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Objectives: To assess quality of life (QOL) outcomes after canal wall up (CWU) and canal wall down (CWD) tympanomastoidectomy in the pediatric population., Methods: A retrospective review tabulated pediatric patients undergoing CWU and CWD tympanomastoidectomy for cholesteatoma by 2 senior surgeons at a single tertiary academic referral center between March 2017 and March 2023. Chronic Ear Survey (CES) and cosmetic survey outcomes were collected post-operatively., Results: A total of 77 ears in 75 patients were identified, with 35 undergoing CWU and 42 undergoing CWD as the most recent (index) otologic surgery. Seventeen patients (23%) participated in the survey. Of this cohort, the mean age was 14.6 years, 12 (71%) were male, and 10 (59%) had CWD as the most recent otologic surgery. The mean time from index surgery to survey completion was 3.4 years (range, 0.1-6.7 years). Regarding QOL outcomes, there were no statistically significant differences in total CES score, CES subscores, and cosmetic survey scores between groups when categorizing by gender or index surgery. Total CES, symptom subscale, medical resource subscale, and cosmetic survey scores showed a tendency to decrease with an increasing number of surgeries ( R  = -.18, -.28, -.53, and -.56, respectively). Pediatric total CES scores appeared comparable to those reported in the published adult literature., Conclusions: It does not appear that there are worse QOL outcomes for pediatric patients who undergo CWD tympanomastoidectomy compared to those who undergo CWU tympanomastoidectomy. There appears to be no difference in QOL outcomes between pediatric and adult patients undergoing tympanomastoidectomy., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: ADS has been a consultant for Cochlear Americas, MED-EL GmbH and Oticon Medical. ADS has been on the surgical advisory board of MED-EL GmbH.

Objective: The recent Field Corrective Action (FCA) for the HiRes Ultra and Ultra 3D (V1) cochlear implants (CIs) triggered much research investigating clinical identification, failure rates, and postrevision outcomes. Pediatric data remain limited, especially with regards to speech outcomes. We aim to characterize the trajectory of children implanted with these devices with specific attention to speech outcomes., Patients: Retrospective cohort study of pediatric patients with FCA-affected CIs from March 2017 to January 2020 at a tertiary children's hospital., Interventions: CI placement, device monitoring, audiologic evaluation, revision surgery., Main Outcome Measures: CI failure rates, revision surgery rate, speech recognition outcomes., Results: Forty-one devices were implanted in 27 pediatric patients. Average age at implantation was 4.01 years (range, 0.87-12.75). To date, 30 devices (73%) are known failures with 90% of these having undergone revision surgery. No statically significant difference was noted on open-set speech testing across best prerevision, immediate prerevision, and best postrevision time points. Best postrevision CNC scores had a mean score of 71% ± 26%, n = 16., Conclusions: Pediatric patients implanted with FCA-affected CI devices have a high risk of device failure. Open-set speech recognition was not significantly different from prerevision to postrevision testing, suggesting preserved speech outcomes. This may be partially attributable to limitations of cohort size and the pediatric population with open-set speech testing. However, we suspect that close follow-up with standardized testing and a low threshold for revision surgery provided by our multidisciplinary team may have mitigated these changes. Postrevision open-set speech testing remains positive for these patients., Competing Interests: Conflict of interest: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Vestibular schwannomas (VS) are benign tumors that lead to significant neurologic and otologic morbidity. How VS heterogeneity and the tumor microenvironment (TME) contribute to VS pathogenesis remains poorly understood. In this study, we perform scRNA-seq on 15 VS, with paired scATAC-seq (n = 6) and exome sequencing (n = 12). We identify diverse Schwann cell (SC), stromal, and immune populations in the VS TME and find that repair-like and MHC-II antigen-presenting SCs are associated with myeloid cell infiltrate, implicating a nerve injury-like process. Deconvolution analysis of RNA-expression data from 175 tumors reveals Injury-like tumors are associated with larger tumor size, and scATAC-seq identifies transcription factors associated with nerve repair SCs from Injury-like tumors. Ligand-receptor analysis and in vitro experiments suggest that Injury-like VS-SCs recruit myeloid cells via CSF1 signaling. Our study indicates that Injury-like SCs may cause tumor growth via myeloid cell recruitment and identifies molecular pathways that may be therapeutically targeted., (© 2023. The Author(s).)

