Recessively Inherited Deficiency of Secreted WFDC2 (HE4) Causes Nasal Polyposis and Bronchiectasis.

Rationale: Bronchiectasis is a pathological dilatation of the bronchi in the respiratory airways associated with environmental or genetic causes (e.g., cystic fibrosis, primary ciliary dyskinesia, and primary immunodeficiency disorders), but most cases remain idiopathic. Objectives: To identify novel genetic defects in unsolved cases of bronchiectasis presenting with severe rhinosinusitis, nasal polyposis, and pulmonary Pseudomonas aeruginosa infection. Methods: DNA was analyzed by next-generation or targeted Sanger sequencing. RNA was analyzed by quantitative PCR and single-cell RNA sequencing. Patient-derived cells, cell cultures, and secretions (mucus, saliva, seminal fluid) were analyzed by Western blotting and immunofluorescence microscopy, and mucociliary activity was measured. Blood serum was analyzed by electrochemiluminescence immunoassay. Protein structure and proteomic analyses were used to assess the impact of a disease-causing founder variant. Measurements and Main Results: We identified biallelic pathogenic variants in WAP four-disulfide core domain 2 (WFDC2) in 11 individuals from 10 unrelated families originating from the United States, Europe, Asia, and Africa. Expression of WFDC2 was detected predominantly in secretory cells of control airway epithelium and also in submucosal glands. We demonstrate that WFDC2 is below the limit of detection in blood serum and hardly detectable in samples of saliva, seminal fluid, and airway surface liquid from WFDC2-deficient individuals. Computer simulations and deglycosylation assays indicate that the disease-causing founder variant p.Cys49Arg structurally hampers glycosylation and, thus, secretion of mature WFDC2. Conclusions: WFDC2 dysfunction defines a novel molecular etiology of bronchiectasis characterized by the deficiency of a secreted component of the airways. A commercially available blood test combined with genetic testing allows its diagnosis.

[Fundamentals and practice of the application of nasal packing in sinonasal surgery]. / Grundlagen und Praxis der Anwendung von Nasentamponaden in der endonasalen Chirurgie.

BACKGROUND AND OBJECTIVES: This paper presents an overview on nasal packing materials which are available in Germany. The current literature is analyzed whether there are robust criteria regarding use nasal packing after sinonasal surgery, whether there are fundamental and proven advantages or disadvantages of products, and what this means in clinical practice. MATERIALS AND METHODS: Selective literature analysis using the PubMed database (key words "nasal packing", "nasal tamponade", "nasal surgery", "sinonasal surgery", or "sinus surgery"), corresponding text books and resulting secondary literature. RESULTS AND CONCLUSIONS: Because of systematic methodological shortcomings, the literature does not help in the decision-making about which nasal packing should be used after which kind of sinonasal surgery. In fact, individual approaches for the many different clinical scenarios are recommended. In principle, nasal packing aims in hemostasis, should promote wound healing, and should not result in secondary morbidity. Nasal packing materials should be smooth (non-absorbable materials), inert (absorbable materials), and should not exert excessive pressure. Using non-absorbable packing entails the risk of potentially lethal aspiration and ingestion. For safety reasons inpatient control is recommended as long as this packing is in situ. With other, uncritical packing materials and in patients with special conditions, outpatient control could be justified.

[Treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) with monoclonal antibodies (biologics): S2k guideline of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery (DGHNO-KHC), and the German College of General Practitioners and Family Physicians (DEGAM)]. / Therapie der chronischen Rhinosinusitis mit Polyposis nasi (CRScNP) mit monoklonalen Antikörpern (Biologika): S2k-Leitlinie der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie (DGHNO-KHC) und der Deutschen Gesellschaft für Allgemeinmedizin und Familienmedizin (DEGAM).

Monoclonal antibodies (so-called biologics) can be prescribed for chronic rhinosinusitis with nasal polyps (CRSwNP) within the scope of their market authorization. However, their prescription is limited to severe CRSwNP without disease control, whereby certain requirements must be met. Dupilumab, omalizumab, and mepolizumab have currently gained market authorization, with adequate evidence for their efficacy and safety available in the literature. It can be assumed that other biologics will be approved for this indication in the future. The severity of disease and the efficacy of treatment should be assessed objectively and subjectively before treatment initiation and after an appropriate duration, respectively. The documentation sheet proposed in this guideline chapter can be used for the assessments. In the presence of relative contraindications, a treatment should only be initiated after differentiated consideration by an experienced physician in the sense of a case-by-case decision. In summary, this guideline chapter aims to contribute to high-quality care of adult patients with these therapies in view of the increasing evidence for treatment with these substances and the increasing number of market authorizations of different biologics.

