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Objectives This document presents the fundamentals of speech audiometry in noise, general requirements for implementation and criteria for choice among the tests available in French according to the health-professional's needs. Material and methods The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors, audiologists and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. Results Eight tests of speech audiometry in noise can be used in France. Conclusion To be complete, evaluation of hearing status requires testing understanding of speech in noise. The examination must begin with a minimum of two measurements familiarizing the subject with the test procedure. For initial diagnosis, adaptive procedures establishing the 50% speech reception threshold (SRT50) in noise are to be preferred in order to obtain a rapid and standardized measurement of perception of speech in noise. When the aim is to measure real-life speech comprehension, tests based on sentences, cocktail-party noise and free-field stimulation are to be preferred. Prosthetic gain is evaluated exclusively in free field. This is the only way to evaluate the contribution of binaurality and to measure perception in noise in an environment as close as possible to real life. In order to avoid acoustic interference in free field, at least five loudspeakers should be used, in particular for evaluating the effectiveness of directional microphones, CROS devices enabling sounds picked up in the damaged ear to be rerouted to the functional ear, or bimodal fitting (i.e., when hearing is enabled by two modalities: for example, hearing aid for one ear, cochlear implant for the other).

Resumen Las exostosis del conducto auditivo externo (CAE) afectan esencialmente a las personas que practican de forma intensiva deportes nauticos. Las indicaciones quirurgicas se basan en la sintomatologia del paciente, la repeticion de las otitis y la repercusion audiometrica. El estudio preoperatorio de las pruebas de imagen es esencial para limitar los riesgos y las complicaciones. La cirugia requiere un fresado prudente de las exostosis despues de la movilizacion de colgajos cutaneos. Esto se puede realizar segun la tecnica de la «cascara de huevo» o por fresado de medial a lateral. Tambien se puede efectuar una reseccion mediante osteotomos. Las complicaciones son infrecuentes. Se trata esencialmente de traumatismos timpanoosiculares o de trastornos de cicatrizacion postoperatorios. En la mayoria de los casos, la cirugia de las exostosis del CAE permite mejorar la calidad de vida de los pacientes, asi como la audiometria tonal postoperatoria en los casos en los que la indicacion corresponda a una sordera.

The aim of the study was to assess a correlation between MRI labyrinthine changes detected with IV-gadolinium optimized high-resolution 3D-FLAIR sequences 4 h after injection (OPT4-3DFLAIR) and the type of SSNHL, in terms of frequency alteration and severity.This was a prospective monocentric study achieved from July 2019 to December 2020. The inclusion criterion was acute hearing loss of at least 30 dB over three contiguous frequencies occurring within a 72-h period, documented by a pure-tone audiometry (PTA). The primary endpoint was the visual assessment of hyperintensity in labyrinthine structures on OPT4-3DFLAIR performed on 3T MRI.Thirty-six affected ears were included (20 men, 15 women; mean age: 54.5 ± 16.3 years) with 69.4% full-spectrum hearing loss. The median hearing loss, expressed as median and interquartile range [IQR] was 91 dB [74-120], with 47.2% of concomitant acute vestibular syndrome. Pathological signal was found in 26 out of 36 ears (72.2%). Basal turn enhancement was found in all abnormal MRIs, with 73.1% of apical turn enhancement and 50% of vestibular enhancement. Seventeen on 19 cases (89.5%) with apical involvement on MRI had low-frequency hearing loss. Vestibular involvement on MRI was significantly associated with a wider frequency range of hearing loss (p = 0.0002) and the severity of SSNHL (84.5 [71.7-92.5] dB versus 120 [85.8-120] dB, p = 0.0158).This report shows that in pathological MRI in SSNHL, a pathologic cochlear base signal is always detected, a cochlear apical turn enhancement matches with low-tone impairment, and a pathological signal within the posterior labyrinth is associated with an impairment of all frequencies and the severity of SSNHL.