Purpose: Patients often have basic audiometry (BA) but not objective diagnostic tests of the vestibular system (VNG) when complaining of symptoms of a vestibular disorder. The relationship of BA results to VNG results is unknown. This study sought to determine if BA scores are related to impaired VNG scores., Materials and Methods: We reviewed electronic medical records at a tertiary care center, for patients seen between 2015 and 2021 who had had both a BA and a VNG (n = 651). BA subtests were pure tone averages, word recognition, and tympanogram. VNG subtests were cervical vestibular evoked myogenic potentials, Dix-Hallpike maneuvers, and bi-thermal caloric tests. All tests were summarized as normal/abnormal., Results: More subjects had abnormal BA than abnormal VNG scores. Age but not sex was significantly related to abnormal scores. High BP was a significant comorbidity in 15 % of the sample, more in patients with abnormal than normal VNG scores. Although the abnormal BA and abnormal VNG were significantly related, pure tone averages and tympanogram scores were not related to VNG subtests. Abnormal word recognition with both ears combined was significantly related to normal and abnormal bi-thermal caloric tests., Conclusions: If the clinician needs to know of any VNG impairment, in general, then performing a BA without a VNG might suffice. If the clinician needs information about the details of possible vestibular impairment, then a VNG should be performed., Competing Interests: Declaration of competing interest There are no conflicts of interest, financial or otherwise., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications., Study Design: Survey., Setting: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery., Results: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting., Conclusions: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: To evaluate the safety and outcomes of cochlear implantation (CI) in patients with ventriculoperitoneal (VP) shunts to inform clinical practice., Study Design: Historical cohort study., Setting: Tertiary referral centers., Methods: A multi-institutional historical cohort of patients with VP shunts and CI was identified and analyzed., Results: A total of 46 patients (median age 8 years [interquratile range, IQR: 2-46]) with VP shunts and CI were identified. Of these, 41 (89%) patients had a VP shunt prior to CI. Based on institutional preference and individual patient factors, CI was performed contralateral to a pre-existing VP shunt in 24 of these 41 cases (59%) and ipsilateral in 17 (41%). Furthermore, pre-CI relocation of the VP shunt was performed in 3 cases (7%), and 2 patients (5%) underwent planned revision of their VP shunt concurrent with CI. In total, 2 of 27 pediatric patients (7%) required unanticipated revision shunt surgery, both contralateral to CI device placement, given VP shunt malfunction. One of 19 adult patients (5%) required shunt revision during CI due to shunt damage noted intraoperatively. Among 43 patients with available follow-up, 38 (88%) are regular CI users, with a median consonant-nucleus vowel-consonant word: score of 58% (IQR: 28-72)., Conclusion: CI can be performed at low risk, either contralateral or ipsilateral, to a VP shunt, and does not mandate shunt revision in most cases. Additional considerations regarding CI receiver-stimulator placement are necessary with programmable shunts to mitigate device interaction. Preoperative planning, including coordination of care with neurosurgery, is important to achieving optimal outcomes., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objective: To present the surgical and quality of life (QOL) outcomes of patients who underwent blind sac closure of the external auditory canal (EAC) via a modified Rambo approach., Study Design: Retrospective case review., Setting: Tertiary academic referral center., Methods: All patients who underwent EAC closure with a modified Rambo approach between 2015 and 2021 were evaluated. Complication rates, QOL estimations from a validated survey, and subjective cosmetic reports were analyzed., Results: Thirty-five ears were closed in 31 patients. The most common indication for surgery was related to cochlear implantation and cochlear malformation or cholesteatoma (31.4%). No case involved an immediate complication requiring revision surgery, and 4 ears (11.4%) were suspected of having cholesteatoma within the surgical cavity at a mean 28.6-month follow-up. Adults (≥18 years) had significantly higher QOL scores than children in the medical resource subscale of the Chronic Ear Survey (P < .01), and patients undergoing concurrent EAC closure and skull base tumor removal scored higher than others (P = .04). Females reported higher cosmetic scores than males (P = .04). QOL and cosmetic scores compared favorably to previously published data involving the management of otologic disease., Conclusions: Ear canal closure can be a useful technique for select adult and pediatric patients. Patient and surgeon concerns regarding QOL and cosmesis in ear canal closure should be explored prior to employing this surgical technique, though the present data suggest that the modified Rambo approach to closure is generally associated with reasonable outcomes in both areas., (© 2022 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Objectives: To determine the incidence and impact of monopolar cautery use in a cohort of pediatric cochlear implant (CI) users., Study Design: Case series from a retrospective chart review and a systematic review of the literature., Setting: Tertiary academic referral center., Methods: CI patient charts from 2012 to 2021 were reviewed from a single pediatric hospital system to determine if monopolar cautery was used during a subsequent surgical procedure. In addition, a systematic review of the literature was performed to identify additional, relevant patients. Postoperative CI function was the primary outcome measure., Results: In total, 190 patients underwent a surgical procedure following cochlear implantation in a single pediatric hospital system. Fifteen patients (7.9%) and 17 distinct surgical procedures were identified in which monopolar cautery was used. Seven of these 17 cases (41.2%) involved the head and neck, and 10 were performed below the clavicles. No patients experienced a device failure or a decline in CI performance following surgery. A systematic review identified an additional 4 patients who underwent a surgery that used monopolar cautery following cochlear implantation, and no change in CI function was identified., Conclusions: The present study adds additional support to the notion that monopolar cautery does not necessarily injure CI functionality. While the most risk adverse strategy when planning a surgical procedure for a CI patient is to avoid monopolar cautery use altogether, the use of cautery should not immediately be associated with implant dysfunction., (© 2022 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Background: Large arachnoid granulations that protrude into dural venous sinuses and partially obstruct outflow are an underappreciated etiology of pulsatile tinnitus (PT). Endovascular dural venous sinus stenting is thought to diminish turbulent venous outflow and may relieve obstruction caused by arachnoid granulations., Methods: Four patients at two institutions were evaluated for unilateral PT. Magnetic resonance imaging and digital subtraction angiography revealed moderate-to-severe stenoses from large arachnoid granulations within the implicated transverse sinus. All patients underwent venous manometry and endovascular sinus stenting., Results: All patients experienced immediate and complete remission of their PT. Stenoses were relieved by a mean of 93% by Warfarin-Aspirin Symptomatic Intracranial Disease criteria. There were no procedural or periprocedural complications. All patients continued to report complete symptom resolution at a mean of 8-month follow-up., Conclusions: PT from arachnoid granulations are an underappreciated pathomechanism. Endovascular dural venous sinus stenting is an effective intervention for treating unilateral PT secondary to large arachnoid granulation., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objectives: To report 4 cases of delayed facial palsy (DFP) after pediatric middle ear (ME) surgery and systematically review and analyze the associated literature to evaluate the effects of age on DFP etiology, management, and prognosis., Methods: Systematic review of PubMed, Cochrane Library, and Embase for articles related to DFP after cochlear implantation (CI) was performed. These articles were assessed for level of evidence, methodological limitations, and number of cases. Meta-analysis was performed to assess the effects of age on DFP incidence. Furthermore, a comprehensive list of all pediatric DFP cases after otologic surgery was assembled through a multi-institutional retrospective review and systematic review of the literature., Results: Twenty-nine articles fit the criteria for inclusion in the meta-analysis. The incidence of DFP after CI was 0.23% and 1.01% for pediatric and adult cases, respectively. This difference was statistically significant ( P  < .001, odds ratio 4.36). Twenty-three cases, adding to the 4 presented herein, were suitable for a comprehensive list. The mean age was 6.9 years. Average postoperative day of paresis onset was 5.4, with an average maximum House-Brackmann grade of 3.5. All patients obtained full facial recovery after an average of 23.5 days., Conclusions: The systematic review demonstrates that DFP after pediatric CI is rare and occurs at a significantly lower rate than in adults, further supporting the viral reactivation hypothesis of DFP. The prognosis for pediatric DFP after otologic surgery is excellent, with a high rate of full recovery in a short time frame. However, steroid administration can be considered., Level of Evidence: IIa.