[Functional disorders of the nose - definition, diagnostics and treatment options]. / Funktionelle Formstörungen der Nase ­ Definition, Diagnostik und Therapieoptionen.

The surgical spectrum of functional rhinosurgery includes nasal septum surgery, septorhinoplasty and nasal concha surgery. Based on the German guideline "Disorders of the inner and/or external nose (with functional and/or aesthetic impairment)" published in April 2022, which was prepared on behalf of the German Society of Otorhinolaryngology, Head and Neck Surgery, we discuss the indications, diagnostic approaches, the planning of the surgery and postoperative care. The most common findings of the external nose with a functional impairment include crooked nose, saddle nose, and tension nose. Combined pathologies occur. Well-documented in-depth consultation is essential for rhinosurgical procedures. In the case of revision surgery, the possible necessity of autologous ear or rib cartilage should be considered. Despite correct surgical performance, no "guarantee" can be given for the surgical (long-term) result in rhinosurgery.

Detecting myositis as a cause of unexplained dysphagia: Proposal for a diagnostic algorithm.

BACKGROUND AND PURPOSE: Idiopathic inflammatory myopathy (IIM) can present with dysphagia as a leading or only symptom. In such cases, diagnostic evaluation may be difficult, especially if serological and electromyographical findings are unsuspicious. In this observational study we propose and evaluate a diagnostic algorithm to identify IIM as a cause of unexplained dysphagia. METHODS: Over a period of 4 years, patients with unexplained dysphagia were offered diagnostic evaluation according to a specific algorithm: The pattern of dysphagia was characterized by instrumental assessment (swallowing endoscopy, videofluoroscopy, high-resolution manometry). Patients with an IIM-compatible dysphagia pattern were subjected to further IIM-focused diagnostic procedures, including whole-body muscle magnetic resonance imaging, electromyography, creatine kinase blood level, IIM antibody panel and, as a final diagnostic step, muscle biopsy. Muscle biopsies were taken from affected muscles. In cases where no other muscles showed abnormalities, the cricopharyngeal muscle was targeted. RESULTS: Seventy-two patients presented with IIM-compatible dysphagia as a leading or only symptom. As a result of the specific diagnostic approach, 19 of these patients were diagnosed with IIM according to the European League Against Rheumatism (EULAR) criteria. Eighteen patients received immunomodulatory therapy as a result of the diagnosis. Of 10 patients with follow-up swallowing examination, dysphagia improved in three patients after therapy, while it remained at least stable in six patients. CONCLUSIONS: Idiopathic inflammatory myopathy constitutes a potentially treatable etiology in patients with unexplained dysphagia. The diagnostic algorithm presented in this study helps to identify patients with an IIM-compatible dysphagia pattern and to assign those patients for further IIM-focused diagnostic and therapeutic procedures.

Framework and baseline examination of the German National Cohort (NAKO).

The German National Cohort (NAKO) is a multidisciplinary, population-based prospective cohort study that aims to investigate the causes of widespread diseases, identify risk factors and improve early detection and prevention of disease. Specifically, NAKO is designed to identify novel and better characterize established risk and protection factors for the development of cardiovascular diseases, cancer, diabetes, neurodegenerative and psychiatric diseases, musculoskeletal diseases, respiratory and infectious diseases in a random sample of the general population. Between 2014 and 2019, a total of 205,415 men and women aged 19-74 years were recruited and examined in 18 study centres in Germany. The baseline assessment included a face-to-face interview, self-administered questionnaires and a wide range of biomedical examinations. Biomaterials were collected from all participants including serum, EDTA plasma, buffy coats, RNA and erythrocytes, urine, saliva, nasal swabs and stool. In 56,971 participants, an intensified examination programme was implemented. Whole-body 3T magnetic resonance imaging was performed in 30,861 participants on dedicated scanners. NAKO collects follow-up information on incident diseases through a combination of active follow-up using self-report via written questionnaires at 2-3 year intervals and passive follow-up via record linkages. All study participants are invited for re-examinations at the study centres in 4-5 year intervals. Thereby, longitudinal information on changes in risk factor profiles and in vascular, cardiac, metabolic, neurocognitive, pulmonary and sensory function is collected. NAKO is a major resource for population-based epidemiology to identify new and tailored strategies for early detection, prediction, prevention and treatment of major diseases for the next 30 years.