International audience; Background: To describe the characteristics of the largest European study of MEC of salivary glands and to determine the prognostic factors for overall and disease free survival.Patients and methods: Patients with MEC were prospectively included in the Réseau d'Expertise Français sur les Cancers ORL Rares (REFCOR, French Network of Rare Head and Neck Tumors) database between 2009 and 2015.Results: A total of 292 patients were included. Tumors were classified as low grade in 175 cases (60%), intermediate in 39 (13%) and high grade in 78 (27%). Median follow-up was 26 months. The 5-year OS and DFS rates were respectively 83% and 69%. In multivariate analysis, age (p = 0.004), diabetes (p = 0.02) and advanced stage (p = 0.03) were found to have a significant negative impact on OS. Diabetes (p = 0.001), alcohol consumption (p = 0.003) and advanced stage (p = 0.001) were found to have a significant negative impact on DFS. Compare to low grade, high grade tended to have a negative impact on OS (p = 0.05) and had a significant effect on DFS (0.002) while intermediate grade had no significant influence on survival. The surgical treatment had a positive impact on both OS (p = 0.00005) and DFS (p = 0.0005). Postoperative radiotherapy had no impact in multivariate analysis.Conclusion: Advanced clinical stage, high grade tumor, high age, the impossibility of carrying out a complete surgical resection, and diabetes are the main prognostic factors in this prospective series of patients with MEC. Such findings open new research perspectives on the influence of these components on initial patient care.

International audience; 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.

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’evaluation des dispositifs medicaux et des technologies de sante – CNEDiMTS) was required [Haute Autorite de sante, 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.

Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France.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.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 (P0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P0.01). 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%, P0.02).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.

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 1st 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. 137;Supplement 1

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, 40–64 years, 65–74 years and > 75 years). 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.

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. 137;Supplement 1

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.

Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label.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.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 (P0.001) and 60% versus 71% in dissyllabic word lists (P0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P0.001) and the APHAB scores were statistically lower after implantation (P0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P0.001), and 48% versus 82% (P0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients.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.

I danni centrali dell’udito comprendono, da una parte, le sordita corticali dovute a lesioni cortico-sotto-corticali, che danno dei quadri semeiologici conosciuti da tempo (sordita verbale, sordita corticale e agnosia uditiva), e, dall’altra, dei disturbi dell’udito meno marcati e di identificazione piu recente, come, per esempio, alcuni ritardi di apprendimento nei bambini e alcune sordita dell’adulto in contrasto con soglie uditive periferiche entro i limiti della norma (obscure auditive dysfonction, sordita latente) e alcune presbiacusie con coinvolgimento centrale. Un danno genetico centrale dell’udito, molto frequente, e rappresentato dall’amusia congenita. Queste lesioni sono difficili da diagnosticare e richiedono, per essere rilevate, degli specifici test soggettivi ed elettrofisiologici. La diagnosi di questi danni centrali dell’udito e, tuttavia, importante da effettuare per migliorare la gestione delle persone colpite, in particolare nel bambino nel pieno periodo dell’apprendimento. Le cause sono molteplici e, tra queste, si annoverano i danni ischemici, emorragici, tumorali, infettivi, degenerativi e iatrogeni (soprattutto chirurgici e radiochirurgici). Alcune cause nei bambini non sono “statiche”, ma in relazione con un’asincronia della maturazione delle vie uditive. L’esplorazione all’esame obiettivo puo aiutare l’elettrofisiologia. Queste tecniche, che utilizzano i potenziali evocati precoci, semiprecoci e tardivi, l’auditory steady state response (ASSR) e la speech-auditory brainstem response (ABR), forniscono delle informazioni limitate sullo stato delle vie acustiche centrali, ma possono aiutare a classificare le diverse patologie responsabili e, soprattutto, a identificare i siti di lesione. La diagnostica per immagini (risonanza magnetica nucleare funzionale [RMf], magnetoencefalografia, tomografia a emissione di positroni [PET]) e promettente ma di applicazione clinica ancora ristretta.

Los trastornos centrales de la audicion engloban, por una parte, las hipoacusias centrales debidas a las lesiones corticosubcorticales, que producen cuadros semiologicos conocidos desde hace mucho tiempo (sordera verbal, sordera cortical y agnosia auditiva) y, por otra parte, los trastornos auditivos menos marcados y que se han identificado mas recientemente, como algunos retrasos del aprendizaje infantiles, algunas hipoacusias del adulto que contrastan con unos umbrales auditivos perifericos dentro de los limites de la normalidad (disfuncion auditiva indefinida [obscure auditive dysfunction]) y algunas presbiacusias con un componente central. Existe tambien una alteracion genetica central de la audicion, que es muy frecuente, denominada amusia congenita. Estas alteraciones son dificiles de diagnosticar y requieren pruebas subjetivas y electrofisiologicas especificas para ser detectadas. Sin embargo, es esencial realizar el diagnostico de estas alteraciones centrales de la audicion para mejorar el tratamiento de las personas afectadas, en especial en los ninos que estan en pleno periodo de aprendizaje. Las etiologias son muy numerosas y engloban las alteraciones geneticas, isquemicas, hemorragicas, tumorales, infecciosas, iatrogenicas (sobre todo quirurgicas y radioquirurgicas) y degenerativas. Algunas causas en la edad pediatrica no son «estaticas», sino que se relacionan con una desincronizacion de la maduracion de las vias auditivas. Las pruebas de imagen funcionales (resonancia magnetica funcional, magnetoencefalografia, tomografia por emision de positrones) son prometedoras, pero su aplicacion clinica aun muy limitada.