Objective: Vestibular schwannomas (VSs) are the most common neoplasm of the cerebellopontine angle in adults. Though these lesions are generally slow growing, their growth patterns and associated symptoms can be unpredictable, which may complicate the decision to pursue conservative management versus active intervention. Additionally, surgical decision-making can be controversial because of limited high-quality evidence and multiple quality-of-life considerations. Machine learning (ML) is a powerful tool that utilizes data sets to essentialize multidimensional clinical processes. In this study, the authors trained multiple tree-based ML algorithms to predict the decision for active treatment versus MRI surveillance of VS in a single institutional cohort. In doing so, they sought to assess which preoperative variables carried the most weight in driving the decision for intervention and could be used to guide future surgical decision-making through an evidence-based approach., Methods: The authors reviewed the records of patients who had undergone evaluation by neurosurgery and otolaryngology with subsequent active treatment (resection or radiation) for unilateral VS in the period from 2009 to 2021, as well as those of patients who had been evaluated for VS and were managed conservatively throughout 2021. Clinical presentation, radiographic data, and management plans were abstracted from each patient record from the time of first evaluation until the last follow-up or surgery. Each encounter with the patient was treated as an instance involving a management decision that depended on demographics, symptoms, and tumor profile. Decision tree and random forest classifiers were trained and tested to predict the decision for treatment versus imaging surveillance on the basis of unseen data using an 80/20 pseudorandom split. Predictor variables were tuned to maximize performance based on lowest Gini impurity indices. Model performance was optimized using fivefold cross-validation., Results: One hundred twenty-four patients with 198 rendered decisions concerning management were included in the study. In the decision tree analysis, only a maximum tumor dimension threshold of 1.6 cm and progressive symptoms were required to predict the decision for treatment with 85% accuracy. Optimizing maximum dimension thresholds and including age at presentation boosted accuracy to 88%. Random forest analysis (n = 500 trees) predicted the decision for treatment with 80% accuracy. Factors with the highest variable importance based on multiple measures of importance, including mean minimal conditional depth and largest Gini impurity reduction, were maximum tumor dimension, age at presentation, Koos grade, and progressive symptoms at presentation., Conclusions: Tree-based ML was used to predict which factors drive the decision for active treatment of VS with 80%-88% accuracy. The most important factors were maximum tumor dimension, age at presentation, Koos grade, and progressive symptoms. These results can assist in surgical decision-making and patient counseling. They also demonstrate the power of ML algorithms in extracting useful insights from limited data sets.

Objective: There is a void in the literature describing reliable surgical landmarks that aid in the dissection of the facial recess in the absence of skeletonizing the mastoid segment of the facial nerve. The posterior ligament of the incus is a readily distinguishable "white dot" along the incus buttress that has been used to guide dissection in a safe and efficient manner. The goal of our study is to describe a surgical approach that utilizes this surgical landmark to drill the facial recess and to take anatomical measurements demonstrating the safety and reliability of this approach., Materials and Methods: After cortical mastoidectomies were performed in 10 cadaveric temporal bones, the white dot was identified at the junction of short process of the incus and the incus buttress. Using the white dot for anatomical reference, a 2 mm diamond drill bit was used to open the facial recess without first identifying the facial nerve or chorda tympani nerve. After photographs were taken, the facial and chorda tympani nerves were definitively identified and skeletonized to delineate the confines of the facial recess. Photographs were once again acquired in a consistent manner for comparison. Finally, calibrated anatomic measurements were acquired from the 10 distinct image sets., Results: The facial recess was successfully drilled in 10 temporal bones using the posterior ligament as a surgical landmark without injury to the chorda tympani or facial nerve. The median angle taken from the axis of the short process of the incus to the facial nerve - chorda tympani junction was 139.2° (IQR 136.8-141). At the widest point in the facial recess, median distances anterior and posterior to an imaginary line connecting the white dot to the facial nerve - chorda tympani junction were 1.6 mm (IQR 1.5-1.7) and 1.6 mm (IQR 1.6-1.7; p = 0.57), indicating at this point, the white dot reference reliably bisects the facial recess width. Similarly, at the level of the round window niche, median anterior and posterior distances from an imaginary line connecting the white dot to the facial nerve - chorda tympani junction were 1.1 mm (IQR 1.1-1.3) and 1.3 mm (IQR 1.1-1.7; p = 0.07), respectively, once again demonstrating the white dot reliably bisecting the facial recess., Conclusions: The white dot, representing the posterior ligament of the incus, is a reliable surgical landmark that aids in safe and efficient drilling of the facial recess without first skeletonizing the facial nerve., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Meningiomas are the most common intracranial neoplasms. Although genomic analysis has helped elucidate differences in survival, there is evidence that racial disparities may influence outcomes. African Americans have a higher incidence of meningiomas and poorer survival outcomes. The etiology of these disparities remains unclear, but may include a combination of pathophysiology and other factors., Objective: To determine factors that contribute to different clinical outcomes in racial populations., Methods: We retrospectively reviewed 305 patients who underwent resection for meningiomas at a single tertiary care facility. We used descriptive statistics and univariate, multivariable, and Kaplan-Meier analyses to study clinical, radiographical, and histopathological differences., Results: Minority patients were more likely to present through the emergency department than an outpatient clinic (P < .0001). They were more likely to present with more advanced clinical symptoms with lower Karnofsky Performance scores, more frequently had peritumoral edema (P = .0031), and experienced longer postoperative stays in the hospital (P = .0053), and African-American patients had higher hospitalization costs (P = .046) and were more likely to be publicly insured. Extent of resection was an independent predictor of recurrence freedom (P = .039). Presentation in clinic setting trended toward an association with recurrence-free survival (P = .055). We observed no significant difference in gross total resection rates, postoperative recurrence, or recurrence-free survival., Conclusion: Minority patients are more likely to present with severe symptoms, require longer perioperative hospitalization, and generate higher hospitalization costs. This may be due to socioeconomic factors that affect access to health care. Targeting barriers to access, especially to subspecialty care, may facilitate more appropriate and timely diagnosis, thereby improving patient care and outcomes., (Copyright © Congress of Neurological Surgeons 2021. All rights reserved.)

