[Chronic rhinosinusitis in people with cystic fibrosis-an up-to-date review from the perspective of otorhinolaryngology]. / Chronische Rhinosinusitis bei Menschen mit zystischer Fibrose ­ eine aktuelle Übersicht aus der Perspektive der Hals­, Nasen­, Ohrenheilkunde.

BACKGROUND: Cystic fibrosis (CF) is a complex systemic disease involving numerous organ systems. With improved treatment options and increasing life expectancy of persons with CF (PwCF), extrapulmonary manifestations are coming increasingly into the focus. From birth, almost all PwCF have radiologically detectable pathologies in the upper airways attributable to CF-associated chronic rhinosinusitis (CF-CRS). OBJECTIVE: The aim of this work is to provide an up-to-date overview of CF-CRS from the otorhinolaryngology perspective and to provide the reader with background knowledge and current developments. PATHOPHYSIOLOGY: The cystic fibrosis transmembrane conductance regulator (CFTR) gene defect leads to increased viscosity of sinonasal secretions and reduced mucociliary clearance, causing chronic infection and inflammation in the upper airway segment and, consequently, to CF-CRS. CLINICAL PICTURE AND DIAGNOSTICS: The clinical picture of CF-CRS comprises a wide spectrum from asymptomatic to symptomatic courses. CF-CRS is diagnosed clinically and radiologically. THERAPY: Sinonasal saline irrigation is recommended as a conservative treatment measure. Topical corticosteroids are also commonly used. Surgical therapy is reserved for highly symptomatic treatment-refractory patients without a sufficient response to conservative treatment including CFTR modulator (CFTRm) therapies. Depending on the CFTR mutation, CFTRm therapies are the treatment of choice. They not only improve the pulmonary and gastrointestinal manifestations in PwCF, but also have positive effects on CF-CRS. CONCLUSION: The ENT specialist is part of the interdisciplinary team caring for PwCF. Depending on symptom burden and treatment responsiveness, CF-CRS should be treated conservatively and/or surgically. Modern CFTRm have a positive effect on the clinical course of CF-CRS.

Qualification of the Low-pressure Cold Gas Spraying for the Additive Manufacturing of Copper-Nickel-Diamond Grinding Wheels.

Grinding wheels are usually manufactured by powder metallurgical processes, i.e., by molding and sintering. Since this requires the production of special molds and the sintering is typically carried out in a continuous furnace, this process is time-consuming and cost-intensive. Therefore, it is only worthwhile for medium and large batches. Another influencing factor of the powder metallurgical process route is the high thermal load during the sintering process. Due to their high thermal sensitivity, superabrasives such as diamond or cubic boron nitride are very difficult to process in this way. In this study, a novel and innovative approach is presented, in which superabrasive grinding wheels are manufactured by thermal spraying. For this purpose, flat samples as well as grinding wheel bodies were coated by low-pressure (LP) cold gas spraying with a blend of a commercial Cu-Al2O3 cold gas spraying powder and nickel-coated diamonds. The coatings were examined metallographically in terms of their composition. A well-embedded superabrasive content of 12 % was achieved. After the spraying process, the grinding wheels were conditioned and tested for the grinding application of cemented carbides and the topographies of both the grinding wheel and the cemented carbide were evaluated. Surface qualities of the ground surface that are comparable to those of other finishing processes were reached. This novel process route offers great flexibility in the combination of binder and hard material as well as a cost-effective single-part and small-batch production.

[Social reimbursement-the Spanish-German ENT Society's (SDGHNO) Latin America project]. / Soziale Rückvergütung ­ das Projekt "Lateinamerika" der Spanisch-Deutschen HNO-Gesellschaft (SDGHNO).

