Immediate CPAP Use after Skull Base Reconstruction with Hydroxyapatite Bone Cement.

OBJECTIVE: The timing for resuming continuous positive airway pressure (CPAP) postoperatively after skull base surgery remains controversial because of the risk of pneumocephalus. We determined the safety of immediate CPAP use after middle cranial fossa (MCF) spontaneous cerebrospinal fluid (sCSF) leak repair with bone cement. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary academic medical center. PATIENTS: Thirteen consecutive patients with CPAP-treated obstructive sleep apnea and temporal bone sCSF leaks who underwent skull base repair with hydroxyapatite bone cement between July 2021 and October 2022. INTERVENTIONS: CPAP use resumed on postoperative day 1 after the confirmation of skull base reconstruction with temporal bone computed tomography (CT). MAIN OUTCOME MEASURES: Postoperative skull base defects on CT, pneumocephalus, or intracranial complications. RESULTS: The average age was 55.5 ± 8.8 years (±standard deviation), and 69.2% were female with a BMI of 45.39 ± 15.1 kg/m 2 . Multiple tegmen defects were identified intraoperatively in 53.9% of patients with an average of 1.85 ± 0.99 defects and an average defect size on preoperative imaging of 6.57 ± 3.45 mm. All patients had an encephalocele identified intraoperatively. No residual skull base defects were observed on CT imaging on postoperative day 1. No postoperative complications occurred. One patient developed a contralateral sCSF leak 2 months after repair. There were no recurrent sCSF leaks 1 month postoperatively. CONCLUSION: Immediate postoperative CPAP use is safe in patients undergoing MCF sCSF leak repair with bone cement because of the robust skull base repair.

Prevalence, trends, and characteristics of trials investigating local therapy in contemporary phase 3 clinical cancer research.

BACKGROUND: Although most patients with cancer are treated with local therapy (LT), the proportion of late-phase clinical trials investigating local therapeutic interventions is unknown. The purpose of this study was to determine the proportion, characteristics, and trends of phase 3 cancer clinical trials assessing the therapeutic value of LT over time. METHODS: This was a cross-sectional analysis of interventional randomized controlled trials in oncology published from 2002 through 2020 and registered on ClinicalTrials.gov. Trends and characteristics of LT trials were compared to all other trials. RESULTS: Of 1877 trials screened, 794 trials enrolling 584,347 patients met inclusion criteria. A total of 27 trials (3%) included a primary randomization assessing LT compared with 767 trials (97%) investigating systemic therapy or supportive care. Annual increase in the number of LT trials (slope [m] = 0.28; 95% confidence interval [CI], 0.15-0.39; p < .001) was outpaced by the increase of trials testing systemic therapy or supportive care (m = 7.57; 95% CI, 6.03-9.11; p < .001). LT trials were more often sponsored by cooperative groups (22 of 27 [81%] vs. 211 of 767 [28%]; p < .001) and less often sponsored by industry (5 of 27 [19%] vs. 609 of 767 [79%]; p < .001). LT trials were more likely to use overall survival as primary end point compared to other trials (13 of 27 [48%] vs. 199 of 767 [26%]; p = .01). CONCLUSIONS: In contemporary late-phase oncology research, LT trials are increasingly under-represented, under-funded, and evaluate more challenging end points compared to other modalities. These findings strongly argue for greater resource allocation and funding mechanisms for LT clinical trials. PLAIN LANGUAGE SUMMARY: Most people who have cancer receive treatments directed at the site of their cancer, such as surgery or radiation. We do not know, however, how many trials test surgery or radiation compared to drug treatments (that go all over the body). We reviewed trials testing the most researched strategies (phase 3) completed between 2002 and 2020. Only 27 trials tested local treatments like surgery or radiation compared to 767 trials testing other treatments. Our study has important implications for funding research and understanding cancer research priorities.

Facial Nerve Preservation With Inferior Long-Axis Dissection of Large Vestibular Schwannomas.