Introduction Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium-sized vessels comprising inflammation of the vessel wall and perivascular and extravascular granulomas, frequently presenting in the form of chronic sinusitis. Observation We report the case of a 27-year-old man who presented with very painful acute frontal sinusitis that was managed medically and surgically. The symptoms rapidly recurred despite treatment and CT scan demonstrated diffuse thickening of the sinus mucosa. Anti-proteinase 3 ANCA were positive. Biopsy of a pulmonary nodule confirmed the diagnosis of GPA. The patient was treated with corticosteroids in combination with rituximab, resulting in improvement of the clinical, laboratory and CT signs. Discussion In the presence of persistent, acute, localized sinusitis despite appropriate treatment, associated systemic signs and/or the presence of other signs suggestive of GPA, the ENT surgeon must request a targeted work-up. In the absence of treatment, GPA can be fatal within a few months. However, with currently available treatment, remission is obtained in 80% of cases with a 75% 10-year survival rate.

Introduction Les adenomes parathyroidiens ectopiques dans le sinus piriforme sont exceptionnels. Cette localisation peut modifier la demarche diagnostique et chirurgicale. Observation Le diagnostic d’hyperparathyroidie primaire est porte chez une patiente de 59 ans asthenique. La scintigraphie 99mTc-MIBI/123I SPECT-CT retrouve une heterogeneite de fixation non specifique a droite du laryngopharynx sans anomalie scanographique en regard, pas d’image d’adenome parathyroidien. A 61 ans, devant une osteoporose fracturaire, une reevaluation endocrinologique montre : calcemie corrigee/albuminemie 2,83 mmol/L, phosphore 0,80 mmol/L, PTH a 119 pg/mL (N 12–88), creatininemie 65 μmol/l, vitamine D 24 ng/mL, calciurie 18,7 mmol/24 h. L’echographie cervicale ne localise pas d’adenome. Un TEP 18F-Choline decrit une structure hyperfixante de 8 mm du sinus piriforme droit compatible avec un adenome parathyroidien ectopique P3 droit. Un scanner 4 T confirme une lesion nodulaire de 9 mm du sinus piriforme droit spontanement hypodense, hyperarterialisee avec un discret washout (densite spontanee Conclusion Un bilan morphologique de premiere intention negatif pour hyperparathyroidie primaire doit faire rechercher un adenome ectopique dans le sinus piriforme par TEP 18F-Choline et nasofibroscopie. Il peut etre gueri par une chirurgie endoscopique.

Objectives The main objective of this retrospective study was to assess functional results in endoscopic Zenker's diverticulum surgery. The secondary objectives were to assess safety, identify recurrence risk factors, and determine optimal management of recurrence. Material and methods From 2000 to 2014, 50 patients underwent endoscopic surgery for marsupialization of Zenker's diverticulum. Regurgitation and dysphagia were assessed on the FOIS scale, pre- and post-operatively. Recurrences and complications rates were determined retrospectively at a minimum 18 months’ follow-up. Results Regurgitation and dysphagia improved in respectively 96% and 86% of patients. There was a 12% rate of complications, mostly minor. Mean hospital stay and time to return to oral feeding were 2.0 and 1.3 days respectively. Nine patients (18%) showed recurrence of symptoms, requiring revision surgery at a mean 2.7 years, performed endoscopically in the majority of cases. Only one recurrence risk factor was identified: small diverticulum size. Conclusion Endoscopic Zenker's diverticulum surgery provided functional improvement in most cases. Safe and effective, it is currently the treatment of choice for Zenker's diverticulum.