Nowadays, social projects are usually oriented in such a way that after a given period of time, they can either support themselves independently or even allow a pecuniary reimbursement. In the latter case, experts speak of a profit-oriented reimbursement. On the other hand, there is so-called social reimbursement, which in contrast to the abovementioned form is not profit oriented, but, for example, considers its task fulfilled by the fact of successful knowledge transfer. The Spanish-German Society for ENT Medicine and Head and Neck Surgery (SDGHNO) launched the Latin America project in 2001 under the patronage of the then President Prof. Dr. Wolfgang Draf (Fulda). The goal of the SDGHNO was and is to create a professional as well as cultural platform for Spanish- and German-speaking ENT doctors. This platform can and should be used for professional purposes, e.g., for knowledge transfer. Since the beginning of its existence, the Latin America project has thus brought numerous scientific events into being and created specific contacts which have lasted until today or have even been continued and further developed. Particularly successful examples are Chile, Colombia, and Peru. This is a vivid example of social reimbursement, because the participating German-speaking members/speakers carried out their tasks on an entirely voluntary basis. Thus, the SDGHNO did not bear any travel, catering, or accommodation costs. The activities of the SDGHNO within the framework of the Latin America project are explained.

Prolongation of working life and its effect on mortality and health in older adults: Propensity score matching.

Many countries are raising the age of pension eligibility because of increases in life expectancy. Given the social gradient in life expectancy and health, it is important to understand the potential late-life health effects of prolonging working life and whether any effects differ by socioeconomic position. We examined the effect of prolonging working life beyond age 65 on mortality and a series of indicators of late-life physical health (the ability to climb stairs without difficulty, self-rated health, ADL limitations, and musculoskeletal pain) in a representative sample of the Swedish population. In addition to average effects, we also examined heterogeneous effects, for instance by occupational social class. To do this, we use propensity score matching, a method suitable for addressing causality in observational data. The data came from two linked Swedish longitudinal surveys based on nationally representative samples with repeated follow-ups; The Swedish Level of Living Survey and the Swedish Panel Study of Living conditions of the Oldest Old, and from national income and mortality registries. The analytical sample for the mortality outcome included 1852 people, and for late-life physical health outcomes 1461 people. We found no significant average treatment effect on the treated (ATT) of working to age 66 or above on the outcomes, measured an average of 12 years after retirement: mortality (ATT -0.039), the ability to climb stairs (ATT -0.023), self-rated health (ATT -0.009), ADL limitations (ATT -0.023), or musculoskeletal pain (ATT -0.009) in late life. Analyses of whether the results varied by occupational social class or the propensity to prolong working life were inconclusive but suggest a positive effect of prolonging working life on health outcomes. Accordingly, more detailed knowledge about the precise mechanisms underlying these results are needed. In conclusion, working to age 66 or above did not have effect on mortality or late-life physical health.

Quality of life after nasal cancer resection - surgical versus prosthetic rehabilitation.

BACKGROUND: Nose reconstruction following resection of nasal carcinomas is controversial. The objective of this study is to investigate the effect of surgical reconstruction versus prosthetic rehabilitation on patient quality of life (QOL). DESIGN: This was a monocentric prospective study of patients diagnosed with nasal carcinoma from 2003 to 2013. QOL was evaluated using two organ-specific questionnaires (Rhinoplasty Outcome Evaluation [ROE] and the Functional Rhinoplasty Outcome Inventory-17 [FROI-17]) and a generic questionnaire, the Short-Form 36 Health Survey (SF-36). MATERIAL AND METHODS: Sixty-four patients were included. Patients completed the ROE, FROI-17, and SF-36 questionnaires after nasal reconstruction. Questionnaires were completed by 62.8% of the 51 alive patients. RESULTS: Recurrence-free survival (RFS) was 89.9%, disease-specific survival was 94.5%, and overall survival was 75.5% after five years according to the Kaplan-Meier method. Considering initial tumor stage, early stage patients had a significantly higher self-confidence score in FROI-17 subgroup analysis. In contrast, advanced stage patients showed a significantly higher score for social functioning in SF-36. Prosthetically fitted patients scored highly on the ROE questionnaire showing a high degree of aesthetic satisfaction. Surgically reconstructed patients showed a high degree of self-confidence on the FROI-17 questionnaire. However, the organ-specific ROE and FROI-17 scores were not significantly different between patients who received surgical reconstruction and prosthetic rehabilitation after oncological resection. When comparing the rehabilitation method as a function of tumor stage, there was significantly better score for physical functioning in early stage surgically reconstructed patients in the SF-36, but no significant differences in organ-specific QOL. CONCLUSION: Surgical reconstruction and prosthetic rehabilitation after nasal cancer resection have the same effect on organ- and non-organ-specific QOL.

