Gene burden analysis identifies genes associated with increased risk and severity of adult-onset hearing loss in a diverse hospital-based cohort.

Loss or absence of hearing is common at both extremes of human lifespan, in the forms of congenital deafness and age-related hearing loss. While these are often studied separately, there is increasing evidence that their genetic basis is at least partially overlapping. In particular, both common and rare variants in genes associated with monogenic forms of hearing loss also contribute to the more polygenic basis of age-related hearing loss. Here, we directly test this model in the Penn Medicine BioBank-a healthcare system cohort of around 40,000 individuals with linked genetic and electronic health record data. We show that increased burden of predicted deleterious variants in Mendelian hearing loss genes is associated with increased risk and severity of adult-onset hearing loss. As a specific example, we identify one gene-TCOF1, responsible for a syndromic form of congenital hearing loss-in which deleterious variants are also associated with adult-onset hearing loss. We also identify four additional novel candidate genes (COL5A1, HMMR, RAPGEF3, and NNT) in which rare variant burden may be associated with hearing loss. Our results confirm that rare variants in Mendelian hearing loss genes contribute to polygenic risk of hearing loss, and emphasize the utility of healthcare system cohorts to study common complex traits and diseases.

Nodal Metastasis in Surgically Treated Oral Cavity Squamous Cell Carcinoma.

INTRODUCTION: Management of the neck in oral cavity squamous cell carcinoma (OCSCC) is essential to oncologic control and survival. The rates of lymph node metastasis (LNM) vary based on oral cavity tumor site and stage and influence treatment decisions. The aim of this paper was to describe clinical LNM for different tumor subsites and stages of surgically managed OCSCC. METHODS: We conducted a retrospective analysis of 25,846 surgically managed OCSCC patients from the National Cancer Database (NCDB) stratified by tumor subsite and clinical T-stage. For cN + patients, rates of pathologic LNM and absence of pathologic LNM were determined. For cN0 patients, outcomes included the rates of elective neck dissection (END) and occult LNM and predictors of occult LNM determined by a multivariable logistic regression model. RESULTS: A total of 25,846 patients (59.1% male, mean age 61.9 years) met inclusion criteria with primary tumor sites including oral tongue (50.8%), floor of mouth (21.2%), lower alveolus (7.6%), buccal mucosa (6.7%), retromolar area (4.9%), upper alveolus (3.6%), hard palate (2.7%), and mucosal lip (2.5%). Among all sites, clinical N+ rates increased with T-stage (8.9% T1, 28.0% T2, 51.6% T3, 52.5% T4); these trends were preserved across subsites. Among patients with cN + disease, the overall rate of concordant positive pathologic LNM was 80.1% and the rate of discordant negative pathologic LNM was 19.6%, which varied based on tumor site and stage. In the overall cohort of cN0 patients, 59.9% received END, and the percentage of patients receiving END increased with higher tumor stage. Occult LNM among those cN0 was found in 25.1% of END cases, with the highest rates in retromolar (28.8%) and oral tongue (27.5%) tumors. Multivariable regression demonstrated significantly increased rates of occult LNM for higher T stage (T2 OR: 2.1 [1.9-2.4]; T3 OR: 3.0 [2.5-3.7]; T4 OR: 2.7 [2.2-3.2]), positive margins (OR: 1.4 [1.2-1.7]), and positive lymphovascular invasion (OR: 5.1 [4.4-5.8]). CONCLUSIONS: Management of the neck in OCSCC should be tailored based on primary tumor factors and considered for early-stage tumors.

Pure tone audiometry as assessed by a commercially-available mobile phone application compared to formal audiometry.