Resume Introduction La granulomatose avec polyangeite (GPA) est une vascularite necrosante des vaisseaux de petit et moyen calibre associant une inflammation de la paroi vasculaire et des granulomes, peri- et extra-vasculaires, frequemment revelee par une atteinte sinusienne chronique. Observation Nous rapportons l’observation d’un patient âge de 27 ans qui a presente une sinusite frontale aigue hyperalgique prise en charge par traitement medical et chirurgical. Malgre le traitement, la symptomatologie recidivait precocement et le scanner retrouvait des epaississements muqueux sinusiens diffus. Les ANCA diriges contre la proteinase 3 etaient positifs. Un nodule pulmonaire a ete biopsie confirmant le diagnostic de GPA. Le patient a ete traite par une corticotherapie en association avec du rituximab, ce qui a permis d’obtenir une amelioration clinique, biologique et scannographique. Discussion En cas d’atteinte sinusienne localisee aigue et persistante malgre un traitement adapte, de signes generaux associes et/ou de survenue de signes evocateurs de la GPA, l’ORL doit savoir demander un bilan oriente. En l’absence de traitement, la GPA pet etre mortelle en quelques mois. Grâce au traitement actuel, la remission est obtenue dans 80 % des cas avec un taux de survie de 75 % a 10 ans.

Half of the patients presenting with head and neck tumor are malnourished at the outset of treatment. Muscle mass loss (sarcopenia) seems to be critical. The aim of the present study was to assess the impact of nutritional status and sarcopenia on postoperative complications in head and neck cancer.A retrospective study for the period November 2014 to May 2016 included 92 patients operated on for head and neck squamous cell carcinoma. Complications at 30 days were graded on the 5-level Clavien-Dindo classification. Nutritional status was assessed anthropometrically (weight), biologically (albuminemia and Nutrition Risk Index (NRI)), on CT (muscle mass index at 3rd lumbar vertebra) and functionally (Short Physical Performance Battery). Assessment was made at the outset of management, and nutritional treatment was initiated as appropriate. Potential risk factors for postoperative complications were assessed: performance status, prior radiation therapy, smoking and alcohol abuse.54% of patients were malnourished on the NRI. 41% had grade≥2 complications. The most frequent complications were infection and healing disorder. The risk of complications was higher in case of malnutrition (62% vs. 17%; P0.001) and sarcopenia (56% vs. 22%; P0.01), with 50% longer hospital stay (P=0.04).Malnutrition and sarcopenia are independent risk factors for postoperative complications.

International audience; BACKGROUND:Head and neck mucosal melanoma (HNMM) is aggressive and rare, with a poor prognosis because of its high metastatic potential. The two main subtypes are sinonasal (sinonasal mucosal melanoma [SNMM]) and oral cavity (oral cavity mucosal melanoma [OCMM]). Consensual therapeutic guidelines considering the primary tumour site and tumour-node-metastasis (TNM) stage are not well established.MATERIAL & METHODS:Patients with HNMM from the prospective national French Rare Head and Neck Cancer Expert Network database between 2000 and 2017 were included. Clinical characteristics, treatment modalities, outcomes and prognostic factors were analysed.RESULTS:In total, 314 patients were included. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 49.4% and 24.7%, respectively, in the surgery group; no long-term survivors were observed when surgery was not feasible. Moreover, even after surgery, a high recurrence rate was reported with a median PFS of 22 months. In multivariate analysis, Union for International Cancer Control (UICC) stage and tumour site correlated with PFS and OS. Postoperative radiotherapy (PORT) improved the PFS but not OS in patients with small (T3) SNMM and OCMM tumours. Nodal involvement was more frequent in patients with OCMM (p < 10-4), although, as in SNMM, it was not a significant prognostic predictor.CONCLUSION:Even early HNMM was associated with poor oncologic outcomes due to distant metastases despite surgical resection with clear margins. Lymph node metastases had no impact on the prognosis, suggesting treatment de-escalation in cervical node management. PORT might be useful for local control.

Fibrous dysplasia of bone is a benign, uncommon, sporadic, congenital skeletal disorder resulting in deformity. This disease arises from activating somatic mutation in GNAS which encodes the α subunit of the G stimulatory protein associated with proliferation of undifferentiated osteogenic cells resulting in marrow fibrosis, abnormal matrix production, and stimulation of osteoclastic resorption upon overproduction of IL-6 observed in dysplastic cells. Fibrous dysplasia may be monostotic or polyostotic. This mutation affecting many tissues, cafe au lait skin macules and endocrinopathies (precocious puberty, hyperthyroidism, growth hormone excess, Cushing syndrome) may be associated in McCune-Albright syndrome, but also myxoma in Mazabraud syndrome or phosphate diabetes. Diagnosis of craniofacial fibrous dysplasia should be considered in the presence of headache, neuralgia, sensory disorders (vision, hearing, balance, smelling), functional disorders (nasal obstruction, nasolacrimal duct obstruction, non-matching occlusion), infectious complications (sinusitis, otitis, mastoiditis). Such symptoms should lead to perform craniofacial CT scan completed with MRI. Bone biopsy is not systematic. Surgical treatment is discussed in cases of nervous complication, facial deformity or active lesions. In case of pain resistant to conventional analgesics, intravenous bisphosphonates can be proposed. In non-responder patients, several case reports suggest the efficacy of a monoclonal antibody directed against the IL-6 receptor which requires to be confirmed by randomized studies.

