Genetic screening as an adjunct to universal newborn hearing screening: literature review and implications for non-congenital pre-lingual hearing loss.

Objective: Universal newborn hearing screening (UNHS) uses otoacoustic emissions testing (OAE) and auditory brainstem response testing (ABR) to screen all newborn infants for hearing loss (HL), but may not identify infants with mild HL at birth or delayed onset HL. The purpose of this review is to examine the role of genetic screening to diagnose children with pre-lingual HL that is not detected at birth by determining the rate of children who pass UNHS but have a positive genetic screening. This includes a summary of the current UNHS and its limitations and a review of genetic mutations and screening technologies used to detect patients with an increased risk of undiagnosed pre-lingual HL.Design: Literature review of studies that compare UNHS with concurrent genetic screening.Study sample: Infants and children with HLResults: Sixteen studies were included encompassing 137,895 infants. Pathogenic mutations were detected in 8.66% of patients. In total, 545 patients passed the UNHS but had a positive genetic screening. The average percentage of patients who passed UNHS but had a positive genetic screening was 1.4%.Conclusions: This review demonstrates the positive impact of concurrent genetic screening with UNHS to identify patients with pre-lingual HL.

Academic Benchmarks for Otolaryngology Leaders.

OBJECTIVE: This study aimed to characterize current benchmarks for academic otolaryngologists serving in positions of leadership and identify factors potentially associated with promotion to these positions. METHODS: Information regarding chairs (or division chiefs), vice chairs, and residency program directors was obtained from faculty listings and organized by degree(s) obtained, academic rank, fellowship training status, sex, and experience. Research productivity was characterized by (a) successful procurement of active grants from the National Institutes of Health and prior grants from the American Academy of Otolaryngology-Head and Neck Surgery Foundation Centralized Otolaryngology Research Efforts program and (b) scholarly impact, as measured by the h-index. RESULTS: Chairs had the greatest amount of experience (32.4 years) and were the least likely to have multiple degrees, with 75.8% having an MD degree only. Program directors were the most likely to be fellowship trained (84.8%). Women represented 16% of program directors, 3% of chairs, and no vice chairs. Chairs had the highest scholarly impact (as measured by the h-index) and the greatest external grant funding. CONCLUSION: This analysis characterizes the current picture of leadership in academic otolaryngology. Chairs, when compared to their vice chair and program director counterparts, had more experience and greater research impact. Women were poorly represented among all academic leadership positions.

Complications Associated with PROPEL Mometasone Furoate Bioabsorbable Drug-eluting Sinus Stents From 2012 to 2020.

BACKGROUND: Till date, there have been no studies that have analyzed a database to examine postmarket adverse events associated with PROPEL mometasone furoate bioabsorbable drug-eluting sinus stents. OBJECTIVE: To determine the postmarket complications associated with PROPEL mometasone furoate bioabsorbable drug-eluting sinus stents. METHODS: The US Food and Drug Administration's Manufacturer and User Facility Device Experience database was searched for adverse events associated with PROPEL bioabsorbable drug-eluting sinus stents between January 1, 2012 and December 31, 2020. Data were extracted and analyzed from medical device reports (MDRs) that involved sinus stents. RESULTS: After 47 MDRs were identified, 25 reports involving PROPEL bioabsorbable drug-eluting sinus stents were reviewed, from which 40 adverse events were recorded. Of these, there were 32 adverse events to patients and 8 device malfunctions. The most common adverse events to patients included infection (21.8%), oropharyngeal obstruction (15.6%), and headache/pain (12.5%). The most common device malfunction reported was migration and expulsion of the stent (87.5%). CONCLUSIONS: PROPEL sinus stents have been shown to be effective in preventing sinus outflow obstruction after functional endoscopic sinus surgery. Both adverse events to patients and device malfunctions are reported infrequently. A more comprehensive understanding of rare postmarket complications seen with PROPEL sinus stents may further aid informed decision-making regarding their usage.

Epithelial-Myoepithelial Carcinoma of the Salivary Glands: An Analysis of 246 Cases.

OBJECTIVE: Epithelial-myoepithelial carcinoma (EMC) is a rare neoplasm of the salivary glands. In this study, we aim to examine the demographic, clinicopathologic, and survival features of EMC using a population-based approach. STUDY DESIGN AND SETTING: Retrospective cohort study. SUBJECTS AND METHODS: The Surveillance, Epidemiology, and End Result (SEER) database (1973-2010) was queried for EMC of the major salivary glands. Data were analyzed with respect to various demographic and clinicopathologic factors. Survival was analyzed using the Kaplan-Meier and Cox proportional hazards models. RESULTS: In total, 246 cases were available for frequency analysis and 207 for survival analysis. Mean ± SD age at diagnosis was 63.8 ± 15.4 years. EMC affected females more frequently (57.3%). Distant metastases were present at diagnosis in only 4.5% of cases. Overall disease-specific survival (DSS) at 60, 120, and 180 months was 91.3%, 90.2%, and 80.7%, respectively. Patients with low-grade histology had significantly better survival at 180 months relative to those with high-grade tumors (90.6% vs 0.0%, P = .0246). When stratified by tumor size, patients with lesions >4 cm had the worst survival at 180 months (58.8%, P = .0003). All but 9 of the 207 cases available for survival analysis underwent surgery. A total of 85 patients (41.1%) received radiotherapy in addition to surgery. No survival benefit was noted for patients who received radiotherapy compared with those who did not (P = .4832). CONCLUSION: This report represents the largest series of EMC to date. Despite being regarded as a low-grade, indolent tumor, a significant fraction of our cohort underwent radiotherapy in addition to surgery, with no apparent added survival benefit.

