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.

Preliminary Evidence for the Effects of Gentamicin on Vertical Semicircular Canals.

INTRODUCTION: Gentamicin is a vestibulotoxic antibiotic often used in patients with Ménière's disease for its vestibular ablative effects. Gentamicin's effect on the horizontal semicircular canal does not always correlate with the degree of vertigo control achieved by patients; its effect on the vertical semicircular canals remains unknown. We sought to examine the effect of intratympanic gentamicin on vertical semicircular canal function in patients with Ménière's disease using video head impulse testing. METHODS: A retrospective case series was carried out at a tertiary academic center. Patients with Ménière's disease who received ≥1 intratympanic gentamicin injection from 2019-2022 and had video head impulse testing performed were included. Outcomes of interest were vertical semicircular canal function following intratympanic gentamicin, correlations between vertical semicircular canal function and horizontal semicircular canal function, and residual symptoms following injection. RESULTS: Ten patients met inclusion criteria. Twenty percent had abnormal V-SCC function prior to any injection and 40% following the first injection. There was an association between abnormal vertical and horizontal semicircular canal function following the first intratympanic gentamicin injection, though the relationship did not reach statistical significance (p = 0.058). While patients with abnormal vertical semicircular canal function following the first injection were less likely to report ongoing vertigo attacks, the relationship was not statistically significant (p = 0.260). CONCLUSIONS: Intratympanic gentamicin leads to changes in vertical semicircular canal function in at least a proportion of patients with Ménière's disease. Further study is required to better assess correlations between vertical semicircular canal function and symptom control following intratympanic gentamicin.

Evaluating the Role of Otologic Biomarkers to Differentiate Meniere's Disease and Vestibular Migraine.

OBJECTIVE: To evaluate the role for prestin and otolin-1 as biomarkers for differentiating Meniere's disease (MD) from vestibular migraine (VM). STUDY DESIGN: It is a cross-sectional, cohort study. RESULTS: There were 19 MD and 11 VM patients. In the 19 MD patients, the mean prestin level was 2.33 ng/ml compared to 0.64 ng/ml in VM patients (p = 0.238). Otolin-1 levels in MD patients were 109.67 pg/ml, while in VM patients, otolin-1 levels were 30.9 pg/ml (p = 0.102). In MD patients, prestin levels were correlated with word recognition scores, being strongest when prestin >2 ng/ml (rho = 0.9; p = 0.019). CONCLUSIONS: Prestin and otolin-1 levels differed between MD patients relative to VM patients. The relationship between prestin and word recognition scores in MD suggests that there may be a role for prestin as a marker for inner ear function, but its role in differentiating MD from VM remains to be elucidated.

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.

Genome-first approach to rare EYA4 variants and cardio-auditory phenotypes in adults.

While newborns and children with hearing loss are routinely offered genetic testing, adults are rarely clinically tested for a genetic etiology. One clinically actionable result from genetic testing in children is the discovery of variants in syndromic hearing loss genes. EYA4 is a known hearing loss gene which is also involved in important pathways in cardiac tissue. The pleiotropic effects of rare EYA4 variants are poorly understood and their prevalence in a large cohort has not been previously reported. We investigated cardio-auditory phenotypes in 11,451 individuals in a large biobank using a rare variant, genome-first approach to EYA4. We filtered 256 EYA4 variants carried by 6737 participants to 26 rare and predicted deleterious variants carried by 42 heterozygotes. We aggregated predicted deleterious EYA4 gene variants into a combined variable (i.e. "gene burden") and performed association studies across phenotypes compared to wildtype controls. We validated findings with replication in three independent cohorts and human tissue expression data. EYA4 gene burden was significantly associated with audiometric-proven HL (p = [Formula: see text], Mobitz Type II AV block (p = [Formula: see text]) and the syndromic presentation of HL and primary cardiomyopathy (p = 0.0194). Analyses on audiogram, echocardiogram, and electrocardiogram data validated these associations. Prior reports have focused on identifying variants in families with severe or syndromic phenotypes. In contrast, we found, using a genotype-first approach, that gene burden in EYA4 is associated with more subtle cardio-auditory phenotypes in an adult medical biobank population, including cardiac conduction disorders which have not been previously reported. We show the value of using a focused approach to uncover human disease related to pleiotropic gene variants and suggest a role for genetic testing in adults presenting with hearing loss.

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.

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.

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.

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.

Patterns of Teprotumumab-Induced Hearing Dysfunction: A Systematic Review.

