Carlson, Matthew L.

Purpose: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. Methods: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. Results: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk–75 yr)] were evaluated for recurrent facial nerve paralysis at the authors’ institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2–20). The median nadir House–Brackmann score was 4, with a median recovery to House–Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1–420 d). Diagnostic evaluation confirmed Melkersson–Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. Conclusion: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

AbstractIntralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

Objectives This video describes the surgical indications, relevant anatomy, and surgical steps of routine translabyrinthine surgery for gross total resection of sporadic vestibular schwannoma. Design The procedure is presented through a surgical instructional video. Setting The surgery took place at tertiary skull base referral center. Parcipant A 47-year-old patient reported with nonserviceable hearing, frequent episodes of vertigo, recurrent severe headache, and a small unilateral right sided vestibular schwannoma. Results Gross total resection with preservation of facial nerve function was achieved. Conclusion This instructional video documents the surgical steps and relevant anatomy for translabyrinthine resection of vestibular schwannoma.The link to the video can be found at: https://youtu.be/CJ2vKMLs7aI.

Objective:There is a common misconception that improvement in sudden sensorineural hearing loss (SSNHL) after treatment with steroid therapy effectively excludes the diagnosis of a vestibular schwannoma (VS) and such cases do not warrant an MRI. Paralleling this, steroids are commonly withheld for SSNHL in patients with an existing diagnosis of VS, believing that this condition is not steroid-responsive. This study seeks to underscore that improvement or recovery of SSNHL with steroid therapy does not exclude the diagnosis of VS and does not preclude the need for magnetic resonance imaging.Methods:A retrospective chart review was performed (2002–2017) of patients with previously untreated sporadic VS who developed SSNHL that improved after steroid treatment. A clinically significant audiometric improvement was defined as an increase of more than or equal to 15% in word recognition score (WRS) and/or decrease of more than or equal to 15 dB in 4-frequency pure-tone average (PTA). To supplement these data, a separate population of patients with incomplete or missing audiometric data, who reported unequivocal subjective improvement in hearing after steroid treatment, were also described to reinforce the study objective. Patient demographics, tumor characteristics, steroid regimen, and data regarding treatment response were recorded.Results:A total of 29 patients (55% women; median age of 47 yr) met inclusion criteria. Fourteen (48%) cases had objective audiometric documentation of SSNHL, while 15 (52%) had either subjective report only or incomplete audiometric data available. Eighteen (62%) had a single event, while 11 (38%) had more than one episode of SSNHL that was treated with steroids. For all patients, the median time between SSNHL and diagnosis of VS was 1.3 months (range, 0.13–148.4 mo). At the time of diagnosis, 15 tumors were purely intracanalicular, while 15 tumors had cerebellopontine angle extension. Of the latter, the median cisternal tumor size was 15.9 mm (range, 5.3–33). Twenty-six (90%) cases received oral steroid therapy alone, two (9%) had intratympanic steroid therapy alone, and one (3%) required combination therapy. The median PTA improvement with steroid therapy was 21 dB HL (range, –10–101.2) and the median WRS improvement was 40% (range, 4–100%).Conclusion:A therapeutic response to steroid therapy for SSNHL does not exclude the diagnosis of VS. All patients with SSNHL should undergo appropriate diagnostic imaging to prevent delays in diagnosis and potential treatment.