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: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon., Patients: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon., Interventions: Computed tomography (CT); exploratory tympanotomy with stapedotomy., Main Outcome Measures: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively., Results: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings., Conclusions: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: To determine the value of standard clinic screening questions and vital signs in predicting abnormal vestibular function, indicated by standard objective diagnostic tests., Study Design: Retrospective records review., Methods: We reviewed electronic medical records of 150 patients seen by the neurotologists or the physician assistant they supervised, in an out-patient tertiary care clinic, between June 2018 and March 2020, and subsequently referred for the complete objective vestibular test battery (VB)., Results: Of standard questions asked during the initial exam about vertigo, disequilibrium, lightheadedness and oscillopsia, only vertigo predicted an abnormal response on the VB. More males than females had abnormal VB responses, P < .05. Pulse was not related to VB score. Significantly more subjects with blood pressure in the range for stage 2 hypertension (blood pressure [BP] stage 2) had abnormal than normal results on the VB, P < .00001. Subjects with BP stage 2 had high rates of diabetes (34.2%) and hypertension (68.4%) as diagnosed by their primary care physicians or cardiologists., Conclusion: Complaints of subjective vertigo and BP in the range of hypertension stage 2 are most likely to predict abnormal findings on the VB. Therefore, during an examination of a patient who comes in complaining of dizziness, two measures may be the most useful for screening: BP in the range of hypertension type 2, when BP is taken by a nurse, and a question to determine whether or not the patient has true vertigo., Level of Evidence: 3 Laryngoscope, 131:1382-1385, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To assess the prevalence of distress and burnout in otolaryngology trainees, including associations with relevant sociodemographic and professional factors, and to compare these results with those of attending otolaryngologists., Study Design: A cross-sectional survey of trainees and attending physicians., Setting: Twelve academic otolaryngology programs., Methods: Distress and burnout were measured with the Expanded Physician Well-being Index and the 2-item Maslach Burnout Inventory. The Patient Health Questionnaire-2 and Generalized Anxiety Disorder-2 were used to screen for depressive disorders and anxiety disorders, respectively. Associations with sociodemographic and professional characteristics were assessed., Results: Of the 613 surveys administered to trainees and attending physicians, 340 were completed (56%). Among 154 trainees, distress was present in 49%, professional burnout in 35%, positive depressive disorder screening in 5%, and positive anxiety disorder screening in 16%. In univariable analysis, female gender, hours worked in a typical week (HW), and nights on call in a typical week (NOC) were significantly associated with distress. In multivariable analysis, female gender (odds ratio, 3.91; P = .001) and HW (odds ratio for each 10 HW, 1.89; P = .003) remained significantly associated with distress. Female gender, HW, and NOC were significantly associated with burnout univariably, although only HW (odds ratio for each 10 HW, 1.92; P = .003) remained significantly associated with burnout in a multivariable setting. Attending physicians had less distress than trainees ( P = .02) and felt less callous and less emotionally hardened than trainees ( P < .001)., Conclusion: Otolaryngology trainees experience significant work-place distress (49%) and burnout (35%). Gender, HW, and NOC had the strongest associations with distress and burnout.

Objective: To ascertain the prevalence of and associations with distress and professional burnout among academic otolaryngology attending physicians., Study Design: Cross-sectional survey., Setting: Twelve US academic otolaryngology programs., Methods: A questionnaire was administered that encompassed sociodemographic and professional features, the Expanded Physician Well-being Index for distress, the 2-item Maslach Burnout Inventory for professional burnout, the Patient Health Questionnaire-2 screen for major depressive disorder, and the Generalized Anxiety Disorder-2 screen for generalized anxiety disorder., Results: The survey response rate was 56% and included 186 attending physicians. The average respondent age was 47 years; 72% were men; 93% were married or partnered; and 86% had children. Distress was present in 40%, professional burnout in 26%, positive depression screening in 8%, and positive anxiety screening in 11%. In a univariable setting, age, hours worked in a typical week, nights on call in a typical week, and years of practice were significantly associated with distress, although in a multivariable setting, only hours worked in a typical week remained significantly associated with a positive Expanded Physician Well-being Index screen (odds ratio for each 10-hour increase, 2.61; 95% CI, 1.73-3.93; P < .001). In a univariable setting, hours worked in a typical week was significantly associated with a positive Maslach Burnout Inventory screen., Conclusion: Distress or professional burnout occurs in more than a quarter of academic otolaryngology attending physicians, whereas the prevalence of depression or anxiety is approximately 10%. The number of hours worked per week had the strongest association with distress and burnout. These findings may be used to develop and implement programs to promote physician well-being and mitigate professional burnout.

Objective: To investigate the evolving prevalence of otosclerosis in a large urban population., Methods: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population., Results: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio [OR] = 3.69, [95% confidence interval [CI], 2.02-6.76], p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics., Conclusion: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The "imported" otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.

