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Objectives: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae., Methods: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures., Results: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases., Conclusions: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

Objective: Describe the safety profile and surgical outcomes of a combined transmastoid-middle cranial fossa suture "pull-through" technique for repair of lateral skull base defects., Study Design: Retrospective., Setting: Tertiary care hospital., Patients: Patients undergoing surgery for cerebrospinal fluid (CSF) fistula and/or encephalocele., Intervention: Combined transmastoid and middle fossa approach using suture pull-through technique., Main Outcome Measures: The primary outcome measures of interest were recurrence of CSF fistula or encephalocele, and postoperative air-bone gap., Results: Twenty-six patients were included; mean age at surgery was 60 ± 14 years and 65% of patients were female. The majority of defects involved both the tegmen mastoideum and tympani (69%); multiple defects were present in 11 patients. Small craniotomy (2 × 3 cm) was performed and defects were repaired using composite grafts constructed with fascia, bone, and/or cartilage, and dural substitute affixed with suture. The suture tail was left long and passed from the middle fossa through the defect into the mastoid. At average follow-up of 8.3 months, no patients of recurrent CSF leak were noted. Significant improvements in both mean pure-tone average and air-bone gap were noted for the entire cohort (p = 0.04 and p = 0.02, respectively)., Conclusion: A combined transmastoid-middle cranial fossa for the repair of lateral skull base CSF fistula and encephaloceles using the suture "pull-through" technique is efficacious and the complication profile is favorable. This method facilitates reliable placement of a composite graft in the center of lateral skull base defects through a small craniotomy that minimizes temporal lobe retraction.

Objective: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD.Methods: Review of all cases at three tertiary-referral centers since disease description in 2003.Results: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy.Conclusions: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.Level Of Evidence: 4 Laryngoscope, 130:2574-2580, 2020. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To estimate the impact of race on disease presentation and treatment of vestibular schwannoma (VS) in the United States.Study Design: Analysis of a national population-based tumor registry.Methods: Analysis of the Surveillance, Epidemiology, and End Results database was performed, including all patients identified with a diagnosis of VS. Associations between race, disease presentation, treatment strategy, and overall survival were analyzed in a univariate and multivariable model.Results: A total of 9,782 patients with VS were identified among 822 million person-years. Of these, 7,400 (75.6%) claimed white, 807 (8.2%) Hispanic, 755 (7.7%) Asian, 397 (4.1%) black, and 423 (4.3%) patients reported other race. The median annual incidence of disease was lowest among black (0.43 per 100,000 persons) and Hispanic populations (0.45 per 100,000 persons) and highest among white (1.61 per 100,000 persons) populations (P < 0.001). Overall, Hispanic patients were diagnosed at the youngest age, and white patients were diagnosed at the oldest age (mean of 50.0 vs. 56.0 years, respectively; P < 0.001). Compared to white populations, black, Hispanic, and Asian populations were more likely to present with larger tumors (P < 0.001). After controlling for tumor size, age, and treatment center in a multivariable model, Hispanic patients were more likely than white patients to undergo surgery (P = 0.010); however, there were no differences between white, black, and Asian populations with regard to treatment modality. Hispanic and black patients had the poorest overall survival following surgery compared to other groups.Conclusion: Racial differences among patients with VS exist within the United States. Further studies are required to determine which factors drive differences in tumor size, age, annual disease incidence, and overall survival between races.Level Of Evidence: 3. Laryngoscope, 126:2128-2133, 2016. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical presentation and management of patients with cholesterol granulomas (CGs) that develop following temporal bone surgery.Study Design: Case series with chart review.Setting: Two independent tertiary academic referral centers.Subjects and Methods: A multicenter retrospective review was performed to identify all patients between 2001 and 2014 who were diagnosed with a CG that developed following temporal bone surgery. Patients with a history of idiopathic petrous apex CGs were excluded, as were those with <6 months of follow-up after diagnosis. Demographic and clinical data were recorded at presentation, and the main outcome measure was symptom evolution over time.Results: A total of 20 patients met inclusion criteria (median age, 55.0 years; 70.0% female), and their cases were analyzed. Diagnosis was made, on average, 149.5 months (median, 94.5; range, 13.0-480.0) following a temporal bone operation, which was for chronic ear disease in 75.0% of cases. The most common presenting symptoms were otalgia (55.0%) and otorrhea (40.0%). In patients initially managed with observation, 71.4% required no surgical intervention through a mean of 56.8 months (median, 30; range, 12.0-178.0) of follow-up. For those who initially underwent surgical excision, durable symptom relief was achieved in 66.7% of cases.Conclusion: Postoperative CG formation in the temporal bone is a rare occurrence that can be encountered years following surgery. The present study suggests that conservative management can be appropriate for many cases in which patients are relatively asymptomatic. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Inverting papillomas (IPs) are benign locally invasive tumors that most commonly present within the sinonasal cavity. Temporal bone involvement is exceedingly rare, with fewer than 30 cases reported within the English literature to date.Study Design: Case series and systematic review of the literature.Methods: Four consecutive subjects with temporal bone inverting papilloma (TBIP) were treated, and an additional 28 previously published cases were identified in the literature. Main outcome measures were disease presentation, diagnostic evaluation, management strategy, and outcome.Results: A total of 32 cases were analyzed. The median age at diagnosis was 54 years (mean 54.1; range 19-81 years). Nineteen (59%) patients had synchronous or metachronous sinonasal IP, whereas 13 (41%) had isolated temporal bone disease without sinus involvement. Over half of the patients undergoing microsurgical resection experienced at least one recurrence. Compared to patients with a history of sinus IP, subjects with primary TBIP were younger at time of presentation (44 vs. 58 years; P=0.012); were more commonly female (62% vs. 32%; P=0.15); and were less likely to have intracranial spread (8% vs. 26%; P=0.36), cranial neuropathy (8% vs. 26%; P=0.36), human papillomavirus positivity (11% vs. 57%; P=0.11), or associated carcinoma (0% vs. 47%; P=0.004).Conclusions: Inverting papilloma of the lateral skull base is rare and can pose a significant therapeutic challenge. Primary lesions of the temporal bone appear to follow a less aggressive clinical course when compared to those arising in association with sinonasal disease. Gross total resection is the preferred method of treatment, when feasible, given the high rate of recurrence with subtotal resection and risk of associated malignancy.Level Of Evidence: 4. [ABSTRACT FROM AUTHOR]