OBJECTIVE: Hearing loss has been reported after administration of the monoclonal antibody teprotumumab. The purpose of this study was to review available evidence regarding the patterns of teprotumumab-related ototoxicity. DATA SOURCES: PubMed, EMBASE, and Cochrane Library. REVIEW METHODS: A systematic review was performed using standardized methodology. Studies were included if they included subjects who were prescribed teprotumumab. Exclusion criteria included non-English articles, abstracts, letters/commentaries, case reports, and reviews. Subjects without both pre- and posttreatment audiometric data were also excluded. Bias was assessed using the Mixed Methods Appraisal Tool. RESULTS: From an initial search of 76 articles, 7 studies reporting on 109 unique patients were included. Four studies were level 4 evidence, 1 study was level 3 evidence, and 2 studies were level 2 evidence. Mean age was 55 ± 14 years with a female predominance (64%). The most commonly reported symptoms were hearing loss (22%), followed by fullness (18%) and tinnitus (14%). In total, 41% of patients with available data met criteria for ototoxicity, all exhibiting shifts in the middle frequencies or higher. Fifteen (14%) patients underwent ultrahigh frequency audiometric testing and 8 (53%, 8/15) demonstrated shifts exclusively in this range. CONCLUSION: Ototoxicity may occur in patients treated with teprotumumab. Hearing loss occurs primarily in higher frequencies, and routine hearing screening with ultrahigh frequency testing may be warranted. The true incidence of ototoxicity with teprotumumab remains unknown, and more data is needed to elucidate underlying mechanisms and develop strategies to minimize risks.

Dizziness and Imbalance Across the Lifespan: Findings of a Pediatric and Adult Vestibular Clinic.

OBJECTIVE: To evaluate diagnostic trends in pediatric and adult patients presenting for multidisciplinary subspecialty evaluation of dizziness and imbalance across the lifespan. STUDY DESIGN: Retrospective chart review. SETTING: Single pediatric and single adult academic tertiary care hospital. METHODS: Retrospective review of electronic health record for patients presenting to an adult or pediatric multidisciplinary vestibular clinic from 2017 to 2020, including clinical data, physical therapy evaluation, and audiovestibular testing. RESULTS: A total of 1934 patients aged 1 to 95 were evaluated. Most patients were female (n = 1188, 61%); the largest cohort was in the fifth decade of life (n = 321, 17%). Seventy-six percent of patients (n = 1470) were assigned a pathologic diagnosis. Central causes of dizziness were most common in children and young adults, comprising 38% to 54% of all diagnoses in ages 1 to 30. The proportion of peripheral vestibular disorders increased with age, peaking at 32% in ages 61 to 70. Vestibular migraine was the most common pathologic diagnosis in ages 6 to 20 (n = 110, 39%) and 31 and 50 (n = 69, 17%) regardless of gender, but was more prevalent in females (21% vs 14%; P < .0001). The prevalence of benign paroxysmal positional vertigo (BPPV) increased throughout the lifespan, peaking at age 71 to 80. Meniere's disease (MD) did not occur within the first decade of life, but increased thereafter, peaking at ages 51 to 60. CONCLUSION: Multidisciplinary vestibular evaluation resulted in a diagnosis for the majority of patients. Vestibular diagnoses vary across the lifespan however among most age groups, central disorders, including migraine disorders, outnumber peripheral vestibulopathies. The prevalence of peripheral vestibular disorders such as BPPV and MD increased with age. LEVEL OF EVIDENCE: Level IV.

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.

Toward a Diagnostic Imaging Algorithm for Undifferentiated Pulsatile Tinnitus.

OBJECTIVE: Decisions around the diagnostic evaluation for pulsatile tinnitus (PT) remain challenging. We describe the usage patterns and diagnostic accuracy of imaging modalities and propose an evidence-based diagnostic approach for undifferentiated PT. STUDY DESIGN: Retrospective. SETTING: Single otology/neurotology clinic. SUBJECTS: Patients with PT presenting between 2009 and 2020. MAIN OUTCOME MEASURES: Sensitivity, specificity, diagnostic yield, and diagnostic accuracy. RESULTS: A total of 315 subjects met inclusion criteria (74% female, mean ± SD age = 52 ± 17 years). Subjects were divided into four cohorts based on exam findings: normal (n = 229), venous cohort (n = 34), arterial cohort (n = 16), and outer/middle ear pathology cohort (n = 40). In total, 53% of patients received a nonidiopathic diagnosis for PT. The most common identifiable cause was sigmoid sinus dehiscence (78%) in the venous cohort, carotid stenosis (36%) in the arterial cohort, and glomus tumor (56%) in the outer/middle ear pathology cohort. There was a higher diagnostic rate among patients with positive exam findings compared to those with unrevealing exams ( p = 0.04). Imaging studies with the highest diagnostic yield were computed tomography (CT) venography (44%), formal angiography (42%), and magnetic resonance venography (40%); studies with the highest specificity were formal angiography (0.82), CT angiography (0.67), and CT venography (0.67). A diagnostic algorithm is proposed. CONCLUSIONS: Reaching a diagnosis in patients with PT requires a systematic approach, taking into account both clinical and radiographic information. Physical examination is a key first step for differentiating patients into venous, arterial, and other cohorts to narrow down the likely pathology and determine which radiographic studies have the highest yield and accuracy.

