No Impairment in Bone Turnover or Executive Functions in Well-Treated Preschoolers with Phenylketonuria-A Pilot Study.

Patients with phenylketonuria (PKU) present signs of impaired executive functioning and bone health in adolescence and adulthood, depending in part on the success of therapy in childhood. Therefore, nine children with well-treated PKU (4-7 years old, 22.2% ♀, seven with a full set of data, two included into partial analysis) and 18 age-, gender- and season-matched controls were analyzed for differences in executive functioning and bone parameters in plasma. Plasma was analyzed with commercially available kits. Cognitive performance in tonic alertness, visuo-spatial working memory, inhibitory control and task switching was assessed by a task battery presented on a touch screen. Regarding cognition, only the performance in incongruent conditions in inhibitory control was significantly better in children with PKU than in controls. No further differences in cognitive tests were detected. Furthermore, no significant difference in the bone turnover markers osteocalcin, undercarboxylated osteocalcin and CTX were detected between children with PKU and controls, while children with PKU had a significantly higher vitamin D concentration (69.44 ± 12.83 nmol/L vs. 41.87 ± 15.99 nmol/L, p < 0.001) and trended towards lower parathyroid hormone concentrations than controls (48.27 ± 15.16 pg/mL vs. 70.61 ± 30.53 pg/mL, p = 0.066). In this small group of well-treated preschoolers with PKU, no impairments in cognitive performance and bone turnover were observed, while vitamin D supplementation of amino acid supplements seems to be sufficient to achieve good vitamin D status.

Electrically evoked compound action potentials are associated with the site of intracochlear stimulation.

OBJECTIVES: Objective measurements to predict the position of a cochlear electrode during cochlear implantation surgery may serve to improve the surgical technique and postoperative speech outcome. There is evidence that electrically evoked compound action potentials (ECAP) are a suitable approach to provide information about the site of stimulation. This study aims to contribute to the knowledge about the association between the intraoperative intracochlear ECAP characteristics and the site of stimulation. METHODS: In a retrospective cohort study, patients undergoing cochlear implant surgery with flexible lateral wall electrode arrays (12 stimulating channels) between 2020 and 2022 were analyzed. The CDL was measured using a CT-based clinical planning software. ECAP were measured for all electrode contacts and associated to the CDL as well as to the site of stimulation in degree. RESULTS: Significant differences among the amplitudes and slopes for the individual stimulated electrode contacts at the stimulation sites of 90°, 180°, 270°, 360°, 450° and 540° were found. The values showed a trend for linearity among the single electrodes. CONCLUSIONS: ECAP characteristics correlate with the electrode's position inside the cochlea. In the future, ECAP may be applied to assess the intracochlear position inside the cochlea and support anatomy-based fitting.

Monitoring mepolizumab treatment in chronic rhinosinusitis with nasal polyps (CRSwNP): Discontinue, change, continue therapy?

BACKGROUND: Chronic rhinosinusitis with nasal polyps (CRSwNP) is a multifactorial inflammatory disease of the mucous membranes of the nose and sinuses. Eosinophilic inflammation is described as a common endotype. The anti-IL-5 antibody mepolizumab was approved in November 2021 as an add-on therapy to intranasal glucocorticosteroids for the treatment of adults with severe chronic rhinosinusitis with nasal polyps when systemic glucocorticosteroids or surgery do not provide adequate disease control. While national and international recommendations exist for the use of mepolizumab in CRSwNP, it has not yet been adequately specified how this therapy should be monitored, what follow-up documentation is necessary, and when it should be discontinued if necessary. MATERIALS AND METHODS: A literature search was performed to analyze previous data on the treatment of CRSwNP with mepolizumab and to determine the available evidence by searching Medline, Pubmed, the national and international trial and guideline registries, and the Cochrane Library. Human studies published in the period up to and including 10/2022 were considered. RESULTS: Based on the international literature and previous experience by an expert panel, recommendations for follow-up, adherence to therapy intervals, and possible therapy breaks as well as discontinuation of therapy when using mepolizumab for the indication CRSwNP in the German healthcare system are given on the basis of a documentation sheet. CONCLUSION: Understanding the immunological basis of CRSwNP opens up new non-surgical therapeutic approaches with biologics for patients with severe, uncontrolled courses. Here, we provide recommendations for follow-up, adherence to therapy intervals, possible therapy pauses, or discontinuation of therapy when mepolizumab is used as add-on therapy with intranasal glucocorticosteroids to treat adult patients with severe CRSwNP that cannot be adequately controlled with systemic glucocorticosteroids and/or surgical intervention.