OBJECTIVE: To describe a tumor resection using the inferior long-axis (ILA) technique for cisternal facial nerve dissection in large vestibular schwannomas (VS). STUDY DESIGN: Retrospective case series from 2018 to 2021. SETTING: Tertiary academic medical center. PATIENTS: Patients who underwent surgical resection with ILA facial nerve dissection of VS (>2.0 cm measured parallel to the petrous ridge) and had at least 3-month follow-up. INTERVENTIONS: Cisternal facial nerve dissection during retrosigmoid or translabyrinthine approach using standardized ILA technique developed by author R.N. MAIN OUTCOME MEASURES: Immediate postoperative and last follow-up facial nerve function with House-Brackmann scores of I to II defined as "good" facial nerve function and House-Brackmann scores III to VI defined as "poor" function. Extent of resection was also assessed. RESULTS: A total of 48 patients underwent large VS resection with ILA dissection of tumor off of the facial nerve from 2018 to 2021. Mean (standard deviation) tumor size was 3.11 (0.76) cm. Mean (standard deviation) follow-up was 9.2 (9.0) months. Gross-total resection or near-total resection were achieved in 75% (radiographic estimate) to 83% (surgeon estimate) of cases. End-of-case facial nerve stimulation at 0.05 mAmp with a response of at least 240 mV was achieved in 80.4% of patients. Good facial nerve function was observed in 72% immediately postoperatively, 70% 1-month postoperatively, and 82% of patients at last follow-up. CONCLUSIONS: The ILA technique is now the method of choice of the senior surgeon (R.N.) when performing microsurgical dissection of the cisternal facial nerve, with which he has achieved high rates of total or near-total resection with excellent facial nerve preservation.

TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation.

BACKGROUND: It is well established that biallelic mutations in transmembrane protease, serine 3 (TMPRSS3) cause hearing loss. Currently, there is controversy regarding the audiological outcomes after cochlear implantation (CI) for TMPRSS3-associated hearing loss. This controversy creates confusion among healthcare providers regarding the best treatment options for individuals with TMPRSS3-related hearing loss. METHODS: A literature review was performed to identify all published cases of patients with TMPRSS3-associated hearing loss who received a CI. CI outcomes of this cohort were compared with published adult CI cohorts using postoperative consonant-nucleus-consonant (CNC) word performance. TMPRSS3 expression in mouse cochlea and human auditory nerves (HAN) was determined by using hybridisation chain reaction and single-cell RNA-sequencing analysis. RESULTS: In aggregate, 27 patients (30 total CI ears) with TMPRSS3-associated hearing loss treated with CI, and 85% of patients reported favourable outcomes. Postoperative CNC word scores in patients with TMPRSS3-associated hearing loss were not significantly different than those seen in adult CI cohorts (8 studies). Robust Tmprss3 expression occurs throughout the mouse organ of Corti, the spindle and root cells of the lateral wall and faint staining within <5% of the HAN, representing type II spiral ganglion neurons. Adult HAN express negligible levels of TMPRSS3. CONCLUSION: The clinical features after CI and physiological expression of TMPRSS3 suggest against a major role of TMPRSS3 in auditory neurons.

Cochlear Implant Electrode Misplacement: A Case Series and Contemporary Review.

OBJECTIVE: To determine root causes leading to misplaced cochlear implant (CI) electrode arrays and discuss their management using a case series and contemporary literature review. STUDY DESIGN: Retrospective case review and contemporary literature review. SETTING: Single tertiary-referral center. PATIENTS: Adult and pediatric patients who were diagnosed with a misplaced CI electrode array, excluding tip-foldover. Literature review was performed via a MEDLINE database PubMed query. All articles that described at least one case of extracochlear electrode array misplacement were included; partial insertions and extrusions were excluded. MAIN OUTCOME MEASURE: Extracochlear misplacement. RESULTS: A total of 61 cases were reviewed, including 4 new cases and 57 cases from 29 previously published articles. We discuss management of CI arrays in the carotid canal, the vestibule, and the modiolus. The rate of CI misplacement is estimated to be 0.49%. The most frequent location of misplacement CI was the vestibular system (50.8%) followed by the internal carotid canal (11.5%). Normal cochlear anatomy was noted on preoperative computer tomography (CT) in 59.0% of patients; abnormalities were noted in 27.9%. The most common technical issue was misidentification or poor visualization of the round window. CONCLUSION: CI electrode misplacement is rare but can cause postoperative complications and may result in permanently diminished CI performance and hearing outcomes, even after revision surgery. Failure to identify the round window is the most common reason for CI misplacement, despite most patients having normal cochlear anatomy. Surgical strategies to localize the round window and basal turn are imperative for proper electrode placement. LEVEL OF EVIDENCE: 4.

