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Objectives: Access to diagnosis and treatments for auditory disorders and related pathologies has regressed in France during the COVID-19 pandemic, posing a risk to the patient's chance of recovery. This best practice recommendations guide aims to list the existing technological solutions for the remote examination of a patient with hearing complaint, and to outline their benefits and, where applicable, their limitations., Methods: The recommendations were developed both from the clinical experience of the medical experts who drafted the guide, and from an extensive review of the literature dealing with clinical practice recommendations for tele-audiology. Tele-audiometry solutions were identified on the basis of a search engine query carried out in April 2020, prior to verification of their availability on the European market., Results: Video otoscopy solutions allow for the teletransmission of images compatible with a high-quality diagnosis, either by connecting via internet to a tele-health platform or using a smartphone or a tablet with an iOS or Android operating system. Using the same telecommunication methods, it is possible to remotely conduct a pure-tone audiometry test in accordance with standard practice, a speech-in-quiet or a speech-in-noise audiometry test, as well as objective measures of hearing. Clinical and paraclinical examinations can be accessed by the physician to be interpreted on a deferred basis (asynchronous tele-audiology). Examinations can also be conducted in real time in a patient, at any age of life, as long as a caregiver can be present during the installation of the transducers or the acoumetry. Tele-audiology solutions also find application in the remote training of future healthcare professionals involved in the management of deafness and hearing impairment., Conclusion: Under French law, tele-otoscopy is a medical procedure that is either a tele-expertise (asynchronous adive) or a teleconsultation act (synchronous advice). Subjective and objective evaluation of the patient's hearing functions can be done remotely provided that the listed precautions are respected., (Copyright © 2020. Published by Elsevier Masson SAS.)

This study concerns the results of cochlear implantation in children and adults from French cochlear implantation centers, monitored at one, two and three years by the Cochlear Implant French Registry EPIIC. This multicenter study enrolled 2603 subjects (1667 adults and 936 children) implanted in one ear. The following parameters were studied: hearing overall performances, monosyllabic or dissyllabic word perception, speech intelligibility, self-assessment questionnaire of Cochlear Implant (CI) benefits (Abbreviated profile of Hearing aid Benefit); professional activity and schooling. This study confirms the ceiling effect in adults' performances after the 1 st year and the progressive growth in children's performances. It also shows that the contralateral hearing aid enhances performances compared to the CI alone condition, in all follow-up sessions. The French register of CIs is the only worldwide register of systematic follow-up on a period of three years and more of all adults and children implanted in a country., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objective: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label., Material and Methods: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded., Results: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients., Conclusion: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: To analyze the performance of cochlear implants in French patients aged 65 and over, implanted between 2012 and 2016, using data from the French national registry for cochlear implants (EPIIC)., Materials and Methods: The French national registry incorporates patient data from before implantation and for three years after implantation, stratified in different age groups (18-39, 40-64years, 65-74years and>75years). Here, we assessed the latter two categories. Hearing was assessed using mono- and disyllabic words in a silent background. The Category of Auditory Performance (CAP) scale was also implemented and subjects took the Abbreviated Profile of Hearing Aid Benefit (Aphab) questionnaire., Results: The population aged over 65 accounted for 38% (n=1193) of the 3178 adult implanted patients. The performance for mono- and disyllabic words in silence, the CAP scores and the APHAB questionnaire answers for ease of communication, background noise and reverberation were dramatically improved at one year post-implantation (P<0.0001 for each score) and remained stable between one and three years thereafter. The percentage improvement was similar across all age groups. The scores for loud-noise intolerance did not change after cochlear implantation in any age group., Conclusion: Cochlear implants improve hearing and communication in subjects aged 65 and over, with comparable