High-Dose Glucocorticoids for the Treatment of Sudden Hearing Loss.

BACKGROUND: Systemic glucocorticoids are commonly used for primary therapy of idiopathic sudden sensorineural hearing loss (ISSNHL). However, the comparative effectiveness and risk profiles of high-dose over lower-dose regimens remain unknown. METHODS: We randomly assigned patients with sudden hearing loss of greater than or equal to 50 dB within 7 days from onset to receive either 5 days of high-dose intravenous prednisolone at 250 mg/d (HD-Pred), 5 days of high-dose oral dexamethasone at 40 mg/d (HD-Dex), or, as a control, 5 days of oral prednisolone (Pred-Control) at 60 mg/d followed by 5 days of tapering doses. The primary outcome was the change in hearing threshold (pure tone average) in the three most affected contiguous frequencies from baseline to day 30. Secondary outcomes included speech understanding, tinnitus, communication competence, quality of life, hypertension, and insulin resistance. RESULTS: A total of 325 patients were randomly assigned. Mean change in 3PTAmost affected hearing threshold from baseline to 30 days was 34.2 dB (95% CI, 28.4 to 40.0) in the HD-Pred group, 41.4 dB (95% CI, 35.6 to 47.2) in the HD-Dex group, and 41.0 dB (95% CI, 35.2 to 46.8) in the Pred-Control group (P=0.09 for analysis of variance). There were more adverse events related to trial medication in the HD-Pred (n=73) and HD-Dex (n=76) groups than in the Pred-Control group (n=46). CONCLUSIONS: Systemic high-dose glucocorticoid therapy was not superior to a lower-dose regimen in patients with ISSNHL, and it was associated with a higher risk of side effects. (Funded by the Federal Ministry of Education and Research [BMBF]; EudraCT number, 2015­002602­36.)

[Providing practical skills in curricular teaching-effect of SkillsLab and flipped classroom]. / Vermittlung praktischer Fähigkeiten in der curricularen Lehre ­ Effekt von SkillsLab und "flipped classroom".

BACKGROUND: In the course of the restructuring of medical studies, practical competencies are clearly defined as learning objectives for the first time. In order to make most effective use of the short attendance time available in otolaryngology, the aim of this study was to teach practical skills with the help of flipped classroom, digital teaching, and a newly established SkillsLab. MATERIALS AND METHODS: During their ENT internship, two groups of students-group A = 93 students (male n = 42, female n = 51) and group B = 113 students (male n = 42, female n = 71)-first worked through material provided online, which explained the individual examinations. This was followed by face-to-face teaching, which consisted of observation and practical exercise of the different examination techniques. While group A practiced on each other or on dummies, group B used structured workstations in the ENT SkillsLab, which was newly built for this purpose. The effects on motivation and subjective competence were measured using a questionnaire developed for this study. RESULTS: After working through the online material, both groups showed a high level of motivation and competence. On the day of face-to-face teaching, there was a gain in motivation and competence, which was statistically significant only in the SkillsLab group (p < 0.001). Although the SkillsLab group was inferior in terms of its subjective competence at the beginning, it was superior to the other group after the face-to-face teaching. CONCLUSION: Combination of digitized teaching in the flipped classroom with structured workstations in the setting of a SkillsLab enables more effective teaching of practical skills, which was reflected by increases in motivation and subjective competence in group B. In particular, the presentation of all collected findings on monitors allows verification of learning success and stimulates discussion.

Comparing linear and non-linear models to estimate the appropriate cochlear implant electrode array length-are current methods precise enough?

