A mutation in ATP11A causes autosomal-dominant auditory neuropathy type 2.

Auditory synaptopathy/neuropathy (AS/AN) is a distinct type of sensorineural hearing loss in which the cochlear sensitivity to sound (i.e. active cochlear amplification by outer hair cells) is preserved whereas sound encoding by inner hair cells and/or auditory nerve fibers is disrupted owing to genetic or environmental factors. Autosomal-dominant auditory neuropathy type 2 (AUNA2) was linked either to chromosomal bands 12q24 or 13q34 in a large German family in 2017. By whole-genome sequencing, we now detected a 5500 bp deletion in ATP11A on chromosome 13q34 segregating with the phenotype in this family. ATP11A encodes a P-type ATPase that translocates phospholipids from the exoplasmic to the cytoplasmic leaflet of the plasma membrane. The deletion affects both isoforms of ATP11A and activates a cryptic splice site leading to the formation of an alternative last exon. ATP11A carrying the altered C-terminus loses its flippase activity for phosphatidylserine. Atp11a is expressed in fibers and synaptic contacts of the auditory nerve and in the cochlear nucleus in mice, and conditional Atp11a knockout mice show a progressive reduction of the spiral ganglion neuron compound action potential, recapitulating the human phenotype of AN. By combining whole-genome sequencing, immunohistochemistry, in vitro functional assays and generation of a mouse model, we could thus identify a partial deletion of ATP11A as the genetic cause of AUNA2.

3D-exoscopic microlaryngoscopy in phonosurgery for glottic insufficiency.

PURPOSE: We hypothesized that using a 3D-exoscope (3Dex) in microlaryngoscopic phonosurgery is non-inferior to using a standard operating microscope (OM). To compare the above, we utilized a 3Dex and an OM for microlaryngoscopic vocal fold augmentation with autologous fat in patients with glottic insufficiency and compared the procedure itself and the long-term impact of vocal fold augmentation on subjective and objective voice parameters in both groups. METHODS: 36 patients with glottic insufficiency received microlaryngoscopic laryngeal augmentation with autologous fat. A 3Dex was utilized in 24 cases for visualization and compared to twelve cases in which an OM was used. Voice parameters were evaluated over a period of twelve months. RESULTS: Comparison of operation time and voice parameters between the 3Dex and OM groups did not reveal significant differences. Significant improvement of mean voice quality in all parameters excluding roughness was observed at 3 and 6 months followed then by a slight decrease of voice quality parameters between the 6 and 12 months interval in both groups. CONCLUSION: Our findings indicate no difference concerning operation time and outcome between the use of a 3Dex and an OM in phonosurgery. Our results highlight a significant voice improvement after vocal fold augmentation with autologous fat in glottic insufficiency mediated dysphonia. The smaller viewing system, better ergonomics for the primary surgeon and the assistant and a direct view for the entire surgical team make a 3Dex an interesting alternative for visualization in microlaryngoscopic phonosurgery.

Referral rate and false-positive rates in a hearing screening program among high-risk newborns.

AIM: More studies exploring referral rates and false-positive rates are needed to make hearing screening programs in newborns better and cost-effective. Our aim was to study the referral and false-positivity rates among high-risk newborns in our hearing screening program and to analyze the factors potentially associated with false-positive hearing screening test results. METHODS: A retrospective cohort study was done among the newborns hospitalized at a university hospital from January 2009 to December 2014 that underwent hearing screening with a two-staged AABR screening protocol. Referral rates and false-positivity rates were calculated and possible risk factors for false-positivity were analyzed. RESULTS: 4512 newborns were screened for hearing loss in the neonatology department. The referral rate for the two-staged AABR-only screening was 3.8% with false-positivity being 2.9%. Our study showed that the higher the birthweight or gestational age of the newborn, the lower the odds of the hearing screening results being false-positive, and the higher the chronological age of the infant at the time of screening, the higher the odds of the results being false-positive. Our study did not show a clear association between the mode of delivery or gender and false-positivity. CONCLUSION: Among high-risk infants, prematurity and low-birthweight increased the rate of false-positivity in the hearing screening, and the chronological age at the time of the test seems to be significantly associated with false-positivity.

Side-of-Implantation Effect on Functional Asymmetry in the Auditory Cortex of Single-Sided Deaf Cochlear-Implant Users.

