, in the diagnosis of PJI. [Orthopedics. 2023;46(5):e291-e297.].

Higher Hospital Frailty Risk Score is associated with increased complications and healthcare resource utilization after endovascular treatment of ruptured intracranial aneurysms.

AIM: To use the Hospital Frailty Risk Score (HFRS) to investigate the impact of frailty on complication rates and healthcare resource utilization in patients who underwent endovascular treatment of ruptured intracranial aneurysms (IAs). METHODS: A retrospective cohort study was performed using the 2016-2019 National Inpatient Sample database. All adult patients (≥18 years) undergoing endovascular treatment for IAs after subarachnoid hemorrhage were identified using ICD-10-CM codes. Patients were categorized into frailty cohorts: low (HFRS <5), intermediate (HFRS 5-15) and high (HFRS >15). Patient demographics, adverse events, length of stay (LOS), discharge disposition, and total cost of admission were assessed. Multivariate logistic regression analysis was used to identify independent predictors of prolonged LOS, increased cost, and non-routine discharge. RESULTS: Of the 33 840 patients identified, 7940 (23.5%) were found to be low, 20 075 (59.3%) intermediate and 5825 (17.2%) high frailty by HFRS criteria. The rate of encountering any adverse event was significantly greater in the higher frailty cohorts (low: 59.9%; intermediate: 92.4%; high: 99.2%, p<0.001). There was a stepwise increase in mean LOS (low: 11.7±8.2 days; intermediate: 18.7±14.1 days; high: 26.6±20.1 days, p<0.001), mean total hospital cost (low: $62 888±37 757; intermediate: $99 670±63 446; high: $134 937±80 331, p<0.001), and non-routine discharge (low: 17.3%; intermediate: 44.4%; high: 69.4%, p<0.001) with increasing frailty. On multivariate regression analysis, a similar stepwise impact was found in prolonged LOS (intermediate: OR 2.38, p<0.001; high: OR 4.49, p<0.001)], total hospital cost (intermediate: OR 2.15, p<0.001; high: OR 3.62, p<0.001), and non-routine discharge (intermediate: OR 2.13, p<0.001; high: OR 4.17, p<0.001). CONCLUSIONS: Our study found that greater frailty as defined by the HFRS was associated with increased complications, LOS, total costs, and non-routine discharge.

Hospital Frailty Risk Score Predicts Adverse Events and Readmission Following a Ventriculoperitoneal Shunt Surgery for Normal Pressure Hydrocephalus.

OBJECTIVE: The aim of this study was to evaluate the impact of a Hospital Frailty Risk Score (HFRS) on unplanned readmission and health care resource utilization in normal pressure hydrocephalus (NPH) patients undergoing a ventriculoperitoneal (VP) shunt surgery. METHODS: A retrospective cohort study was performed using the 2016-2019 Nationwide Readmission Database. All NPH patients (≥60 years) undergoing a VP shunt surgery were identified using ICD-10-CM diagnostic and procedural codes. Patients were dichotomized into 2 cohorts as follows: Low HFRS (<5) and Intermediate-High HFRS (≥5). A multivariate logistic regression analysis was then used to identify independent predictors of adverse event (AE) and 30- and 90-day readmission. RESULTS: Of 13,262 patients, 4386 (33.1%) had an Intermediate-High HFRS score. A greater proportion of the Intermediate-High HFRS cohort experienced at least one AE (1.9 vs. 22.1, P < 0.001). The Intermediate-High HFRS cohort also had a longer length of stay (2.3 ± 2.4 days vs. 7.0 ± 7.7 days, P < 0.001), higher non-routine discharge rate (19.9% vs. 39.9%, P < 0.001), and greater admission cost ($14,634 ± 5703 vs. $21,749 ± 15,234, P < 0.001). The Intermediate-High HFRS cohort had higher rates of 30- (7.6% vs. 11.0%, P < 0.001) and 90-day (6.8% vs. 8.3%, P < 0.001) readmissions. On a multivariate regression analysis, Intermediate-High HFRS compared to Low HFRS was an independent predictor of any AE (odds ratio, 16.6; 95% confidence interval, [12.9-21.5]; P < 0.001) and 30-day readmission (odds ratio, 1.4; 95% confidence interval, [1.2-1.7]; P < 0.001). CONCLUSIONS: Our study suggests that frailty, as defined by HFRS, is associated with increased resource utilization in NPH patients undergoing VP shunt surgery. Furthermore, HFRS was an independent predictor of adverse events and 30-day hospital readmission.

Stereotactic radiosurgery and local control of brain metastases from triple-negative breast cancer.