PURPOSE: In cochlear implantation with flexible lateral wall electrode arrays, a cochlear coverage (CC) range between 70% and 80% is considered ideal for optimal speech perception. To achieve this CC, the cochlear implant (CI) electrode array has to be chosen according to the individual cochlear duct length (CDL). Here, we mathematically analyzed the suitability of different flexible lateral wall electrode array lengths covering between 70% and 80% of the CDL. METHODS: In a retrospective cross-sectional study preoperative high-resolution computed tomography (HRCT) from patients undergoing cochlear implantation was investigated. The CDL was estimated using an otosurgical planning software and the CI electrode array lengths covering 70-80% of the CDL was calculated using (i) linear and (ii) non-linear models. RESULTS: The analysis of 120 HRCT data sets showed significantly different model-dependent CDL. Significant differences between the CC of 70% assessed from linear and non-linear models (mean difference: 2.5 mm, p < 0.001) and the CC of 80% assessed from linear and non-linear models (mean difference: 1.5 mm, p < 0.001) were found. In up to 25% of the patients none of the existing flexible lateral wall electrode arrays fit into this range. In 59 cases (49,2%) the models did not agree on the suitable electrode arrays. CONCLUSIONS: The CC varies depending on the underlying CDL approximation, which critically influences electrode array choice. Based on the literature, we hypothesize that the non-linear method systematically overestimates the CC and may lead to rather too short electrode array choices. Future studies need to assess the accuracy of the individual mathematical models.

Longitudinal trajectories of memory among middle-aged and older people with hearing loss: the influence of cochlear implant use on cognitive functioning.

Introduction: Cochlear implants (CI) are the gold standard intervention for severe to profound hearing loss, a known modifiable risk factor for dementia. However, it remains unknown whether CI use might prevent the age-related cognitive decline. Recent studies are encouraging but are limited, mainly by short follow-up periods and, for ethical reasons, lack of appropriate control groups. Further, as age-related cognitive decline is multifaceted and not linear, other statistical approaches have to be evaluated. Materials and methods: Immediate and delayed recall as measures of cognitive function were assessed in 75 newly implanted CI users (mean age 65.41 years ± 9.19) for up to 5 years (mean 4.5 ± 0.5) of CI use and compared to 8,077 subjects of the same age range from two longitudinal cohort studies, the Health and Retirement Study (HRS) and the English Longitudinal Study of Aging (ELSA). Linear and quadratic changes in cognitive trajectories were analyzed in detail using mixed growth models, considering possible confounders. Results: For CI users, the linear time slope showed a significant improvement in the specific domains (recall and delayed recall) over time. The quadratic time slope clearly indicated that the predicted change after CI provision followed an inverted U-shape with a predicted decline 2 years after CI provision. In the hearing-impaired group, a significant decline over time was found, with steeper declines early on and the tendency to flatten out in the follow-up. Conclusion: Cochlear implant use seems to boost cognitive trajectories in the first years after implantation. However, long-term prevention of dementia seems to need far more than restoration of hearing loss.

Performing Repeated Intraoperative Impedance Telemetry Measurements during Cochlear Implantation.

Impedance measurements are routinely performed during cochlear implantation (CI) after finalized electrode insertion. They may allow conclusions on the electrode's and implant's function. In the postoperative setting, the analysis of impedance changes enables the identification of scarring or inflammation processes around the electrode. Recent studies report associations between impedance telemetry and the site of stimulation. Consequently, repeated impedance measurements during cochlear implant electrode insertion may enable objective feedback on whether the electrode is positioned inside the perilymph or outside the inner ear. With the presented novel method, impedances can be measured in real-time during cochlear implantation. This protocol systematically explains how to perform repeated impedance recordings during CI surgery. These repeated measurements are challenging since they depend on multiple intraoperative methodological factors starting with the draping of the patient. Thus, for successful recordings, a standardized procedure is mandatory. In this article, we comprehensively illustrate the system setup and procedure of performing intraoperative measurements during CI surgery.

Accuracy of Preoperative Cochlear Duct Length Estimation and Angular Insertion Depth Prediction.