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., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To discuss the natural history and management of pneumolabyrinth, with particular attention to illustrative cases where hearing improved after a fluid-filled vestibule was restored., Patients: Adults at two academic medical centers with documented pneumolabyrinth on computed tomography (CT) imaging., Intervention: Middle ear surgery or observation., Main Outcome Measures: Cochlear and vestibular symptoms are reported. Inner ear function was documented with audiometry and vestibular testing. Resolution of pneumolabyrinth was documented on imaging for a subset of patients., Results: All patients presented with severe hearing loss following temporal bone fracture (three cases), penetrating trauma (one case), or stapedectomy (three cases). The radiographic finding was typically seen when imaging was obtained in the first few days after the injury. In two cases, vestibular symptoms resolved and partial recovery of hearing was seen despite a relatively long time interval between the inciting event and repair., Conclusion: Management of pneumolabyrinth is individualized based on the mechanism of injury, severity of symptoms, and timing of the imaging study. In most cases, the barrier between the middle ear and inner ear is expected to heal with rapid resolution of pneumolabyrinth. Late repair is demonstrated to result in favorable recovery in selected cases. The presence of air in the vestibule cannot be assumed to be indicative of irreversible inner ear damage.

Objective: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature., Methods: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases., Results: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL., Conclusions: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling., Competing Interests: Declaration of competing interest ADS is a consultant for Advanced Bionics Corp., Cochlear Corp., MED-EL Corp., and Oticon Medical. ADS is on the Surgical Advisory Board for MED-EL Corp., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients., Data Sources: A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay.", Review Methods: Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up., Results: Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%., Conclusion: Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.

Objective: Acquired encephaloceles of the temporal bone may be traumatic, spontaneous, or from chronic ear disease or previous surgery. Iatrogenic encephaloceles arise in the setting of previous mastoidectomy and traditionally involve both bony dehiscence and dural injury. We aim to classify and analyze the pathogenesis, clinical presentation, and treatment options for patients with iatrogenic tegmen defects and encephaloceles of the temporal bone., Study Design: Retrospective chart review., Setting: Single tertiary academic center., Patients: Subjects with iatrogenic tegmen defects and encephaloceles of the temporal bone were included., Intervention: Patient demographics, history, symptoms, radiographic data, intraoperative findings, management, follow-up, and outcomes were recorded., Outcome Measures: Primary outcome measures included patient characteristics, time from primary otologic surgery to surgical repair, location of the defect, and management strategy including surgical approach, methods, and follow-up., Results: Iatrogenic tegmen injuries or encephaloceles were identified in 18 patients and divided into intentional or unintentional. The latter group presented immediately, early, or late, as determined by intraoperative identification or from delayed symptoms. Eleven patients presented late with previously unrecognized encephaloceles. Compared with patients presenting with incidentally noted tegmen dehiscence during revision mastoidectomy, a significant proportion of patients with late encephaloceles had BMI ≥30 kg/m (p = 0.03)., Conclusion: The majority of iatrogenic encephaloceles are unintentional, unrecognized, and may present many years after primary surgery. Similar to spontaneous encephaloceles of the temporal bone, obesity is associated with iatrogenic encephaloceles, although the rate of progression and timing is unclear. We recommend primary repair of any unintentional tegmen defect encountered during mastoid surgery, especially in obese patients.

Mutations in the neurofibromin 2 ( NF2 ) gene were among the first genetic alterations implicated in meningioma tumorigenesis, based on analysis of neurofibromatosis type 2 (NF2) patients who not only develop vestibular schwannomas but later have a high incidence of meningiomas. The NF2 gene product, merlin, is a tumor suppressor that is thought to link the actin cytoskeleton with plasma membrane proteins and mediate contact-dependent inhibition of proliferation. However, the early recognition of the crucial role of NF2 mutations in the pathogenesis of the majority of meningiomas has not yet translated into useful clinical insights, due to the complexity of merlin's many interacting partners and signaling pathways. Next-generation sequencing studies and increasingly sophisticated NF2 -deletion-based in vitro and in vivo models have helped elucidate the consequences of merlin loss in meningioma pathogenesis. In this review, we seek to summarize recent findings and provide future directions toward potential therapeutics for this tumor.

Bone conduction implants transfer sound to the inner ear through direct vibration of the skull. In patients with skull base tumors and infections, these devices can bypass a dysfunctional ear canal and/or middle ear. Though not all skull base surgery patients opt for bone conduction hearing rehabilitation, a variety of these devices have been developed and marketed over time. This article reviews the evolution and existing state of bone conduction technology.

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: 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.

Question 1: Does intraoperative facial nerve monitoring during vestibular schwannoma surgery lead to better long-term facial nerve function?, Target Population: This recommendation applies to adult patients undergoing vestibular schwannoma surgery regardless of tumor characteristics., Recommendation: Level 3: It is recommended that intraoperative facial nerve monitoring be routinely utilized during vestibular schwannoma surgery to improve long-term facial nerve function., Question 2: Can intraoperative facial nerve monitoring be used to accurately predict favorable long-term facial nerve function after vestibular schwannoma surgery?, Target Population: This recommendation applies to adult patients undergoing vestibular schwannoma surgery., Recommendation: Level 3: Intraoperative facial nerve can be used to accurately predict favorable long-term facial nerve function after vestibular schwannoma surgery. Specifically, the presence of favorable testing reliably portends a good long-term facial nerve outcome. However, the absence of favorable testing in the setting of an anatomically intact facial nerve does not reliably predict poor long-term function and therefore cannot be used to direct decision-making regarding the need for early reinnervation procedures., Question 3: Does an anatomically intact facial nerve with poor electromyogram (EMG) electrical responses during intraoperative testing reliably predict poor long-term facial nerve function?, Target Population: This recommendation applies to adult patients undergoing vestibular schwannoma surgery., Recommendation: Level 3: Poor intraoperative EMG electrical response of the facial nerve should not be used as a reliable predictor of poor long-term facial nerve function., Question 4: Should intraoperative eighth cranial nerve monitoring be used during vestibular schwannoma surgery?, Target Population: This recommendation applies to adult patients undergoing vestibular schwannoma surgery with measurable preoperative hearing levels and tumors smaller than 1.5 cm., Recommendation: Level 3: Intraoperative eighth cranial nerve monitoring should be used during vestibular schwannoma surgery when hearing preservation is attempted., Question 5: Is direct monitoring of the eighth cranial nerve superior to the use of far-field auditory brain stem responses?, Target Population: This recommendation applies to adult patients undergoing vestibular schwannoma surgery with measurable preoperative hearing levels and tumors smaller than 1.5 cm., Recommendation: Level 3: There is insufficient evidence to make a definitive recommendation.  The full guideline can be found at: https://www.cns.org/guidelines/guidelines-manage-ment-patients-vestibular-schwannoma/chapter&#95;4., (Copyright © 2017 by the Congress of Neurological Surgeons)