Comparative Analysis of Recidivism After Endoscopic and Microscopic-Based Cholesteatoma Resection.

OBJECTIVES: 1) To analyze outcomes of cholesteatoma resection utilizing postauricular microscopic and endoscopic ear surgery (EES) approaches.2) To analyze predictors of residual and recurrent cholesteatoma. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Three hundred seventy-five adult and pediatric patients with cholesteatoma (2012-2017). INTERVENTIONS: Patients underwent surgical resection of cholesteatoma with EES (n = 122) and microscopic (n = 253) approach. MAIN OUTCOME MEASURES: Residual cholesteatoma, recurrent cholesteatoma, second-look procedures. RESULTS: The endoscopic cohort included significantly more pediatric cases (p = 0.0008). There was no difference in laterality, gender distribution, congenital or acquired cholesteatoma, and revision cases between the cohorts. Out of 122 EES cases, 16 (13%) developed residual disease and 9 (7%) developed recurrent disease. Of 253 microscopic cases 16 (6%) developed residual disease while 11 (4%) developed recurrent disease. Second look procedures were more commonly used in EES cohort (50 vs 18%). Single predictor analysis revealed 12 predictors for residual disease and 5 for recurrent disease. Multivariable model identified pediatric case distribution and higher disease stage to be significant predictors for both residual (p = 0.04, 0.007) and recurrent disease (p = 0.02, 0.01). EES approach was associated with a weak significance for residual disease (p = 0.049) but not recurrent disease (p = 0.34). CONCLUSIONS: EES approach for cholesteatoma resection seems to perform similarly to microscopic approach with no difference in rates of recurrent disease. However, it is associated with a higher rate of residual disease; this may be a reflection of a greater rate of second look procedures done in this group.

Speech Recognition Outcomes in Adults With Slim Straight and Slim Modiolar Cochlear Implant Electrode Arrays.

OBJECTIVE: To compare differences in audiologic outcomes between slim modiolar electrode (SME) CI532 and slim lateral wall electrode (SLW) CI522 cochlear implant recipients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic hospital. METHODS: Comparison of postoperative AzBio sentence scores in quiet (percentage correct) in adult cochlear implant recipients with SME or SLW matched for preoperative AzBio sentence scores in quiet and aided and unaided pure tone average. RESULTS: Patients with SLW (n = 52) and patients with SME (n = 37) had a similar mean (SD) age (62.0 [18.2] vs 62.6 [14.6] years, respectively), mean preoperative aided pure tone average (55.9 [20.4] vs 58.1 [16.4] dB; P = .59), and mean AzBio score (percentage correct, 11.1% [13.3%] vs 8.0% [11.5%]; P = .25). At last follow-up (SLW vs SME, 9.0 [2.9] vs 9.9 [2.6] months), postoperative mean AzBio scores in quiet were not significantly different (percentage correct, 70.8% [21.3%] vs 65.6% [24.5%]; P = .29), and data log usage was similar (12.9 [4.0] vs 11.3 [4.1] hours; P = .07). In patients with preoperative AzBio <10% correct, the 6-month mean AzBio scores were significantly better with SLW than SME (percentage correct, 70.6% [22.9%] vs 53.9% [30.3%]; P = .02). The intraoperative tip rollover rate was 8% for SME and 0% for SLW. CONCLUSIONS: Cochlear implantation with SLW and SME provides comparable improvement in audiologic functioning. SME does not exhibit superior speech recognition outcomes when compared with SLW.

