Patel, Neil

BACKGROUND: Given the increasing prevalence and shifting disease demographic of vestibular schwannoma toward smaller tumors in people with less advanced symptoms, increasing emphasis has been placed on functional hearing preservation. OBJECTIVE: To provide an update to the 2018 CNS Guideline on hearing preservation outcomes in patients with sporadic vestibular schwannoma. METHODS: Systematic review and clinical practice guideline summarizing data pertaining to hearing preservation outcomes within the first 10 years after contemporary stereotactic radiation, microsurgery, or observation with serial imaging. The analysis incorporates studies included in the 2018 guideline and additional literature published up to May 20, 2022, providing a comprehensive up-to-date review of published clinical outcome data over time. RESULTS: Pooled estimated rates of serviceable hearing preservation are presented for observation, radiosurgery, and microsurgery for adults with sporadic vestibular schwannoma who have documented serviceable hearing in the ipsilateral ear at the time of diagnosis. Overall estimated rates of maintaining serviceable hearing are 78% at 2 years, 59% at 5 years, and 47% at 10 years during observation; 71% at 2 years, 59% at 5 years, and 38% at 10 years after radiosurgery; and 48% at 2 years, 40% at 5 years, and 32% at 10 years after microsurgery. In addition, features portending hearing outcome among management modalities are reported to guide accurate patient counseling. CONCLUSION: Regardless of treatment modality, fewer than half of patients with sporadic vestibular schwannoma who present with serviceable hearing will maintain useful hearing by 10 years. Across all studies, microsurgery and radiosurgery seem to accelerate this decline over the natural history, although further research is needed given limitations of available evidence. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/4-hearing-preservation-outcomes-in-patients-with-s

Abstract Rationale: Lymphoid Interstitial Pneumonia (LIP) is a rare interstitial lung disease characterized as a benign lymphoproliferative pulmonary condition. It is often associated with autoimmune conditions such as Sjögren's syndrome, immune dysregulatory syndromes, and common variable immunodeficiency. The incidence and prevalence of LIP remain unclear. While fluorodeoxyglucose (FDG) Positron Emission Tomography (PET/CT) findings have been documented for various diffuse parenchymal lung diseases, data specific to LIP is limited. This study aims to assess the utility of PET/CT in the evaluation of malignancy in patients with LIP, in addition to describing PET/CT findings in patients with known LIP who underwent PET/CT for any reason. Methods: This retrospective study involved a reviewof electronic medical records within the Mayo Clinic Enterprise to identify patients with an established diagnosis of LIP who had undergone PET/CT. Using ICD code J84.2, we identified patients with a diagnosis of LIP, and further selected for those who had undergone PET/CT between 2009 and 2023. We analyzed descriptive statistics, including the mean and prevalence of common PET/CT findings. Results: We identified 10 patients, all of whom had anestablished expert diagnosis of LIP and had undergone PET/CT for any reason. The mean age was 66 years, and all patients were female. Six of the ten patients (60%) underwent a lung biopsy, and six (60%) exhibited FDG uptake in the lungs, with maximum standard uptake value (SUVmax) uptake ranging from 2.0 to 8.2. Six of the imaging studies (60%) showed abnormal FDG uptake in one or more lymph nodes, with SUV maximum ranging from 2.4 to 7.8, and four (40%) demonstrated extrapulmonary FDG uptake. Four patients were diagnosed with a malignancy, the majority being MALT, with one being lung squamous cell carcinoma (SCC). Conclusion: Given the rarity of LIP, there is limiteddata on the utility and findings of PET/CT in these patients. Our study reveals that in patients with LIP, despite abnormal parenchyma in the background of inflammatory disease, malignancy was still likely in abnormally hypermetabolic regions. In this study, lymphoma was the most common malignancy isolated in patients with LIP. Nonetheless, malignancy other than lymphoma can occur, namely squamous cell carcinoma (SCC), and PET positivity can occur in LIP patients even in the absence of tissue proven malignancy. In patients with LIP, SUVmax does not appear to be a differentiating factor between those later diagnosed with lymphoma (SUVmax 2.0-8.2) and SCC (SUVmax=3.1).

ABSTRACTObjectiveThe primary objective of this study is to review the evidence for aspirin use to prevent the growth of vestibular schwannomas (VS) and to propose a prospective trial to determine the progression‐free survival of VS patients after up to 42 months of treatment with aspirin. Secondary study objectives are to determine the effect of aspirin on VS growth, hearing function, serum biomarker levels, and the quality of life, as well as to determine the tolerability of aspirin treatment and whether serum biomarker and salicylate levels predict the response to aspirin.Study DesignLiterature review.SettingSix academic and private medical centers.MethodsReview of recent English literature regarding prophylactic aspirin use for VS.ResultsThe retrospective reviews on the utility of aspirin to prevent VS growth are inconclusive. Eighty‐four patients have been enrolled in a prospective double‐blinded, placebo‐controlled trial thus far to determine the effect of aspirin on VS.ConclusionsCompletion of a robust study design is necessary. The current aspirin dose has been well tolerated, with minimal adverse events to date.Level of EvidenceReview of retrospective studies: Level 4; Proposed randomized, placebo‐controlled, double‐blinded clinical trial: Level 2.

