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.

Socioeconomic Status and Melanoma in Canada: A Systematic Review.

As melanoma is one of the leading cancers in average years of life lost per death from disease, screening and early diagnosis are imperative to decrease morbidity and mortality. Socioeconomic status (SES) has been shown to be associated with melanoma incidence. However, it is unclear if this association holds true in universal healthcare systems where screening, diagnostic, and treatment services are available to all patients. The objective of this systematic review was to evaluate the evidence on the association of SES and melanoma incidence in Canada. A comprehensive search of PubMed and EMBASE yielded 7 studies reporting on melanoma incidence or outcomes with respect to SES in Canada. High SES was associated with increased melanoma incidence across all studies, which encompassed all Canadian provinces, and time periods spanning from 1979 to 2012. Studies also reported an increasing incidence of melanoma over time. There were substantial discrepancies in melanoma incidence across Canadian provinces, after controlling for SES and demographic characteristics. Populations of lower SES and living within certain healthcare regions had increased risks of advanced melanoma at diagnosis. This review highlights the potential for inequities in access to care even within a universal healthcare system. Future research is needed to characterize specific risk factors within different patient groups and within the universal health system context in order to implement targeted strategies to lower melanoma incidence, morbidity, and mortality.

The Canadian Management of Bovine Spongiform Encephalopathy in Historical and Scientific Perspective, 1990-2014.

On February 11, 2015, the Canadian Food Inspection Agency announced that a cow born and raised in Alberta had tested positive for bovine spongiform encephalopathy (BSE), commonly known as mad cow disease. BSE is a prion disease of cattle that, when transmitted to humans, produces a fatal neurodegenerative disease known as variant Creutzfeldt-Jakob disease. We believe that this latest case of BSE in Canadian cattle suggests the timeliness of a review of the management of BSE in Canada from a historically and scientifically informed perspective. In this article, we ask: how did the Canadian management of BSE between 1990 and 2014 engage with the contemporary understanding of BSE's human health implications? We propose that Canadian policies largely ignored the implicit medical nature of BSE, treating it as a purely agricultural and veterinary issue. In this way, policies to protect Canadians were often delayed and incomplete, in a manner disturbingly reminiscent of Britain's failed management of BSE. Despite assurances to the contrary, it is premature to conclude that BSE (and with it the risk of variant Creutzfeldt-Jakob disease) is a thing of Canada's past: BSE remains very much an issue in Canada's present.

An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024.

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.

The Impact of Monoclonal Antibody Usage on Hearing Outcomes: A Systematic Review.

OBJECTIVE: To ascertain trends in ototoxicity observed with monoclonal antibodies (mABs) and understand the impact they may have on hearing function. DATA SOURCES: PubMed, Embase, Scopus. REVIEW METHODS: A systematic review was performed following PRISMA guidelines. Data were reviewed for demographics, utilized mABs with respective indication and dosing, audiometric outcomes, and treatment for otologic effects. RESULTS: Of 757 studies reviewed, a total of 44 were included, encompassing 18,046 patients treated with mABs. Mean age of the sample was 57.8 years old. The search yielded 18 agents of ototoxicity, with reported symptoms of ototoxicity such as hearing loss, tinnitus, and/or aural fullness occurring in 1079 of total patients. Main agents causing ototoxicity were teprotumumab (n = 17/44 studies), nivolumab (n = 10/44), ipilimumab (n = 9/44), pembrolizumab (n = 5/44), and rituximab (n = 4/44). Thirty-one of 44 studies encompassing eight agents reported audiometric data for ototoxic agents, showing sensorineural hearing loss primarily in the high-frequency range. Only two articles performed ultrahigh-frequency audiograms. CONCLUSION: Monoclonal antibody usage is expanding, but the vast majority of studies lack substantial audiometric data. Where reported, study design and inclusion criteria vary greatly. Future studies would benefit from rigid inclusion of audiometric data, prospective study design, and consideration of formal ototoxicity screening. Otolaryngologists should be aware of the cochlear immune response and potential impact of this expanding medication class on hearing function. 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.