Socioeconomic Influences on Short-term Postoperative Outcomes in Patients With Oral Cavity Cancer Undergoing Free Flap Reconstruction.

OBJECTIVE: To evaluate the associations between median household income (MHI) and area deprivation index (ADI) on postoperative outcomes in oral cavity cancer. STUDY DESIGN: Retrospective review (2000-2019). SETTING: Single-institution tertiary medical center. METHODS: MHI and ADI were matched from home zip codes. Main postoperative outcomes of interest were length of tracheostomy use, length of hospital stay, return to oral intake, discharge disposition, and 60-day readmissions. Linear and logistic regression controlled for age, sex, race, body mass index, tobacco and alcohol use history, primary tumor location, disease staging at presentation, and length of surgery. A secondary outcome was clinical disease staging (I-IV) at time of presentation. RESULTS: The cohort (N = 681) was 91.3% White and 38.0% female, and 51.7% presented with stage IV disease. The median age at the time of surgery was 62 years (interquartile range [IQR], 53-71). The median MHI was $47,659 (IQR, $39,324-$58,917), and the median ADI was 67 (IQR, 48-79). ADI and MHI were independently associated with time to return of oral intake (ß = 0.130, P = .022; ß = -0.092, P = .045, respectively). Neither was associated with length of tracheostomy, hospital stay, discharge disposition, or readmissions. MHI quartiles were associated with a lower risk of presenting with more advanced disease (Q3 vs Q1: adjusted odds ratio, 0.56 [95% CI, 0.32-0.97]). CONCLUSION: MHI is associated with oral cavity cancer staging at the time of presentation. MHI and ADI are independently associated with postoperative return to oral intake following intraoral tumor resection and free flap reconstruction.

Endoscope-Assisted Superior Semicircular Canal Dehiscence Repair: Single Institution Outcomes.

OBJECTIVE: To compare outcomes of endoscope-assisted middle cranial fossa MCF) repair of superior semicircular canal dehiscence (SSCD) compared to microscopic MCF repair. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary medical center neurotology practice. METHODS: Retrospective chart review and cohort study of patients who underwent surgical repair of SSCD via MCF approach from 2010 to 2019 at our institution. Patients were categorized according to use of endoscope intraoperatively. Pre- and post-operative symptom number was calculated from 8 patient-reported symptoms. Pre- and post-operative changes in symptom number were assessed using paired t-tests. Single-predictor binary logistic regression was used to compare final reported symptoms between cohorts. Linear regression was performed to assess air-bone gap (ABG) changes postoperatively between cohorts. RESULTS: Forty-six patients received surgical management for SSCD. Of these, 27 (59%) were male and 19 (41%) were female. Bilateral SSCD was present in 14 cases (29%), of which 3 underwent surgical management bilaterally, for a total of 49 surgical ears. Surgery was performed on the right ear in 19 cases (39%) and on the left in 30 cases (61%). Forty ears (82%) underwent microscopic repair while 9 (18%) underwent endoscope-assisted repair. Microscopic and endoscope-assisted MCF repair both demonstrated significantly improved symptom number postoperatively (P < .001 for each). There was no significant difference in change in ABG between the 2 cohorts. On average, patient-reported symptoms and audiometrically-tested hearing improved postoperatively in both groups. CONCLUSION: While endoscopic-assisted MCF repair has the potential to provide better visualization of medial and downslope defects, repair via this technique yields similar results and is equivalent to MCF repair utilizing the microscope alone.

Sinonasal Small Cell Carcinoma-Case Series of a Rare Malignancy.

