Anke Tropitzsch

Occupational Noise, Smoking, and a High Body Mass Index are Risk Factors for Age-related Hearing Impairment and Moderate Alcohol Consumption is Protective: A European Population-based Multicenter Study

A multicenter study was set up to elucidate the environmental and medical risk factors contributing to age-related hearing impairment (ARHI). Nine subsamples, collected by nine audiological centers across Europe, added up to a total of 4,083 subjects between 53 and 67 years. Audiometric data (pure-tone average [PTA]) were collected and the participants filled out a questionnaire on environmental risk factors and medical history. People with a history of disease that could affect hearing were excluded. PTAs were adjusted for age and sex and tested for association with exposure to risk factors. Noise exposure was associated with a significant loss of hearing at high sound frequencies (>1 kHz). Smoking significantly increased high-frequency hearing loss, and the effect was dose-dependent. The effect of smoking remained significant when accounting for cardiovascular disease events. Taller people had better hearing on average with a more pronounced effect at low sound frequencies (<2 kHz). A high body mass index (BMI) correlated with hearing loss across the frequency range tested. Moderate alcohol consumption was inversely correlated with hearing loss. Significant associations were found in the high as well as in the low frequencies. The results suggest that a healthy lifestyle can protect against age-related hearing impairment.

Biofunctionalized peptide-based hydrogels provide permissive scaffolds to attract neurite outgrowth from spiral ganglion neurons

Cochlear implants (CI) allow for hearing rehabilitation in patients with sensorineural hearing loss or deafness. Restricted CI performance results from the spatial gap between spiral ganglion neurons and the CI, causing current spread that limits spatially restricted stimulation and impairs frequency resolution. This may be substantially improved by guiding peripheral processes of spiral ganglion neurons towards and onto the CI electrode contacts. An injectable, peptide-based hydrogel was developed which may provide a permissive scaffold to facilitate neurite growth towards the CI. To test hydrogel capacity to attract spiral ganglion neurites, neurite outgrowth was quantified in an in vitro model using a custom-designed hydrogel scaffold and PuraMatrix®. Neurite attachment to native hydrogels is poor, but significantly improved by incorporation of brain-derived neurotrophic factor (BDNF), covalent coupling of the bioactive laminin epitope IKVAV and the incorporation a full length laminin to hydrogel scaffolds. Incorporation of full length laminin protein into a novel custom-designed biofunctionalized hydrogel (IKVAV-GGG-SIINFEKL) allows for neurite outgrowth into the hydrogel scaffold. The study demonstrates that peptide-based hydrogels can be specifically biofunctionalized to provide a permissive scaffold to attract neurite outgrowth from spiral ganglion neurons. Such biomaterials appear suitable to bridge the spatial gap between neurons and the CI.

Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities.

Objective Goldenhar syndrome is a developmental disorder presenting with orofacial and vertebral anomalies, which are also accompanied by abnormalities in other organs. We examined temporal bone changes with special emphasis on inner ear abnormalities in these patients. Study Design A retrospective review of 7 new cases in addition to a previously published series of 14 cases with clinically diagnosed Goldenhar syndrome was carried out to search for inner ear anomalies. In addition, temporal bone imaging studies from the literature were summarized and compared with our results. Setting Departments of Neuroradiology and Otorhinolaryngology at a university hospital. Patients In addition to the previous series of 14 patients, 7 new patients with Goldenhar syndrome were identified. Interventions Patients underwent otologic examination, audiometric studies, and high-resolution computed tomography (CT) or magnetic resonance imaging (MRI) of the temporal bone. Main Outcome Measure Temporal bone changes and specifically inner ear malformations. Results Nineteen of 21 patients showed changes of the external and middle ear correlating with the literature. Seven of 21 patients showed inner ear abnormalities constituting one-third of all patients. These ranged from mild such as vestibular enlargement to severe defects such as cochlear hypoplasia and common cavity. Conclusion Inner ear abnormalities were present in one-third of patients. Although in some cases, these might not be of clinical significance, some patients show severe defects of the inner ear requiring more complex hearing loss therapy. Therefore, imaging of the temporal bone structures is important in the care of these patients.