A multi-institutional machine learning algorithm for prognosticating facial nerve injury following microsurgical resection of vestibular schwannoma.

Vestibular schwannomas (VS) are the most common tumor of the skull base with available treatment options that carry a risk of iatrogenic injury to the facial nerve, which can significantly impact patients' quality of life. As facial nerve outcomes remain challenging to prognosticate, we endeavored to utilize machine learning to decipher predictive factors relevant to facial nerve outcomes following microsurgical resection of VS. A database of patient-, tumor- and surgery-specific features was constructed via retrospective chart review of 242 consecutive patients who underwent microsurgical resection of VS over a 7-year study period. This database was then used to train non-linear supervised machine learning classifiers to predict facial nerve preservation, defined as House-Brackmann (HB) I vs. facial nerve injury, defined as HB II-VI, as determined at 6-month outpatient follow-up. A random forest algorithm demonstrated 90.5% accuracy, 90% sensitivity and 90% specificity in facial nerve injury prognostication. A random variable (rv) was generated by randomly sampling a Gaussian distribution and used as a benchmark to compare the predictiveness of other features. This analysis revealed age, body mass index (BMI), case length and the tumor dimension representing tumor growth towards the brainstem as prognosticators of facial nerve injury. When validated via prospective assessment of facial nerve injury risk, this model demonstrated 84% accuracy. Here, we describe the development of a machine learning algorithm to predict the likelihood of facial nerve injury following microsurgical resection of VS. In addition to serving as a clinically applicable tool, this highlights the potential of machine learning to reveal non-linear relationships between variables which may have clinical value in prognostication of outcomes for high-risk surgical procedures.

Results of Baha® implantation using a small horizontal incision.

PURPOSE: Techniques for Baha® implantation continue to evolve. The Weber technique, utilizing a 1.5 cm horizontal incision for Baha® implantation is evaluated. METHODS: Retrospective review of patients undergoing Baha® implantation by a single surgeon over three years. RESULTS: 33 Baha®s implanted in 30 patients. Fourteen used an Inverted J (IJ) incision with an anteriorly-based skin-flap, 13 with the Weber technique (W). Five were not included as other techniques were used. Demographics and weeks to activation (14.58 vs 13.4, p = 0.12) were similar. There were no differences in the number of patients with minor complications (5 vs 2, p = 0.22) or number of minor complications (20 vs 4, p = 0.09). One patient in the IJ Group required operative revision for overgrowth. There were no infections in the IJ Group, and one requiring oral antibiotics in the W Group. Follow-up was longer in the IJ Group (41 vs 13 weeks, p = 0.016), no complications occurred after 14 weeks post-op. Mean operative times were similar (43 vs 39 min, p = 0.59). There were no cases of skin flap necrosis in either group. CONCLUSION: A small incision for Baha® implantation proved as effective, without increased complications as a skin-flap based technique.

Caloric function as a predictor of cochlear implant performance.

OBJECTIVES: Changes in vestibular function have been demonstrated following cochlear implantation (CI). The functional impact of these changes on CI performance has not been well-studied. We sought to assess whether caloric changes postimplantation could predict CI performance. METHODS: Retrospective review of a prospectively collected database at a tertiary care hospital. Patients who underwent CI over a 22-year period (1999-2021) and had videonystagmography (VNG) testing pre- and postimplantation were included. Caloric responses were compared pre- versus post-implantation, and assessed for their ability to predict CI performance as evaluated using AzBio +10 decibels signal-to-noise ratio (dB S/N) scores. RESULTS: 43 CI recipients were included. There was a significant difference in the average maximal slow-phase velocity in response to caloric irrigation in the implanted ear pre- versus post-operatively (21.2 vs. 18.5 deg/s; p = 0.02). Controlling for age and pre-implantation speech recognition performance, pre- and post-implantation caloric responses in the implanted ear significantly predicted CI performance at 0-6 months and >6 months post-implantation. Caloric changes following implantation were not significantly correlated with CI performance. CONCLUSION: CI impacts vestibular function as evidenced by changes in caloric responses. Vestibular function pre- and possibly post-CI may be clinically useful metrics for predicting some proportion of CI performance variability.

Predictive Ability of First-Side Cochlear Implant Performance in Adult Sequential Bilateral Cochlear Implantation.