[State of the art of quality-of-life measurement in patients with chronic otitis media and conductive hearing loss]. / Aktueller Stand der Lebensqualitätsmessung bei Patienten mit chronischer Otitis media und Schallleitungsschwerhörigkeit.

The evaluation of results after middle ear reconstruction has been mainly based on functional parameters. In clinical practice as well as in otological research, the pure tone audiogram represents the gold standard in the assessment of the postoperative outcome. In order to assess the patient's subjective outcome, outcome analyzes focus increasingly on the health-related quality of life (HRQOL). However, the evaluation of HRQOL requires reliable and validated measuring instruments. A modest number of validated questionnaires for determination of the disease-specific HRQOL in patients with chronic otitis media and/or conductive hearing loss are currently available. Three of seven available questionnaires were developed and validated in the German-speaking countries, the Zurich Chronic Middle Ear Inventory 21 (ZCMEI-21), the Chronic Otitis Media Outcome Test 15 (COMOT-15) and the Stapesplasty Outcome Test 25 (SPOT-25). In this review, all seven available disease-specific measuring instruments as well as the generic questionnaires, which were used in previous clinical trials, are explained and current findings of quality-of-life research in patients with chronic otitis media and/or conductive hearing loss are presented.

[CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa]. / S3-Leitlinie: Lungenerkrankung bei Mukoviszidose ­ Modul 2: Diagnostik und Therapie bei der chronischen Infektion mit Pseudomonas aeruginosa.

Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection. This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.

[Diagnostics and management of choanal atresia]. / Diagnostik und Management der Choanalatresie.

Choanal atresia is a rare malformation that represents a special challenge. While bilateral choanal atresia usually needs to be surgically treated within a few days of birth, the intervention for one-sided choanal atresia can be postponed for years. Treatment planning requires adequate imaging (CT or MRI), which also serves to exclude other skull base malformities. Surgical treatment currently focuses on transnasal endoscopic techniques. Simultaneous resection of the parts of the vomer involved in the atresia seems to be important surgical success. Postoperative stenting is still controversially discussed. Postoperative application of corticosteroid nasal sprays and saline nasal rinsing for several weeks is of great importance. Due to the rarity of the diagnosis, the absence of prospective randomized controlled trials does not allow definitive statements regarding the optimal surgical technique or stenting.

[Development and validation of the Stapesplasty Outcome Test 25 (SPOT-25)]. / Entwicklung und Validierung des Stapesplasty Outcome Test 25 (SPOT-25).

BACKGROUND: Otosclerosis affects health-related quality of life (HRQOL). Until now, measurements have only been performed using audiometric data or non-validated quality of life questionnaires. OBJECTIVE: The objective of this study was to develop the first validated disease-specific HRQOL measuring instrument to determine HRQOL in otosclerosis. MATERIALS AND METHODS: After sequential analysis and item reduction of the initial Stapesplasty Outcome Test 47 (SPOT-47), the SPOT-25 was validated prospectively on 52 otosclerosis patients undergoing stapes surgery. In addition to the overall score, four subscores were defined (hearing function, tinnitus, social restrictions, mental condition). RESULTS: The SPOT-25 showed a high internal consistency (Cronbach's α > 0.7), allowed discrimination between otosclerosis patients and healthy subjects, and demonstrated acceptable test-retest reliability (r = 0.85). After stapes surgery, the HRQOL improved significantly. The responsiveness was high. CONCLUSION: The SPOT-25 is the first validated disease-specific instrument for HRQOL measurement in otosclerosis patients. It should be used routinely for quality control.