Sinonasal extramedullary plasmacytoma: a systematic review of 175 patients.

BACKGROUND: This study reviews the published literature related to extramedullary sinonasal plasmacytomas. Clinical presentation, demographics, treatment, and outcomes of this uncommon disease are reported. METHODS: A systematic review of studies for sinonasal plasmacytomas from 1950 to 2012 was conducted. A PubMed database search, both for articles related to this condition along with bibliographies of those selected articles, was performed. Articles were examined for patient data that reported disease outcome. RESULTS: Sixty-seven journal articles were included in this analysis, comprising a total of 175 cases. Radiotherapy was the most common treatment modality, used in 89 cases, followed by a combination of surgery and radiotherapy, and surgery alone. A total of 71.8% of patients were alive after a median follow-up of 39 months, independent of treatment modality. A combination of radiotherapy and chemotherapy was rarely used but had the best treatment outcome, with 88.9% of patients (8/9 patients) alive. Of the 3 most common treatment modalities, a combination of radiotherapy and surgery had the most favorable outcomes. Sixteen patients (9.1%) converted to multiple myeloma, with the majority of these patients (75.0%) receiving radiotherapy alone as their treatment modality. CONCLUSION: This review contains the largest pool of sinonasal plasmacytoma patients to date and suggests aggressive radiotherapy is the most common treatment modality for this condition. Of the 3 most common treatment modalities, a combination of surgery and radiotherapy was shown to have the best survival outcomes.

Demographics and survival trends of sinonasal adenocarcinoma from 1973 to 2009.

BACKGROUND: The purpose of this work was to study the demographics and survival of patients diagnosed with sinonasal adenocarcinoma (SNAC) within the time period of 1973 to 2009 using the Surveillance, Epidemiology, and End Result (SEER) database. METHODS: A retrospective cohort study using the U.S. National Cancer Institute's SEER registry was performed to study the demographics and survival for SNAC from 1973 to 2009. Analysis was conducted based on race, gender, and stage. RESULTS: In total, 1270 cases of SNAC were analyzed for demographics and survival. Males accounted for 51.6% of cases, while females accounted for 48.4% of cases, amounting to a male to female ratio of 1.06:1.00. Disease specific survival at 5, 10, 15, and 20 years was 65.2%, 50.9%, 40.9%, and 36.5%, respectively. When analyzed by gender, females had higher survival than males, although this difference was not statistically significant. When analyzed by race, the category of other, which encompasses American Indian, Asian, Hispanic, and unknown or unspecified race, was shown to have the best survival, followed by whites and blacks, respectively. CONCLUSION: SNAC is a rare tumor classically associated with occupational exposure and carries a variable prognosis. This is the first dedicated large-scale, retrospective analysis of a North American SNAC population. SNAC appears to affect both males and females equally and predominantly affects whites. Patients categorized as other had significantly better survival outcomes, while gender appeared to have no significant effect on survival.

Gender differences in successful National Institutes of Health funding in ophthalmology.

OBJECTIVE: To determine whether gender differences in individual National Institutes of Health (NIH) awards and in funding totals exist in ophthalmology, and to further characterize whether factors such as experience, academic rank, and terminal degree play a role. DESIGN: A retrospective review of awards granted to primary investigators (PIs) in ophthalmology departments from 2011 through the present was conducted. PIs were classified by gender, degree, experience, and academic position. The NIH funding database was used to gather award data. SETTING: Academic medical center. RESULTS: Men had higher mean NIH awards ($418,605) than their female colleagues ($353,170; p = 0.005) and had higher total funding per PI (p = 0.004). Men had statistically higher awards at the level of assistant professor than their female counterparts (p < 0.05). A gender difference was statistically significant and most marked among researchers holding an MD (or equivalent) degree. When controlled for publication experience, men had higher NIH awards throughout their careers, although this difference only reached statistical significance on comparison of faculty with 10 or fewer years of experience. CONCLUSIONS: Male PIs receiving grants since 2011 had higher awards than their female colleagues did, most markedly among PIs in the earlier portions of their career. Differences in gender representation among senior faculty and in positions of leadership in academic ophthalmology may be partially a result of disparities in research output, as scholarly productivity is an important component of the academic advancement process in ophthalmology.

Image-guidance in endoscopic sinus surgery: is it associated with decreased medicolegal liability?

BACKGROUND: The use of image-guidance (IG) in endoscopic sinus surgery (ESS) has escalated over the last decade despite a lack of consensus that its use improves outcomes or decreases complications. One theoretical reason for using IG in ESS is its potential to minimize legal liability should an adverse outcome occur. In this study, we aimed to characterize the role of IG in ESS litigation, and further detail other factors in pertinent cases. A secondary objective was to characterize recent malpractice litigation for other relevant factors. METHODS: Relevant cases from Westlaw were examined to determine whether the use of IG played a role in initiating litigation in ESS malpractice suits. Factors such as patient demographics and alleged cause(s) of malpractice litigation were examined. RESULTS: Out of 30 malpractice cases from 2004 to April 2013, 4 (13.3%) mentioned the use of IG during ESS, although this did not appear to be a factor affecting the plaintiff's decision to initiate litigation, nor the case outcomes. In the 26 cases (86.7%) in which IG was not used, its non-use was not specified as an alleged cause of negligence. Eleven (36.7%) cases were resolved in the defendant's favor. Frequently-cited factors included iatrogenic injury (83.3%), permanent deficits (66.7%), needing additional surgery (63.3%), orbital and intracranial injury, and perceived deficits in informed consent (40.0%). CONCLUSION: The use of IG was not found to be a factor in ESS litigation. This suggests that not using IG does not necessarily make one more vulnerable to malpractice litigation.