Introduction Sinonasal mucosal melanoma is a rare disease associated with a very poor prognosis. The purpose of this study was to assess the prognostic value of the 2 staging systems published in the literature for these tumours: the American Joint Committee on Cancer (AJCC) Cancer Staging Manual for mucosal melanoma of the head and neck published in 2009 (7th edition) and the AJCC Cancer Staging Manual for cancers of the nasal cavity and paranasal sinuses published in 2002 (6th edition) and the prognostic value of tumour site, either limited to the nasal cavities or with paranasal sinus invasion. Methods A retrospective study was conducted on 18 patients treated between August 1998 and June 2014. Each lesion was staged according to the AJCC Cancer Staging Manual 2002 and 2009 and the following data were collected: age, sex, tumour site, initial symptoms, treatment modalities, follow-up, recurrences and overall survival. Patient survival, from the date of discovery of the melanoma until death, was analysed by Kaplan-Meier survival curves and between-group comparison of survival was performed with a log rank test. Results The mean age at diagnosis was 72 years (range: 54–94) and the cohort comprised 11 women and 7 men. The median overall survival was 80 months, the 1-year overall survival was 82.6% and the 5-year overall survival was 54.5%. The AJCC 2002 staging system presented a statistically significant prognostic value (P = 0.0476), while no statistically significant prognostic value was observed for the AJCC 2009 staging system (P = 0.108). Paranasal sinus invasion was significantly associated with a poor prognosis (P = 0.0039). Conclusion This study demonstrates the superiority of the non-specific AJCC 2002 Cancer Staging Manual. Medical and surgical management must take paranasal sinus invasion into account, as it constitutes a major prognostic factor.

Objectives In patients with Friedreich ataxia (FRDA), mitochondrial failure leads to impaired cellular energetics. Since many FRDA patients have impaired hearing in noise, we investigated the objective consequences on standard auditory brainstem-evoked responses (ABRs). Methods In 37 FRDA patients, among whom 34 with abnormal standard ABRs, hearing sensitivity, speech-in-noise intelligibility and otoacoustic emissions were controlled. ABR recordings were split into four consecutive segments of the total time frame used for data collection, thus allowing the dynamics of ABR averaging to be observed. Results Most ears showed features of an auditory neuropathy spectrum disorder with flattened ABRs and impaired speech-in-noise intelligibility contrasting with near-normal hearing sensitivity and normal preneural responses. Yet split-ABRs revealed short-lived wave patterns in 26 out of 68 ears with flattened standard ABRs (38%). While averaging went on, the pattern of waves shifted so that interwave latencies increased by 35% on average. Conclusions In FRDA, the assumption of stationarity used for extracting standard ABRs is invalid. The preservation of early split-ABRs indicates no short-term dyssynchrony of action potentials. A large decrease in conduction velocity along auditory neurons occurs within seconds, attributed to fast energetic failure. Significance This model of metabolic sensory neuropathy warns against exposure of metabolically-impaired patients to sustained auditory stimulation.

The aim of our study was to identify clinical criteria for optimizing rehabilitation of patients with unilateral deafness using the Baha device. We made a retrospective study of 102 patients with unilateral deafness requesting auditory rehabilitation over a period of 5 years. All subjects underwent a series of stereo audiometric tests, with and without Baha worn on a headband, and were then referred to a hearing care specialist for a real life trial of 15 days. The Glasgow Health Status Inventory (GHSI) questionnaire was administered. Patients refusing the implantation were retrospectively submitted to a questionnaire specifically designed to ask the reasons for refusal. We measured stereo audiometric test results, age, aetiology of deafness, duration of auditory deprivation on the rehabilitated ear, and GHSI score. At the conclusion of testing, the implantation rate was 29%. During preoperative testing, the improvement in understanding of speech-in-noise was 22 ± 11% for patients agreeing to the implantation versus 13 ± 11% for patients refusing the implantation. Age, aetiology of deafness and duration of auditory deprivation had no influence on the implantation decision. Speech-in-noise testing and aided stereo audiometric gain were the only two measures showing statistically significant differences between the groups agreeing to and refusing the implantation. There were multiple reasons for refusal of the implantation. Among these, the four principal reasons were: absence of perceived benefit during stereo audiometric testing (59%), requirement for surgery (35%), cost of the solution (44%), and aesthetics (41%). Hence, no other criteria except the preoperative improvement in understanding of speech-in-noise and the aided gain from Baha worn on a headband were found to be predictive of the patient's acceptance of surgical implantation of a bone-anchored implant/abutment for Baha. Speech-in-noise testing with and without Baha worn on a headband has a role to play in deciding on the implantation of a bone-anchored hearing solution.