PURPOSE: Comparison of audiometric measurements of commercially available smartphone audiogram application thresholds as compared to gold standard audiometric evaluation. MATERIALS AND METHODS: A single-institution, original contribution. Ninety consecutive adult patients presenting to a tertiary care auditory clinic with auditory complaints were evaluated using standard audiometric testing and an application-based hearing test. Correlation between app results and standard audiogram for air conduction pure tone thresholds was evaluated. RESULTS: Mimi™ (Berlin, Germany) results for audiometric thresholds were moderately correlated with standard audiogram (r = 0.51-0.68) depending on severity. The percentage of patients whose hearing loss severity on formal audiometry results were accurately reflected in the Mimi™ (app-based hearing test: ABHT)1 results ranged from 18.2 to 80 %. Among patients whose results were at the extremes of hearing performance, app and standard audiogram results were similar. ABHT yielded an overall sensitivity of 35.5 % and specificity of 97.1 % for normal hearing, and an overall sensitivity of 80 % and specificity of 96 % for severe hearing loss. CONCLUSIONS: Results from an audiometric smart phone application showed accurate categorization of hearing loss at the high and extremes as compared to standard audiometry. However, correlation of pure tone values was more variable and dependent on hearing level.

Role of elective neck dissection and adjuvant radiation therapy in patients with polymorphous adenocarcinoma.

PURPOSE: To evaluate the role of elective neck dissection (END) and of adjuvant radiation (aRT) in polymorphous adenocarcinoma (PAC), previously known as polymorphous low-grade adenocarcinoma (PLGA). METHODS: Retrospective cohort study of patients in the National Cancer Database with a histology of PAC (coded as PLGA) at a head and neck site diagnosed between 2004 and 2015. Multivariable Cox proportional hazard modeling was used to assess overall survival in the overall population, and in sub-analyses of clinically N0 disease, positive resection margins, and late stage disease. RESULTS: A total of 922 patients [66.8% female; mean (SD) age, 60.9 (13.9) years] met inclusion criteria. 74.7% of patients received surgery alone, and 18.0% received surgery and aRT. Only 7.6% of patients with clinically N0 disease received an END, with 10.6% of these having at least one positive node. END did not have a survival benefit compared to no END [HR 1.28 (0.61-2.68)]. Compared to surgery alone, aRT did not have significantly increased survival in the overall population or in late stage [HR 0.68 (0.39-1.19) and HR 0.46 (0.18-1.22), respectively]. On sub-analysis of patients with positive resection margins, aRT had a significant survival benefit compared to surgery alone [HR 0.37 (0.14-0.99)]. CONCLUSION: PAC is a rare, slow-growing malignant tumor typically treated with surgical excision, with undefined indications for END or aRT. Our findings show END to not have a benefit to overall survival. In patients with positive resection margins, there was a survival benefit for aRT.

Social determinants of health and survivorship in parotid cancer: An analysis of the National Cancer Database.

BACKGROUND: Given the rarity of parotid cancer, there is relatively few data published regarding outcomes. Utilizing the large sample population of the National Cancer Database (NCDB), we aim to examine the relationship between two key social determinants of health, demographics and socioeconomic status (SES), and parotid malignancy survival rates. METHODS: Our analytic sample consists of patients with a diagnosis of primary malignancy of the parotid gland between 2004 and 2012 in the NCDB. We used univariable and multivariable Cox proportional hazard models to evaluate the relationship between overall survival rate and two key social determinants of health: demographics and SES. RESULTS: 15,815 cases met inclusion criteria. Average age was 60.1 years and 8255 were male (52.2%). Median overall survival was 121 months with 5-year overall survival of 67.4%. Male sex and older age at diagnosis were associated with poorer overall survival (p < 0.0001). We found that Asian Americans compared to whites had better overall survival (HR 0.75; 95% CI [0.58-0.95]). Black patients had improved survival compared to whites on univariate (HR 0.71; 95% CI [0.64-0.79]); but not multivariate analysis. Hispanic ethnicity and higher education level were protective (HR 0.76 95% CI [0.63-0.91] and HR 0.84 95% CI [0.74-0.96], respectively). We found no significant survival association based on income level. CONCLUSION: In this national sample of patients with parotid malignancy, a rare form of cancer, we found a significant correlation between important social determinants of health and overall survival rate. Females, Asian-Americans, Hispanics, and patients with higher education level have better overall survival.

Impact of payer status on survival in parotid malignancy.