Cochlear implants (CIs) allow to restore the hearing function in profoundly deaf individuals. Due to the degradation of the stimulus by CI signal processing, implanted individuals with single-sided deafness (SSD) have the specific challenge that the input highly differs between their ears. The present study compared normal-hearing (NH) listeners (N = 10) and left- and right-ear implanted SSD CI users (N = 10 left, N = 9 right), to evaluate cortical speech processing between CI- and NH-ears and to explore for side-of-implantation effects. The participants performed a two-deviant oddball task, separately with the left and the right ear. Auditory event-related potentials (ERPs) in response to syllables were compared between proficient and non-proficient CI users, as well as between CI and NH ears. The effect of the side of implantation was analysed on the sensor and the source level. CI proficiency could be distinguished based on the ERP amplitudes of the N1 and the P3b. Moreover, syllable processing via the CI ear, when compared to the NH ear, resulted in attenuated and delayed ERPs. In addition, the left-ear implanted SSD CI users revealed an enhanced functional asymmetry in the auditory cortex than right-ear implanted SSD CI users, regardless of whether the syllables were perceived via the CI or the NH ear. Our findings reveal that speech-discrimination proficiency in SSD CI users can be assessed by N1 and P3b ERPs. The results contribute to a better understanding of the rehabilitation success in SSD CI users by showing that cortical speech processing in SSD CI users is affected by CI-related stimulus degradation and experience-related functional changes in the auditory cortex.

[Is Flying or Diving risky after cochlear implantation? Examination in a pressure chamber]. / Ist Fliegen oder Tauchen riskant für CI-Träger? ­ Untersuchungen in einer Druckkammer.

OBJECTIVE: Complications after cochlear implantation are relatively rare but patients are asked to follow certain behavioral rules. Until now there is no evidence if pressure changes that occur during flying and diving are safe for patients after cochlear implantation. For example, no recommendation exists for SCUBA- diving activities. MATERIALS AND METHODS: In a hypo-/hyperbaric pressure chamber patients after cochlear implantation were exposed to a standardized profile of decompression and compression simulating a flight in a common airplane and a dive. Pre and after the exposure in the pressure chamber, ENT examination and a control of impedances of the cochlear implant were performed. Additionally, patients filled in a questionnaire about complaints they had experienced in the pressure chamber or after. RESULTS: A total of 11 subjects (17 ears with CI) were examined in the pressure chamber. 2/3 of the subjects stated that they had slight complaints during the examination. Only in one patient the measurement needed to be stopped due to pain. Seven patients showed a vascular injection of the malleus in ear microscopy. In none of the patients there was a subjective change in hearing after the examination. Objective measurements of the impedance did not show relevant changes. CONCLUSION: The exposure of cochlear implanted patients to overpressure and underpressure in a pressure chamber did not result in any significant deviation of the impedances, so that no further adjustment was necessary. Since there are no certified in vivo studies on pressure changes while diving and flying with a cochlear implant, further studies are required in order to be able to adequately advise CI patients about diving and flying.

Voice fundamental frequency differences and speech recognition with noise and speech maskers in cochlear implant recipients.

Cochlear implant (CI) recipients are limited in their perception of voice cues, such as the fundamental frequency (F0). This has important consequences for speech recognition when several talkers speak simultaneously. This examination considered the comparison of clear speech and noise-vocoded sentences as maskers. For the speech maskers it could be shown that good CI performers are able to benefit from F0 differences between target and masker. This was due to the fact that a F0 difference of 80 Hz significantly reduced target-masker confusions, an effect that was slightly more pronounced in bimodal than in bilateral users.

A TUBB6 mutation is associated with autosomal dominant non-progressive congenital facial palsy, bilateral ptosis and velopharyngeal dysfunction.

Congenital cranial dysinnervation disorders (CCDDs) comprise a heterogeneous spectrum of diseases characterized by congenital, non-progressive impairment of eye, eyelid and/or facial movements including Möbius syndrome, Duane retraction syndrome, congenital ptosis, and congenital fibrosis of the extraocular muscles. Over the last 20 years, several CCDDs have been identified as neurodevelopmental disorders that are caused by mutations of genes involved in brain and cranial nerve development, e.g. KIF21A and TUBB3 that each plays a pivotal role for microtubule function. In a five-generation pedigree, we identified a heterozygous mutation of TUBB6, a gene encoding a class V tubulin which has not been linked to a human hereditary disease so far. The missense mutation (p.Phe394Ser) affects an amino acid residue highly conserved in evolution, and co-segregates with a phenotype characterized by congenital non-progressive bilateral facial palsy and congenital velopharyngeal dysfunction presenting with varying degrees of hypomimia, rhinophonia, impaired gag reflex and bilateral ptosis. Expression of the mutated protein in yeast led to an impaired viability compared to wildtype cells when exposed to the microtubule-poison benomyl. Our findings enlarge the spectrum of tubulinopathies and emphasize that mutations of TUBB6 should be considered in patients with congenital non-progressive facial palsy. Further studies are needed to verify whether this phenotype is indeed part of the CCDD spectrum.