efficiency to that achieved in younger subjects. Cochlear implantation should thus be proposed whenever hearing aids provide only limited benefit. However, between 2012 and 2016, cochlear implantation was given to less than 1% of the French population aged 65 and over with profound deafness., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France., Materials and Methods: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations., Results: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02)., Conclusion: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016., Patients and Methods: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared., Results: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling., Conclusion: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Cochlear and brainstem implants have been included on the list of reimbursable products (LPPR) in France since March of 2009. The implants were initially inscribed for 5 years, after which an application for renewal with the French National Commission for the Evaluation of Medical Devices and Health Technologies (Commission Nationale d'évaluation des dispositifs médicaux et des technologies de santé - CNEDiMTS) was required [Haute Autorité de santé, 2009]. Upon registration to the list of reimbursable products, the companies and the reference centers for cochlear and brainstem implants were asked to set up a post-registration registry called EPIIC. This article reports the evolution in the EPIIC registry of the general indicators for 5051 patients over the five years from 2012-2016., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objectives: The aim of this study was to evaluate peri- and post-operative complications related to cochlear implantations. We searched for risk factors predicting these complications and analyzed the complications in the youngest and most elderly., Study Design: Retrospective analysis of cochlear implant patients., Materials and Methods: All patients who underwent cochlear implantation in France between January 2012 and December 2016 were anonymized and registered in the EPIIC database. This population included 3483 adults and 2245 children. Their demographic and surgical data and their incidence of peri- or post-operative complications, including their severity, whether major or minor, were all indicated., Results: The global complication rate was 6.84%. The risk of complication was higher in initial implantation versus reimplantation (P<0.0001). The risk was also higher for bilateral implantation versus unilateral (P<0.0001). Complications were more frequent for patients with cochlear malformation (P=0.002). There was no difference in complication rates across age groups; babies under 1 year old, and the elderly over 80 and even over 90, did not have more complications than the rest of the population. Patients treated in the daily care unit had no more complications than those who were hospitalized for one night or more (P=0.64)., Conclusion: Cochlear implantation is a safe technique with a low incidence of complications. The absence of increased risk in patients at the extremes of the age spectrum justifies offering this solution to all, without age limitation., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Evaluation of endoscopic ethmoidectomy performed as a day-case in terms of security, quality, and satisfaction of the patient. This prospective observatory bi-centric study over 1 year included 74 patients undergoing an ethmoidectomy respecting the eligibility criteria of ambulatory care. We recorded patients' demographic data, operative details, satisfaction, postoperative course, and follow-up results. Nasal symptoms were evaluated by SNOT-22 on preoperative appointment and postoperatively at D30. No non-absorbable nasal packing was used, eventually in the case of preoperative-bleeding absorbable gelatine packing. The postoperative follow-up took place at D1 by phone call and at D10 and D30 to assess complications, Visual Analogue Scale, and state of ethmoidal corridors by endoscopic exam. Patients benefited of bilateral ethmoidectomy in 82.4 % cases associated with septoplasty in 42 %. The majority (95 %) was discharged on the same day. Only one patient had bleeding at D0 and was kept in standard hospitalization, such as three other patients for medical or organizational reasons not related to surgery. At D1, 23 % described postoperative light bleeding but needed no revisit and pain was estimated at 1.3 (VAS). No readmission was observed, and no major complication was noted. SNOT-22 decreased successfully by 56 %, statistically related to postoperative treatment of corticosteroids and in the case of Samter triad. 97 % of patients were satisfied of the ambulatory care. These results suggest that within an experienced and dedicated day-case medical and paramedical team, ethmoidectomy can be safely performed on a day-case basis with high quality of taking care and satisfaction of patients.

