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BackgroundMénière's disease (MD) is a disorder of the inner ear that causes episodic bouts of severe dizziness, roaring tinnitus, and fluctuating hearing loss. To date, no targeted therapy exists. As such, we have undertaken a large whole genome sequencing study on carefully phenotyped unilateral MD patients with the goal of gene/pathway discovery and a move towards targeted intervention. This study was a retrospective review of patients with a history of Ménière's disease. Genomic DNA, acquired from saliva samples, was purified and subjected to whole genome sequencing.ResultsStringent variant calling, performed on 511 samples passing quality checks, followed by gene-based filtering by recurrence and proximity in molecular interaction networks, led to 481 high priority MD genes. These high priority genes, including MPHOSPH8, MYO18A, TRIOBP, OTOGL, TNC, and MYO6, were previously implicated in hearing loss, balance, and cochlear function, and were significantly enriched in common variant studies of hearing loss. Validation in an independent MD cohort confirmed 82 recurrent genes. Pathway analysis pointed to cell-cell adhesion, extracellular matrix, and cellular energy maintenance as key mediators of MD. Furthermore, the MD-prioritized genes were highly expressed in human inner ear hair cells and dark/vestibular cells, and were differentially expressed in a mouse model of hearing loss.ConclusionBy enabling the development of model systems that may lead to targeted therapies and MD screening panels, the genes and variants identified in this study will inform diagnosis and treatment of MD.

OBJECTIVES: This study aimed to evaluate the outcomes of a hands-on simulation-based course with emphasis on procedural techniques, clinical reasoning, and communication skills developed to improve junior Otolaryngology - Head and Neck Surgery (OHNS) residents preparedness in managing otolaryngologic emergencies. METHODS: Junior OHNS residents and faculty from residency programs in California, Nevada, and Arizona participated in this workshop in 2020 and 2021. The stations featured airway management techniques, ultrasound-guided needle aspiration, nasoseptal hematoma evacuation, and facial fracture repair using various models and cadavers. Participants completed a pre-workshop survey, post-workshop survey, and 2-month follow-up survey that assessed resident anxiety and confidence in three OHNS emergency situations across knowledge, manual skills, and teamwork using a 5-point Likert scale. RESULTS: Pre-workshop surveys reported the least anxiety and most confidence in teamwork, but the most anxiety and least confidence in technical skills and knowledge related to foreign body retrieval and airway management. Immediately post-workshop participants reported significant reductions in anxiety and increases in confidence, largest in the manual skills domain, in foreign body retrieval (anxiety: -0.99, confidence: +0.95, p

The national policy context regarding transgender students' rights has been volatile in recent years. New York State's Department of Education has a transgender policy in place, yet local school districts around New York State do not. This qualitative case study examined how district and school staff perceived the knowledge and effectiveness of transgender policies. Findings suggest a policy breakdown created by key stakeholders at both the district and school levels that leave transgender students vulnerable.

Objectives  Dispersion of bone dust in the posterior fossa during retrosigmoid craniectomy for vestibular schwannoma (VS) resection could be a source of meningeal irritation and lead to development of persistent postoperative headaches (POH). We aim to determine risk factors, including whether the presence of bone spicules that influence POH after retrosigmoid VS resection. Design  Present study is a retrospective case series. Setting  The study was conducted at a tertiary skull-base referral center. Participants  Adult patients undergoing VS resection via a retrosigmoid approach between November 2017 and February 2020 were included for this study. Main Outcome Measures  Development of POH lasting ≥ 3 months is the primary outcome of this study. Results  Of 64 patients undergoing surgery, 49 had complete data (mean age, 49 years; 53% female). Mean follow-up time was 2.4 years. At latest follow up, 16 (33%) had no headaches, 14 (29%) experienced headaches lasting