Objective: To identify racial and/or health insurance disparities in the population undergoing ablation for paroxysmal supraventricular tachycardia(PSVT). Method: We identified adult patients (pts) admitted with principle diagnosis of PSVT by ICD 9 code 427.0 who had catheter ablation (ICD 9 code - 37.34), using the Nationwide Inpatient Sample (2000-2011). We stratified patients by race (White, Black, Hispanic, and other minority racial groups)and insurance status, and a hierarchical mixed effect multivariate model was created to identify independent predictors of PSVT ablation. Results: 64,564(20.2%) patients underwent ablation out of 319,579 patients admitted for PSVT during the period of 2000-2011. In absolute numbers, the majority of ablations were performed in Whites (58.7%) and patients with Medicare (56.3%) or private insurance (30.1%). After adjusting for confounding factors, the odds of having ablation were lower in Black pts (compare to White), and Medicare, Medicaid, and Uninsured pts (compare to private insurance) (see table). Other predictors of lower rate of ablations were: older age and female patients while odds of receiving ablation were higher for elective admissions and in teaching hospitals (p<0.001 for all comparisons). Conclusion: In a population of pts admitted for PSVT, those without private insurance and Black pts were less like to be treated with catheter ablation; and, this disparity persisted from 2000-2011. Since catheter ablation for PSVT is generally considered a curative procedure, future studies are needed to identify the factors that lead to these disparities of care.

Abstract Trigeminal nerve schwannomas (TNSs) are rare lesions that typically present with symptoms of trigeminal neuropathy or other cranial nerve palsies. These lesions classically have a dumbbell shape, with an anterior component within Meckel's cave and posterior component extending into the posterior fossa through the porus trigeminus. Surgical resection of TNSs can often be achieved via an extradural subtemporal approach to Meckel's cave without an anterior petrousectomy, even for tumors with a significant posterior fossa component, as the tumor often erodes a portion of the petrous apex. 1 We present the case of a 53-yr-old female presenting to our institution with complete trigeminal neuropathy secondary to a right-sided, previously resected and radiated TNS. Serial imaging demonstrated an interval growth of significant residual tumor despite multiple adjuvant therapies, and, thus, the patient was recommended to undergo additional surgical resection. The lesion was approached through a right-sided subtemporal approach to Meckel's cave, 2 with a plan to utilize an anterior petrousectomy only if difficulty resecting the posterior fossa component of the tumor was encountered. Intraoperatively, the posterior fossa component was found to be densely adherent to the adjacent brainstem, likely secondary to prior surgery and radiation therapy, and, thus, an anterior petrousectomy was performed. Postoperatively, the patient had stable trigeminal neuropathy without any new neurological deficits and a magnetic resonance imaging (MRI) confirmed a gross total resection. In the accompanying video, we hope to demonstrate the steps and nuances of both the subtemporal approach to accessing Meckel's cave and anterior petrousectomy when employed for the resection of TNSs. The patient in question provided formal consent for the making of this video.

Background: Previous cross-sectional studies analyzing quality of life (QOL) outcomes in patients with sporadic vestibular schwannoma (VS) have shown surprisingly little difference among treatment modalities. To date, there is limited prospective QOL outcome data available comparing baseline to posttreatment scores. Study Design: Prospective longitudinal study using the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) scale. Setting: Large academic skull base center. Patients: Patients diagnosed with unilateral VS who completed a baseline survey before treatment and at least one posttreatment survey. Main Outcome Measures: Change in PANQOL scores from baseline to most recent survey. Results: A total of 244 patients were studied, including 78 (32%) who elected observation, 118 (48%) microsurgery, and 48 (20%) stereotactic radiosurgery. Patients who underwent microsurgery were younger (p < 0.001) and had larger tumors (p < 0.001) than those who underwent observation or radiosurgery; there was no significant difference in duration of follow-up among management groups (mean 2.1 yrs; p = 0.28). When comparing the total PANQOL score at baseline to the most recent survey, the net change was only –1.1, –0.1, and 0.3 points on a 100-point scale for observation, microsurgery, and radiosurgery, respectively (p = 0.89). After multivariable adjustment for baseline features, there were no statistically significant changes when comparing baseline to most recent scores within each management group for facial function, general health, balance, hearing loss, energy, and pain domains or total score. However, the microsurgical group experienced a 10.8-point improvement (p = 0.002) in anxiety following treatment, compared with 1.5 (p = 0.73) and 5.3 (p = 0.31) for observation and radiosurgery, respectively. Conclusions: In this prospective longitudinal study investigating differences in QOL outcomes among VS treatment groups using the disease-specific PANQOL instrument, treatment did not modify QOL in most domains. Microsurgery may confer an advantage with regard to patient anxiety, presumably relating to the psychological benefit of “cure” from having the tumor removed.

