Objectives: The variability in outcomes of cochlear implantation is largely unexplained, and clinical factors are not sufficient for predicting performance. Genetic factors have been suggested to impact outcomes, but the clinical and genetic heterogeneity of hereditary hearing loss makes it difficult to determine and interpret postoperative performance. It is hypothesized that genetic mutations that affect the neuronal components of the cochlea and auditory pathway, targeted by the cochlear implant (CI), may lead to poor performance. A large cohort of CI recipients was studied to verify this hypothesis. Design: This study included a large German cohort of CI recipients (n = 123 implanted ears; n = 76 probands) with a definitive genetic etiology of hearing loss according to the American College of Medical Genetics (ACMG)/Association for Molecular Pathology (AMP) guidelines and documented postoperative audiological outcomes. All patients underwent preoperative clinical and audiological examinations. Postoperative CI outcome measures were based on at least 1 year of postoperative audiological follow-up for patients with postlingual hearing loss onset (>6 years) and 5 years for children with congenital or pre/perilingual hearing loss onset (≤6 years). Genetic analysis was performed based on three different methods that included single-gene screening, custom-designed hearing loss gene panel sequencing, targeting known syndromic and nonsyndromic hearing loss genes, and whole-genome sequencing. Results: The genetic diagnosis of the 76 probands in the genetic cohort involved 35 genes and 61 different clinically relevant (pathogenic, likely pathogenic) variants. With regard to implanted ears (n = 123), the six most frequently affected genes affecting nearly one-half of implanted ears were GJB2 (21%; n = 26), TMPRSS3 (7%; n = 9), MYO15A (7%; n = 8), SLC26A4 (5%; n = 6), and LOXHD1 and USH2A (each 4%; n = 5). CI recipients with pathogenic variants that influence the sensory nonneural structures performed at or above the median level of speech performance of all ears at 70% [monosyllable word recognition score in quiet at 65 decibels sound pressure level (SPL)]. When gene expression categories were compared to demographic and clinical categories (total number of compared categories: n = 30), mutations in genes expressed in the spiral ganglion emerged as a significant factor more negatively affecting cochlear implantation outcomes than all clinical parameters. An ANOVA of a reduced set of genetic and clinical categories (n = 10) identified five detrimental factors leading to poorer performance with highly significant effects (p < 0.001), accounting for a total of 11.8% of the observed variance. The single strongest category was neural gene expression accounting for 3.1% of the variance. Conclusions: The analysis of the relationship between the molecular genetic diagnoses of a hereditary etiology of hearing loss and cochlear implantation outcomes in a large German cohort of CI recipients revealed significant variabilities. Poor performance was observed with genetic mutations that affected the neural components of the cochlea, supporting the “spiral ganglion hypothesis.”

Human stapedius muscle (SM) can be directly and safely accessed via retrofacial approach, opening new approaches to directly measure the electrically evoked stapedius reflex threshold (eSRT). The measurement of the SM activity via direct surgical access represents a potential tool for objective eSRT fitting of cochlear implants (CI), increasing the benefit experienced by the CI users and leading to new perspectives in the development of smart implantable neurostimulators. 3D middle-ear reconstructions created after manual segmentation and related SM accessibility metrics were evaluated before the CI surgery for 16 candidates with assessed stapedius reflex. Retrofacial approach to access the SM was performed after facial recess exposure. In cases of poor exposition of SM, the access was performed anteriorly to the FN via drilling of the pyramidal eminence (PE). The total access rate of the SM via both the retrofacial and anterior approach of the FN was 100%. In 81.2% of cases (13/16), the retrofacial approach allowed to access the SM on previously categorized well exposed (8/8), partially exposed (4/5), and wholly concealed (1/3) SM with respect to FN. Following intraoperative evaluation in the remaining 18.8% (3/16), the SM was accessed anteriorly via drilling of the PE. Exposure of SM with respect to the FN and the sigmoid sinus’s prominence was a predictor for the suitable surgical approach. The retrofacial approach offers feasible and reproducible access to the SM belly, opening direct access to electromyographic sensing of the eSRT. Surgical planner tools can quantitatively assist pre-surgical assessment.