Einfluss exogener Faktoren auf Altersschwerhörigkeit

BACKGROUND Age-related hearing impairment is a complex disorder, the causes for which have been insufficiently studied. Genetic and environmental factors all play a role. METHODS A total of 406 persons aged between 53 and 67 years old were interviewed about various causes and audiometric data were collected. The audiometric pure tone data were adjusted for sex and age and tested for association with exposure to risk factors. RESULTS Significant negative effects of noise exposure, painkillers, overweight, and cardiovascular diseases on hearing loss were found. A positive effect of moderate alcohol consumption could also be shown in the elderly. These results suggest that a healthy lifestyle can positively affect age-related hearing impairment.ZusammenfassungHintergrundAltersschwerhörigkeit ist eine komplexe Erkrankung, deren Ursachen nur unzureichend erforscht sind. Sowohl genetische als auch Umweltfaktoren spielen eine Rolle.MethodenEs wurden 406 Personen im Alter von 53–67 Jahren zu verschiedenen Ursachen befragt und audiometrisch untersucht. Die audiometrischen Daten wurden nach Geschlecht und Alter bereinigt und auf Assoziationen mit einer Risikofaktorexposition untersucht.ErgebnisseEs konnten signifikante negative Effekte von Lärmexposition, Schmerzmitteln, Übergewicht und Herz-Kreislauf-Erkrankungen auf das Gehör gezeigt werden. Moderater Alkoholgenuss ist mit einem besseren Hörvermögen im Alter assoziiert. Diese Ergebnisse deuten an, dass ein gesunder Lebensstil sich positiv auf Altersschwerhörigkeit auswirken kann.AbstractBackgroundAge-related hearing impairment is a complex disorder, the causes for which have been insufficiently studied. Genetic and environmental factors all play a role.MethodsA total of 406 persons aged between 53 and 67 years old were interviewed about various causes and audiometric data were collected. The audiometric pure tone data were adjusted for sex and age and tested for association with exposure to risk factors.ResultsSignificant negative effects of noise exposure, painkillers, overweight, and cardiovascular diseases on hearing loss were found. A positive effect of moderate alcohol consumption could also be shown in the elderly. These results suggest that a healthy lifestyle can positively affect age-related hearing impairment.

Multicenter Clinical Trial of Vibroplasty Couplers to Treat Mixed/Conductive Hearing Loss: First Results

Objective: To evaluate the safety and effectiveness of round window (RW), oval window (OW), CliP and Bell couplers for use with an active middle ear implant. Methods: This is a multicenter, long-term, prospective trial with consecutive enrollment, involving 6 university hospitals in Germany. Bone conduction, air conduction, implant-aided warble-tone thresholds and Freiburger monosyllable word recognition scores were compared with unaided preimplantation results in 28 moderate-to-profound hearing-impaired patients after 12 months of follow-up. All patients had previously undergone failed reconstruction surgeries (up to 5 or more). In a subset of patients, additional speech tests at 12 months postoperatively were used to compare the aided with the unaided condition after implantation with the processor switched off. An established quality-of-life questionnaire for hearing aids was used to determine patient satisfaction. Results: Postoperative bone conduction remained stable. Mean functional gain for all couplers was 37 dB HL (RW = 42 dB, OW = 35 dB, Bell = 38 dB, CliP = 27 dB). The mean postoperative Freiburger monosyllable score was 71% at 65 dB SPL. The postimplantation mean SRT50 (speech reception in quiet for 50% understanding of words in sentences) improved on average by 23 dB over unaided testing and signal-to-noise ratios also improved in all patients. The International Outcome Inventory for Hearing Aids (IOI-HA)quality-of-life questionnaire was scored very positively by all patients. Conclusion: A significant improvement was seen with all couplers, and patients were satisfied with the device at 12 months postoperatively. These results demonstrate that an active implant is an advantage in achieving good hearing benefit in patients with prior failed reconstruction surgery.

[Influence of exogenic factors on age-related hearing impairment].

BACKGROUND Age-related hearing impairment is a complex disorder, the causes for which have been insufficiently studied. Genetic and environmental factors all play a role. METHODS A total of 406 persons aged between 53 and 67 years old were interviewed about various causes and audiometric data were collected. The audiometric pure tone data were adjusted for sex and age and tested for association with exposure to risk factors. RESULTS Significant negative effects of noise exposure, painkillers, overweight, and cardiovascular diseases on hearing loss were found. A positive effect of moderate alcohol consumption could also be shown in the elderly. These results suggest that a healthy lifestyle can positively affect age-related hearing impairment.ZusammenfassungHintergrundAltersschwerhörigkeit ist eine komplexe Erkrankung, deren Ursachen nur unzureichend erforscht sind. Sowohl genetische als auch Umweltfaktoren spielen eine Rolle.MethodenEs wurden 406 Personen im Alter von 53–67 Jahren zu verschiedenen Ursachen befragt und audiometrisch untersucht. Die audiometrischen Daten wurden nach Geschlecht und Alter bereinigt und auf Assoziationen mit einer Risikofaktorexposition untersucht.ErgebnisseEs konnten signifikante negative Effekte von Lärmexposition, Schmerzmitteln, Übergewicht und Herz-Kreislauf-Erkrankungen auf das Gehör gezeigt werden. Moderater Alkoholgenuss ist mit einem besseren Hörvermögen im Alter assoziiert. Diese Ergebnisse deuten an, dass ein gesunder Lebensstil sich positiv auf Altersschwerhörigkeit auswirken kann.AbstractBackgroundAge-related hearing impairment is a complex disorder, the causes for which have been insufficiently studied. Genetic and environmental factors all play a role.MethodsA total of 406 persons aged between 53 and 67 years old were interviewed about various causes and audiometric data were collected. The audiometric pure tone data were adjusted for sex and age and tested for association with exposure to risk factors.ResultsSignificant negative effects of noise exposure, painkillers, overweight, and cardiovascular diseases on hearing loss were found. A positive effect of moderate alcohol consumption could also be shown in the elderly. These results suggest that a healthy lifestyle can positively affect age-related hearing impairment.