OBJECTIVE: In the setting of current national healthcare reform, it becomes especially relevant to understand the current state of healthcare disparities with regards to insurance status. To determine the impact of payer status on survival in parotid malignancy, we utilized the National Cancer Database (NCDB). STUDY DESIGN: Retrospective database review. SETTING: National Cancer Database (2004-2012). SUBJECTS AND METHODS: The NCDB was queried for cases of primary malignancy of the parotid gland between 2004 and 2012. The impact of payer status on overall survival was evaluated, as well as the relationship of insurance status with patient and tumor variables. RESULTS: 15,815 cases met inclusion criteria. A majority had private insurance (47.8%), followed by Medicare (40.9%), Medicaid (5.0%), uninsured (3.2%) and other government sources (1.3%). Medicare patients had the lowest 5 and 10-year survival rates (50.7% (95% CI [49.3-52.1]) and 27.8% (95% CI [25.0-30.9]), respectively). On multivariable analysis, uninsured, Medicare, and Medicaid patients had worse overall survival than the privately insured (HR 1.42, 95% CI [1.17-1.74]; HR 1.29, 95% CI [1.17-1.42]; HR 1.36, 95% CI [1.13-1.62], respectively). Uninsured and Medicaid patients were more likely than the privately insured to present with advanced stage disease, nodal metastasis and longer times to treatment following diagnosis. CONCLUSION: In parotid malignancy, uninsured, Medicaid, and Medicare patients have worse survival outcomes compared to those with private insurance. Uninsured and Medicaid patients also present with more advanced stage disease and have increased wait times before definitive treatment is initiated.

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

Challenges and opportunities in developing targeted molecular imaging to determine inner ear defects of sensorineural hearing loss.

The development of inner ear gene carriers and delivery systems has enabled genetic defects to be repaired and hearing to be restored in mouse models. Today, promising advances in translational therapies provide confidence that targeted molecular therapy for inner ear diseases will be developed. Unfortunately, the currently available non-invasive modalities, such as Computerized Tomography scan or Magnetic Resonance Imaging provide insufficient resolution to identify most pathologies of the human inner ear, even when the current generation of contrast agents is utilized. The development of targeted contrast agents may play a critical role in determining the cause of, and treatment for, sensorineural hearing loss. Such agents should be able to pass through the cochlea barriers, possess minimal cytotoxicity, and easily conjugate to a targeting agent, without distorting the anatomic details. This review focuses on a series of contrast agents which may fit these criteria for potential clinical application.

Cribriform adenocarcinoma of the tongue and minor salivary gland: transoral robotic surgical resection.

BACKGROUND/AIMS: Cribriform adenocarcinoma of the tongue and minor salivary gland (CATMSG) is a rare, locally invasive, and poorly recognized tumor, typically occurring on the base of the tongue. This case report describes the previously unreported use of transoral robotic surgery (TORS) for the local resection of CATMSG in a novel location, the palatine tonsil, and leverages follow-up information to compare TORS to conventional surgical approaches. METHODS: We performed transoral radical tonsillectomy, limited pharyngectomy, and base-of-tongue resection with staged left selective neck dissection. RESULTS: Tumor pathology revealed an infiltrating salivary gland carcinoma with perineural invasion and a histologically similar adenocarcinoma in 1 of 64 left neck lymph nodes. TORS was performed with no perioperative complications, and the patient was subsequently discharge on postoperative day 3 with a Dobhoff tube. Postoperatively, the Dobhoff tube was removed at 1 month, the patient was advanced to soft foods by mouth at 2 months, and 3-month positron emission tomography-computed tomography scan showed no evidence of distant metastases and evolving postsurgical changes in the left tonsillectomy bed. CONCLUSIONS: This case report highlights the use of TORS resection with minimal acute and long-term morbidity compared to conventional approaches for the resection of this rare, locally invasive salivary gland carcinoma in the palatine tonsil.

An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024.

Development and Initial Validation of a Meniere's Disease Quality of Life Instrument: The MenQOL.