Cross-cultural adaptation and validation of the 22-item sinonasal outcome test (SNOT-22) in German-speaking patients: a prospective, multicenter cohort study.

PURPOSE: Chronic rhinosinusitis (CRS) is a common condition associated with a significant reduction of the health-related quality of life. One of the most widely used assessment tools in CRS is the disease-specific, health-related questionnaire SNOT-22. The aim of this study was to translate and validate the SNOT-22 into the German language. METHODS: The questionnaire was translated using the forward-backward translation technique. After the translation its reliability, validity, and sensitivity were evaluated. For this purpose, the questionnaire was completed by patients diagnosed with CRS before, 3 months and 1 year after endoscopic sinus surgery and by healthy individuals as controls at three university hospitals in Germany. The individual scores of the questionnaire before surgery was correlated with the Lund-Mackay score as well as a global disease-specific question. RESULTS: A total of 139 CRS patients and 31 healthy individuals participated in the study. Internal consistency at all timepoints was very good, with Cronbach's alpha scores of 0.897, 0.941, and 0.945. The questionnaire was able to discriminate between CRS patients and control subjects (p < 0.0001) and scores improved significantly 3 month and 1 year after sinus surgery (p < 0.0001), indicating a good test-retest reliability, validity, and responsiveness. A significant correlation to the single global disease-specific question could be found (p < 0.0001), but no correlation with the Lund-Mackay score. CONCLUSION: The German Version of the SNOT-22 is a reliable, valid, and sensitive instrument for measuring health-related quality of life in patients with CRS. It can be recommended for clinical practice and outcome research for German-speaking patients.

Awake Rhinology Surgery in Response to the COVID-19 Pandemic in Europe.

BACKGROUND: European health-care systems are faced with a backlog of surgical procedures following the suspension of routine surgery during the COVID-19 crisis. Routine rhinology surgery under general anaesthetic (GA) is now faced with significant challenges which include limited theatre capacity, the negative ramifications of surgical prioritization, reduced patient throughput in secondary care, and additional personal protective equipment requirements. Delayed surgery in rhinology, particularly with regards to chronic rhinosinusitis, has previously been shown to have poorer surgical outcomes, a detrimental effect on quality of life and long-term negative health socio-economic effects. Awake rhinology surgery under local anaesthetic (LA) provides an ideal alternative to GA. It provides a means of operating on patients in a setting alternative to currently oversubscribed main theatres, by utilizing satellite facilities, while ensuring identical surgical outcomes for patients who may otherwise have been forced to wait a long time for their procedure. It also confers additional benefits in terms of shorter recovery time and hospital stay for patients. OBJECTIVES: We have developed a set of recommendations that are intended to help support clinicians and managers to better adopt LA rhinology protocols and minimize the risk to the patient and health-care professionals involved. METHODOLOGY: International roundtable forums were conducted and supplemented by individual interviews. The international board consisted of 12 rhinologists experienced in awake rhinology surgery. Feedback was analysed and shared to develop a consensus of best practice. RECOMMENDATIONS: Local and national guidelines need to be adhered to with specific focus on patient and clinician safety. When performing awake rhinology procedures in the COVID-19 recovery process, consider implementing specific safety measures and workflow practices to safeguard patients and staff and minimize the risk of infection. CONCLUSION: Awake surgery potentially provides quicker access to routine rhinology surgery in the post-COVID-19 recovery phase, ensuring patients are treated in a timely matter, thereby avoiding higher downstream costs, and improving outcomes.

Results of a national web-based survey on the SARS-CoV-2 infectious state of otorhinolaryngologists in Germany.