International audience; Importance: Infection after cochlear implantation is a rare but serious event that can lead to meningitis. There is no consensus on prevention of infection in these patients, and each center applies its own strategy.Objective: To describe the rates of major surgical site infection for patients undergoing cochlear implantation who receive prolonged antibiotic treatment compared with those who receive a single perioperative dose of antibiotic prophylaxis.Design, setting, and participants: Retrospective cohort study of patients who underwent cochlear implantation between January 1, 2011, and July 8, 2015, with a postoperative follow-up of 1 to 3 years. In this multicenter study at 8 French university centers, 1180 patients (509 children and 671 adults) who underwent cochlear implantation during this period were included.Interventions: Prolonged antibiotic treatment vs single-dose antibiotic prophylaxis.Main outcomes and measures: Major infection and explantation.Results: Among 1180 patients (509 children [51.7% female] with a mean [SD] age of 4.6 [3.8] years and 671 adults [54.9% female] with a mean [SD] age of 54.8 [17.0] years), 12 patients (1.0%) developed a major infection, with 4 infections occurring in the prolonged antibiotic treatment group and 8 infections occurring in the antibiotic prophylaxis group (odds ratio, 2.45; 95% CI, 0.73-8.17). Children (9 of 509 [1.8%]) were more likely to develop infection than adults (3 of 671 [0.4%]). Among children, 4 infections occurred in the prolonged antibiotic group (n = 344), and 5 infections occurred in the antibiotic prophylaxis group (n = 158) (odds ratio, 2.78; 95% CI, 0.74-10.49). Among adults, 3 infections occurred in the antibiotic prophylaxis group (n = 365), whereas no infections occurred in the prolonged antibiotic treatment group (n = 290).Conclusions and relevance: After cochlear implantation, infection was rare, was less common among those who received prolonged antibiotic treatment, and was less likely to occur in adults than in children.

Resume Objectifs Exposer les donnees connues des consequences fonctionnelles de l’ischemie cochleaire et les moyens disponibles permettant de les reconnaitre. Methodes Revue des principaux modeles integres in vivo d’ischemie cochleaire decrits chez le mammifere. Resultats Les principaux modeles integres d’ischemie cochleaire utilisent la velocimetrie laser doppler pour quantifier le degre d’ischemie cochleaire. La fonction cochleaire est controlee efficacement par l’analyse au cours de l’ischemie cochleaire des potentiels cochleaires globaux et des otoemissions acoustiques, chacun de ces signaux apportant des informations qui leur sont specifiques. La cochlee s’avere particulierement tolerante a l’ischemie reversible de plusieurs minutes. Le controle indirect de l’ischemie cochleaire par l’analyse des produits de distorsion acoustique est possible au cours d’interventions chirurgicales de l’angle pontocerebelleux. Il est encore impossible de maniere non invasive de detecter directement l’ischemie cochleaire en pratique clinique non chirurgicale. Conclusions Les modeles integres d’ischemie cochleaire ont beaucoup contribue a la connaissance du comportement fonctionnel de la cochlee dans cette situation. Un pas important a franchir en pratique clinique sera de reconnaitre precocement les situations d’ischemie cochleaire de maniere non invasive, par exemple en cas de surdite brusque.