Objectives/hypothesisTo better understand the obstacles facing residents and K-awardee faculty in choosing a clinician-scientist career in otolaryngology.Study designAnonymous survey.MethodsAn anonymous, online Qualtrics survey was sent to residents participating in T32 training grants and K-awardee junior faculty. The survey was sent to the residents with the permission of their program chair. The results of this survey were compiled and analyzed. A separate survey was sent to current K-awardees in U.S. academic programs. Both surveys were then compared to a survey published in 2008 to determine if the concerns and obstacles faced by aspiring clinician-scientists are still present.ResultsResidents felt that combining a research and clinical career presented many obstacles, including a lower salary, competition with PhDs for grant funding, and the lack of departmental support. Prolonging their training to include a fellowship was not a deterrent. Family/spousal issues which ranked as the primary concern previously were no longer given the same level of importance. The major concerns of K-awardees were the economic disparity of clinician-scientists with their clinical counterparts, the lack of mentors, and department support. Forty percent received their K-award after first try, 100% after two revisions, and one has received an R grant funding.ConclusionsThe obstacles facing clinician-scientists in otolaryngology are highlighted by this survey and require attention by our academic programs, National Institutes of Health, and specialty societies.Level of evidenceNA Laryngoscope, 132:1555-1560, 2022.

Purpose of reviewThis review briefly covers the history of stapedectomy, discusses the indications and problems encountered with revision surgery, and provides case examples with solutions.Recent findingsRevision surgery is challenging and successful outcome even in the most experienced specialists is 45-71%, which is far less than that of primary surgery.SummaryCareful evaluation of the reasons for reoperation, anticipation of the common problems, and patient education on reasonable expectations are all very important for success.

ObjectiveWe describe three rare cases of spontaneous intracranial hypotension (SIH) presenting with symptoms of endolymphatic hydrops (EH) and perform a literature review to bring attention to a rare link between SIH and EH.PatientA 59-year-old female presented with postural headache, aural fullness, vertigo, hearing loss, and abnormal electrocochleography after being diagnosed with SIH by magnetic resonance imaging. The site of cerebrospinal fluid leak was identified in this individual. Two additional patients with vertigo, hearing loss, and SIH were identified by retrospective chart review.InterventionAll patients underwent blood patches. One patient also had diuretic treatment while another had fibrin glue injection.Main outcome measuresThe outcomes of interest were resolution of headache, vertigo, aural fullness, and hearing loss.ResultsAll patients eventually improved with time. Literature review suggests that overall outcome is excellent.ConclusionsSIH may be an under-recognized cause of EH. We support the theory that negative intracranial pressure transmitted through the cochlear aqueduct and perilymph leads to EH. Despite the variations in treatments, the overall prognosis is excellent.

BackgroundHearing impairment is prevalent among older adults and has been identified as a risk factor for cognitive impairment and dementia. We evaluated the association of hearing impairment with long-term cognitive decline among community-dwelling older adults.MethodsA population-based longitudinal study of adults not using hearing aids who had hearing acuity and cognitive function assessed in 1992-1996, and were followed for a maximum of 24 years with up to five additional cognitive assessments. Hearing acuity was categorized based on pure-tone average (PTA) thresholds: normal (PTA ≤ 25 dB), mild impairment (PTA > 25-40 dB), moderate/severe impairment (PTA > 40 dB).ResultsOf 1,164 participants (mean age 73.5 years, 64% women), 580 (49.8%) had mild hearing impairment and 196 (16.8%) had moderate/severe hearing impairment. In fully adjusted models, hearing impairment was associated with steeper decline on the Mini-Mental State Examination (MMSE) (mild impairment β = -0.04, p = .01; moderate/severe impairment β = -0.08, p = .002) and Trails B (mild impairment β = 1.21, p = .003; moderate/severe impairment β = 2.16, p = .003). Associations did not differ by sex or apolipoprotein E (APOE) ϵ4 status and were not influenced by social engagement. The MMSE-hearing association was modified by education: mild hearing impairment was associated with steeper decline on the MMSE among participants without college education but not among those with college education. Moderate/severe hearing impairment was associated with steeper MMSE decline regardless of education level.ConclusionsHearing impairment is associated with accelerated cognitive decline with age, and should be screened for routinely. Higher education may provide sufficient cognitive reserve to counter effects of mild, but not more severe, hearing impairment.