OBJECTIVE: Stereotactic radiosurgery (SRS) is an effective treatment for intracranial metastatic disease, but its role in triple-negative breast cancer requires further study. Herein, the authors report overall survival (OS) and local tumor control in a multiinstitutional cohort with triple-negative breast cancer metastases treated with SRS. METHODS: Patients treated from 2010 to 2019 at 9 institutions were included in this retrospective study if they had biopsy-proven triple-negative breast cancer with intracranial metastatic lesions treated with SRS. Patients were excluded if they had undergone prior SRS, whole-brain radiation therapy, or resection of the metastatic lesions. A retrospective chart review was conducted to determine OS, local control, and treatment efficacy. RESULTS: Sixty-eight patients with 315 treated lesions were assessed. Patients had a median Karnofsky Performance Status of 80 (IQR 70-90) and age of 57 years (IQR 48-67 years). Most treated patients had 5 or fewer intracranial lesions, with 34% of patients having a single lesion. Treated lesions were small, having a median volume owf 0.11 cm3 (IQR 0.03-0.60 cm3). Patients were treated with a median margin dose of 18 Gy (IQR 18-20 Gy) to the median 71% isodose line (IQR 50%-84%). Overall, patients had a 1-year OS of 43% and 2-year OS of 20%. Most patients (88%) were followed until death, by which time local tumor progression had occurred in only 7% of cases. Furthermore, 76% of the lesions demonstrated regression. Tumor volume was correlated with local tumor progression (p = 0.012). SRS was very well tolerated, and only 3 patients (5%) developed symptomatic radiation necrosis. CONCLUSIONS: SRS is a safe and efficacious treatment for well-selected patients with triple-negative breast cancer, especially for those with a favorable performance status and small- to moderate-volume metastatic lesions.

Biallelic inactivation of PBRM1 as a molecular driver in a rare pineoblastoma case: illustrative case.

BACKGROUND: Pineoblastomas are a rare and aggressive pediatric neuroectodermal tumor subtype. Because of their rarity, pineoblastomas are still poorly understood, and there is little research delineating their molecular development and underlying genetic phenotype. Recent multiomic studies in pineoblastomas and pineal parenchymal tumors identified four clinically and biologically relevant consensus groups driven by signaling/processing pathways; however, molecular level alterations leading to these pathway changes are yet to be discovered, hence the importance of individually profiling every case of this rare tumor type. OBSERVATIONS: The authors present the comprehensive somatic genomic profiling of a patient with pineoblastoma presenting with the loss of protein polybromo-1 (PBRM1) as a candidate genomic driver. Loss of PBRM1, a tumor suppressor, has been reported as a driver event in various cancer types, including renal cell carcinoma, bladder carcinoma, and meningiomas with papillary features. LESSONS: This is the first report presenting biallelic loss of PBRM1 as a candidate molecular driver in relation to pineoblastoma.

Midterm Survivorship of Robotic-Assisted Lateral Unicompartmental Knee Arthroplasty.

BACKGROUND: Lateral unicompartmental knee arthroplasty (UKA) is a popular alternative to total knee arthroplasty (TKA) for patients with isolated lateral compartment osteoarthritis. Few studies have investigated outcomes following robotic-assisted lateral UKA. The purpose of this study is to evaluate mid-term survivorship and patient-reported outcomes of robotic-assisted lateral UKA. METHODS: A retrospective case series was conducted on all robotic-assisted lateral UKAs performed by a single surgeon between 2013 and 2019. Patient demographics, surgical variables, and Kozinn and Scott criteria were collected. Implant survivorship was estimated using the Kaplan-Meier method with all-cause reoperation and conversion to TKA as endpoints. Participating patients were assessed for patient satisfaction and the Forgotten Joint Score-12. Correlations between patient demographics and patient outcome scores were investigated. RESULTS: In total, 120 lateral UKAs were identified, 84 of which met inclusion criteria, with a mean follow-up of 4.0 years (range 2.0-7.0). Five-year survivorship was 92.9% (95% confidence interval [CI] 84.5-96.7) with all-cause reoperation as the endpoint, and 100% (95% CI 95.0-100) with conversion to TKA as the endpoint. One patient was converted to TKA after the 5-year mark, resulting in a 6-year survival for conversion to TKA of 88.9% (95% CI 44.9-98.5). Average Forgotten Joint Score-12 score was 82.7/100, and patient satisfaction 4.7/5. Mean coronal plane correction was 2.5° ± 1.9° toward the mechanical axis. Neither final postoperative alignment nor failure to meet classic Kozinn and Scott criteria for UKA resulted in differences in patient-reported outcomes. CONCLUSION: The current study demonstrates high mid-term survivorship and excellent patient-reported outcomes with robotic-assisted lateral UKA. Robotic-assisted lateral UKA is a viable treatment option for isolated lateral compartment arthritis even in patients who do not meet classic indications.

Transcarotid artery revascularization (TCAR): a technical video.