Olfactory Dysfunction in Patients With CNGB1-Associated Retinitis Pigmentosa

Importance Co-occurrence of retinitis pigmentosa (RP) and olfactory dysfunction may have a common genetic cause. Objective To report olfactory function and the retinal phenotype in patients with biallelic mutations in CNGB1, a gene coding for a signal transduction channel subunit expressed in rod photoreceptors and olfactory sensory neurons. Design, Setting, and Participants This case series was conducted from August 2015 through July 2017. The setting was a multicenter study involving 4 tertiary referral centers for inherited retinal dystrophies. Participants were 9 patients with CNGB1-associated RP. Main Outcomes and Measures Results of olfactory testing, ocular phenotyping, and molecular genetic testing using targeted next-generation sequencing. Results Nine patients were included in the study, 3 of whom were female. Their ages ranged between 34 and 79 years. All patients had an early onset of night blindness but were usually not diagnosed as having RP before the fourth decade because of slow retinal degeneration. Retinal features were characteristic of a rod-cone dystrophy. Olfactory testing revealed reduced or absent olfactory function, with all except one patient scoring in the lowest quartile in relation to age-related norms. Brain magnetic resonance imaging and electroencephalography measurements in response to olfactory stimulation were available for 1 patient and revealed no visible olfactory bulbs and reduced responses to odor, respectively. Molecular genetic testing identified 5 novel (c.1312C>T, c.2210G>A, c.2492+1G>A, c.2763C>G, and c.3044_3050delGGAAATC) and 5 previously reported mutations in CNGB1. Conclusions and Relevance Mutations in CNGB1 may cause an autosomal recessive RP–olfactory dysfunction syndrome characterized by a slow progression of retinal degeneration and variable anosmia or hyposmia.

Myelin-induced inhibition in a spiral ganglion organ culture – Approaching a natural environment in vitro

The performance of a cochlear implant depends on the defined interaction between afferent neurons of the spiral ganglion and the inserted electrode. Neurite outgrowth can be induced by neurotrophins such as brain-derived neurotrophic factor (BDNF) via tropomyosin kinase receptor B (TrkB). However, neurotrophin signaling through the p75 neurotrophin receptor (p75) inhibits neurite outgrowth in the presence of myelin. Organotypic cultures derived from postnatal (P3-5) mice were used to study myelin-induced inhibition in the cochlear spiral ganglion. Neurite outgrowth was analyzed and quantified utilizing an adapted Sholl analysis. Stimulation of neurite outgrowth was quantified after application of BDNF, the selective TrkB agonist 7,8-dihydroxyflavone (7,8-DHF) and a selective inhibitor of the Rho-associated kinase (Y27632), which inhibits the p75 pathway. Myelin-induced inhibition was assessed by application of myelin-associated glycoprotein (MAG-Fc) to stimulate the inhibitory p75 pathway. Inhibition of neurite outgrowth was achieved by the selective TrkB inhibitor K252a. Stimulation of neurite outgrowth was observed after treatment with BDNF, 7,8 DHF and a combination of BDNF and Y27632. The 7,8-DHF-induced growth effects could be inhibited by K252a. Furthermore, inhibition of neurite outgrowth was observed after supplementation with MAG-Fc. Myelin-induced inhibition could be overcome by 7,8-DHF and the combination of BDNF and Y27632. In this study, myelin-induced inhibition of neurite outgrowth was established in a spiral ganglion model. We reveal that 7,8-DHF is a viable novel compound for the stimulation of neurite outgrowth in a myelin-induced inhibitory environment. The combination of TrkB stimulation and ROCK inhibition can be used to overcome myelin inhibition.

