Health Disparities in Otology: A PRISMA-Based Systematic Review.

OBJECTIVE: Social determinants of health (SDOHs), including but not limited to sex, race, socioeconomic status, insurance status, and education level, play a significant role in health disparities and affect health outcomes. The purpose of this systematic review is to examine health disparities in otology within the United States and highlight areas warranting further research. DATA SOURCES: PubMed, Ovid MEDLINE. REVIEW METHODS: Our search encompassed all years through January 10, 2021. All peer-reviewed primary literature of any design and publication date regarding health disparities and otology outcomes in the United States was eligible for inclusion. Eligibility assessment was performed via 3 independent investigators. RESULTS: Of the 6326 unique abstracts identified, 188 studies underwent full-text review, and 52 remained in the final review. The most frequently examined otologic condition was hearing loss (36.5%), followed by cochlear implantation (28.8%) and infection/effusion (15.4%). Vertigo/dizziness (1.9%), Ménière's disease (1.9%), and tinnitus (1.9%) were the least represented otologic conditions. Comprehensive articles on multiple disparity topics were the most common (n = 18), followed by articles on race/ethnicity (n = 11) and socioeconomic status (n = 9). Language (n = 2), education (n = 2), and gender (n = 1) were the least discussed. Over 5-fold the number of articles were published between 2011 and 2020 compared to the preceding decade (42 vs 8). CONCLUSION: This study captures the existing literature regarding health disparities and outcomes in otology. The lack of robust data suggests the need for future quality studies aimed at investigating disparities in otologic care, as well as a broader push for recording and reporting SDOHs.

Cochlear Implantation in Noonan Syndrome With and Without Multiple Lentigines: A Case Report and Systematic Review.

Objectives: To describe outcomes after bilateral cochlear implantation (CI) in a patient with a pathologic PTPN11 variant associated with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML). Additionally, to assess the utility of CI in this specific population based on our outcome and previous reports. Study Design: Retrospective case report with literature review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Patients: A young boy with various multiorgan abnormalities, speech and language delay, and persistent hearing loss who was found to have a heterozygous PTPN11 gene mutation at age 2. Interventions: Bilateral tympanostomy tube placement, diagnostic imaging, and eventual staged bilateral CI. Main Outcome Measures: Objective audiometric testing and developmental milestone attainment. Results: Bilateral CI was successfully completed over a 2-month period. The patient illustrated significant improvement in objective audiologic measurement. However, he continues to sign as his main form of communication without significant speech progression. Conclusions: Early diagnostic and therapeutic intervention in patients with NS/NSML can help improve long-term audiologic and speech development. Given the heterogeneity of NS/NSML, a multidisciplinary approach is needed for optimal outcomes.

Reconstruction of the Anterior External Auditory Canal With Mastoid Cortex Autologous Bone Graft.

OBJECTIVE: To describe the surgical management of temporomandibular joint (TMJ) herniation with external auditory canal (EAC) reconstruction using autologous bone grafting from the mastoid cortex. STUDY DESIGN: Retrospective case series. SETTING: A tertiary university medical center. PATIENTS: Three patients who presented to our Otolaryngology clinic with evidence of TMJ herniation through an anterior EAC defect, both on otoscopy and computed tomography (CT) imaging. INTERVENTIONS: Reconstruction of the anterior EAC with mastoid cortex bone grafting using an endaural approach. MAIN OUTCOME MEASURES: Successful reconstruction of anterior EAC bony defect without recurrence of herniation. RESULTS: All three patients presented with otalgia, hearing loss, and either tinnitus or a clicking sensation with jaw movement. Etiologies for TMJ herniation included osteoradionecrosis following external beam radiation therapy for head and neck carcinoma and iatrogenic injury following multiple tympanoplasties and canalplasties. A mastoid cortex bone graft was placed and secured anterior to the bony EAC defect through an endaural approach. Two patients wore a dental retainer postoperatively to keep the condyle in an open position. After reconstruction, patients reported an improvement in their presenting symptoms. There was no recurrence of TMJ herniation in all cases after 1, 4, and 9 years. CONCLUSIONS: Anterior EAC reconstruction with autologous bone grafting can be an effective definitive treatment in TMJ herniation. To our knowledge, this is the first report of the use of bone grafting to reconstruct the canal defect in TMJ herniation.Level of Evidence: V.

In silico Single-Cell Analysis of Steroid-Responsive Gene Targets in the Mammalian Cochlea.

BACKGROUND: Treatment of many types of hearing instability in humans, including sudden sensorineural hearing loss, Meniere's disease, and autoimmune inner ear disease, rely heavily on the utilization of corticosteroids delivered both by oral and transtympanic routes. Despite this use, there is heterogeneity in the response to treatment with corticosteroids in humans with these diseases. The mechanisms by which corticosteroids exert their effect and the cell types in which they exert their effects in the inner ear remain poorly characterized. In this study, we localize steroid-responsive genes to cochlear cell types using previously published transcriptome datasets from the mammalian cochlea. METHODS: Steroid-responsive genes were localized to specific cochlear cell types using existing transcriptome datasets from wild-type mammalian cochlea exposed to systemic and transtympanic steroids, as well as previously published single-cell and single-nucleus RNA-sequencing datasets from the mammalian cochlea. Gene ontology (GO) analysis of differentially expressed genes (DEGs) was performed using PANTHER to investigate cellular processes implicated in transtympanic vs. systemic steroid action in the cochlea. RESULTS: Steroid-responsive genes were localized to specific cell types and regions in the cochlea including the stria vascularis, organ of Corti, and spiral ganglion neurons (SGN). Analyses demonstrate differential prevalence of steroid-responsive genes. GO analysis demonstrated steroid-responsive DEGs in the SGN to be associated with angiogenesis, apoptosis, and cytokine-mediated anti-inflammatory pathways. CONCLUSIONS: Single-cell and single-nucleus transcriptome datasets localize steroid-responsive genes to specific regions in the cochlea. Further study of these regionally-specific steroid-responsive genes may provide insight into the mechanisms of and clinical response to corticosteroids in diseases of hearing instability.