Carotid revascularization is an important method of stroke prevention and includes carotid endarterectomy and transfemoral carotid angioplasty and stenting. More recently, a hybrid open-endovascular approach, termed transcarotid artery revascularization (TCAR), is garnering increased attention. Although fundamentally a 'stenting procedure', unlike transfemoral carotid angioplasty and stenting, TCAR allows for a proximal neuroprotection strategy based on flow reversal. In this technical video, we will review operative techniques and nuances of the TCAR procedure, with a particular focus on the neurovascular proceduralist looking to adopt this technique into routine clinical practice(video 1). neurintsurg;14/8/842/V1F1V1Video 1TCAR Technical Video.

Utilization of technology-assisted total hip arthroplasty in the United States from 2005 to 2018.

BACKGROUND: Successful outcomes in total hip arthroplasty (THA) rely in part on accurate component positioning, which may be optimized through the use of computer navigation and robot-assistance. Therefore, we queried a large national database to characterize national trends in technology-assisted THA utilization, determine whether these technologies were associated with increased hospital charges, and identify demographic factors associated with technology-assisted THA. METHODS: Using the Nationwide Inpatient Sample database, patients that underwent conventional THA, computer-navigated THA, and robot-assisted THA from 2005 to 2018 were identified. Patient and hospital demographics, charge data, and payer characteristics were collected. Temporal trends in utilization were reported. Univariate analyses were performed to compare differences between groups with multiple logistic regression analysis to account for confounders. RESULTS: In total, 3,428,208 patients undergoing THA from 2005 to 2018 were identified, of which 63,136 (1.8%) used computer navigation and 32,660 (1.0%) used robot-assistance. National utilization of computer navigation in THA increased from 0.1% to 1.9% between 2005 and 2018, while utilization of robot-assisted THA increased from <0.1% to 2.1% from 2008 to 2018. On multivariate analysis, technology-assisted THA was most commonly performed in urban hospitals in the Northeastern United States. Median hospital charges were increased for technology-assisted THAs relative to conventional THAs ($66,089 ± $254 vs $55,418 ± $43). CONCLUSIONS: Computer navigation and robot-assistance in THA demonstrated a consistent increase in utilization during the period examined, representing 4.0% of THAs performed in 2018. Patient and hospital characteristics including risk of mortality, geographic region, and teaching status were associated with increased utilization. Utilization of computer navigation was associated with increased hospital charges.

Cost-effectiveness of Single vs Double Debridement and Implant Retention for Acute Periprosthetic Joint Infections in Total Knee Arthroplasty: A Markov Model.

BACKGROUND: Periprosthetic joint infection (PJI) is a common cause of revision total knee surgery. Although debridement and implant retention (DAIR) has lower success rates in the chronic setting, it is an accepted treatment of acute PJI, whether postoperatively or with late hematogenous seeding. There are two broad DAIR strategies: single debridement and planned double debridement. The purpose of this study is to evaluate the cost-effectiveness of single vs double DAIR for acute PJI in total knee arthroplasty. METHODS: A decision tree using single or double DAIR as the treatment strategy for acute PJI was constructed. Quality-adjusted life years and costs associated with the two treatment arms were calculated. Treatment success rates, failure rates, and mortality rates were derived from the literature. Medical costs were derived from both the literature and Medicare data. A cost-effectiveness plane was constructed from multiple Monte Carlo trials. A sensitivity analysis identified parameters most influencing the optimal strategy decision. RESULTS: Double DAIR was the optimal treatment strategy both in terms of the health utility state (82% of trials) and medical cost (97% of trials). Strategy tables demonstrated that as long as the success rate of double debridement is 10% or greater than the success rate of a single debridement, the two-stage protocol is cost-effective. CONCLUSIONS: A double DAIR protocol is more cost-effective than single DAIR from a societal perspective.

Surgical strategies for older patients with glioblastoma.

OBJECTIVE: While adjuvant treatment regimens have been modified for older patients with glioblastoma (GBM), surgical strategies have not been tailored. METHODS: Clinical data of 48 consecutive patients aged 70 years or older, who underwent surgical resection for GBM with intraoperative ultrasonography (IoUS) alone or combination with intraoperative MRI (IoMRI) at Yale New Haven Hospital were retrospectively reviewed. Variables were analyzed, and comparative analyses were performed. RESULTS: The addition of IoMRI was not superior to IoUS alone in terms of overall survival (OS) (P = 0.306), Karnofsky Performance Score (KPS) at postoperative 6 weeks (P = 0.704) or extent of resection (P = 0.263). Length of surgery (LOSx), however, was significantly longer (P = 0.0002) in the IoMRI group. LOSx (P = 0.015) and hospital stay (P = 0.025) were predictors of postoperative complications. Increased EOR (GTR or NTR) (P = 0.030), postoperative adjuvant treatment (P < 0.0001) and postoperative complications (P = 0.006) were predictive for OS. Patients with relatively lower preoperative KPS scores (<70) showed significant improvement at postoperative 6 weeks (P<0.0001). Patients with complications (P = 0.038) were more likely to have lower KPS at postoperative 6 weeks. CONCLUSIONS: Aggressive management with surgical resection should be considered in older patients with GBM, even those with relatively poor KPS. The use of ioMRI in this population does not appear to confer any measurable benefit over ioUS in experienced hands, but prolongs the length of surgery significantly, which is a preventable prognostic factor for impeding care.