Sinonasal small cell neuroendocrine carcinoma (SNEC) is an extremely rare and aggressive neoplasm that can arise in the sinonasal region. These tumors are associated with high morbidity and mortality, are difficult to diagnose, and are hard to treat. We describe 2 cases of this poorly understood malignancy and review imaging, pathology, and treatment decisions. A 41-year-old male and a 64-year-old female presented to a tertiary center in 2019 after developing nasal obstruction and were found to have sinonasal masses on imaging. Both biopsies showed strong expression of pancytokeratin with dot-like reactivity and expression of neuroendocrine markers chromogranin and synaptophysin. The findings were diagnostic of SNEC. Staging positron emission tomography/computed tomography and brain MRI were performed, and patients were discussed at a multidisciplinary tumor board. Neither had distant metastatic disease at presentation. One patient had no intracranial or orbital disease and underwent a subtotal endoscopic resection with adjuvant chemoradiation. The other patient demonstrated middle cranial fossa, dural, and orbital involvement as well as cranial nerve V palsy. This patient was treated with induction chemotherapy, followed by concurrent chemoradiation. Both patients are presently alive at 4 months follow-up, but one with persistent local disease and the other distant metastasis. Sinonasal small cell neuroendocrine carcinoma is a rare and poorly understood malignancy with an aggressive clinical course. Continued careful review of pathology and study of molecular features are needed for improved understanding of SNEC, and particularly for head and neck SNEC, to establish a staging system and better formulate treatment protocols.

Comparison of Small Intestinal Submucosal Graft and Autologous Tissue in Prevention of CSF leak after Posterior Fossa Craniotomy.

Objective To compare the use of porcine small intestinal submucosal grafts (SISG) and standard autologous material (fascia) in prevention of cerebrospinal fluid (CSF) leak and pseudomeningocele formation after translabyrinthine resection. Setting Set at the tertiary skull base center. Methods This is a retrospective chart review. After Institutional Review Board approval, we performed a retrospective cohort study evaluating CSF leak in patients who underwent resection of lateral skull base defects with multilayered reconstruction using either fascia autograft or porcine SISGs. Demographics were summarized with descriptive statistics. Logistic regression was used to compare autograft and xenograft cohorts in terms of CSF complications. Results Seventy-seven patients underwent lateral skull base resection, followed by reconstruction of the posterior cranial fossa. Of these patients, 21 (27.3%) underwent multilayer repair using SISG xenograft. There were no significant differences in leak-associated complications between autograft and xenograft cohorts. Ventriculoperitoneal shunt was necessary in one (1.8%) autograft and one (4.8) xenograft cases ( p = 0.49). Operative repair to revise surgical defect was necessary in three (5.4%) autograft cases and none in xenograft cases. Conclusion The use of SISG as a component of complex skull base reconstruction after translabyrinthine tumor resection may help reduce CSF leak rates and need for further intervention.

Progressive Vestibular Schwannoma following Subtotal or Near-Total Resection: Dose-Escalated versus Standard-Dose Salvage Stereotactic Radiosurgery.

Objective Local failure of incompletely resected vestibular schwannoma (VS) following salvage stereotactic radiosurgery (SRS) using standard doses of 12 to 13 Gy is common. We hypothesized that dose-escalated SRS, corrected for biologically effective dose, would have superior local control of high-grade VS progressing after subtotal or near-total resection compared with standard-dose SRS. Design Retrospective cohort study. Setting Tertiary academic referral center. Participants Adult patients treated with linear accelerator-based SRS for progressive VS following subtotal or near-total resection. Main Outcome Measures Dose-escalated SRS was defined by a biologically effective dose exceeding a single-fraction 13-Gy regimen. Study outcomes were local control and neurologic sequelae of SRS. Binary logistic regression was used to evaluate predictors of study outcomes. Results A total of 18 patients with progressive disease following subtotal (71%) and near-total (39%) resection of Koos grade IV disease (94%) were enrolled. Of the 18 patients, 7 were treated with dose-escalated SRS and 11 with standard-dose SRS. Over a median follow-up of 32 months after SRS, local control was 100% in the dose-escalated cohort and 91% in the standard-dose cohort ( p = 0.95). Neurologic sequelae occurred in 28% of patients, including 60% of dose-escalated cohort and 40% of the standard-dose cohort ( p = 0.12), although permanent neurologic sequelae were low at 6%. Conclusions Dose-escalated SRS has similar local control of recurrent VS following progression after subtotal or near-total resection and does not appear to have higher neurologic sequalae. Larger studies are needed.

The Influence of Extent of Resection and Tumor Morphology on Facial Nerve Outcomes Following Vestibular Schwannoma Surgery.