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.

A Rare Case of Cochlear Implant Electrode Array Misplacement Into the Posterior Semicircular Canal.

Cochlear implant electrode array misplacement is a rare but serious complication that may result in failure of hearing rehabilitation, non-auditory percepts, vestibular disturbance, or damage to adjacent neurovascular structures. We present a case of an elderly patient who suffered electrode array misplacement into the posterior semicircular canal, resulting in vestibular symptoms and severe downstream sequelae. The risk of misplacement may be higher in patients with a history of chronic otitis media or prior otologic surgery, and with the use of pre-curved electrode arrays. Electrophysiological testing and intraoperative imaging may allow for early detection and intervention in these cases. Laryngoscope, 133:175-177, 2023.

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.

Signal processing and stimulation potential within the ascending auditory pathway: a review.

The human auditory system encodes sound with a high degree of temporal and spectral resolution. When hearing fails, existing neuroprosthetics such as cochlear implants may partially restore hearing through stimulation of auditory neurons at the level of the cochlea, though not without limitations inherent to electrical stimulation. Novel approaches to hearing restoration, such as optogenetics, offer the potential of improved performance. We review signal processing in the ascending auditory pathway and the current state of conventional and emerging neural stimulation strategies at various levels of the auditory system.

Predictors of Surgical Site Infection in Pediatric Cochlear Implantation.

OBJECTIVE: To determine risk factors for surgical site infection (SSI) after cochlear implantation (CI) in pediatric patients. STUDY DESIGN: Case-control study. SETTING: A total of 150 hospitals contributing data to the ACS-NSQIP Pediatric database (American College of Surgeons National Surgical Quality Improvement Program) in North America and worldwide. METHODS: Pediatric patients (aged <18 years) undergoing CI during the years 2012 to 2017 were identified in the ACS-NSQIP Pediatric database. Uni- and multivariable logistic regression analyses were used to determine the odds ratios (ORs) of SSI (including superficial incisional, deep incisional, organ/space) occurring up to 30 days postoperatively. RESULTS: A total of 79 SSIs occurred over a 5-year period (n = 5146). Longer operative time significantly increased the odds of SSI (OR, 1.965; 95% CI, 1.205-3.289). Younger age was also found to raise the odds of SSI, with decreased odds associated with each 6-month increase in age (OR, 0.887; 95% CI, 0.814-0.958). CONCLUSION: Longer operative time and younger age appear to significantly increase the odds of SSI in pediatric CI. Body mass index, recent steroid use, American Society of Anesthesiologists class, bilateral vs unilateral implantation, and hospital length of stay do not appear to significantly influence SSI risk. These findings must be interpreted in the context of the limitations inherent to adverse events reporting, which are mitigated by the stringent manner of data collection by the ACS-NSQIP, and those inherent to the definition of SSI. Future prospective studies should investigate the impact of reducing operative time on the risk of SSI and other complications in pediatric CI.

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.

The effect of insurance status on treatment modality in advanced oral cavity cancer.

BACKGROUND: Insurance status has been shown to impact survival outcomes. We sought to determine whether insurance affects the choice of treatment modality among patients with advanced (T4) oral cavity squamous cell carcinoma. METHODS: This is a retrospective, population-based cohort study using the Survival, Epidemiology, and End Results Program database. The population included all adult (age ≥ 18) patients with advanced (T4a or T4b) oral cavity squamous cell carcinoma diagnosed from 2007 to 2016. The main outcome was the odds of receiving definitive treatment, defined as primary surgical resection. Insurance status was categorized into uninsured, any Medicaid, and insured groups. Univariable, multivariable, and subgroup analyses were performed. RESULTS: The study population consisted of 2628 patients, of whom 1915 (72.9%) were insured, 561 (21.3%) had Medicaid, and 152 (5.8%) were uninsured. The multivariable model showed that patients who were 80 years or older, unmarried, received treatment in the pre-Affordable Care Act (ACA) period, and who were on Medicaid or uninsured were significantly less likely to receive definitive treatment. Insured patients were significantly more likely to receive definitive treatment compared to those on Medicaid or uninsured (OR = 0.59, 95% CI 0.46-0.77, p < 0.0001 [Medicaid vs. Insured]; and OR = 0.48, 95% CI 0.31-0.73 p = 0.001 [Uninsured vs. Insured]), however these differences did not persist when considering only those patients treated following the 2014 expansion of the ACA. CONCLUSIONS: Insurance status is significantly associated with treatment modality among adults with advanced stage (T4a) oral cavity squamous cell carcinoma. These findings support the premise of expanding insurance coverage in the US.