Portable, bedside, low-field magnetic resonance imaging for evaluation of intracerebral hemorrhage.

Radiological examination of the brain is a critical determinant of stroke care pathways. Accessible neuroimaging is essential to detect the presence of intracerebral hemorrhage (ICH). Conventional magnetic resonance imaging (MRI) operates at high magnetic field strength (1.5-3 T), which requires an access-controlled environment, rendering MRI often inaccessible. We demonstrate the use of a low-field MRI (0.064 T) for ICH evaluation. Patients were imaged using conventional neuroimaging (non-contrast computerized tomography (CT) or 1.5/3 T MRI) and portable MRI (pMRI) at Yale New Haven Hospital from July 2018 to November 2020. Two board-certified neuroradiologists evaluated a total of 144 pMRI examinations (56 ICH, 48 acute ischemic stroke, 40 healthy controls) and one ICH imaging core lab researcher reviewed the cases of disagreement. Raters correctly detected ICH in 45 of 56 cases (80.4% sensitivity, 95%CI: [0.68-0.90]). Blood-negative cases were correctly identified in 85 of 88 cases (96.6% specificity, 95%CI: [0.90-0.99]). Manually segmented hematoma volumes and ABC/2 estimated volumes on pMRI correlate with conventional imaging volumes (ICC = 0.955, p = 1.69e-30 and ICC = 0.875, p = 1.66e-8, respectively). Hematoma volumes measured on pMRI correlate with NIH stroke scale (NIHSS) and clinical outcome (mRS) at discharge for manual and ABC/2 volumes. Low-field pMRI may be useful in bringing advanced MRI technology to resource-limited settings.

A Prospective Evaluation of Swallowing and Speech in Patients with Neurofibromatosis Type 2.

Objective Neurofibromatosis type 2 (NF2) patients report that swallowing and speech problems significantly affect their quality of life, but the etiology of these phenomena is poorly understood. Swallowing and speech deficits may arise due to the neuropathy of involved nerves, due to posterior fossa tumor growth, or as iatrogenic effects from neurosurgical procedures to remove these tumors. This study aims to identify the natural history of swallowing and speech deficits in an NF2 cohort and to characterize the factors that may lead to those deficits. Methods Subjects ( n = 168) were enrolled in a prospective, longitudinal study of NF2 with yearly imaging and clinical exams. The patients completed a self-reported questionnaire that included responses regarding subjective swallowing and speech dysfunction. A formal speech-language pathology evaluation and modified barium swallow (MBS) study (reported as American Speech-Language Hearing Association [ASHA] swallowing independency score from 1 through 7) was obtained when a speech/swallowing deficit was reported on the questionnaire. Results Of the 168 enrolled subjects, 55 (33%, median age = 31 years) reported subjective speech and/or swallowing deficits. These patients underwent one ( n = 37) or multiple ( n = 18) MBS studies during 44.8 ± 10.4 months follow-up. During MBS, a majority demonstrated near-normal swallowing (ASHA score >6, 82%), and no evidence of aspiration (aspiration/laryngeal penetration score = 1, 96%). Prior to initial MBS consultation, 38 (69%) patients had undergone relevant neurosurgical procedures. In those with recent (<1 week) posterior fossa surgery ( n = 12), 2 (17%) patients had severe dysphagia and high aspiration risk on postoperative MBS. Both of these patients recovered to functionally independent swallowing status. Unilateral ( n = 10) or bilateral ( n = 6) tongue deficits unrelated to previous history suggestive of hypoglossal nerve injury were detected on clinical examination. There was a correlation between the presence of dysarthria and tongue deficits and tumors associated with the hypoglossal canal noted on imaging. Conclusion A large proportion of patients with NF2 report speech and swallow deficits that are not evident on objective measurements. We also found hypoglossal neuropathy unrelated to prior surgical interventions. Our findings suggest that swallowing and speech problems in NF2 are associated with lower cranial nerve neuropathy, some due to compressive effects of posterior fossa tumors. Adaptation over the course of the disease allows for the compensation of these deficits and subsequent normal findings on objective testing.