PURPOSE: SARS-CoV-2 is detected on the mucosa of the upper airways to a high degree. In the course of the COVID-19 pandemic, otorhinolaryngologists (ORL) are assumed to be at high risk due to close contact with the mucosa of the upper airways. No data are yet available providing evidence that ORLs have an increased risk of infection. METHODS: German ORLs were invited via e-mail through the German Society of ORL, Head and Neck Surgery and the German ENT Association to participate in a web-based survey about infection with SARS-CoV-2 and development of COVID-19. Data of infections and concomitant parameters in German ORLs were collected and compared to the total number of infections in Germany. RESULTS: Out of 6383 German ORLs, 970 (15%) participated. 54 ORLs reported testing positive for SARS-CoV-2. Compared to the total population of Germany, ORLs have a relative risk of 3.67 (95% CI 2.82; 4.79) of contracting SARS-CoV-2. Domestic quarantine was conducted in 96.3% of cases. Two individuals were admitted to hospital without intensive care. No casualties were reported. In 31 cases, the source of infection was not identifiable whereas 23 had a clear medical aetiology: infected patients: n = 5, 9.26%; medical staff: n = 13, 14.1%. 9.26% (n = 5) of the identified cases were related to contact to infected family members (n = 3), closer neighbourhood (n = 1) or general public (n = 1). There was no identified increased risk of infection due to performing surgery. CONCLUSION: German ORLs have an almost 3.7-fold risk of contracting SARS-CoV-2 compared to the population baseline level. Appropriate protection appears to be necessary for this occupational group.

Follow-up of a national web-based survey on the SARS-CoV-2 infectious state of otorhinolaryngologists in Germany.

PURPOSE: The SARS-CoV­2 pandemic has affected the health and practice of otorhinolaryngologists (ORLs) for over 1 year. Follow-up data of a national survey with German ORLs were evaluated regarding differences between the two waves of the pandemic. METHODS: As in the initial survey, German ORLs were addressed via e­mail through the German Society of ORL, Head and Neck Surgery and the German ENT Association. All ORLs afflicted with SARS-CoV­2 were invited to participate in a web-based survey. General data on infections and concomitant parameters were evaluated. RESULTS: Since the start of the pandemic, 129 ORLs reported testing positive for SARS-CoV­2 in Germany. The ORLs infected during the first wave had a relative risk (RR) of 4.07 (95% CI: 3.20; 5.19) of contracting SARS-CoV­2. During the second wave, the RR decreased to 0.35 (95% CI: 0.28; 0.45). The availability of personal protective equipment (PPE) increased from the first to the second wave along with an increased perception of protection in the professional environment. The source of infection shifted from infections via medical staff during the first wave to patients and household exposure during the second wave. Regular medical practice was resumed by clinicians and general practitioners in the second wave. Nevertheless, a proportionally lower infection rate was observed compared with the German population as a whole. CONCLUSION: The data reflect a unique long-term survey of ORLs during the pandemic. Differences in the source of infection were seen between the first and second wave, confirming the need for appropriate PPE for medical professionals working in high-risk environments. Further strategies to reduce the risk of infection include consistent testing for SARS-CoV­2 in healthcare professionals, patients, and the general public as well as vaccination of high-risk medical groups.

Influence of the Subjective Body Image on the Outcome of Functional Rhinoplasty.

Improvement of nasal breathing is considered to be the major aspect of functional rhinoplasty (fRPL). Nevertheless, simultaneous aesthetic modifications can be required to achieve sufficient functional enhancement. Thus, the aim of this study was to assess the influence of the subjective perception of the nasal appearance on the outcome of fRPL. Patients undergoing fRPL were asked to complete the German version of the Utrecht Questionnaire for Outcome Assessment in Aesthetic Rhinoplasty (D-OAR) preoperatively, 1, 3 and 12 months after surgery. The patients' satisfaction with the procedure's result was determined using a five-point Likert scale 1, 3 and 12 months after rhinoplasty. In total, 87 patients (42 males and 45 females) with a median age of 25 years undergoing fRPL were included in this study. Compared to males, females showed diminished VAS scores (4.03 ± 2.02 vs 2.71 ± 1.96, p = 0.006) and higher D-OAR scores during preoperative outpatient consultation (13.34 ± 5.00 vs 16.07 ± 5.62, p = 0.020). An increase in the VAS score and a decrease in the D-OAR score were observed independent of gender post-operatively. Significant correlations between the patients' satisfaction and the D-OAR score at each time point of assessment were demonstrated, whereas no significant correlation between the post-operative patients' satisfaction and the initial D-OAR score could be identified. These results demonstrate the importance of body image and the subjective perception of the nasal appearance in particular in patients undergoing fRPL which should be taken into consideration of surgeons preoperatively. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Telemedical Methods in Otorhinolaryngology. / Telemedizinische Methoden in der Hals-Nasen-Ohrenheilkunde.