ImportanceOtosclerosis can be managed through surgical treatment, such as stapedectomy, or through hearing amplification with hearing aids. To our knowledge, there has been no cost-effectiveness analysis of these 2 treatment methods.ObjectiveTo determine the cost-effectiveness of stapedectomy vs hearing aid use for the treatment of otosclerosis.Design and settingIn this cost-effectiveness analysis, a decision tree was built to model the treatment choices for otosclerosis. The tree was run as a Markov model of a case patient aged 30 years. The model spanned the patient's lifetime to determine total costs of management of otosclerosis with stapedectomy or hearing aids. Cost-effectiveness was measured using an incremental cost-effectiveness ratio, with a willingness to pay of $50 000 per quality-adjusted life-year (QALY) considered cost-effective. One-way sensitivity analyses were performed for all variables. A 2-way sensitivity analysis was performed for the cost of stapedectomy vs the cost of hearing aids. Probabilistic sensitivity analysis was performed to determine the likelihood that stapedectomy would be cost-effective across a range of model inputs.InterventionsStapedectomy vs hearing aid use.Main outcomes and measuresThe primary objective of this study was to determine the cost-effectiveness of stapedectomy vs hearing aids in the treatment of otosclerosis. The secondary objectives were to determine which factors are associated with the cost-effectiveness of the interventions.ResultsStapedectomy had an estimated lifetime cost of $19 417.95, while hearing aids had an average lifetime cost of $16 439.94. Stapedectomy also had a benefit of 16.58 QALYs, and hearing aids had a benefit of 15.82 QALYs. Stapedectomy increases lifetime costs by $2978.01, with a benefit of 0.76 QALYs compared with hearing aids. The incremental cost-effectiveness ratio for stapedectomy is $3918.43 per QALY. The model was sensitive to the cost of stapedectomy and the cost of stapedectomy revision surgery. Probabilistic sensitivity analysis showed that stapedectomy was cost-effective compared with hearing aids 99.98% of the time.Conclusions and relevanceStapedectomy appears to be a cost-effective option for treating otosclerosis compared with hearing aid use, from the patient perspective.

ObjectiveOtogenic brain abscess is a well-recognized clinical condition that describes brain abscess secondary to an ear infection or mastoiditis. Current evidence remains limited on risk factors associated with mortality as most data are from case series. We aimed to 1) report the mortality rate among patients who did and did not receive mastoidectomy 2) identify factors associated with inpatient mortality.Study designRetrospective cohort study.SettingMulti-institutional.PatientsWe identified a cohort of patients for years 2008 to 2014 who in their inpatient hospitalization carried the diagnoses of both brain abscess and infectious ear disease.InterventionsInpatient neurotology and neurosurgical procedures.Main outcome measuresA multivariable logistics regression model was built to identify the factors associated with inpatient mortality.ResultsThe final analysis included 252 patients, of which 84 (33.3%) underwent mastoidectomy. The rate of inpatient morbidity and mortality were 17.5% and 4.0%, respectively. The rate of mortality in patients without mastoidectomy versus those with mastoidectomy was 4.2% versus 3.6%, respectively (p > 0.99). The odds of inpatient mortality were significantly increased for every 10-year increase in age (odds ratio [OR] 2.73, 95% confidence interval [CI]: 1.39-7.01, p = 0.011) and for Black compared to White patients (OR: 45.81, 95% CI: 4.56-890.92, p = 0.003).ConclusionOlder age and Black race were associated with increased odds of inpatient mortality and there were no significant differences in mortality between mastoidectomy cohorts. This research serves to generate further hypotheses for larger observational studies to investigate the association between sociodemographic factors and surgical variables with outcomes among this surgical population.

OBJECTIVE: AIED (autoimmune inner ear disease) is an autoimmune process that leads to the dysfunction of the inner ear, resulting in fluctuating, audiovestibular symptoms. Although the pathogenesis is likely heterogeneous, immune processes within the inner ear ultimately lead to histopathologic changes and sensorineural hearing loss. This review will discuss the latest evidence on treatment options. METHODS: A literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed. RESULTS: Corticosteroid treatment continues to remain as first line therapy for AIED but long-term responsiveness is poor. Cytotoxic chemotherapies can be effective alternatives for steroid nonresponsive patients, but significant side effects may limit their use. Intratympanic steroid injections are beneficial and although there is not enough evidence currently to supplant oral steroid trial they may be a useful adjunct if steroid toxicity is an issue. The efficacy of biologic agents has been variable. Compared to placebo, etanercept does not improve the hearing improvement already attained by steroids alone. However, open pilot studies of other biologic agents show hearing improvements, improvements in tinnitus/aural fullness/vertigo, ability to wean steroid dependency, or benefits in steroid-resistant AIED. CONCLUSION: There is currently not enough evidence that alternative treatments supersede the use of initial steroid treatment. Biologic agents and intratympanic steroid injections are relatively well tolerated and should be considered as adjunctive therapy. More studies on the efficacy of various biologics and more studies on the treatment of steroid resistant disease especially after initial benefit are still needed. For those who eventually lose their hearing, cochlear implantation remains as a viable option. LEVEL OF EVIDENCE: expert opinion.