[Quality of life in patients with vestibular schwannoma]. / Lebensqualität von Patienten mit Vestibularisschwannom.

BACKGROUND: Vestibular schwannoma (VS) is a disease which might affect health-related quality of life (HR-QOL) in a negative manner. For many years, only generic quality of life instruments such as SF-36 were available to measure HR-QOL. However, some years ago, the Penn Acoustic Neuroma Quality Of Life (PANQOL) tool, a disease-specific instrument, was developed and validated. It is expected that the application of this instrument will be able to better assess relevant aspects of the HR-QOL of VS patients in the future. A validated German version of the instrument does not exist yet. The disease-specific symptoms most frequently named by patients are headache and dizziness. RESULTS AND DISCUSSION: The available literature shows that the therapeutic approaches affect HR-QOL differently. In particular, radiation therapy of small and medium-sized tumors has no pronounced negative effects on HR-QOL. However, restrictions after surgery become similar to those after radiotherapy over the course of several years. For large VS with a diameter >3 cm, no guiding data on this aspect are currently available. To clarify the outstanding issues, future prospective studies with long-term follow-up of 10 years and more are desirable.

Acupuncture in posttonsillectomy pain : A prospective, double-blinded, randomized, controlled trial.

BACKGROUND: Postoperative swallowing pain is one of the most unpleasant after-effects of tonsillectomy. During recent years, the demand for alternatives to drug-based pain therapy has continued to grow, although the topic has received little research attention until now. MATERIALS AND METHODS: A total of 46 patients were randomized into verum acupuncture, control acupuncture, and drug-based treatment groups. All patients received nonsteroidal antirheumatic drugs (NSAIDs). One hour after drug intake, the verum group also received acupuncture according to classical acupuncture rules (S34, S44 and PC5). The control group had acupuncture needles placed at nonspecific acupuncture points in the midaxillary line. Acupuncture was performed by a blinded acupuncturist, who had learnt exclusively these techniques in the run up to the study. Patients were asked to evaluate their pain before, and at intervals of 20 min, 1 h, 2 h, and 3 h after drug intake/acupuncture treatment using a visual analog scale (VAS). RESULTS: The analgesic effect of acupuncture was significant up to 3 hours in the verum group (p < 0.05). The analgesic effect in the control acupuncture group was significant for up to 1 h after acupuncture (p < 0.05). With reference to the time point before acupuncture, the differences between both acupuncture groups and the drug group were significant (p < 0.01) over the whole time. CONCLUSION: Acupuncture is an effective complement to NSAIDs in the treatment of posttonsillectomy pain. Particularly patients with allergies, drug intolerance, or reduced response to the commonly administered drugs may benefit from acupuncture.

[Acupuncture in posttonsillectomy pain : A prospective double-blind randomized controlled trial. German version]. / Einsatz von Akupunktur bei Posttonsillektomieschmerz : Eine prospektive, randomisierte und kontrollierte Doppelblindstudie.

BACKGROUND: Postoperative swallowing pain is one of the most unpleasant after-effects of tonsillectomy. During recent years, the demand for alternatives to drug-based pain therapy has continued to grow, although the topic has received little research attention until now. MATERIALS AND METHODS: A total of 46 patients were randomized into verum acupuncture, control acupuncture, and drug-based treatment groups. All patients received nonsteroidal antirheumatic drugs (NSAIDs). One hour after drug intake, the verum group also received acupuncture according to classical acupuncture rules (S34, S44 and PC5). The control group had acupuncture needles placed at nonspecific acupuncture points in the midaxillary line. Acupuncture was performed by a blinded acupuncturist, who had learnt exclusively these techniques in the run up to the study. Patients were asked to evaluate their pain before, and at intervals of 20 min, 1 h, 2 h, and 3 h after drug intake/acupuncture treatment using a visual analog scale (VAS). RESULTS: The analgesic effect of acupuncture was significant up to 3 hours in the verum group (p < 0.05). The analgesic effect in the control acupuncture group was significant for up to 1 h after acupuncture (p < 0.05). With reference to the time point before acupuncture, the differences between both acupuncture groups and the drug group were significant (p < 0.01) over the whole time. CONCLUSION: Acupuncture is an effective complement to NSAIDs in the treatment of posttonsillectomy pain. Particularly patients with allergies, drug intolerance, or reduced response to the commonly administered drugs may benefit from acupuncture.