Telemedicine is a generic term summarizing all communication techniques assisting to provide medical services over distance. This rapidly developing medical area provides especially for the field of otorhinolaryngology with its high dependency on endoscopy and microcopy vaulable options. For clinical examination of ear, nose, throat and larynx, but also for postoperative care and surveillance of head and neck tumor patients data exist using the telemedical approach. Furthermore, audiological telemedical measurements even after Cochlear implantation are deemed feasible. While first data indicate interesting approaches, they also demonstrate problems, if a telemedical approach is used without proper preparation. Especially the observers at the remote location of the patients and their training are highly important in this regard. This review summarizes the current state of science of this rapidly changing are of research and clinical care.

[Survey on the need for an e-learning-platform for ENT residents]. / Umfrage zum Bedarf einer e-Learning-Plattform für Ärzte in der HNO-Facharzt-Weiterbildung.

OBJECTIVE: Interactive e-learning-platforms may replace the classical textbook in the future. Such media have the possible advantage of including video and audio files in a more comprehensive way, but ENT-specific platforms do not currently exist. So far, the actual needs and wishes of ENT residents are unclear and may be affected by the so called digital revolution. MATERIAL AND METHODS: An online survey was carried out addressing all ENT residents in Germany known to the German society of oto-rhino-laryngology, head and neck surgery. A 17-items survey was developed by ENT doctors receiving and providing training and distributed by e-mail. The survey was available to answer in April and May 2019. RESULTS: A total of 150 out of 671 ENT specialists and residents took part in the study. Of these, 80 % were residents and 20 % were ENT specialists. 63-80 % of the respondents already use online media in general at work, in preparation for the ENT specialist examination, for training purposes and for support as a clinician. 92-95 % of the participants indicated the willingness to use an interactive platform for their ENT specialist examination preparation and further training. On average, e-learning media are used by the responders to prepare for the ENT specialist examination or in clinical everyday life and for further training 108 or 130 min/week. The desire for surgical instruction videos is also very high. CONCLUSION: There is a high demand for a structured e-learning-platform especially for ENT. An interactive e-learning-platform would ensure, supplement and support qualified education and training.

Staphylococcus aureus α-Toxin Induces Actin Filament Remodeling in Human Airway Epithelial Model Cells.

Exposure of cultured human airway epithelial model cells (16HBE14o-, S9) to Staphylococcus aureus α-toxin (hemolysin A, Hla) induces changes in cell morphology and cell layer integrity that are due to the inability of the cells to maintain stable cell-cell or focal contacts and to properly organize their actin cytoskeletons. The aim of this study was to identify Hla-activated signaling pathways involved in regulating the phosphorylation level of the actin-depolymerizing factor cofilin. We used recombinant wild-type hemolysin A (rHla) and a variant of Hla (rHla-H35L) that is unable to form functional transmembrane pores to treat immortalized human airway epithelial cells (16HBE14o-, S9) as well as freshly isolated human nasal tissue. Our results indicate that rHla-mediated changes in cofilin phosphorylation require the formation of functional Hla pores in the host cell membrane. Formation of functional transmembrane pores induced hypophosphorylation of cofilin at Ser3, which was mediated by rHla-induced attenuation of p21-activated protein kinase and LIM kinase activities. Because dephosphorylation of pSer3-cofilin results in activation of this actin-depolymerizing factor, treatment of cells with rHla resulted in loss of actin stress fibers from the cells and destabilization of cell shape followed by the appearance of paracellular gaps in the cell layers. Activation of protein kinase A or activation of small GTPases (Rho, Rac, Cdc42) do not seem to be involved in this response.

[Management and complications of tumor resections of the midface]. / Management und Komplikationen von Mittelgesichtstumorresektionen.