Question 1: What is the overall probability of maintaining serviceable hearing following stereotactic radiosurgery utilizing modern dose planning, at 2, 5, and 10 yr following treatment?, Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is moderately high probability (>50%-75%) of hearing preservation at 2 yr, moderately high probability (>50%-75%) of hearing preservation at 5 yr, and moderately low probability (>25%-50%) of hearing preservation at 10 yr., Question 2: Among patients with AAO-HNS (American Academy of Otolaryngology-Head and Neck Surgery hearing classification) class A or GR (Gardner-Robertson hearing classification) grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing following stereotactic radiosurgery, utilizing modern dose planning, at 2, 5, and 10 yr following treatment?, Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is a high probability (>75%-100%) of hearing preservation at 2 yr, moderately high probability (>50%-75%) of hearing preservation at 5 yr, and moderately low probability (>25%-50%) of hearing preservation at 10 yr., Question 3: What patient- and tumor-related factors influence progression to nonserviceable hearing following stereotactic radiosurgery using ≤13 Gy to the tumor margin?, Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled regarding the probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut-points reported, smaller tumor size, marginal tumor dose ≤12 Gy, and cochlear dose ≤4 Gy. Age and sex are not strong predictors of hearing preservation outcome., Question 4: What is the overall probability of maintaining serviceable hearing following microsurgical resection of small to medium-sized sporadic vestibular schwannomas early after surgery, at 2, 5, and 10 yr following treatment?, Recommendation: Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled that there is a moderately low probability (>25%-50%) of hearing preservation immediately following surgery, moderately low probability (>25%-50%) of hearing preservation at 2 yr, moderately low probability (>25%-50%) of hearing preservation at 5 yr, and moderately low probability (>25%-50%) of hearing preservation at 10 yr., Question 5: Among patients with AAO-HNS class A or GR grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing following microsurgical resection of small to medium-sized sporadic vestibular schwannomas early after surgery, at 2, 5, and 10 yr following treatment?, Recommendation: Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled that there is a moderately high probability (>50%-75%) of hearing preservation immediately following surgery, moderately high probability (>50%-75%) of hearing preservation at 2 yr, moderately high probability (>50%-75%) of hearing preservation at 5 yr, and moderately low probability (>25%-50%) of hearing preservation at 10 yr., Question 6: What patient- and tumor-related factors influence progression to nonserviceable hearing following microsurgical resection of small to medium-sized sporadic vestibular schwannomas?, Recommendation: Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled regarding the probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut-points reported, smaller tumor size commonly less than 1 cm, and presence of a distal internal auditory canal cerebrospinal fluid fundal cap. Age and sex are not strong predictors of hearing preservation outcome., Question 7: What is the overall probability of maintaining serviceable hearing with conservative observation of vestibular schwannomas at 2, 5, and 10 yr following diagnosis?, Recommendation: Level 3: Individuals who meet these criteria and are considering observation should be counseled that there is a high probability (>75%-100%) of hearing preservation at 2 yr, moderately high probability (>50%-75%) of hearing preservation at 5 yr, and moderately low probability (>25%-50%) of hearing preservation at 10 yr., Question 8: Among patients with AAO-HNS class A or GR grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing with conservative observation at 2 and 5 yr following diagnosis?, Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is a high probability (>75%-100%) of hearing preservation at 2 yr, and moderately high probability (>50%-75%) of hearing preservation at 5 yr. Insufficient data were available to determine the probability of hearing preservation at 10 yr for this population subset., Question 9: What patient and tumor-related factors influence progression to nonserviceable hearing during conservative observation?, Recommendation: Level 3: Individuals who meet these criteria and are considering observation should be counseled regarding probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut-points reported, as well as nongrowth of the tumor. Tumor size at the time of diagnosis, age, and sex do not predict future development of nonserviceable hearing during observation.  The full guideline can be found at: https://www.cns.org/guidelines/guidelines-manage-ment-patients-vestibular-schwannoma/chapter&#95;3., (Copyright © 2017 by the Congress of Neurological Surgeons)

Question 1: What is the expected diagnostic yield for vestibular schwannomas when using a magnetic resonance imaging (MRI) to evaluate patients with previously published definitions of asymmetric sensorineural hearing loss?, Target Population: These recommendations apply to adults with an asymmetric sensorineural hearing loss on audiometric testing., Recommendation: Level 3: On the basis of an audiogram, it is recommended that MRI screening on patients with ≥10 decibels (dB) of interaural difference at 2 or more contiguous frequencies or ≥15 dB at 1 frequency be pursued to minimize the incidence of undiagnosed vestibular schwannomas. However, selectively screening patients with ≥15 dB of interaural difference at 3000 Hz alone may minimize the incidence of MRIs performed that do not diagnose a vestibular schwannoma., Question 2: What is the expected diagnostic yield for vestibular schwannomas when using an MRI to evaluate patients with asymmetric tinnitus, as defined as either purely unilateral tinnitus or bilateral tinnitus with subjective asymmetry?, Target Population: These recommendations apply to adults with subjective complaints of asymmetric tinnitus., Recommendation: Level 3: It is recommended that MRI be used to evaluate patients with asymmetric tinnitus. However, this practice is low yielding in terms of vestibular schwannoma diagnosis (<1%)., Question 3: What is the expected diagnostic yield for vestibular schwannomas when using an MRI to evaluate patients with a sudden sensorineural hearing loss?, Target Population: These recommendations apply to adults with a verified sudden sensorineural hearing loss on an audiogram., Recommendation: Level 3: It is recommended that MRI be used to evaluate patients with a sudden sensorineural hearing loss. However, this practice is low yielding in terms of vestibular schwannoma diagnosis (<3%).  The full guideline can be found at: https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter&#95;2., (Copyright © 2017 by the Congress of Neurological Surgeons)