Background: Usher syndrome (USH) combines sensorineural deafness with blindness. It is inherited in an autosomal recessive mode. Early diagnosis is critical for adapted educational and patient management choices, and for genetic counseling. To date, nine causative genes have been identified for the three clinical subtypes (USH1, USH2 and USH3). Current diagnostic strategies make use of a genotyping microarray that is based on the previously reported mutations. The purpose of this study was to design a more accurate molecular diagnosis tool., Methods: We sequenced the 366 coding exons and flanking regions of the nine known USH genes, in 54 USH patients (27 USH1, 21 USH2 and 6 USH3)., Results: Biallelic mutations were detected in 39 patients (72%) and monoallelic mutations in an additional 10 patients (18.5%). In addition to biallelic mutations in one of the USH genes, presumably pathogenic mutations in another USH gene were detected in seven patients (13%), and another patient carried monoallelic mutations in three different USH genes. Notably, none of the USH3 patients carried detectable mutations in the only known USH3 gene, whereas they all carried mutations in USH2 genes. Most importantly, the currently used microarray would have detected only 30 of the 81 different mutations that we found, of which 39 (48%) were novel., Conclusions: Based on these results, complete exon sequencing of the currently known USH genes stands as a definite improvement for molecular diagnosis of this disease, which is of utmost importance in the perspective of gene therapy.

Objectives: To determine the clinical, histological, epidemiological and occupational data related to exposure to wood dust in a series of 100 nasal sinus malignant tumors., Material and Methods: We conducted a retrospective and descriptive study of cases diagnosed between 1st January 1981 and 31 December 2000, in the Auvergne region of France. Individual, medical, and occupational data were collected from a questionnaire completed by the patient (or the patient's family in case of death) and from the medical documents available., Results: Forty-six cases (46 men), with an average age of 63+/-9.2 years [range, 43-82], had been exposed to occupational wood dust before the diagnosis. Fifty-four cases (30 men, 24 women), with an average age of 64.3+/-8.7 years [range, 40-96], had never been exposed. The average annual incidence increased, either for the total population or for the two subgroups distinguished on the basis of occupational exposure to wood dust. The majority of the patients presented different functional symptoms at the time of the diagnosis. For the 46 patients exposed to wood dust, the tumors were primarily ethmoid adenocarcinomas (92%). For the 54 non-exposed patients, the tumors observed were mainly epidermoid carcinomas (57%), then adenocarcinomas (15%). On the 46 patients exposed to wood dust, 85% were carpenters or cabinetmakers. For the majority of the patients, wood dust exposure started before the age of 20 (average age: 17+/-4.5) and the longest exposure began before 1981. The exposure time to wood dust before diagnosis was in the majority of cases greater than 20 years (mean exposure time: 37 years+/-11.4). Only 15% were exposed at the time of the diagnosis (mean time between the end of the exposure to the diagnosis was 11 years+/-2.8). Of the 54 non-exposed patients, no professional risk factor was evidenced., Conclusion: Epidemiologic data, such as the increasing incidence, and clinical and professional data, such as the occupational exposure to wood dust, were in agreement with the French and European literature. It is still probably too early to appreciate the effectiveness of prevention, established in France since 1980, on nasal sinus tumor incidence.

Objectives: Determinate the different prognostic factors of survival in ethmoidal sinus adenocarcinomas (ADK)., Material and Methods: 60 patients with ethmoidal sinus ADK. 59 men and one woman. Average of 62.2 years (41-82). Retrospective study between 1985 and 2005. The following data were analyzed: exposure time to wood dust, disease incidence, primary clinical symptoms and ASA score. Radiological data were recovered by tomodensitometry and magnetic resonance imaging. Histological groups were described. TNM classification according to UICC 2002 and Roux/Brasnu was established on clinical and radiological constatations. Different treatments used were analyzed. Estimate of survival rate and impact of different prognostic factors were based on Kaplan-Meier actuarial method and multivariate analysis., Results: Incidence rate was 2.86 patients a year. Exposure average time to wood dust was 25.6 years (2-44). T3/T4 stages were predominant (66.7%). the survival rate was 46.5% at 5 years. The survival rate was significantly superior respectively in T1 and T2 stages than in T3 and T4 stages, and in T4a than in T4b stages. Extension of the lesion to the sphenoid sinus was revealed as a significant bad prognostic factor. The ASA score and the exposure time to wood dust were not identified as statistically significant prognosis factors., Conclusion: Survival factors of ethmoïd sinus ADK were T stage and the extension of the tumor to the sphenoid sinus. On the results of this study, we consider that extension in sphenoïd sinus could be include in TNM classification of ethmoïd sinus adenocarcinomas.