OBJECTIVE: To determine the influence extent of resection and tumor characteristics on facial nerve (FN) outcomes following microsurgical resection of vestibular schwannoma (VS). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Three hundred eighty-five patients who underwent VS microsurgical resection. INTERVENTIONS: Microsurgical VS resection. MAIN OUTCOME MEASURES: House-Brackmann (HB) scores postoperatively. Good FN function was defined as HB grade I and II and poor FN function was defined as HB grade III and VI. Gross total resection (GTR) versus subtotal resection (STR). Propensity-score matching was used in subset analysis to balance tumor volume between the surgical cohorts, followed by multivariable analysis. RESULTS: Seventy-one patients (18%) underwent STR and 314 patients (82%) underwent GTR. Two hundred fourteen patients (63%) had good FN function at 2 to 3 weeks postoperatively, and 80% had good FN function at 1 year. In single predictor analysis, STR did not influence FN function at 2 to 3 weeks (p = 0.65). In propensity-score matched subset analysis (N = 178), patients with STR were less likely to have poor FN function at 2 to 3 weeks (p = 0.02) independent of tumor volume (p = 0.004), but there was no correlation between STR and FN function at 1 year (p = 0.09). Ventral extension of tumor relative to the internal auditory canal plane was associated with poor FN outcomes at 2 to 3 weeks (p = 0.0001) and 1-year postop (p = 0.002). CONCLUSIONS: When accounting for tumor volume, STR is protective in immediate postoperative FN function compared to GTR. Ventral extension of the tumor is a clinical predictor of long-term FN outcomes.

Diameter-Based Volumetric Models May Inadequately Calculate Jugular Paraganglioma Volume Following Sub-Total Resection.

BACKGROUND: As gross total resection of jugular paragangliomas (JPs) may result in cranial nerve deficits, JPs are increasingly managed with subtotal resection (STR) with postoperative radiological monitoring. However, the validity of commonly used diameter-based models that calculate postoperative volume to determine residual tumor growth is dubious. The purpose of this study was to assess the accuracy of these models compared to manual volumetric slice-by-slice segmentation. METHODS: A senior neuroradiologist measured volumes via slice-by-slice segmentation of JPs pre- and postoperatively from patients who underwent STR from 2007 to 2019. Volumes from three linear-based models were calculated. Models with absolute percent error (APE) > 20% were considered unsatisfactory based on a common volumetric definition for residual growth. Bland-Altman plots were used to evaluate reproducibility, and Wilcoxon matched-pairs signed rank test evaluated model bias. RESULTS: Twenty-one patients were included. Median postoperative APE exceeded the established 20% threshold for each of the volumetric models as cuboidal, ellipsoidal, and spherical model APE were 63%, 28%, and 27%, respectively. The postoperative cuboidal model had significant systematic bias overestimating volume (p = 0.002) whereas the postoperative ellipsoidal and spherical models lacked systematic bias (p = 0.11 and p = 0.82). CONCLUSION: Cuboidal, ellipsoidal, and spherical models do not provide accurate assessments of postoperative JP tumor volume and may result in salvage therapies that are unnecessary or inappropriately withheld due to inaccurate assessment of residual tumor growth. While more time-consuming, slice-by-slice segmentation by an experienced neuroradiologist provides a substantially more accurate and precise measurement of tumor volume that may optimize clinical management.

Outcomes of Initial Observation Versus Upfront Microsurgical Resection for Small to Medium-sized Vestibular Schwannomas.