Objective: To examine surgical and device-related cochlear implant practice patterns across centers in the United States., Methods: Cross-sectional survey of the American Neurotology Society (ANS)., Results: A total of 81 surveys were returned from ANS members who report regular involvement in cochlear implant care. Overall there was a broad distribution in age and clinical experience, with most respondents reporting Accreditation Council for Graduate Medical Education (ACGME) accreditation in neurotology and employment at an academic center. The annual volume of cochlear implant surgeries varied considerably across centers. Eighty percent of respondents report to offer all three Food and Drug Administration (FDA) approved device brands at their center.In cases where hearing preservation is not a goal, 56% of respondents prefer a perimodiolar electrode design, while 44% prefer a lateral wall electrode. With regard to insertion technique for scala tympani access, 64% prefer inserting through the round window (RW) membrane, 26% prefer an extended round window (ERW) approach, while only 10% prefer a cochleostomy. In cases where hearing preservation is a goal of surgery, 86% of respondents prefer a lateral wall electrode design, while only 14% prefer a perimodiolar electrode design. With regard to insertion technique, 86% prefer RW insertion, while only 9% prefer an ERW approach, and only 5% prefer a cochleostomy. Respondents who prefer RW electrode insertion more commonly use a lateral wall electrode, whereas those who prefer an ERW or cochleostomy approach more commonly use a periomodiolar electrode (p < 0.001). There was a statistically significant greater number of surgeons that prefer lateral wall electrodes (p < 0.001) and RW insertion (p < 0.001) for hearing preservation cases compared with non-hearing preservation cases. For implantation of a hypothetical patient with significant residual hearing, within the range of hybrid device candidacy, the great majority of surgeons prefer a conventional length electrode and only 29% prefer to implant a hybrid device.In cases where hearing preservation is a goal, the overwhelming majority of respondents (96%, n = 74) reported using at least one form of steroid application. Ninety-two percent (n = 71) used intraoperative intravenous steroids, 55% (n = 42) prescribed postoperative oral steroids, 44% (n = 34) bathed the middle ear with steroids during surgery, and 30% (n = 23) prescribed preoperative oral steroids.Together, 65% of respondents use a variation of the tight subperiosteal pocket technique for internal device fixation, 19% prefer a bony well and trough with tie-downs, 10% prefer a bony well and trough without tie-downs, and 5% prefer a screw fixation system. Of the 50 who prefer utilizing a subperiosteal pocket, 31 (62%) incorporate a bony trough and 19 (38%) perform a subperiosteal pocket alone without a bony trough., Conclusion: The results of this survey reflect the evolution in surgical and device-related preferences for cochlear implantation in the United States. An increasing number of surgeons prefer the RW approach for electrode insertion. When hearing preservation is not a goal, there is a near even preference for lateral wall and perimodiolar electrode designs; however, the great majority of surgeons prefer a lateral wall design when attempting to preserve residual acoustic hearing. Even in cases where substantial residual hearing exists, many surgeons prefer conventional length electrodes to hybrid designs. These changes reflect the prioritization of atraumatic surgery and parallel the development of thinner and more atraumatic electrodes.