Prevalence and risk factors for hearing loss in high-risk neonates in Germany.

AIM: Hearing loss in infants is often diagnosed late, despite universal screening programmes. Risk factors of hearing impairment in high-risk neonates, identified from population-based studies, can inform policy around targeted screening. Our aim was to determine the prevalence and the risk factors of hearing loss in a high-risk neonatal population. METHODS: This was a retrospective cohort study of neonates hospitalised at the University Hospital Cologne, Germany from January 2009 to December 2014 and were part of the newborn hearing screening programme. Multivariable regression analyses using the lasso approach was performed. RESULTS: Data were available for 4512 (43% female) neonates with a mean gestational age at birth of 35.5 weeks. The prevalence of hearing loss was 1.6%, and 42 (0.9%) neonates had permanent hearing loss. Craniofacial anomalies, hyperbilirubinaemia requiring exchange transfusion, oxygen supplementation after 36 weeks of gestation and hydrops fetalis showed associations with permanent hearing loss. CONCLUSION: Our findings of risk factors for hearing loss were consistent with other studies. However, some commonly demonstrated risk factors such as perinatal infections, meningitis, sepsis and ototoxic drugs did not show significant associations in our cohort. Targeted screening based on risk factors may help early identification of hearing loss in neonates.

Cochlear implantation using the underwater technique: long-term results.

INTRODUCTION: The opening of the round window and the insertion of the electrode array into the scala tympani during cochlear implant surgery can lead to a pressure shock of the delicate inner ear structures. By filling the tympanic cavity with Ringer Solution during these surgical steps (underwater technique), the hydrostatic pressure of the fluid acts as a smooth pressure stabilizer, avoiding a pressure shock of the inner ear structures. The aim of this retrospective study was to present long-term results of this new method of cochlear implantation in underwater technique. METHODS: Altogether, 47 implantations in 43 patients with residual hearing at the frequencies 250, 500 and 1000 Hz in the unaided preoperative pure tone audiometry were included. A cochlear implantation via round window with a conventional full-length electrode was performed in underwater technique. Changes of residual hearing 7 weeks and 24 months after surgery were analyzed. RESULTS: Overall postimplant hearing preservation 7 weeks after implantation was achieved in 22 ears (47%). Subsequent follow-up was performed on average 24 months after surgery (range 12 months-4.2 years) in all patients. At this late postoperative evaluation, preservation of hearing was recorded in 18 ears (38%). Neither the follow-up time nor the type of electrode had a significant impact on the postoperative hearing loss. CONCLUSION: The underwater technique is an atraumatic cochlear implantation technique with hearing preservation rates comparable to results in literature and a very small hearing preservation decline rate over time even when using full-length CI electrodes.

AUNA2: A Novel Type of Non-Syndromic Slowly Progressive Auditory Synaptopathy/Auditory Neuropathy with Autosomal-Dominant Inheritance.

BACKGROUND: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative. MATERIALS AND METHODS: Here, we report detailed clinical data on a large German AS/AN family with slowly progressive postlingual hearing loss. Affected family members developed their first symptoms in their second decade. Moderate hearing loss in the fourth decade then progressed to profound hearing impairment in older family members. Comprehensive audiological and neurological tests were performed in the affected family members. Genetic testing comprised linkage analyses with polymorphic markers and a genome-wide linkage analysis using the Affymetrix GeneChip® Human Mapping 250K. RESULTS AND CONCLUSION: We identified a large family with autosomal-dominant AS/AN. By means of linkage analyses, the AUNA1 locus was excluded, and putatively linked regions on chromosomal bands 12q24 and 13q34 were identified as likely carrying the second locus for autosomal-dominant AS/AN (AUNA2). AUNA2 is associated with a slowly progressive postlingual hearing loss without any evidence for additional symptoms in other organ systems.

The correlation between ECochG parameters and early auditory behavior after cochlear implantation in children.