Resume Objectifs Determiner les aspects cliniques, histologiques, epidemiologiques et professionnels, en particulier ceux lies a une exposition aux poussieres de bois, d’une serie de 100 tumeurs malignes naso-sinusiennes. Materiel et methodes Il s’agit d’une etude retrospective et descriptive de cas diagnostiques entre le 1er janvier 1981 et le 31 decembre 2000, dans la region Auvergne. Les donnees personnelles, medicales et professionnelles ont ete recueillies a partir de l’interrogatoire du patient, de sa famille lorsqu’il etait decede et des dossiers medicaux disponibles. Resultats Quarante-six cas (46 hommes), d’âge moyen de 63 ± 9,2 ans [43–82] avaient ete exposes aux poussieres de bois dans le cadre du travail avant le diagnostic. Cinquante-quatre cas (30 hommes, 24 femmes), d’âge moyen de 64,3 ± 8,7 ans [40–96] n’avaient jamais ete exposes. L’incidence annuelle moyenne augmente, que ce soit pour la population totale, ou que ce soit pour les deux sous-groupes distingues selon l’exposition professionnelle aux poussieres de bois. La plupart des patients presentaient au moment du diagnostic des signes d’appel multiples. Pour les 46 patients exposes aux poussieres de bois, les tumeurs etaient essentiellement des adenocarcinomes (92 %), avec une localisation ethmoidale. Pour les 54 patients non exposes, les tumeurs observees etaient majoritairement des carcinomes epidermoides (57 %), suivis des adenocarcinomes (15 %). Sur les 46 patients exposes aux poussieres de bois, 85 % avait exerce les metiers de menuisiers ou d’ebenistes. Pour la majorite des patients l’exposition a commence avant 20 ans (âge moyen : 17 ans ± 4,5) et l’essentiel de l’exposition s’est deroulee avant 1981. La duree de l’exposition aux poussieres de bois avant le diagnostic est dans la plupart des cas superieure a 20 ans (duree moyenne : 37 ans ± 11,4). Seuls 15 % etaient encore en activite et exposes au moment du diagnostic (delai moyen entre la fin de l’exposition et le diagnostic : 11 ans ± 2,8). Pour les 54 patients non exposes, aucune profession n’etait particulierement representee. Conclusion Les aspects epidemiologiques, comme l’augmentation de l’incidence, cliniques, professionnels, en particulier sur l’exposition professionnelle aux poussieres de bois de cette serie sont en accord avec la litterature francaise et europeenne. Il est encore probablement trop tot pour juger de l’efficacite des actions de prevention entreprises en France a partir des annees 1980 sur l’incidence de ces tumeurs.

Annales Francaises d'Oto-Rhino-Laryngologie et de pathologie cervico-faciale - Vol. 124 - N° 6 - p. 314-317

Resume Objectif Les otoemissions acoustiques (OEA) connues depuis 1978 ont une origine endocochleaire bien etablie. Elles dependent etroitement du travail des cellules ciliees externes et servent largement en recherche experimentale pour tester la vitalite cochleaire. Mais les OEA ne sont que tres peu utilisees en pratique clinique, a part pour le depistage des nourrissons. L'objectif de ce dossier thematique est de montrer leur grande utilite clinique. Materiel et methodes Une revue des connaissances physiologiques et biophysiques des OEA, sous leurs differentes formes, provoquees transitoires, ou produits de distorsion acoustiques, precise la nature et l'origine de ce signal acoustique. Differentes situations cliniques sont ensuite exposees et les alterations des OEA expliquees pour chacune. Il est question, notamment du depistage neonatal de la surdite, du diagnostic de surdite en fonction de l'âge, de situations critiques pour la cochlee comme un traitement ototoxique ou une intervention chirurgicale dans l'angle pontocerebelleux. Resultats Les OEA apparaissent potentiellement tres utiles en pratique clinique, notamment pour l'aide au depistage et au diagnostic de surdite. Elles servent aussi a controler l'audition en periode peroperatoire lors de resections tumorales de l'angle pontocerebelleux. Conclusion Les OEA sont encore sous-utilisees par le clinicien dans un but diagnostique alors que leur aide clinique potentielle devrait leur conferer un role primordial.

Fibrous dysplasia of bone is a benign, uncommon, sporadic, congenital skeletal disorder resulting in deformity. This disease arises from activating somatic mutation in GNAS which encodes the α subunit of the G stimulatory protein associated with proliferation of undifferentiated osteogenic cells resulting in marrow fibrosis, abnormal matrix production, and stimulation of osteoclastic resorption upon overproduction of IL-6 observed in dysplastic cells. Fibrous dysplasia may be monostotic or polyostotic. This mutation affecting many tissues, café au lait skin macules and endocrinopathies (precocious puberty, hyperthyroidism, growth hormone excess, Cushing syndrome) may be associated in McCune-Albright syndrome, but also myxoma in Mazabraud syndrome or phosphate diabetes. Diagnosis of craniofacial fibrous dysplasia should be considered in the presence of headache, neuralgia, sensory disorders (vision, hearing, balance, smelling), functional disorders (nasal obstruction, nasolacrimal duct obstruction, non-matching occlusion), infectious complications (sinusitis, otitis, mastoiditis). Such symptoms should lead to perform craniofacial CT scan completed with MRI. Bone biopsy is not systematic. Surgical treatment is discussed in cases of nervous complication, facial deformity or active lesions. In case of pain resistant to conventional analgesics, intravenous bisphosphonates can be proposed. In non-responder patients, several case reports suggest the efficacy of a monoclonal antibody directed against the IL-6 receptor which requires to be confirmed by randomized studies.