OBJECTIVE: To design and validate a disease-specific quality of life instrument for Meniere's disease. METHODS: We used a sequential process of expert input, patient focus groups, and analyses of responses to draft questionnaires to create a 24-item Meniere's disease quality of life (MenQOL) instrument. The MenQOL and the SF-36v2 were administered to a cohort of 50 patients with Meniere's disease and 60 comparison patients with tinnitus, vertigo, or hearing loss from other causes identified at a tertiary academic center. We performed exploratory factor analysis, Cronbach's α, between group comparisons of total MenQOL scores, and regression analyses between the MenQOL and SF-36v2 to evaluate the instrument's factor structure, internal consistency, face validity, and external validity. Segregation of the instrument into domains was assessed by exploratory factor analysis. RESULTS: Exploratory factor analysis revealed that the MenQOL has a single domain. Cronbach's α = 0.914 indicated high internal consistency for the instrument as a whole. Mean MenQOL scores showing significantly worse quality of life among patients with Meniere's disease than comparison participants (52.5 ± 15.8 vs. 43.2 ± 12.6; p = 0.0051), indicating good construct validity. Significant inverse relationships in bivariate linear regressions between total MenQOL scores and SF-36v2 physical (slope = -0.94, p < 0.0001) and mental (slope = -1.16, p < 0.0001) composite scores showed acceptable concurrent validity. CONCLUSIONS: We have described the initial development of the MenQOL, a simple, valid patient-reported outcome measure that, subject to further study, may be used to assess the effects of treatment on disease-specific quality of life in patients with Meniere's disease. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Surgical Resection Improves Overall Survival in cT4b Major Salivary Gland Cancer.

OBJECTIVE: To compare surgical and nonsurgical definitive treatment in cT4b major salivary gland cancer (MSGC). STUDY DESIGN: Retrospective cohort study. SETTING: The 2004 to 2019 National Cancer Database. METHODS: The NCDB was queried for patients with cT4b MSGC (N = 976). Patients undergoing definitive treatment with (1) surgical resection + adjuvant therapy, (2) radiotherapy (RT) alone, or (3) chemoradiotherapy (CRT) were included in Kaplan-Meier and Cox survival analyses. RESULTS: Of 219 patients undergoing definitive treatment, 148 (67.6%) underwent surgical resection + adjuvant therapy and 71 (32.4%) underwent RT or CRT. There were no documented mortalities within 90 days of surgical resection. Tumor diameter and nodal metastasis were associated with decreased odds of undergoing definitive treatment (P < 0.025). Patients with positive surgical margins (PSM) had higher 5-year overall survival (OS) than those undergoing definitive RT or CRT (48.5% vs 30.1%, P = 0.018) and similar 5-year OS as those with negative margins (48.5% vs 54.0%, P = 0.205). Surgical resection + adjuvant therapy (adjusted hazard ratio: 0.55, 95% confidence interval [CI]: 0.37-0.84) was associated with higher OS than definitive RT or CRT (P < 0.025). A separate cohort of 961 patients with cT4a tumors undergoing surgical resection + adjuvant therapy was created; cT4a and cT4b (hazard ratio: 1.02, 95% CI: 0.80-1.29, P = 0.896) tumors had similar OS. CONCLUSION: A minority of patients with cT4b MSGC undergo definitive treatment. Surgical resection + adjuvant therapy was safe and associated with higher OS than definitive RT or CRT, despite high rate of PSM. In the absence of clinical trial data, appropriately selected patients with cT4b MSGC may benefit from surgical resection.

Elective Neck Dissection in cT1-4 N0M0 Head and Neck Basaloid Carcinoma.