OBJECTIVE The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm 3 (range 84–6080 mm 3 , n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1–143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%–95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS.

Intracranial extension of rhinosinusitis is rare in children. We report a 9-year-old immunocompetent boy with central precocious puberty and obstructive sleep apnea-hypopnea syndrome who developed an intracranial epidural abscess secondary to rhinosinusitis while on continuous positive airway pressure (CPAP) treatment. A retrospective review of the medical record and imaging studies was performed. MEDLINE and Cochrane databases were searched for reports of epidural abscess developing in patients receiving CPAP treatment or in patients with precocious puberty. Intracranial extension of frontal rhinosinusitis is more common during puberty probably because of the active growth of the frontal sinuses and their rich blood supply. Controlled studies show no increase with rhinosinusitis in adults on CPAP; no published studies assess intracranial extension of rhinosinusitis in CPAP use. Patients with unexplained, severe headache and fever following CPAP use may require neuroimaging (magnetic resonance imaging [MRI] / contrast computed tomography) to rule out intracranial extension of sinusitis.

Objective: Describe the first case of cochlear implantation (CI) for auditory rehabilitation of a patient with craniometaphyseal dysplasia (CMD) and progressive mixed hearing loss. Patients: A 65-year-old woman with known autosomal dominant CMD presented with progressive mixed hearing loss and declining benefit from conventional hearing aids. Computed tomography and magnetic resonance imaging revealed hyperostosis of the entire craniofacial skeleton. Hearing evaluation demonstrated pure-tone thresholds in the profound range bilaterally by air conduction, and bone conduction thresholds that matched aided thresholds for her left ear, though testing was somewhat limited by inability to mask at high air-conduction thresholds. CI candidacy testing confirmed poor word and sentence scores in the right ear. Intervention: Due to the inability to access the cochlea via a conventional mastoidectomy and facial recess approach, cochlear implantation via a postauricular subtotal petrosectomy approach with ear canal overclosure was performed. Main Outcome Measures: Post-implantation word and sentence testing. Results: Despite extensive internal auditory canal stenosis, the patient demonstrated excellent early speech understanding results 5 weeks after device activation. Postimplantation audiologic evaluation showed thresholds between 20 and 30 dB HL from 250 to 6000 Hz. Word and sentence testing scores were 76% Consonant-nucleus-consonant in quiet (up from 2% preoperatively) and 77% AzBio sentences in quiet (up from 10% preoperatively). Conclusion: This report describes the first description of CI for CMD. Despite the extensive radiologic abnormalities, the patient has demonstrated excellent benefit from implantation. Further study of rare temporal bone dysplasias, such as CMD, is critical to better characterize the progression of otologic disease and determine optimal treatment.

Objective: The treatment paradigm for jugular paraganglioma (JP) has changed considerably over time with the wider adoption of stereotactic radiosurgery (SRS). To the best of the authors’ knowledge, there are no published studies that use validated patient-reported outcome measures to ascertain quality of life (QoL) outcomes following SRS for JP when used in single or combined modality treatment regimens. Study Design: Cross-sectional survey. Setting: Tertiary referral center. Patients: Adult patients with JP treated with primary SRS or SRS following primary surgery between 1990 and 2017. Interventions(S): Surgery and/or Gamma Knife SRS. Main Outcome Measures: Global and treatment-related QoL and differences in QoL based on treatment approach. Results: Sixty-nine surveys were distributed and a total of 26 completed surveys were received (38% response rate). Among respondents, the median age at SRS was 53 years and 16 of the 26 patients (62%) were female. Median follow-up was 97 months. Nineteen patients (73%) were treated with primary SRS or staged SRS following intentional subtotal resection (STR; hereafter referred to as “staged SRS”), while the remainder (n = 7, 27%) were treated with SRS for recurrent JP. Median physical and mental health QoL PROMIS-10 T-scores regardless of treatment strategy were 39.8 and 38.8, respectively, while median SF36 physical and mental component subscores were similar to national averages and non-tumor controls. When comparing general physical and mental health QoL scores, there was no significant difference between patients treated with primary or staged SRS and those treated with SRS for recurrent JP. However, age-adjusted swallowing function among patients treated with primary or staged SRS was better than in those patients treated with SRS for recurrent JP (p = 0.05). Conclusions: Patients treated with primary or staged SRS for JP tend to exhibit better swallowing outcomes than those treated with SRS for recurrent JP. However, the majority of overall and disease-specific quality of life measures were not different between groups. Based on the low incidence of new cranial neuropathy following SRS, it is likely that initial surgical morbidity is the primary contributor to this outcome. Disease-specific overall quality of life measures, akin to those already used for other benign skull base tumors, are necessary to better gauge physical and mental health outcomes following treatment for JP. Though limited by small sample size, this represents the first study to gauge QoL outcomes following treatment for JP.