Poor Online Patient Ratings of Otolaryngologists in the United States: What are Patients Saying?

OBJECTIVES: Online patient forums have become a platform for patient education and advocacy in many areas of medicine. The anonymity provided by such forums may encourage honest, candid responses. Using patient online reviews, this study sought to explore themes that arose from negatively perceived care interactions with American otolaryngologists using the Accreditation Council for Graduate Medical Education (ACGME) competency framework. STUDY DESIGN: Qualitative thematic analysis. METHODS: Through an iterative multistep process, a qualitative thematic analysis was conducted on negative reviews (defined as ratings of two or less out of five) of all American otolaryngologists found on a popular online physician-rating website (RateMDs.com). RESULTS: A systematic search through the RateMDs website revealed 2950 separate comments of negative reviews. Of these negative reviews, 350 were randomly selected for thematic analysis. The predominant themes that emerged aligned closely with the Accreditation Council for Graduate Medical Education (ACGME) competencies, in particularly with professionalism and interprofessional skills and communication. CONCLUSIONS: The negative reviews of American otolaryngologists revealed a number of areas where improvements could be made to quality of care. Patients value evidence-based medicine delivered by compassionate and respectful physicians. Isolating and aligning predominant themes within the ACGME framework proved a productive method to collect and organize pertinent patient feedback and integrate teaching into the post-graduate training and continuing professional development in order to avoid such negatively perceived interactions in the future.

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.

Impact of Anticholinergic Medications on Speech Perception Performance after Cochlear Implantation.

OBJECTIVE: To identify and characterize the impact of anticholinergic medications, which have known adverse effects on cognition in older adults, on speech perception after cochlear implantation. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. SUBJECT POPULATION: Adult patients who underwent cochlear implantation between January 2010 and September 2020 with speech perception scores at 3, 6, and 12 months. INTERVENTIONS: Anticholinergic burden of patients' prescribed medications. MAIN OUTCOME MEASURES: AzBio speech perception scores after implantation. RESULTS: One hundred twenty-six patients had documented AzBio in quiet speech perception score at all three postactivation time points. Patients were divided into three groups by anticholinergic burden (ACB) score, including ACB = 0 (90 patients), 1 (23 patients), and ≥2 (13 patients). There was no statistically significant difference between ACB groups in audiologic performance at candidacy testing ( p = 0.77) or at 3 months after implantation ( p = 0.13). Beginning at 6 months, a lower mean AzBio was seen in patients with higher ACB scores (68% ACB = 0; 62% ACB = 1; 48.1% ACB ≥ 2; p = 0.03). At 12 months, there were further differences between the groups (71.0% ACB = 0, 69.5% ACB = 1, 48.0% ACB ≥2, p < 0.01). Controlling for the effects of age using multivariate linear regression showed persistent effects of ACB score on learning-related AzBio improvements. Comparatively, the negative impact of a single ACB score point was equivalent to nearly 10 years of aging ( p = 0.03). CONCLUSIONS: Increased ACB is associated with worse speech perception scores after cochlear implantation, an effect that persists even when accounting for patient age, suggesting that these medications may have cognitive and learning effects that reduce cochlear implant performance.