Only about 8% of head and neck tumors affect the midface. This anatomical area is characterized by an enormous number of functionally important structures, and it is frequently only possible to achieve small resection margin distances. To avoid complications and unfavorable results, a dedicated preparation of both patient and surgeon is warranted. This review aims to provide encouragement for the postgraduate training of interested ENT specialists. Following the clinical course and based on recent literature, strategies to avoid complications of tumor surgery to the midface are given.

[The Microbiome - The Unscheduled Parameter for Future Therapies]. / Das Mikrobiom ­ die unplanbare Größe zukünftiger Therapien.

The microbiome is defined as the total of cellular microorganisms of baczerial, viral or e. g., parasite origin living on the surface of a body. Within the anatomical areas of otorhinolaryngology, a significant divergence and variance can be demonstrated. For ear, nose, throat, larynx and cutis different interactions of microbiome and common factors like age, diet and live style factors (e. g., smoking) have been detected in recent years. Besides, new insights hint at a passible pathognomic role of the microbiome towards diseases in the ENT area. This review article resumes the present findings of this rapidly devloping scientific area.

[The new German guideline on rhinosinusitis - an abbreviated version]. / Die neue Leitlinie Rhinosinusitis ­ eine Miniaturversion.

At the beginning of this year, the new German guideline on rhinosinusitis was published as a joint guideline of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery and the German College of General Practitioners and Family Physicians. The guideline was designed for the treatment of adult patients with inflammatory diseases of the paranasal sinuses and is addressed to all medical specialties involved in the management of these diseases. The current challenge is the implementation of this guideline in the clinical daily routine. For this purpose, an abbreviated version (miniature) was designed.

Staphylococcus aureus hemolysin A disrupts cell-matrix adhesions in human airway epithelial cells.

Treatment of primary or immortalized human airway epithelial cells (16HBE14o-, S9) or alveolar cancer cells (A549) with recombinant hemolysin A (rHla), a major virulence-associated factor of Staphylococcus aureus, induces alterations in cell shape and formation of paracellular gaps in the cell layer. Semiquantitative Western blotting using extracts of freshly isolated airway tissue (nasal epithelium) or 16HBE14o- model cells revealed that phosphorylation levels of focal adhesion kinase (Fak) and paxillin were altered upon treatment of tissue or cells with rHla. Immune fluorescence analyses showed that rHla treatment of 16HBE14o- cells results in losses of vinculin and paxillin from focal contacts and a net reduction in the number of focal contacts. The actin cytoskeleton was strongly remodeled. We concluded that treatment of cells with rHla activates Fak signaling, which accelerates focal contact turnover and prevents newly formed focal contacts (focal complexes) from maturation to focal adhesions. The inability of rHla-treated cells to form stable focal adhesions may be one factor that contributes to gap formation in the cell layer. In vivo, such changes may disturb the defensive barrier function of the airway epithelium and may facilitate lung infections by S. aureus.

Influence of Anatomy-Based Fitting in Cochlear Implant Users on Music Perception Using the Montreal Battery of Evaluation of Amusia.

OBJECTIVES: The aim is to investigate the influence of the anatomy-based fitting (ABF) in cochlear implants (CI) on the perception of specific musical features. DESIGN: The Montreal Battery of Evaluation of Amusia (MBEA) test battery was performed with a collective of 16 CI users with and without an anatomy-based fitting and 9 normal hearing subjects. The insertion angles of the intracochlear electrode arrays were calculated using Otoplan® (MED-EL, Innsbruck, Austria) software. RESULTS: CI users with an anatomy-based fitting achieve better results in the MBEA in all categories, especially in rhythm, meter, and memory. There is no effect of insertion depth alone on the ability to perceive musical features, even after anatomy-based fitting. CONCLUSIONS: The results of the CI users in comparison to normal hearing people confirm previous studies. Apart from better speech understanding in CI users, which has been demonstrated in other studies, the anatomy-based fitting also contributes to a better perception of musical features. Currently, there are no comparable studies on this relationship. Investigations with further parameters with the help of the MBEA are pending. SIGNIFICANCE: Our results show that anatomy-based fitting of cochlear implants significantly improves the perception of specific musical attributes and thus leads to an increased enjoyment of music.