Quality of life after primary septorhinoplasty in deviated- and non-deviated nose measured with ROE, FROI-17 and SF-36.

BACKGROUND: Quality of life measurements are gaining in importance. The present study was conducted with the aim to compare patient satisfaction after septorhinoplasty according to their preoperative nasal deformity. METHODS: The patients completed two disease-specific questionnaires before their surgery: the Functional Rhinoplasty Outcome Inventory (FROI-17), the Rhinoplasty Outcome Evaluation (ROE) and as a general instrument, the Short Form 36 Health Survey (SF-36). The second measurement was taken during an outpatient examination 12 months after their primary septorhinoplasty. Patients were grouped in nasal axis deviation (NAD), nasal hump deformity (NHD) and NAD plus NHD. Additionally the patients with preoperative NAD and NAD+NHD were combined as deviated nose and compared to the non-deviation group. RESULTS: One hundred and two patients (51 male and 51 female) underwent primary septorhinoplasty. The ROE- and the FROI-17 overall score including all three subgroups showed significant postoperative improvements. Regarding the SF-36, the postoperative score improved significantly only in two scales (role-functioning physical and mental health). Looking at the the different QoL questionnaires, there were significant postoperative differences in regards to deviated versus non-deviated nose in the FROI-17 overall- and FROI-17 subscores (nasal and general symptoms) and in three scales of the SF-36 (vitality, social functioning, role-functioning emotional), showing a greater postoperative satisfaction in the deviated-nose patient. CONCLUSION: Patients with and without nasal deviation showed improved QoL after their surgery, as measured with the ROE, the FROI-17 and the SF-36. The patients with a nasal deviation showed a significantly better outcome, as measured with the FROI-17, in comparison with the non-deviated group.

[Multicenter trial for sudden hearing loss therapy - planning and concept]. / Multizentrische Studie zur Hörsturztherapie - Planung und Konzeption.

Systemic steroids are widely used worldwide as a standard of care for primary therapy of idiopathic sudden sensorineural hearing loss (ISSHL). The German ISSHL guideline recommends high-dose steroids for primary therapy of ISSHL, without evidence from randomized controlled trials (RCTs). The rationale for the treatment of ISSHL using high dose steroids is only based on retrospective cohort studies.This article describes the planning and initiation of a multicenter, national, randomized, controlled clinical trial entitled Efficacy and safety of high dose glucocorticosteroid treatment for idiopathic sudden sensorineural hearing loss - a three-armed, randomized, triple-blind, multicenter trial (HODOKORT). This clinical trial aims to compare standard dose with two types of high-dose steroids for primary systemic therapy with respect to their efficacy in improving hearing, and thus communication ability, in patients with idiopathic sudden sensorineural hearing loss.This study is funded by the "Clinical Trials with High Patient Relevance" research program in the health research framework of the German Federal Ministry of Education and Research. It is one of two studies by the German Study Center of Clinical Trials of the German Society of Otorhinolaryngology, Head and Neck Surgery (DSZ-HNO). Planning and initiation was done in cooperation with the DSZ-HNO, the Coordination Center of Clinical Trials of the Martin-Luther-University Halle-Wittenberg, and the Study Center of the University Hospital Freiburg.