OBJECTIVE: To assess postoperative outcomes and predictive factors of patients observed prior to microsurgery and those undergoing upfront resection for small and medium-sized VS. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. PATIENTS: VS patients who had microsurgery from 2003 to 2018 for tumors up to 2.5 cm. MAIN OUTCOME MEASURES: Postoperative outcomes including facial nerve function and interventions, complications, extent of resection, and salvage therapy. RESULTS: Of 220 patients, 120 were initially observed, and 100 pursued upfront microsurgery. There was no significant association between initial observation and upfront microsurgery for postoperative facial nerve function at 2 to 3 weeks (p = 0.18) or 12 months (p = 0.5), facial nerve intervention (p = 0.5), major/minor complications (p = 0.48/0.63), recurrence (p = 0.8), subtotal resection (p = 0.6), or salvage therapy (p = 0.9). Time from initial consultation to surgery did not significantly impact outcomes. Intrameatal tumors were more likely to be observed (odds ratios [OR] 2.93; 95% CI 1.53-5.63; p = 0.001). Patients with larger tumor volume (OR 0.52; 95% CI 0.37-0.72; p < 0.0001), brainstem compression (OR 0.28; 95% CI 0.09-0.91; p = 0.03), or higher PTA were less likely to undergo observation (OR 0.99; 95% CI 0.97-0.997; p = 0.02). On multivariable analysis, predictive factors for observation were smaller tumor volume (OR 0.53; 95% CI 0.38-0.75; p < 0.001), lower PTA (OR 0.99; 95% CI 0.98-0.999; p = 0.04), and diabetes (OR 2.54; 95% CI 0.95-6.83; p = 0.06). CONCLUSIONS: Patients with worse hearing, larger tumor volume, and brainstem compression were more likely to pursue upfront microsurgery. A watchful waiting period does not appear to worsen outcomes and can be considered for patients with better hearing and smaller tumors without brainstem compression.

Cerebellopontine Angle and Internal Auditory Canal Lipomas: Case Series and Systematic Review.

OBJECTIVES/HYPOTHESIS: Cerebellopontine angle (CPA) and internal auditory canal (IAC) lipomas are rare, benign tumors comprising 0.08% of all intracranial tumors and can be mistaken for other, more common lesions of the CPA/IAC such as vestibular schwannoma. The purpose of this study was to review the literature and assess the evolution of CPA/IAC lipoma diagnosis and management. In addition, we present 17 new lipomas, matching the largest known case series of this rare tumor. STUDY DESIGN: Retrospective case series and systematic review. METHODS: Systematic review of the literature was performed using PubMed and Google Scholar. References from identified articles were also reviewed to identify potential additional manuscripts. Manuscripts and abstracts were reviewed to identify unique cases. For the case series, the charts of all CPA/IAC lipoma patients seen at a single institution from 2006-2019 were manually reviewed. Logistic regression and chi-squared analysis were performed where appropriate. RESULTS: A total of 219 unique lipomas have been reported in the literature, including 17 presented in this study. Surgical management has been performed in 46% of cases and has been conducted less often in recent decades, likely due to improved radiographic diagnostic capabilities and understanding of surgical outcomes. Surgical management is associated with worse neurologic outcomes (P = .002) and has become less common in recent decades. Although growth is unlikely, it has been demonstrated in patients into their 30s. CONCLUSIONS: Accurate radiographic diagnosis is imperative for appropriate patient management, as CPA/IAC lipomas should typically be managed through observation and serial imaging whereas vestibular schwannomas and other CPA/IAC lesions may require microsurgical or radiosurgical intervention depending on growth and symptomatology. Laryngoscope, 131:2081-2087, 2021.

Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Persistent Conductive Hearing Loss After Tympanostomy Tube Placement Due to High-Riding Jugular Bulb.

High-riding jugular bulb (HRJB) is a rare condition not often observed in the clinical setting that occurs in 1% to 3% of cases. The jugular bulb is not present at birth, and the precise size and location likely depends on a myriad of postnatal events. This report describes the case of a male adolescent who experienced persistent conductive hearing loss (CHL) unilaterally following bilateral tympanostomy tube placement. Subsequent workup included computed tomography, which identified a very high jugular bulb eroding the posterior semicircular canal and occluding the round window niche. The patient had no hearing or vestibular symptoms aside from CHL and continues to be observed on a regular basis. HRJB is a rare disorder that has been known to erode the posterior semicircular canal, resulting in possible tinnitus, vertigo, dizziness, and/or sensorineural hearing loss. CHL has been reported in HRJB cases, although it is uncommon. HRJB may result in CHL through a third-window defect shunting hydromechanical energy away from the round window or due to middle ear blockage. Imaging is useful in ascertaining rare causes of CHL, such as HRJB. Because HRJB is not easily fixable, it is important to recognize it as a rare cause of CHL for appropriate patient counseling. Possible interventions should be tailored to the patient after careful consideration of contralateral anatomy and likely benefits. Laryngoscope, 131:E1272-E1274, 2021.