OBJECTIVE: Predictors of second-side cochlear implant performance have not been well studied. We sought to assess whether speech recognition scores from first-side cochlear implant (CI1) could predict second-side cochlear implant (CI2) scores in sequential bilaterally implanted adults. STUDY DESIGN: Retrospective review using a prospectively collected database. SETTING: Academic tertiary care hospital. PATIENTS: Fifty-seven adults with postimplantation speech recognition testing performed at least 12 months after CI2. INTERVENTION: Sequential bilateral CI. MAIN OUTCOME MEASURES: CI2 performance at ≥12 months as measured using consonant-nucleus-consonant (CNC) words and AzBio sentences in quiet and +10 dB signal-to-noise ratio (S/N). RESULTS: CI1 performance scores at ≥12 months were independently associated with CI2 performance scores at ≥12 months for CNC words (ß = 0.371 [0.136-0.606], p = 0.003), AzBio sentences in quiet (ß = 0.614 [0.429-0.80], p < 0.0001), and AzBio +10 dB S/N (ß = 0.712 [0.459-0.964], p < 0.0001). CI1 scores on AzBio in quiet at 0 to 6 months were also independently associated with CI2 AzBio in quiet scores at ≥12 months (ß = 0.389 [0.004-0.774], p = 0.048). Hearing loss etiology and duration, age at implantation, interval between CI1 and CI2, duration of hearing aid use, and preimplantation speech recognition testing scores were not consistently associated with CI2 scores at ≥12 months. CONCLUSIONS: CI1 performance is an independent predictor of second-side performance as measured ≥12 months postimplantation. This may be a clinically useful metric when considering adult sequential bilateral implantation.

Flipping the Otolaryngology Didactics Curriculum: Results of a Novel Resident Course Structure.

OBJECTIVE: To assess the impact of a novel flipped-classroom (FC) otolaryngology resident didactic curriculum on resident learning. METHODS: Based on the preferences elicited in a survey of preferred learning styles of residents, a hybrid web-based and in-person FC otolaryngology didactic curriculum was implemented over a 6-month period in 2020-2021. Resident reactions to the new curriculum were assessed via a second survey. Kruskal Wallis tests were used to compare mean stanine scores on the Otolaryngology Training Examination (OTE) exam for topics taught in the new format with topics delivered in the old format. RESULTS: After instituting the curriculum reform, engagement in at least a moderate amount of the pre-didactic work and synchronous case discussions was reported by 67% and 88% of the residents, respectively. After the curriculum change, residents in years PGY2 and above reported statistically significant increases in self-reported ability to retain information from didactics, to feel prepared for the OTE, and to feel confident in fund of knowledge (p < 0.001, p = 0.004, and p = 0.004, respectively). Compared to the prior year, mean stanine increased on OTE scores for residents in years PGY2-PGY5 to a statistically significant degree (5.45 vs. 4.41, p = 0.001) for the topics delivered in the new format compared to topics delivered in the traditional format (5.13 vs. 4.70, p = 0.07). CONCLUSION: By organizing a didactic curriculum through online modules that incorporate pre-recorded lectures and external resources, residents reported and demonstrated improved knowledge retention. LEVEL OF EVIDENCE: NA Laryngoscope, 133:2129-2134, 2023.

Long-Term Hearing Outcomes After Hybrid Cochlear Implantation.

OBJECTIVE: To examine long-term (>5 yr) low-frequency hearing preservation after hybrid cochlear implantation. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Tertiary care center outpatient clinic. PATIENTS: All patients older than 21 years implanted with a Cochlear Hybrid L24 device from 2014 to 2021. MAIN OUTCOME MEASURE: Changes in low-frequency pure-tone average (LFPTA) were calculated at each of several time points relative to the date of implantation. The proportion of patients with preserved LFPTA at last follow-up and Kaplan-Meier estimates for loss of residual hearing were calculated in addition to hazard ratios for hearing loss according to patient- and surgery-specific factors. RESULTS: Thirty ears in 29 patients underwent hybrid cochlear implantation and were eligible for inclusion (mean age, 59 yr; 65% female). Mean preoperative LFPTA was 31.7 dB. Mean LFPTA across all implanted ears at first follow-up was 45.1 dB; no patient had experienced loss of residual hearing at first follow-up. Six patients had loss of residual hearing during the follow-up period, with Kaplan-Meier probability estimates of preserved hearing of 100% at 1 month, 90% at 12 months, 87% at 24 months, and 80% at 48 months. There was no association between loss of residual hearing and patient age, preoperative LFPTA, surgeon, or use of topical steroids intraoperatively (hazard ratios, 1.05 [0.96-1.15], 0.97 [0.88-1.05], 1.39 [0.20-9.46], 0.93 [0.09-9.74], respectively). CONCLUSIONS: Long-term (>5 yr) outcomes after hybrid cochlear implantation demonstrate good preservation of low-frequency hearing, with only modest decline in the long-term postimplantation, and a low proportion of loss of residual low-frequency hearing.