OBJECTIVE: The individual outcome after cochlear implantation in children with auditory synaptopathy/neuropathy (AS/AN) is difficult to predict. A tool for preoperative assessment would be helpful for counseling parents. This study evaluates the outcome after CI in children with AS/AN and with sensorineural hearing loss (SNHL), and correlates it with the preoperative ECochG results in order to find specific parameters of prognostic value. DESIGN: The improvement of auditory behavior after CI was retrospectively assessed using the LittlEARS questionnaire and quantified in a score (LS). This score was correlated with the CAP/SP ratio in the preoperative ECochG. The score was further correlated with the patient's age six months following CI. STUDY SAMPLE: Nine children with AS/AN were compared to nine children with SNHL. RESULTS: Both groups showed a significant improvement in LS following CI. There was a significant positive correlation between the CAP/SP ratio and the improvement in LS in all children. The correlation between age and LS was significantly negative in the SNHL group and positive in the AS/AN group. CONCLUSION: All children with AS/AN and SNHL benefit to a similar extent from CI. The preoperatively assessed CAP/SP ratio has a prognostic value for the development of auditory behavior following CI.

Adaptation of the Cochlear Implant Quality of Life-35 Profile Into German.

PURPOSE: The adaptation of existing questionnaires is a valuable method to make instruments available in multiple languages. It is necessary to assure the quality of an adaptation by following adaptation guidelines. The Cochlear Implant Quality of Life-35 Profile (CIQOL-35 Profile) was developed and validated to measure the functional abilities in English-speaking adult CI users but is not yet available in German. In this study, we performed a cross-cultural adaptation of this instrument to make it applicable in research and rehabilitation with German-speaking patients. METHOD: This study followed established practice guidelines for translating and adapting hearing-related questionnaires. Professional translators and health care professionals with experience with patients with hearing loss translated all items forward and backward multiple times. A committee reviewed the process and decided when a satisfactory consensus was achieved. Next, we examined the intelligibility of the German version using cognitive interviews with 15 adult CI users. RESULTS: For most items, there was no difficulty with direct translation. In items that turned out to be more difficult to translate, it proved to be very helpful to compare the back translation to the original version, discuss the wording in the committee, and ask the source-language questionnaire developer. During the interviews, issues of comprehension for some phrases were identified. These phrases were changed according to the participant's questions and suggestions. CONCLUSIONS: The CIQOL-35 Profile was successfully adapted into German. The German version of the questionnaire is now available for research and clinical practice. Further validation of the German CIQOL-35 Profile is in progress. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25386571.

Changes in visually and auditory attended audiovisual speech processing in cochlear implant users: A longitudinal ERP study.

Limited auditory input, whether caused by hearing loss or by electrical stimulation through a cochlear implant (CI), can be compensated by the remaining senses. Specifically for CI users, previous studies reported not only improved visual skills, but also altered cortical processing of unisensory visual and auditory stimuli. However, in multisensory scenarios, it is still unclear how auditory deprivation (before implantation) and electrical hearing experience (after implantation) affect cortical audiovisual speech processing. Here, we present a prospective longitudinal electroencephalography (EEG) study which systematically examined the deprivation- and CI-induced alterations of cortical processing of audiovisual words by comparing event-related potentials (ERPs) in postlingually deafened CI users before and after implantation (five weeks and six months of CI use). A group of matched normal-hearing (NH) listeners served as controls. The participants performed a word-identification task with congruent and incongruent audiovisual words, focusing their attention on either the visual (lip movement) or the auditory speech signal. This allowed us to study the (top-down) attention effect on the (bottom-up) sensory cortical processing of audiovisual speech. When compared to the NH listeners, the CI candidates (before implantation) and the CI users (after implantation) exhibited enhanced lipreading abilities and an altered cortical response at the N1 latency range (90-150 ms) that was characterized by a decreased theta oscillation power (4-8 Hz) and a smaller amplitude in the auditory cortex. After implantation, however, the auditory-cortex response gradually increased and developed a stronger intra-modal connectivity. Nevertheless, task efficiency and activation in the visual cortex was significantly modulated in both groups by focusing attention on the visual as compared to the auditory speech signal, with the NH listeners additionally showing an attention-dependent decrease in beta oscillation power (13-30 Hz). In sum, these results suggest remarkable deprivation effects on audiovisual speech processing in the auditory cortex, which partially reverse after implantation. Although even experienced CI users still show distinct audiovisual speech processing compared to NH listeners, pronounced effects of (top-down) direction of attention on (bottom-up) audiovisual processing can be observed in both groups. However, NH listeners but not CI users appear to show enhanced allocation of cognitive resources in visually as compared to auditory attended audiovisual speech conditions, which supports our behavioural observations of poorer lipreading abilities and reduced visual influence on audition in NH listeners as compared to CI users.