Hypothesis: Photon-counting detector CT (PCD-CT) with deep learning-supported denoising can significantly reduce the radiation dose for cochlear implant (CI) planning without compromising the accuracy of cochlear duct length (CDL) measurements. Background: Optimal electrode placement in CI surgery requires detailed cochlear anatomy from CT scans, but reducing radiation exposure is critical. This study explores PCD-CT with denoising algorithms to lower doses while preserving diagnostic accuracy. Methods: Four body donors without inner ear malformations were scanned using PCD-CT at 100%, 50%, 25%, 10%, and 5% dose levels. Images were denoised with ClariAce, a deep learning algorithm, and CDL was measured using OTOPLAN software. Neurotologists compared the results to manual segmentations. Statistical analyses evaluated accuracy across dose levels, with Bland-Altman plots assessing systematic errors. Results: Automatic segmentation succeeded across all doses but showed increased failure below 50%. At 100% and 50% doses, CDL measurements closely matched the gold standard, with minor deviations (eg, -0.17 mm at 50%). Below 50%, CDL underestimation increased (−1.25 mm at 25% and −4.0 mm at 5%). Denoising improved segmentation but minimally affected CDL accuracy at low doses, where manual segmentation performed better. Conclusions: PCD-CT enables significant dose reduction for CI planning, with reliable CDL accuracy down to 50%. Deep learning denoising enhances image quality but is less effective below 50%, necessitating manual segmentation. These findings align with ALARA principles and suggest further refinement of AI algorithms for lower-dose applicability in CI diagnostics.

This study was designed to examine the changes in emergency room visits in otolaryngology, head and neck surgery, during the COVID-19 pandemic. The study included 11,277 patients who presented to a tertiary care hospital (ER) and an emergency practice (EP) during on-call hours in the first half of 2018, 2019, and 2020. The epidemiologic parameters, diagnoses, and level of urgency were recorded using a four-step scale. A comparison was made between the pre-pandemic years and 2020. The findings revealed a significant decrease in the frequency of ER visits in the second quarter of 2020 compared to 2019 (ER: 30.8%, EP: 37.8%), mainly due to the fact that there were significantly fewer patients, with low levels of urgency. Certain diagnoses, such as epistaxis (−3.0%) and globus sensation (−3.2%), were made at similar frequencies to 2019, while inflammatory diseases like skin infections (−51.2%), tonsillitis (−55.6%), sinusitis (−59%), and otitis media (−70.4%) showed a significant reduction. The study concludes that patients with a low triage level were less likely to visit the ER during the early stages of the pandemic, but some diagnoses were still observed at comparable rates. This suggests a disparity in perception between patients and ER staff regarding urgency. Many of the issues discussed were also emphasized in the 2024 proposal by the German Ministry of Health to reform emergency care in Germany.

Objective: To determine the effect on the frequency of revision surgery caused by electrode migration (EM) in cochlear implantation by use of an electrode fixation clip combined with a novel surgical technique. Study Design: Retrospective chart review. Setting: Tertiary referral center. Patients: The investigated institutional cohort included 1052 cases of cochlear implantation with straight electrode arrays from a single manufacturer between 2011 and 2023. Until 2016, the fixation clip was not used, whereas since 2017, the fixation clip and the described novel surgical technique have been applied as an institutional standard in nearly all cases. Intervention: The titanium electrode fixation clip was first introduced and described in 1998 and was CE marked in Europe in 2014. In modification of the original description, a “Reverse Fixation Clip Technique” was applied to optimize the surgical handling and fixation stability. A similar approach has been described recently. Main Outcome Measure: Cumulative incidence of revision surgery caused by EM. Results: The cumulative incidence of revision surgery caused by EM at 3, 5, and 7 years was 0.5%, 1.0%, and 1.9% in the group without the fixation clip (n=461; 43.4%) and 0.0% at all time points in the group with the fixation clip using the “Reverse Fixation Clip Technique” (n=591; 56.6%) (log-rank test P =0.054). No fixation clip-related complications were noted. Conclusion: The application of the fixation clip using the “Reverse Fixation Clip Technique” prevents revision surgery caused by EM in cochlear implantation.