Effect of betahistine on pro-inflammatory cytokine expression in autoimmune inner ear disease and Meniere's disease patients.

Objective: Betahistine is a partial H1 receptor agonist and a potent H3 receptor antagonist commonly used for the treatment of MD and peripheral vertigo. The aim of this study was to investigate the impact of betahistine on the salt induced cytokine expression profiles of AIED and MD patients.

Methods: Peripheral blood mononuclear cells (PBMCs) were obtained from 24 patients with autoimmune inner ear disease (AIED) or Meniere's disease (MD) during an acute exacerbation of hearing loss.

NaCl exposure results in increased expression and processing of IL-1β in Meniere's disease patients.

Meniere's disease (MD) is a chronic disease that causes episodic vertigo, fluctuating hearing loss, and aural fullness, initially managed by dietary salt reduction, and use of diuretics. Our prior research in autoimmune inner ear disease (AIED) demonstrated that in peripheral blood mononuclear cell (PBMC) from corticosteroid-resistant AIED patients, increased production, processing and release of interleukin-1β (IL-1β) is observed and hearing could be improved with use of anakinra, an interleukin-1 receptor antagonist. We have further identified that in these AIED patients, IL-1β is uniquely processed to a 28 kDa pro-inflammatory product by caspase-7.

Direct SARS-CoV-2 infection of the human inner ear may underlie COVID-19-associated audiovestibular dysfunction.

Background: COVID-19 is a pandemic respiratory and vascular disease caused by SARS-CoV-2 virus. There is a growing number of sensory deficits associated with COVID-19 and molecular mechanisms underlying these deficits are incompletely understood.

Methods: We report a series of ten COVID-19 patients with audiovestibular symptoms such as hearing loss, vestibular dysfunction and tinnitus.

Immune-Mediated Sensorineural Hearing Loss in the Pediatric Population.

A case series with chart review of pediatric patients with immune-mediated sensorineural hearing loss, including sudden sensorineural hearing loss and autoimmune-mediated hearing loss, was performed. Ninety-eight patients with sensorineural hearing loss were reviewed, and 41 patients met the inclusion criteria, which included corticosteroid therapy for a decline in sensorineural hearing. The primary outcome was the corticosteroid response rate: 61% of patients responded.

Biologics for Immune-Mediated Sensorineural Hearing Loss.

Immune-mediated hearing losses include autoimmune inner ear disease, sudden sensorineural hearing loss, and Meniere's disease. Standard therapy for an acute decline in hearing is timely use of corticosteroids. Although 60% to 70% of patients are initially corticosteroid-responsive, that responsiveness is lost over time.

The Correlation of Clinical Corticosteroid Responsiveness With Expression of IL-6 in Peripheral Blood Immune Cells (PBMC) in Patients With Autoimmune Inner Ear Disease (AIED).

Hypothesis: Autoimmune inner ear disease (AIED) patients will differentially express interleukin (IL)-6 based on corticosteroid responsiveness.

Background: AIED is characterized by periods of acute sensorineural hearing loss (SNHL). In a majority of patients corticosteroid responsiveness is lost over time.

Autoimmune inner ear disease patient-associated 28-kDa proinflammatory IL-1β fragment results from caspase-7-mediated cleavage in vitro.

Interleukin-1β (IL-1β) is a key proinflammatory cytokine involved in the progression of many autoinflammatory and autoimmune diseases, including autoimmune inner ear disease (AIED). IL-1β inhibition has been shown to result in clinical hearing improvement in a small cohort of corticosteroid-resistant patients with AIED. Canonical processing of pro-IL-1β by caspase-1 generates an active 17-kDa fragment, capable of instigating a proinflammatory microenvironment.

Medical and Audiological Indications for Implantable Auditory Devices.

Implantable auditory devices (IADs) are a viable hearing restoration option for patients with hearing loss. Conditions such as chronic otitis externa, congenital aural atresia, and chronic otitis media can be treated with a variety of implants. Progressive disease are also amenable to restoration with IADs, providing stabilized hearing.