OBJECTIVE: To investigate the survival benefit of elective neck dissection (END) over neck observation in surgically resected cT1-4 N0M0 head and neck basaloid carcinoma (HNBC). STUDY DESIGN: Retrospective cohort study. SETTING: The 2006 to 2017 hospital-based National Cancer Database. METHODS: Patients with surgically resected cT1-4 N0M0 HNBC were selected. Linear, binary logistic, Kaplan-Meier, and Cox proportional hazards regression models were implemented. RESULTS: Of 857 patients satisfying inclusion criteria, the majority were male (77.0%) and white (88.1%) with disease of the oral cavity (21.5%) or oropharynx (42.9%) classified as high grade (76.9%) and cT1-2 (72.9%). 389 (45.4%) patients underwent END. END utilization between 2006 and 2017 increased for cT1-2 disease (33.3% vs 56.9%, R2 = .699) but remained relatively constant for cT3-4 disease (66.7% vs 57.9%, R2 = .062). One-hundred and fifteen (29.6%) ENDs detected occult nodal metastases (ONMs). The 5-year overall survival (OS) of patients undergoing neck observation and END was 65.6% and 66.8%, respectively (P = .652). END was not associated with improved OS in survival analyses stratified by patient demographics, clinicopathologic features, and adjuvant therapy. Compared with surgery alone, adjuvant radiotherapy (adjusted hazard ratio: 0.74, 95% confidence interval [CI]: 0.57-0.97, P = .031) was associated with improved OS. END (hazard ratio [HR]: 0.96, 95% CI: 0.71-1.28, P = .770) and ONM (HR: 1.12, 95% CI: 0.78-1.61, P = .551) were not associated with OS. CONCLUSION: END is performed in nearly half of patients with HNBC but is not associated with improved OS, even after stratifying survival analyses by patient demographics, clinicopathologic features, and adjuvant therapy. The rate of ONM approaching 30%, however, justifies inclusion of END in the surgical management of HNBC.

Socioeconomic determinants impact quality of life at vestibular schwannoma diagnosis.

BACKGROUND: Socioeconomic variables including race, education, and income have been shown to affect vestibular schwannoma incidence, treatment, and outcomes. We sought to determine the impact of socioeconomic factors on quality of life at the time of vestibular schwannoma diagnosis. METHODS: Retrospective cohort study conducted at a tertiary academic center. All patients evaluated for vestibular schwannoma from March 1, 2010 to December 31, 2021 who completed at least one Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire at presentation or prior to any intervention were included. PANQOL scores were compared across income quintiles, racial groups, and health insurance categories. RESULTS: Two-hundred and ninety-six patients who had non-missing information on variables of interest were included. Compared to White/Caucasian patients (84.5 %), Black/African American patients (4.7 %) had significantly lower PANQOL total scores (b = -12.8[-21.7, -4.0], p = 0.005). Compared to patients with Commercial insurance (53 %), patients who were Uninsured/ Self-pay (1.7 %) had significantly lower PANQOL total scores (b = -16.7[-31.4, -1.9], p = 0.027). Patients in higher income quintiles had significantly higher PANQOL total scores (b = 11.7[3.9, 19.5], p = 0.004 comparing highest income quintile to lowest). After controlling for potential confounders, income quintile (b = 9.6[1.3, 17.9], p = 0.023 comparing highest quintile to lowest) and insurance (b = -17.0[-31.9, -21], p = 0.025 comparing Uninsured/Self-pay to Commercial insurance) remained predictors of total PANQOL score. CONCLUSIONS: Socioeconomic factors including race, health insurance, and income appear to contribute to quality of life at the time of vestibular schwannoma diagnosis. These variables are interrelated and the effects of race may be mediated in part by differences in income and health insurance coverage.

Primary Site Surgery in Distantly Metastatic Oropharyngeal Squamous Cell Carcinoma.