OBJECTIVE: In cochlear implantation with flexible lateral wall electrodes, a cochlear coverage of 70% to 80% is assumed to yield an optimal speech perception. Therefore, fitting the cochlear implant (CI) to the patient's individual anatomy has gained importance in recent years. For these reasons, the optimal angular insertion depth (AID) has to be calculated before cochlear implantation. One CI manufacturer offers a software that allows to visualize the AID of different electrode arrays. Here, it is hypothesized that these preoperative AID models overestimate the postoperatively measured insertion angle. This study aims to investigate the agreement between preoperatively estimated and postoperatively measured AID. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Single-center tertiary referral center. PATIENTS: Patients undergoing cochlear implantation. INTERVENTION: Preoperative and postoperative high-resolution computed tomography (HRCT). MAIN OUTCOME MEASURES: The cochlear duct length was estimated by determining cochlear parameters ( A value and B value), and the AID for the chosen electrode was (i) estimated by elliptic circular approximation by the software and (ii) measured manually postoperatively by detecting the electrode contacts after insertion. RESULTS: A total of 80 HRCT imaging data sets from 69 patients were analyzed. The mean preoperative AID estimation was 662.0° (standard deviation [SD], 61.5°), and the mean postoperatively measured AID was 583.9° (SD, 73.6°). In all cases (100%), preoperative AID estimation significantly overestimated the postoperative determined insertion angle (mean difference, 38.1°). A correcting factor of 5% on preoperative AID estimation dissolves these differences. CONCLUSIONS: The use of an electrode visualization tool may lead to shorter electrode array choices because of an overestimation of the insertion angle. Applying a correction factor of 0.95 on preoperative AID estimation is recommended.

[Health-related quality of life in chronic otitis media-measurement methods and their application in surgical therapy]. / Gesundheitsbezogene Lebensqualität bei der chronischen Otitis media ­ Messmethoden und deren Anwendung bei chirurgischen Therapien.

BACKGROUND: Chronic otitis media (COM) can lead to significant impairment of health-related quality of life (HRQoL) due to symptoms such as otorrhea, pain, hearing loss, tinnitus, or dizziness. A systematic assessment of HRQoL in COM is becoming increasingly important as it complements (semi-)objective outcome parameters in clinical practice and research. HRQoL is measured by means of patient-reported outcome measures (PROMs). There are two disease-specific validated PROMs available for COM in German-the Chronic Otitis Media Outcome Test (COMOT-15) and the Zurich Chronic Middle Ear Inventory (ZCMEI-21)-which have become increasingly popular in recent years. OBJECTIVE: The purpose of this narrative review is to present the current state of research on measuring HRQoL in COM before and after surgical procedures. RESULTS AND CONCLUSION: Hearing is the most important factor influencing HRQoL in COM. Surgical procedures usually result in a clinically relevant improvement in HRQoL in COM with or without cholesteatoma. However, if cholesteatoma is present, its extent does not correlate with HRQoL. While HRQoL plays a secondary role in establishment of the indication for surgical therapy in COM with cholesteatoma, it plays an important role in terms of relative surgical indications, e.g., a symptomatic open mastoid cavity after resection of the posterior canal wall. We encourage the regular use of disease-specific PROMs preoperatively as well as during follow-up to assess HRQoL in COM in individual patients, in research, and in the context of quality monitoring.

Intra- and Interrater Reliability of CT- versus MRI-Based Cochlear Duct Length Measurement in Pediatric Cochlear Implant Candidates and Its Impact on Personalized Electrode Array Selection.

BACKGROUND: Radiological high-resolution computed tomography-based evaluation of cochlear implant candidates' cochlear duct length (CDL) has become the method of choice for electrode array selection. The aim of the present study was to evaluate if MRI-based data match CT-based data and if this impacts on electrode array choice. METHODS: Participants were 39 children. CDL, length at two turns, diameters, and height of the cochlea were determined via CT and MRI by three raters using tablet-based otosurgical planning software. Personalized electrode array length, angular insertion depth (AID), intra- and interrater differences, and reliability were calculated. RESULTS: Mean intrarater difference of CT- versus MRI-based CDL was 0.528 ± 0.483 mm without significant differences. Individual length at two turns differed between 28.0 mm and 36.6 mm. Intrarater reliability between CT versus MRI measurements was high (intra-class correlation coefficient (ICC): 0.929-0.938). Selection of the optimal electrode array based on CT and MRI matched in 90.1% of cases. Mean AID was 629.5° based on the CT and 634.6° based on the MRI; this is not a significant difference. ICC of the mean interrater reliability was 0.887 for the CT-based evaluation and 0.82 for the MRI-based evaluation. CONCLUSION: MRI-based CDL measurement shows a low intrarater difference and a high interrater reliability and is therefore suitable for personalized electrode array selection.