Treatment of Sinonasal Teratocarcinosarcoma: A Systematic Review and Survival Analysis.

BACKGROUND: Sinonasal teratocarcinosarcoma (SNTCS) is a rare malignancy of the anterior skull base with only 127 cases described in the English literature. Given the rarity of this tumor, new cases and analysis of published reports may assist in future management of SNTCS. OBJECTIVES: 1) Describe findings from a systematic review of all available literature for malignant SNTCS including the clinical presentation, treatment modalities and outcomes. 2) Present two new cases of this rare anterior skull base tumor. 3) Compare treatment outcomes with respect to recurrence and mortality. METHODS: A systematic review of all English literature available in 2 comprehensive databases was conducted by two independent reviewers using PRISMA guidelines. 85 publications were identified. Each case was reviewed for demographics, treatment and survival, and aggregate treatment outcomes were compared using Kaplan-Meier analysis. RESULTS: A total of 64 articles meeting inclusion criteria were reported in the literature between 1977-2018. This represented a total of 127 patients, with a strong male predominance (83%) and mean age of 50 years (range 10-82). Mean follow-up was 21 months. Recurrence rate was 38%, with mean survival at 2 years of 55%. Almost all patients underwent surgery as a primary treatment modality (90%). The majority of cases were treated with multimodal therapy, with 55% receiving surgery and radiation and 20% receiving surgery with adjuvant chemoradiation. Kaplan-Meier analysis demonstrated a significant survival advantage for patients treated with combined therapy compared to surgery alone (p < 0.001) but did not show differences in recurrence (p = 0.085). CONCLUSION: Two-year survival rates for SNTCS are 55%. Multimodality treatment outcomes appear to be superior to surgery alone based on the published data of this rare skull base tumor, although heterogeneity of treatment methods and reporting bias limits the generalizability of these findings.

Pregnancy, Estrogen Exposure, and the Development of Otosclerosis: A Case-Control Study of 1196 Women.

OBJECTIVE: This study sought to determine whether a history of pregnancy or bilateral oophorectomy is associated with subsequent otosclerosis development or disease severity. STUDY DESIGN: Population-based case-control study. SETTING: Olmsted County, Minnesota. METHODS: Women diagnosed with otosclerosis were matched to 3 women without otosclerosis based on age and historical depth of medical records. Associations of prior delivery and bilateral oophorectomy with subsequent development of otosclerosis and with pure-tone average (PTA) at the time of otosclerosis diagnosis were evaluated. RESULTS: We studied 1196 women: 299 cases of otosclerosis and 897 matched controls. The odds ratio for the association of ≥1 delivery with otosclerosis was 1.16 (95% confidence interval [CI] 0.85-1.60; P = .35). Odds ratios for the associations of 1, 2, 3, or ≥4 deliveries with otosclerosis were 1.22 (0.83-1.80), 1.09 (0.71-1.68), 1.28 (0.77-2.12), and 1.00 (0.54-1.84), respectively. The odds ratio for the association of prior bilateral oophorectomy with otosclerosis was 1.12 (0.58-2.18; P = .73). In cases with otosclerosis, PTA at diagnosis was not significantly higher for women with ≥1 delivery as compared with those without (median 45 dB hearing loss [HL] [interquartile range {IQR} 36-55] vs 43 [IQR 34-53]; P = 0.18) but was significantly higher for women with bilateral oophorectomy compared with those without (median 54 dB HL [IQR 44-61] vs 44 [IQR 34-53]; P = .03). CONCLUSION: These data do not support a relationship between endogenous estrogen exposure and development of otosclerosis. Women with otosclerosis who had a history of pregnancy did not have significantly worse hearing at the time of diagnosis, suggesting that pregnancy is not associated with disease severity.