Objectives: To test the hypothesis that bipolar electrical impedance measurements in perimodiolar cochlear implants (CIs) may be used to differentiate between perimodiolar insertion technique favoring proximity to the modiolus or lateral wall., Study Design and Methods: Bipolar impedances are a measure of electrical resistance between pairs of electrode contacts in a CI. Stimulation is through biphasic pulses at fixed frequency. Impedance measurements were made in real time through sequential sampling of electrode pairs. Perimodiolar electrodes were inserted in temporal bones using one of two techniques: 1) In the standard insertion technique (SIT), the electrode array slides along the lateral wall during insertion. 2) In the Advance Off Stylet (Cochlear Ltd. Sydney) technique (AOS), the electrode maintains modiolar contact throughout the insertion process. A set of 22 insertions were performed in temporal bone specimens using perimodiolar electrode arrays with both AOS and SIT. Buffered saline was used as a substitute for natural perilymph based on similar electrical conductivity properties. Impedance with and without stylet removal were recorded with a 30-second sampling window at final insertion depth., Results: There is a significant difference in bipolar impedance measures between AOS and SIT, with impedances rising in measurements with stylet removal. Evaluation was based on two-sided analysis of variance considering technique and electrode with P < 0.025., Conclusion: Bipolar electrical impedance can be used to detect relative motion toward the modiolus inside the cochlea. This detection method has the potential to optimize intraoperative placement of perimodiolar electrode arrays during implantation. We anticipate that this will result in lower excitation thresholds and improved hearing outcome., Level of Evidence: NA. Laryngoscope, 127:1413-1419, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: To highlight the phenomenon of cholesteatoma arising from the vascular strip following tympanoplasty and tympanomastoidectomy., Study Design: Multi-institutional retrospective chart review., Methods: Consecutive adult and pediatric patients evaluated between 2000 and 2015 with acquired cholesteatoma arising from the skin of a prior vascular strip were identified. Patients with evidence of residual or recurrent cholesteatoma elsewhere in the middle ear, mastoid, or ear canal were excluded., Results: Seventeen cases (71% female, 53% right-sided) were identified, and the mean age at presentation was 39.2 years. Patients presented on average 12.9 years following prior otologic surgery, which was most commonly tympanoplasty without mastoidectomy (59%). However, younger patients presented with symptoms sooner following prior surgery (r = 0.61, r 2 = 0.37, P = 0.0095). The most common presenting symptom was otorrhea (76%). All patients were found to have mastoid cholesteatoma with bony erosion, and three patients exhibited additional tegmen or posterior fossa bone defects. The middle ear was not involved with cholesteatoma in any case. A canal wall down procedure was performed due to extensive bony canal erosion in 29% of cases., Conclusion: The vascular strip is an uncommon source of iatrogenic cholesteatoma that can present years following an otherwise uncomplicated otologic surgery. The findings presented herein highlight the importance of careful vascular strip orientation at the conclusion of otologic surgery., Level of Evidence: 4. Laryngoscope, 127:698-701, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: To describe the clinical presentation, radiological features, and outcome of patients with plasmacytoma of the temporal bone., Methods: Multicenter retrospective case series of all patients diagnosed with plasmacytoma of the temporal bone between 1990 and 2015. Comprehensive literature review of all previously published cases., Results: A total of seven patients (average age, 57.3 yr; 57% women) met inclusion criteria. Three (43%) had a known history of multiple myeloma (MM). The most common symptoms at presentation included otalgia, headache, and dizziness (43%, each). Five (71%) presented with a vascular appearing middle ear mass visible on otoscopy. Four of these patients did not have an established history of MM and were referred with presumptive diagnoses of paraganglioma or endolymphatic sac tumor (ELST). The average maximum tumor diameter was 3.7 cm (median, 3.5 cm; range, 0.9-6.6 cm) and the most commonly involved temporal bone subsites were the middle ear and mastoid (71% each). MM was ultimately diagnosed in six cases (86%). In addition to the new cases presented herein, 18 previously published reports were analyzed. In the 25 aggregate cases, 44% of cases were associated with MM, and the mastoid (72%) and middle ear (53%) were the most commonly involved temporal bone subsites., Conclusion: Plasmacytoma of the temporal bone is rare, and the clinical presentation can closely mimic that of other primary temporal bone tumors. In patients with an established history of MM, it is imperative to have a high index of suspicion for temporal bone plasmacytoma, even when radiological features suggest an alternate diagnosis.

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.

Cholesterol granuloma is the most common primary lesion of the petrous apex. Management of petrous apex cholesterol granuloma has evolved in recent years from primary surgical treatment to conservative observation. In this article, a case of spontaneous resolution of a petrous apex cholesterol granuloma is described. To the authors' knowledge, this is the first known report of spontaneous involution of a petrous apex cholesterol granuloma. Radiographic differential diagnosis of petrous apex lesions and the natural history of cholesterol granulomas are discussed., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To evaluate the influence of marital status on sporadic vestibular schwannoma (VS) in the United States., Study Design: Analysis of the Surveillance, Epidemiology, and End Results (SEER) database., Setting: National, population-based tumor registry., Subjects and Methods: The SEER database was queried to identify all patients with sporadic VS between 2004 and 2012. Univariable and multivariable analyses were used to identify differences in tumor size at presentation, management strategy, and mortality on the basis of patient marital status., Results: Eight thousand and eight hundred thirty eight patients met inclusion criteria. When comparing patient groups on the basis of marital status, significant differences emerged. Univariable and multivariable analyses revealed that married subjects were older, had smaller tumors at presentation, and had better overall survival compared with non-married patients. After adjusting for baseline differences between groups, non-married subjects were more likely to undergo observation (odds ratio [OR] 1.2, 95% confidence interval [CI] 1.08-1.35, p = 0.009), and were less likely to undergo surgery (OR 0.85, 95% CI 0.76-0.94, p = 0.002) than married subjects, while there was no difference between groups with regard to radiation treatment (OR 0.99, 95% CI 0.88-1.11, p = 0.828). Individual differences between non-married subgroups (i.e., single, separated/divorced, and widowed) are also reported., Conclusion: Marital status influences disease presentation, treatment, and outcome in patients with sporadic VS in the United States. The authors speculate that greater social support of married subjects may drive many of these differences between groups. Future research is needed to further elucidate the underlying causes for these findings as well as the influence of other important demographic variables such as socioeconomic status and general health status.

Objectives: (1) To describe and review a single center's pediatric endoscopic cholesteatoma experience, including surgical and audiologic outcomes. (2) To assess the most common locations of residual cholesteatoma following endoscopic removal., Study Design: Case series with chart review., Setting: Tertiary otologic referral center., Subjects: Patients <19 years of age who underwent cholesteatoma removal with either endoscopic or microscopic visualization., Methods: In a comparison of patients who underwent total endoscopic ear surgery (TEES), combined endoscopic-microscopic surgery, or microscopic surgery, analyzed outcomes included locations and incidence of recurrent and residual cholesteatoma, complications, and audiometric testing., Results: Sixty-six patients (mean age, 10.9 years; range, 4-18 years; 43.4% female) with 76 ears met inclusion criteria. The average overall follow-up was 18.8 months (range, 6.7-48.3). Forty-seven (61.8%) ears underwent microscopic removal of cholesteatoma; 29 (38.1%) ears underwent combined endoscopic-microscopic removal; and 8 (10.5%) ears underwent TEES removal. Significantly more mastoidectomies were completed in microscopic cases as compared with endoscopic cases (P = .049). Though second-look procedures occurred in 15 (51.7%) endoscopic cases and 10 (21.3%) microscopic cases (P = .006), the rate of residual disease was 20.0% and 40.0% in endoscopic and microscopic cases, respectively (P = .38). When controlling for preoperative hearing, only the air-bone gap for TEES demonstrated significant improvement (P = .009). No complications were noted., Conclusion: The present report describes our experience with pediatric endoscopic cholesteatoma surgery, demonstrating similar hearing outcomes, rates of recurrence and residual disease, and complication rates as compared with traditional microscopic techniques., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.)

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., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.)