Idiopathic Sudden Sensorineural Hearing Loss Is Not a Sentinel Event for Acute Myocardial Infarction.

Objective: Given ongoing debate about the suggested association, the primary objective was to determine if idiopathic sudden sensorineural hearing loss (ISSNHL) was a sentinel event for acute myocardial infarction (AMI) in adults.

Study Design: Case-control study.

Setting: United States MarketScan administrative health claims database.

Characterization of newborn hearing screening failures in multigestational births.

Objective: To define the rate and characterize the type of newborn hearing screening failures in multigestational births.

Methods: Retrospective chart review of all multigestational births that occurred in a 10-year period (2002-2012) in which at least one newborn failed newborn hearing screening at two tertiary care hospitals in the Northwell Health System.

Results: Out of 125,405 total births, we identified 2961 multigestational births, of which 59 (2.

The Balance of Tissue Inhibitor of Metalloproteinase-1 and Matrix Metalloproteinase-9 in the Autoimmune Inner Ear Disease Patients.

Tissue inhibitor of metalloproteinase-1 (TIMP-1) is a protein implicated in the control of inflammation in a number of autoimmune diseases. We hypothesized that the balance of TIMP-1 and matrix metalloproteinase-9 (MMP-9) may influence the control or perpetuation of inflammation in corticosteroid-responsive (RES) and corticosteroid-resistant (NR) autoimmune inner ear disease (AIED) patients. In the present study, we observed that plasma from AIED patients exhibited greater levels of TIMP-1 values compared with normal healthy controls.

CHEER National Study of Chronic Rhinosinusitis Practice Patterns: Disease Comorbidities and Factors Associated with Surgery.

Objectives (1) Describe national patterns of chronic rhinosinusitis (CRS) care across academic and community practices. (2) Determine the prevalence of comorbid disorders in CRS patients, including nasal polyposis, allergic rhinitis, asthma, and cystic fibrosis. (3) Identify demographic, clinical, and practice type factors associated with endoscopic sinus surgery (ESS).

AAO: Autoimmune and Autoinflammatory (Disease) in Otology: What is New in Immune-Mediated Hearing Loss.

Objectives: Autoinflammatory diseases are a family of immune-mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory-mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinflammatory diseases that affect hearing, discuss the limitations of our knowledge, and highlight potential new disease mechanisms and therapeutics.

Data Sources: Pubmed Literature Review; Google Scholar Literature review.

The experience of bilateral cochlear implantation in a child with LEOPARD syndrome.

We present a 3-year old boy with Leopard syndrome. His clinical manifestations included a congenital bilateral sensorineural hearing loss. He underwent cochlear implantation on the right side at age 1 year and on the left side at age 1.

Does an Otolaryngology-Specific Database Have Added Value? A Comparative Feasibility Analysis.

Objectives: There are multiple nationally representative databases that support epidemiologic and outcomes research, and it is unknown whether an otolaryngology-specific resource would prove indispensable or superfluous. Therefore, our objective was to determine the feasibility of analyses in the National Ambulatory Medical Care Survey (NAMCS) and National Hospital Ambulatory Medical Care Survey (NHAMCS) databases as compared with the otolaryngology-specific Creating Healthcare Excellence through Education and Research (CHEER) database.

Study Design: Parallel analyses in 2 data sets.

Variation in the Use of Vestibular Diagnostic Testing for Patients Presenting to Otolaryngology Clinics with Dizziness.

Objective: We used a national otolaryngology practice-based research network database to characterize the utilization of vestibular function testing in patients diagnosed with dizziness and/or a vestibular disorder.

Study Design: Database review.

Setting: The Creating Healthcare Excellence through Education and Research (CHEER) practice-based research network of academic and community providers

Subjects And Methods: Dizzy patients in the CHEER retrospective database were identified through ICD-9 codes; vestibular testing procedures were identified with CPT codes.