eHealth in otorhinolaryngology: a disruptive innovation

Thank you very much for your interest and your valuable comments on our article ‘‘The potential of eHealth in otorhinolaryngology-head and neck surgery: patients’ perspectives’’ [1]. The authors strongly believe that further integration of eHealth in the field of otorhinolaryngology (ORL) will follow the typical characteristics of a disruptive innovation. This is the reason why we assume that eHealth has the fundamental potential to improve quality and safety as well as efficacy and efficiency of care and patient engagement. An essential basis for this is that the use of eHealth applications may allow not only the patients, but also the otorhinolaryngologists to get a better understanding of the ORL diseases and therefore can support the diagnostic decision-making and treatment processes. These positive effects of eHealth have already been shown in other medical fields [2, 3]. Because many eHealth applications are developed by young information technology (IT) savvy and healthy people, they often fail to show continuous use and improvement in intersectoral patient care [4]. Therefore, we recommend that eHealth applications in the field of ORL be developed by interdisciplinary and interprofessional teams based on the users’—the patients’ and health-care providers’—needs. Furthermore, experts in data safety, data security, data availability and interface experts for selected and secure transfer of personal medical information between common IT systems, as well as clinical quality and risk managers, health service researchers and health economic experts must be integrated in the development and implementation processes of eHealth applications in ORL. Successful eHealth projects in ORL essentially require clearly defined professional objectives with measurable and predefined outcome parameters regarding patient safety, treatment quality, treatment outcome and health-care costs. In addition to a high usability for ORL patients and physicians to support their engagement, eHealth applications must also meet the highest standards of data security, data safety and data availability for personal medical information and provide standardized interfaces to common IT systems used by otorhinolaryngologists. Though eHealth applications may also contribute to the reduction of health-care costs (e.g., by reduced travel costs or online transfer of discharge summaries), multiple reward and especially adequate reimbursement systems, research funding and a closer focus on eHealth topics by the ORL societies are needed to support the development of the disruptive innovation to benefit patients and health-care providers in the field of ORL.

Personalisierte Medizin in der Otologie

BACKGROUND Classification of diseases on the molecular level is the basis for personalized medicine. Personalized medicine proposes to improve efficiency and quality of care, to reduce side effects and to increase long-term cost-effectiveness. OBJECTIVES This paper is concerned with the role of genetic diagnostics in patients with a cochlear implant. MATERIAL AND METHODS A selective literature search in PubMed was performed. RESULTS Genetic diagnosis allows ruling out syndromic hearing loss and thus prevents follow-up studies. It allows genetic counseling, prognosis and advice on family planning and targeted prevention. Due to its minimal invasiveness, it is suitable for evaluation of factors accounting for hearing loss in children. CONCLUSIONS Molecular medicine plays a major role in the treatment of sensorineural hearings loss with cochlear implants.ZusammenfassungHintergrundDie Klassifizierung von Krankheiten auf molekularer Ebene ist Grundlage für die personalisierte Medizin, die es sich zum Ziel gesetzt hat, Wirksamkeit und Qualität der Behandlung zu verbessern, Nebenwirkungen zu reduzieren und langfristig die Kosteneffektivität zu steigern.Ziel der ArbeitDie Arbeit beschäftigt sich mit der Rolle der genetischen Diagnostik bei der mit Cochlear Implant versorgten Schwerhörigkeit.Material und MethodenEs erfolgte eine selektive Literaturrecherche in Pubmed.ErgebnisseDie genetische Diagnostik kann syndromale Erkrankungen ausschließen und so belastende Folgeuntersuchungen vermeiden. Sie ermöglicht eine Beratung bezüglich Prognose und Familienplanung sowie zielgerichtete Prävention. Durch ihre Minimalinvasivität ist sie geeignet zur Abklärung der kindlichen Schwerhörigkeit.DiskussionIm Bereich der Cochlearimplantationen spielt die molekulare Medizin bereits heute eine große Rolle.AbstractBackgroundClassification of diseases on the molecular level is the basis for personalized medicine. Personalized medicine proposes to improve efficiency and quality of care, to reduce side effects and to increase long-term cost-effectiveness.ObjectivesThis paper is concerned with the role of genetic diagnostics in patients with a cochlear implant.Material and MethodsA selective literature search in PubMed was performed.ResultsGenetic diagnosis allows ruling out syndromic hearing loss and thus prevents follow-up studies. It allows genetic counseling, prognosis and advice on family planning and targeted prevention. Due to its minimal invasiveness, it is suitable for evaluation of factors accounting for hearing loss in children.ConclusionsMolecular medicine plays a major role in the treatment of sensorineural hearings loss with cochlear implants.