Citations

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.

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.

Objectives: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. Study Design: Multicenter retrospective review. Setting: Three tertiary referral centers. Patients: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. Intervention: Surgical repair or observation. Main Outcome Measure: Radiographic findings and clinical symptoms were analyzed. Results: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9–75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. Conclusions: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

Objective: Evaluate prevalence and pattern of occipital pneumatization (OP). Patients: Individuals with imaging evidence of OP on computed tomography (CT). Intervention(s): High resolution CT scans of the temporal bone. Main Outcome Measure: The prevalence and pattern of OP on 1000 CT scans performed at a large academic healthcare system. Results: OP had a prevalence of 11.8% (n = 118) while occipital condyle pneumatization occurred in only 0.3%. Occipital air cells were right-sided in 27.1% (n = 32), left-sided in 51.7% (n = 61), and bilateral in 21.2% (n = 25), and OP was contiguous with the occipitomastoid suture. In cases of unilateral OP, the contralateral jugular foramen demonstrated relative enlargement ( p = 0.006), but a direct association could not be established. Conclusions: The occipital bone is an accessory site of skull base pneumatization in 11.8% of the population undergoing temporal bone CT, while air cell extension into the occipital condyle occurs more rarely. These occipital air cells have a left-sided predilection and are always in direct proximity to the occipitomastoid suture, suggesting developmental egress from the mastoid. OP is a normal variant and lacks features of craniocervical pneumatization, which is a rare disorder.

Objective: To date, less than 150 cases of middle ear adenomatous neuroendocrine tumors (MEANTs) have been reported in the English literature. The objective of this study was to provide a contemporary analysis of these rare lesions and develop a consensus-driven staging system. Study Design: Multi-institutional retrospective histopathologic, radiologic, and clinical review. Setting: Six tertiary referral centers. Patients: Thirty-two patients with pathologically confirmed MEANT. Intervention: Surgical resection, adjuvant therapy. Main Outcome Measures: Clinical manifestations, outcomes, staging system. Results: Patients commonly presented with progressive conductive or mixed hearing loss (90%), aural fullness (50%), and tonal tinnitus (46%). Pulsatile tinnitus (16%), carcinoid syndrome (4%), and facial nerve paresis (4%) were less commonly observed. MEANTs frequently mimicked temporal bone paraganglioma (31%) and cholesteatoma (15%) at presentation. According to a novel T/N/M/S staging system (S = secretory tumor) proposed herein, there were 6 (19%) T1, 19 (59%) T2, and 7 (22%) T3 MEANTs. T3 tumors were significantly more likely to undergo subtotal or near total resection compared with lower staged tumors secondary to adherence to critical neurovascular structures such as the petrous internal carotid artery and facial nerve (p = 0.027). Patients with T3 MEANTs were more likely to experience multiple recurrences, require adjuvant therapy with a somatostatin analogue, or develop permanent facial nerve paresis compared with lower staged tumors. At last follow up, no patients with T1 MEANTs had developed recurrence, whereas 37% (7/19) of patients with T2 MEANT and 57% (4/7) of patients with T3 MEANT experienced either disease recurrence after gross total resection (GTR) or regrowth of known residual tumor requiring additional surgery at a median duration of 72 months (95% CI, 24–84). Conclusions: Patients with MEANTs can present with nonspecific symptomatology that overlaps with more commonly encountered middle ear lesions. MEANT exhibits a proclivity for recurrence according to T-stage and long-term clinical follow up is necessary, particularly for advanced stage tumors.

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.