[Rhinosinusitis in cystic fibrosis]. / Rhinosinusitis bei Mukoviszidose

In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients.

Butyric acid fermentation from pretreated and hydrolysed wheat straw by an adapted Clostridium tyrobutyricum strain.

Butyric acid is a valuable building-block for the production of chemicals and materials and nowadays it is produced exclusively from petroleum. The aim of this study was to develop a suitable and robust strain of Clostridium tyrobutyricum that produces butyric acid at a high yield and selectivity from lignocellulosic biomasses. Pretreated (by wet explosion) and enzymatically hydrolysed wheat straw (PHWS), rich in C6 and C5 sugars (71.6 and 55.4 g l(-1) of glucose and xylose respectively), was used as substrate. After one year of serial selections, an adapted strain of C. tyrobutyricum was developed. The adapted strain was able to grow in 80% (v v(-1) ) PHWS without addition of yeast extract compared with an initial tolerance to less than 10% PHWS and was able to ferment both glucose and xylose. It is noticeable that the adapted C. tyrobutyricum strain was characterized by a high yield and selectivity to butyric acid. Specifically, the butyric acid yield at 60-80% PHWS lie between 0.37 and 0.46 g g(-1) of sugar, while the selectivity for butyric acid was as high as 0.9-1.0 g g(-1) of acid. Moreover, the strain exhibited a robust response in regards to growth and product profile at pH 6 and 7.

Outcomes of mismatched and unrelated donor hematopoietic stem cell transplantation in Fanconi anemia conditioned with chemotherapy only.

Fanconi anemia (FA) is a genomic instability syndrome associated with bone marrow failure, myelodysplastic syndrome (MDS), and/or acute myeloid leukemia (AML) requiring hematopoietic stem cell transplantation (HSCT) to restore normal hematopoiesis. Although low-intensity fludarabine-based preparative regimens without radiation confer excellent outcomes in FA HSCTs with HLA-matched sibling donors, outcomes for FA patients with alternative donors are less encouraging, albeit improving. We present our experience with 17 FA patients who completed mismatched related or unrelated donor HSCT using a non-radiation fludarabine-based preparative regimen at Charité University Medicine Berlin. All patients engrafted; however, one patient had unstable chimerism in the setting of multi-viral infections that necessitated a stem cell boost to revert to full donor chimerism. Forty-seven percent of patients developed grade I acute graft-verus-host disease (aGVHD). No grade II-IV aGVHD or chronic graft-versus-host disease of any severity occurred. At a median follow-up of 30 months, 88 % of patients are alive with normal hematopoiesis. Two patients died of infections 4 months post-transplantation. These results demonstrate that short-term outcomes for FA patients with mismatched and unrelated donor HSCTs can be excellent using chemotherapy only conditioning. Viral reactivation, however, was a major treatment-related complication.

[The impact of cochlear implants on the quality of life of patients with single-sided deafness]. / Der Einfluss der CI-Versorgung von einseitig tauben Patienten auf die Lebensqualität.

BACKGROUND: Unilaterally deaf patients are increasingly supplied with a cochlear implant (CI). In the present study the change in quality of life following implantation was investigated. PATIENTS AND METHODS: The study comprised 20 postlingual CI recipients with single-sided deafness, who were divided into two groups: "normal hearing" (group 1) and "moderate impairment" (group 2) contralaterally. Their quality of life was determined using four standardized questionnaires (NCIQ, HPS, APHAB, HHIE) and a visual analogue scale. RESULTS: With the exception of the sensation of loud everyday sounds (APHAB AV scale) reported by patients from group 2, all study participants benefited significantly from the implantation. DISCUSSION: The problems caused by the aversiveness of loud everyday sounds can be explained by the enhanced transmission of high frequencies and the associated unfamiliar sensations by the CI system. The effects of deafness duration and response shift remain to be investigated. CONCLUSION: Cochlear implantation for unilaterally deaf patients was found to be a legitimate and meaningful rehabilitation measure.