Tophaceous gout of the middle ear is a rare occurrence that presents as a granular white-colored mass. It is frequently misdiagnosed as cholesteatoma or tympanosclerosis in patients who otherwise may not manifest any clinical or biochemical signs of gout. While uncommon, it can lead to clinically significant disease such as conductive hearing loss. The present report describes 2 cases of middle ear gouty tophi initially mistaken for another entity. Both patients underwent surgery, and the diagnosis of gout was revealed after final histopathological analysis. A review of the literature is also presented.

ObjectiveThis is the first report of multifocal inflammatory pseudotumor (IPT) involving the temporal bone, orbit and paranasal sinus, and the use of rituximab as adjunctive therapy in multifocal temporal bone IPT.PatientWe describe a 46-year-old man with orbital and maxillary sinus IPT, whose disease progressed despite radiation and steroid burst. He then developed contralateral mastoid disease, otalgia, aural fullness, and hearing loss.InterventionHe was initiated on rituximab and prednisone therapy. Mastoidectomy with near-total tumor removal was accomplished and histopathology confirmed IPT. A literature review was also performed.Main outcome measureTumor regression or recurrence.ResultDespite disease progression after radiation and steroids, his orbital, sinus, and mastoid disease improved after surgery, steroids, and rituximab. A review of four other previously reported cases of multifocal disease involving the temporal bone suggest that multifocal disease may be a more aggressive entity with higher recurrence rate compared with solitary disease. Although surgery and steroids are typically recommended, there is currently no consensus treatment recommendation.ConclusionsMultifocal IPT of the temporal bone is a rare but aggressive entity for which surgery and steroid combination therapy should be first line treatment. We suggest rituximab may be an effective adjunctive treatment particularly for recurrent disease or where systemic therapy may be favored.

Cor triatriatum sinister (CTS) is a rare congenital cardiac malformation. In CTS, a fibromuscular membrane subdivides the left atrium into 2 chambers. The communication between the 2 chambers is through 1 or more orifices in the dividing membrane. We present an interesting case of a 2-month-old infant with obstructed CTS membrane who first presented on account of poor feeding and failure to thrive. Echocardiography showed a persistent levoatrial cardinal vein (LACV) connecting the left atrium and the innominate vein. This allowed the proximal left atrial chamber to decompress its blood volume into the innominate vein and subsequently the superior vena cava. There was minimal prograde blood flow across the Cor triatriatum membrane, so the majority of pulmonary venous blood ultimately returned to the heart by way of the decompressing vertical vein into the systemic venous circulation. Surgical repair was performed with an uneventful postoperative course. The specific anatomical variant of Cor triatriatum found in our subject has rarely been reported.

We previously identified peptides that are actively transported across the intact tympanic membrane (TM) of rats with infected middle ears. To assess the possibility that this transport would also occur across the human TM, we first developed and validated an assay to evaluate transport in vitro using fragments of the TM. Using this assay, we demonstrated the ability of phage bearing a TM-transiting peptide to cross freshly dissected TM fragments from infected rats or from uninfected rats, guinea pigs and rabbits. We then evaluated transport across fragments of the human TM that were discarded during otologic surgery. Human trans-TM transport was similar to that seen in the animal species. Finally, we found that free peptide, unconnected to phage, was transported across the TM at a rate comparable to that seen for peptide-bearing phage. These studies provide evidence supporting the concept of peptide-mediated drug delivery across the intact TM and into the middle ears of patients.

We previously identified peptides that are actively transported across the intact tympanic membrane (TM) of rats with infected middle ears. To assess the possibility that this transport would also occur across the human TM, we first developed and validated an assay to evaluate transport in vitro using fragments of the TM. Using this assay, we demonstrated the ability of phage bearing a TM-transiting peptide to cross freshly dissected TM fragments from infected rats or from uninfected rats, guinea pigs and rabbits. We then evaluated transport across fragments of the human TM that were discarded during otologic surgery. Human trans-TM transport was similar to that seen in the animal species. Finally, we found that free peptide, unconnected to phage, was transported across the TM at a rate comparable to that seen for peptide-bearing phage. These studies provide evidence supporting the concept of peptide-mediated drug delivery across the intact TM and into the middle ears of patients.