Cochlear measurement in computed tomography and magnetic resonance imaging data sets by the Otoplan measurement tool: a retrospective comparative study.

BACKGROUND: Using Otoplan software, it is possible to measure the cochlea before cochlear implant surgery. Until now, computed tomography (CT) of the cochlea has been necessary for this purpose. The aim of this study was to find out whether measuring the cochlea with magnetic resonance imaging (MRI) using Otoplan is possible with the same accuracy. METHODS: The cochlea of 44 patients of the local cochlear implant centre was measured by Otoplan using high-resolution CT-bone and MRI images, and the determined lengths were compared. RESULTS: No significant difference was found between the cochlear lengths measured, regardless of whether the length measurement was based on a CT or an MRI data set. CONCLUSION: For the determination of cochlear length prior to cochlear implant surgery, MRI images are just as suitable as CT images, therefore CT is not mandatory for length measurement by Otoplan, which could reduce the patient's radiation exposure.

A novel MYO6 splice site mutation causes autosomal dominant sensorineural hearing loss type DFNA22 with a favourable outcome after cochlear implantation.

Mutations in MYO6 encoding an atypical myosin motor protein important for inner ear hair cell function have been associated with autosomal recessive (DFNB37) and autosomal dominant (DFNA22) types of hearing loss in a few families worldwide. After genome-wide linkage analysis, we identified a novel MYO6 mutation at the splice acceptor site of exon 7 (c.554-1G>A) in an extended German family with autosomal dominant postlingual non-syndromic hearing impairment. Analysis of blood-derived cDNA revealed different aberrantly spliced mRNAs caused by the mutation, which are predicted to severely interfere with protein function. Two of the family members underwent cochlear implantation at ages 53 and 65. Here, we present detailed clinical data of this family which suggest a favourable outcome of cochlear implantation in hearing-impaired individuals with a MYO6 mutation.

Cochlear implant fixation: are sutures really necessary?

The objective of this study was to evaluate retrospectively the risk of cochlear implant migration and complications related to the here presented alternative surgical fixation technique of the receiver/stimulator without any foreign body materials. Fixation of the implant was achieved by an "L-shaped" muscle-periosteal flap and an exactly shaped bony well. Between January 2006 and December 2009, 247 consecutive primary cochlear implantations have been performed with the described technique in the Department of Otorhinolaryngology, Head and Neck surgery of the University of Cologne (tertiary referral center). Devices from different manufacturers have been implanted. Implantation age ranged from 6 months to 78 years (mean age: 24.50 years). Follow-up time ranged from 12 to 60 months. Neither implant dislocations nor migrations were observed in our patients. In four very young children (1.6 %), additional suture fixation of the implant was performed. Postoperative complications, i.e. seroma or hematoma were observed in a total of six cases (2.4 %). The here presented surgical fixation technique is a sufficient and reliable way for fixation of cochlear implants independent of the device type.

Universal Newborn Hearing Screening Program: 10-Year Outcome and Follow-Up from a Screening Center in Germany.

Regular reporting of quality control is important in newborn hearing screening, ensuring early diagnosis and intervention. This study reports on a population-based newborn hearing screening program in North-Rhine, Germany and a hospital-based screening at a University Hospital for 2007-2016. The two-staged 'screening' and 'follow-up' program involving TEOAE and AABR recruited newborns through participating birth facilities. Results were sent to the regional tracking center, and the data were analyzed based on recommended benchmarks. The percentage of newborns from the participating birth facilities in the region increased from 1.4% in 2007 to 57.5% in 2016. The 10-year coverage rate for these newborns was 98.7%, the referral rate after a failed two-step screening was 3.4%, and the lost-to-follow-up rate was 1%. At the hospital, >95% of the screened newborns completed screening within 30 days, the 10-year referral rate was 5%, and 64% were referred within 3 months of age. The median time for screening completion was 6 days after birth, for referral it was 74 days after birth, and for diagnosis it was 55 days after birth. Regional-centralized tracking centers with uniform structure are necessary for proper quality control. Obligatory participation of birthing facilities and quality reports may improve performance, but the recommended quality criteria need considerable financial and infrastructural expenditure.

Effects of noise and noise reduction on audiovisual speech perception in cochlear implant users: An ERP study.