Tonsillectomy Bleed Rates across the CHEER Practice Research Network: Pursuing Guideline Adherence and Quality Improvement.

Objectives: (1) Compare postoperative bleeding in the CHEER network (Creating Healthcare Excellence through Education and Research) among age groups, diagnoses, and practice types. (2) Report the incidence of bleeding by individual CHEER practice site based on practice guidelines.

Study Design: Retrospective data collection database review of the CHEER network based on ICD-9 and CPT codes related to tonsillectomy patients.

Thyroid Disease and Surgery in CHEER: The Nation's Otolaryngology-Head and Neck Surgery Practice-Based Network.

Objectives: (1) Describe thyroid-related diagnoses and procedures in Creating Healthcare Excellence through Education and Research (CHEER) across academic and community sites. (2) Compare management of malignant thyroid disease across these sites. (3) Provide practice-based data related to flexible laryngoscopy vocal fold assessment before and after thyroid surgery based on the American Academy of Otolaryngology-Head and Neck Surgery Foundation's clinical practice guidelines.

Ménière's Disease: A CHEER Database Study of Local and Regional Patient Encounter and Procedure Patterns.

Objective: (1) Integrate practice-based patient encounters using the Dartmouth Atlas Medicare database to understand practice treatments for Ménière's disease (MD). (2) Describe differences in the practice patterns between academic and community providers for MD.

Study Design: Practice-based research database review.

N-Acetylcysteine attenuates tumor necrosis factor alpha levels in autoimmune inner ear disease patients.

Autoimmune inner ear disease (AIED) is a poorly understood disease marked by bilateral, rapidly progressive hearing loss triggered by unknown stimuli, which is corticosteroid responsive in 60 % of patients. Although the mechanism of the disease is not precisely understood, a complex interaction of cytokines is believed to contribute toward the inflammatory disease process and hearing loss. Previously, we showed the role of TNF-α in steroid-sensitive and IL-1β in steroid-resistant immune-mediated hearing loss.

Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease.

Background: Autoimmune inner ear disease (AIED) is a rare disease that results in progressive sensorineural hearing loss. Patients with AIED initially respond to corticosteroids; however, many patients become unresponsive to this treatment over time, and there is no effective alternative therapy for these individuals.

Methods: We performed a phase I/II open-label, single-arm clinical trial of the IL-1 receptor antagonist anakinra in corticosteroid-resistant AIED patients.

Resident Research Experience and Career Path Association: A National Survey of Recent Otolaryngology Graduates.

Objective: To determine whether the research rotation experience affects the career path of otolaryngology residents.

Study Design/setting: Two web-based surveys were disseminated by the AAO-HNS; one to current and former resident trainees and the other to current residency program directors.

Subjects And Methods: A web-based survey was disseminated to all AAO-HNS members classified as otolaryngology residents or residency graduates within the last 6 years, regarding their research rotation and its potential influence on their career path.

Innate immune recognition of molds and homology to the inner ear protein, cochlin, in patients with autoimmune inner ear disease.

Autoimmune Inner Ear Disease (AIED) is characterized by bilateral, fluctuating sensorineural hearing loss with periods of hearing decline triggered by unknown stimuli. Here we examined whether an environmental exposure to mold in these AIED patients is sufficient to generate a pro-inflammatory response that may, in part, explain periods of acute exacerbation of disease. We hypothesized that molds may stimulate an aberrant immune response in these patients as both several Aspergillus species and penecillium share homology with the LCCL domain of the inner ear protein, cochlin.

Diagnostic and prognostic utility of measuring tumor necrosis factor in the peripheral circulation of patients with immune-mediated sensorineural hearing loss.

Objectives: To characterize levels of tumor necrosis factor (TNF; formerly known as tumor necrosis factor α), a well-established proinflammatory cytokine, in patients with immune-mediated sensorineural hearing loss (IM-SNHL) and to determine the role of this cytokine in identifying steroid-responsive hearing loss.

Design: Prospective case-control study.

Setting: Tertiary care academic medical center.