Objectifs Determiner les differents facteurs pronostiques de survie des adenocarcinomes (ADK) de l’ethmoide Materiel et methodes Soixante patients atteints d’un ADK de l’ethmoide. 59 hommes et une femme. Age moyen de 62,2 ans (41-82). Etude retrospective sur 20 ans. Les donnees suivantes ont ete analysees : duree d’exposition au bois, incidence de la maladie, signes d’appel de la maladie et donnees sur l’etat general du patient (score ASA). Les signes radiologiques recueillis par tomodensitometrie et imagerie par resonance magnetiquenucleaire ont ete analyses. Les caracteristiques histologiques ont ete notees. La classification TNM selon UICC 2002 et selon Roux/Brasnu a ete etablie sur la base des constatations cliniques et radiologiques. Les differents traitements utilises ont ete repertories. L’evaluation des taux de survie et l’impact des differents facteurs pronostiques ont ete realises par la methode de Kaplan-Meier et analyse multivariable. Resultats L’incidence etait de 2,86 nouveaux cas par an. La duree moyenne d’expositiona la poussiere de bois etait de 25,6 ans (2-44). Les tumeurs T3/T4 etaient predominantes (66,7 %). La survie globale brute etait de 46,5 % a 5 ans. La survie etait significativement. Conclusion Les facteurs de survie des ADK de l’ethmoide retrouves dans cette etude sont le stade tumoral et l’envahissement du sinussphenoidal. L’atteinte du sinus sphenoidal devrait, selon cette etude, venir completer les criteres de stadification de l’adenocarcinome de l’ethmoide.

Objectif Le but de cette etude etait de rapporter deux cas de tumeurs cervicale et para-pharyngee d’origine osseuse. Materiel et methodes Les patients etaient âges de 29 et 67 ans. Les lesions se presentaient respectivement sous la forme d’une masse cervicale droite et d’une masse para-pharyngee gauche. Les caracteristiques cliniques, radiologiques, anatomo-pathologiques et therapeutiques ont ete analysees de facon retrospective. Resultats Un abord cervical a ete realise dans les deux cas. Les biopsies ont respectivement mis en evidence un kyste anevrysmal osseux primitif vertebral et un chordome corporeo-pediculaire vertebral. Conclusion Les tumeurs osseuses vertebrales revelees par une masse cervicale sont des entites tres rares. Le diagnostic doit etre systematiquement evoque devant une tumeur cervicale ou para-pharyngee associee a une lyse osseuse vertebrale.

Objectifs Evaluer la prise en charge des patients traites d’un paragangliome tympanique. Patients et methodes De 1996 a 2003, nous avons pris en charge neuf patients porteurs de paragangliomes tympaniques. On comptait 8 femmes et 1 homme (âge moyen : 65 ans). Ces 9 patients furent operes. Cette etude retrospective s’est interessee aux signes revelateurs, aux moyens diagnostiques utilises, a la technique chirurgicale, ainsi qu’aux resultats fonctionnels a court-, moyen-, et long-termes. Resultats L’intervention chirurgicale fut toujours bien supportee. Il n’y eut aucun cas de mastoidite post-operatoire. Aucun patient n’eut de trouble de l’equilibre post-operatoire. La resection tumorale fut obtenue dans 7 cas. Dans un cas, la resection fut incomplete afin de preserver la motricite faciale. Tous les patients sauf un furent soulages de leurs acouphenes apres chirurgie. Toutefois, dans quatre cas, la surdite fut aggravee par la resection chirurgicale, essentiellement par une augmentation de la composante transmissionnelle. Conclusion Le diagnostic de paragangliome tympanique, a evoquer devant tout acouphene pulsatile, est fortement oriente par l’imagerie. Il doit etre le plus precoce possible pour minimiser la morbidite post-operatoire. Si le traitement chirurgical permet la guerison et supprime l’acouphene, il comporte un risque auditif eleve.

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 129 - N° 4 - p. 211-213