OBJECTIVE: Hearing with a cochlear implant (CI) is difficult in noisy environments, but the use of noise reduction algorithms, specifically ForwardFocus, can improve speech intelligibility. The current event-related potentials (ERP) study examined the electrophysiological correlates of this perceptual improvement. METHODS: Ten bimodal CI users performed a syllable-identification task in auditory and audiovisual conditions, with syllables presented from the front and stationary noise presented from the sides. Brainstorm was used for spatio-temporal evaluation of ERPs. RESULTS: CI users revealed an audiovisual benefit as reflected by shorter response times and greater activation in temporal and occipital regions at P2 latency. However, in auditory and audiovisual conditions, background noise hampered speech processing, leading to longer response times and delayed auditory-cortex-activation at N1 latency. Nevertheless, activating ForwardFocus resulted in shorter response times, reduced listening effort and enhanced superior-frontal-cortex-activation at P2 latency, particularly in audiovisual conditions. CONCLUSIONS: ForwardFocus enhances speech intelligibility in audiovisual speech conditions by potentially allowing the reallocation of attentional resources to relevant auditory speech cues. SIGNIFICANCE: This study shows for CI users that background noise and ForwardFocus differentially affect spatio-temporal cortical response patterns, both in auditory and audiovisual speech conditions.

Effectiveness and costs of a low-threshold hearing screening programme (HörGeist) for individuals with intellectual disabilities: protocol for a screening study.

INTRODUCTION: Individuals with intellectual disabilities (ID) often suffer from hearing loss, in most cases undiagnosed or inappropriately treated. The implementation of a programme of systematic hearing screening, diagnostics, therapy initiation or allocation and long-term monitoring within the living environments of individuals with ID (nurseries, schools, workshops, homes), therefore, seems beneficial. METHODS AND ANALYSIS: The study aims to assess the effectiveness and costs of a low-threshold screening programme for individuals with ID. Within this programme 1050 individuals with ID of all ages will undergo hearing screening and an immediate reference diagnosis in their living environment (outreach cohort). The recruitment of participants in the outreach group will take place within 158 institutions, for example, schools, kindergartens and places of living or work. If an individual fails the screening assessment, subsequent full audiometric diagnostics will follow and, if hearing loss is confirmed, initiation of therapy or referral to and monitoring of such therapy. A control cohort of 141 participants will receive an invitation from their health insurance provider via their family for the same procedure but within a clinic (clinical cohort). A second screening measurement will be performed with both cohorts 1 year later and the previous therapy outcome will be checked. It is hypothesised that this programme leads to a relevant reduction in the number of untreated or inadequately treated cases of hearing loss and strengthens the communication skills of the newly or better-treated individuals. Secondary outcomes include the age-dependent prevalence of hearing loss in individuals with ID, the costs associated with this programme, cost of illness before-and-after enrolment and modelling of the programme's cost-effectiveness compared with regular care. ETHICS AND DISSEMINATION: The study has been approved by the Institutional Ethics Review Board of the Medical Association of Westphalia-Lippe and the University of Münster (No. 2020-843 f-S). Participants or guardians will provide written informed consent. Findings will be disseminated through presentations, peer-reviewed journals and conferences. TRIAL REGISTRATION NUMBER: DRKS00024804.

Laryngeal electromyography: a proposal for guidelines of the European Laryngological Society.

Although recognized as a valuable diagnostic tool for more than 60 years, many laryngologists do not routinely use laryngeal electromyography (LEMG). This may be due to a persisting lack of agreement on methodology, interpretation, validity, and clinical application of LEMG. To achieve consensus in these fields, a laryngeal electromyography working group of European neurolaryngologic experts was formed in order to (1) evaluate guidelines for LEMG performance and (2) identify issues requiring further clarification. To obtain an overview of existing knowledge and research, English-language literature about LEMG was identified using Medline. Additionally, cited works not detected in the initial search were screened. Evidence-based recommendations for the performance and interpretation of LEMG and also for electrostimulation for functional evaluation were considered, as well as published reports based on expert opinion and single-institution retrospective case series. To assess the data obtained by this literature evaluation, the working group met five times and performed LEMG together on more than 20 patients. Subsequently, the results were presented and discussed at the 8th Congress of the European Laryngological Society in Vienna, Austria, September 1-4, 2010, and consensus was achieved in the following areas: (1) minimum requirements for the technical equipment required to perform and record LEMG; (2) best practical implementation of LEMG; (3) criteria for interpreting LEMG. Based on this consensus, prospective trials are planned to improve the quality of evidence guiding the proceedings of practitioners.