OBJECTIVES: Determine if intensive local therapy (i.e., local surgery or radiation) has a survival benefit for patients presenting with distantly metastatic oropharyngeal squamous cell carcinoma (OPSCC). METHODS: Retrospective population-based cohort study of patients in the National Cancer Database presenting with distantly metastatic OPSCC. Overall survival (OS) was compared for patients receiving systemic therapy alone or in combination with local surgery or curative dose radiation, controlling for various clinicodemographic factors. RESULTS: Between 2010 and 2015, 627 patients presented with newly diagnosed, metastatic OPSCC and an initial treatment course including systemic chemotherapy. Multivariable analysis demonstrated that local radiation therapy was independently associated with improved OS (OR 0.64, CI [0.51-0.81]); local surgery was not independently associated with improved OS (OR 0.99, CI [0.65-1.53]). Higher T stages were associated with worse OS (OR 1.69, CI [1.14-2.50] for T3 and OR 1.77, CI [1.22-2.58] for T4 compared to T1). HPV-positive (HPV+) tumors were associated with improved OS compared to HPV- (OR 0.79, CI [0.64-0.97]). Multiagent chemotherapy was associated with improved OS compared to single-agent (OR 0.78, CI [0.62-1.00]). The best survival for the entire cohort and for HPV+ patients was for radiation with systemic therapy and the worst survival for systemic therapy alone. CONCLUSIONS: Curative dose local radiotherapy in addition to systemic therapy is associated with improved OS compared to systemic therapy alone in patients presenting with distantly metastatic OPSCC. There is not a significant survival benefit for local surgery in addition to systemic therapy in this patient population, regardless of HPV status. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:2243-2251, 2024.

Predicting reoperation and readmission for head and neck free flap patients using machine learning.

BACKGROUND: To develop machine learning (ML) models predicting unplanned readmission and reoperation among patients undergoing free flap reconstruction for head and neck (HN) surgery. METHODS: Data were extracted from the 2012-2019 NSQIP database. eXtreme Gradient Boosting (XGBoost) was used to develop ML models predicting 30-day readmission and reoperation based on demographic and perioperative factors. Models were validated using 2019 data and evaluated. RESULTS: Four-hundred and sixty-six (10.7%) of 4333 included patients were readmitted within 30 days of initial surgery. The ML model demonstrated 82% accuracy, 63% sensitivity, 85% specificity, and AUC of 0.78. Nine-hundred and four (18.3%) of 4931 patients underwent reoperation within 30 days of index surgery. The ML model demonstrated 62% accuracy, 51% sensitivity, 64% specificity, and AUC of 0.58. CONCLUSION: XGBoost was used to predict 30-day readmission and reoperation for HN free flap patients. Findings may be used to assist clinicians and patients in shared decision-making and improve data collection in future database iterations.

Choice of Adjuvant Radiotherapy Facility in Major Salivary Gland Cancer.

OBJECTIVE: Undergoing surgery and adjuvant radiotherapy (aRT) at the same facility has been associated with higher overall survival (OS) in head and neck squamous cell carcinoma. Our study investigates whether undergoing surgery and aRT at the same academic facility is associated with higher OS in major salivary gland cancer (MSGC). METHODS: The 2006-2018 National Cancer Database was queried for patients with MSGC undergoing surgery at an academic facility and then aRT. Multivariable binary logistic and Cox proportional hazards regression models were implemented. RESULTS: Of 2801 patients satisfying inclusion criteria, 2130 (76.0%) underwent surgery and aRT at the same academic facility. Residence in a less populated area (adjusted odds ratio [aOR] 1.69, 95% confidence interval [CI] 1.16-2.45), treatment without adjuvant chemotherapy (aOR 1.97, 95% CI 1.41-2.76), and aRT duration (aOR 1.02, 95% CI 1.01-1.04) were associated with undergoing surgery and aRT at different facilities on multivariable logistic regression adjusting for patient demographics, clinicopathologic features, and adjuvant therapy (p < 0.01). Five-year OS was higher in patients undergoing surgery and aRT at the same academic facility (68.8% vs. 61.9%, p < 0.001). Undergoing surgery and aRT at different facilities remained associated with worse OS on multivariable Cox regression (aHR 1.41, 95% CI 1.10-1.81, p = 0.007). CONCLUSION: Undergoing surgery and aRT at the same academic facility is associated with higher OS in MSGC. Although undergoing surgery and aRT at the same academic facility is impractical for all patients, academic physicians should consider same-facility treatment for complex patients who would most benefit from clear multidisciplinary communication. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3620-3632, 2024.

An Update on the Epidemiology and Clinicodemographic Features of Meniere's Disease.