Background: The Acoustic Neuroma Association (ANA) is a national, nonprofit organization, focused on the education and support of patients with vestibular schwannoma (VS). The aim of the present study is to characterize the profile of ANA survey respondents and compare them with non-ANA patients evaluated at a single tertiary academic referral center to investigate the potential influence of selection bias. Methods: A prospectively maintained VS quality-of-life (QOL) database, comprised of patients evaluated at the authors’ center and members of the ANA, was queried. Demographic variables, patient-reported symptoms and tumor characteristics, as well as patient-reported outcome scores were captured. Health-related QOL was evaluated using the disease-specific Penn Acoustic Neuroma QOL (PANQOL) questionnaire. Multivariable regression models were fitted for PANQOL domain and total scores as well as satisfaction with treatment adjusting for baseline demographics, symptoms, and PANQOL scores. Results: A total of 1,060 patients (802 [76%] ANA respondents) were analyzed. Overall, ANA patients were slightly younger (mean age: 59 vs 60 yr, p = 0.145), more likely to be women (72 vs 55%, p < 0.001), and had a larger tumor size (overall p < 0.001). Furthermore, a significantly higher proportion of ANA patients were more likely to undergo microsurgery (57 vs 21%) or radiation (21 vs 8%) and less likely to be managed with observation (16 vs 65%, overall p < 0.001). A significantly higher proportion of ANA patients reported hearing loss (95 vs 88%, p < 0.001), tinnitus (80 vs 73%, p = 0.034), dizziness (78 vs 64%, p < 0.001), headache (56 vs 45% p = 0.003), and facial paralysis (37 vs 12%, p < 0.001). On multivariable analysis, ANA respondents exhibited significantly lower PANQOL scores for hearing (OR: 0.47, 95% CI: 0.35–0.64, p < 0.001), balance (OR: 0.51, 95% CI: 0.38–0.70, p < 0.001), pain (OR: 0.63, 95% CI: 0.46–0.86, p = 0.004), facial function (OR: 0.58, 95% CI: 0.42–0.80, p = 0.001), energy (OR: 0.44, 95% CI: 0.32–0.59, p < 0.001), anxiety (OR: 0.54, 95% CI: 0.40–0.74, p < 0.001), general (OR: 0.72, 95% CI: 0.53–0.98, p = 0.03), and total QOL (OR: 0.40, 95% CI: 0.30–0.55, p < 0.001). No statistically significant difference was seen with regard to treatment satisfaction. To determine the true clinical relevance of these differences, the two groups were compared using the minimal clinically important difference (MCID) for each domain. MCID is defined as the smallest difference in score in the domain of interest that patients perceive as important, either beneficial or harmful. The domains for hearing, balance, energy, anxiety, and total QOL reached their respective MCID thresholds, indicating that the ANA cohort has QOL scores that are clinically, perceptually worse for these domains compared to the non-ANA group. Conclusion: These data help delineate some of the inherent limitations and biases associated with survey studies incorporating data from national patient support organizations. The population profile of ANA survey respondents likely differs significantly from the greater population of patients with VS that may be encountered at a tertiary referral center.

ObjectivesConflicting research exists surrounding the utility of aspirin to prevent tumor growth in the medical management of vestibular schwannoma (VS). Recent studies demonstrated no association between aspirin and VS growth using linear tumor measurements. Given the heightened sensitivity of volumetric analyses to monitor tumor growth, the current study was conceived with the chief objective of assessing the association between aspirin or other nonsteroidal anti‐inflammatory drug (NSAID) use and VS growth using volumetric analyses.Study DesignRetrospective review.SettingTertiary referral center.Subjects and MethodsA total of 361 patients totaling 1601 volumetrically analyzed magnetic resonance imaging studies who underwent initial observation since January 1, 2003.ResultsIn total, 123 (35%) patients took 81 mg aspirin daily, 23 (7%) took 325 mg aspirin daily, and 41 (11%) reported other NSAID use. Among those taking aspirin, 112 (72%) exhibited volumetric tumor growth during observation compared to 33 (80%) among other NSAID users and 137 (67%) among nonaspirin users. Patients taking aspirin or other NSAIDs were significantly older at time of diagnosis (median, 66 vs 56 years; P <. 001). Neither aspirin use (hazard ratio [HR], 0.96; P =. 73) nor other NSAID use (HR, 1.39; P =. 081) was significantly associated with a reduced risk of volumetric tumor growth. These results were similar following age adjustment (P =. 81 and. 087, respectively). When separating aspirin users by 81‐mg or 325‐mg dosing, neither group exhibited a reduced risk of growth (P =. 95 and. 73, respectively).ConclusionDespite promising initial results, the preponderance of existing literature suggests that aspirin and other NSAID use does not prevent tumor growth in VS.

Abstract BACKGROUND Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses. OBJECTIVE To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation. METHODS Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors’ institution since January 1, 1996 was also performed to supplement these data. RESULTS Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%). CONCLUSION Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and/or surgical debulking to relieve mass effect.