OBJECTIVE: To characterize the estimated prevalence and clinicodemographic features of Ménière's disease (MD) using current diagnostic criteria. METHODS: A cross-sectional study was undertaken at our tertiary academic referral center. All patients seen in Otolaryngology clinic with ICD-10 diagnoses of MD, from January 1, 2013 to July 31, 2022 were identified. Chart review was undertaken to determine the estimated prevalence of MD meeting AAO-HNS diagnostic criteria. Clinicodemographic features were evaluated against a comparator group without MD seen in our health system. RESULTS: Of 806 ICD-10 diagnoses of MD, we identified 480 MD cases meeting diagnostic criteria (168 definite). Mean age at presentation for MD cases was 49 years. Forty-seven percent of cases were male. A significantly higher proportion of MD cases than comparators were white (76% vs. 66%, p < 0.001). Mean time since MD symptom onset was 6.7 years, with a mean attack duration of 4.6 h; 7.5% of MD cases reported a positive family history, and 7% had bilateral disease. The odds of reporting migraine were significantly greater among MD patients than comparators (OR 1.74 [1.26-2.42]); the odds of having autoimmune conditions were lower (OR 0.45 [0.28-0.74]); and the odds of reporting allergies were no different (OR 0.96 [0.74-1.25]) versus comparator patients after controlling for demographic characteristics. CONCLUSIONS: Among MD diagnoses, there is a low estimated prevalence of MD meeting diagnostic criteria, and an even lower prevalence of definite MD. Compared to a comparator group of patients seen for any disorder, patients with MD are more likely to be white, male, and have a history of migraine. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3310-3315, 2024.

Reassessing the Utility of Surgical Intervention for Skull Base Osteomyelitis: A 16-Year Experience.

OBJECTIVE: The role of surgery in lateral skull base osteomyelitis (SBO) is controversial. Surgical intervention is often requested by consulting services in the interest of additional culture data to inform medical management. However, whether surgery alters subsequent antibiotic treatment or modifies disease outcome remains unknown. The aim of this study was to investigate the role of surgical intervention in the treatment of SBO by (1) comparing nonsurgical and surgical culture data and (2) assessing clinical outcomes and treatment course following surgical intervention. STUDY DESIGN: Retrospective. SETTING: Tertiary care center. METHODS: The electronic record was queried for all patients with SBO who presented to a single institution over a 16-year period (2007-2023). Information recorded included history and exam, bedside and intraoperative culture data, antibiotic course, and disease outcomes. Primary outcome measures included change in medical management based on intraoperative cultures, recurrence rates, and mortality rates. RESULTS: Forty patients (41 ears, average age 73 ± 13 years) met inclusion criteria. Out of 13 (32%) patients who underwent surgical intervention, one intraoperative culture changed the antibiotic course due to identification of resistance to the original antibiotic used. Surgery did not demonstrate a benefit in overall mortality (23% vs 18%, P = 0.36) or facial nerve function (33% vs 50%, P = 0.56) compared to medical management, and was associated with increased recurrence rates (54% vs 11%, P = 0.05). CONCLUSION: Surgical cultures rarely changed antibiotic selection. Surgical debridement in treatment-refractory SBO was also not associated with improvement in recurrence or mortality rates, though this may reflect underlying differences in disease severity.

Head and Neck Paragangliomas: Overview of Institutional Experience.

Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with 69.0% female and 26.5% patients with multiple PGLs. Among 119 patients undergoing genetic testing, 70 (58.8%) patients had mutations, with SDHB (30) and SDHD (26) being the most common. The rates of metastasis and recurrence were higher among patients with SDHB mutations or SDHD mutations associated with multiple PGLs. Metabolic evaluation showed elevated plasma dopamine levels were the most common derangements in HNPGL. MRI and CT were the most common anatomic imaging modalities and DOTATATE was the most common functional scan used in this cohort. Most patients (81.5%) received surgery as the primary definitive treatment, while 22.5% patients received radiation treatment, mostly as an adjuvant therapy or for surgically challenging or inoperable cases. Systemic treatment was rarely used in our cohort. Our single-center experience highlights the need for referral for genetic testing and metabolic evaluation and for a team-based approach to improve the clinical outcomes of patients with HNPGLs.