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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.

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.

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.

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.

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.

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.

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

Resume Propos. – Les surdites brusques idiopathiques sont des surdites neurosensorielles sans cause declenchante reconnue au moment de leur installation. L’atteinte neurosensorielle est habituellement cochleaire, mais certaines lesions retrocochleaires (tumorales de l’angle pontocerebelleux, degeneratives, ou ischemiques du nevraxe) peuvent se reveler ainsi. La prise en charge vise a rechercher une cause et a instaurer un traitement en urgence. Lorsqu’aucune cause n’est trouvee, on pose le diagnostic de surdite brusque idiopathique. Habituellement l’atteinte est cochleaire. La physiopathologie de cette atteinte sensorielle est encore inconnue. Il est tres probable que plusieurs causes soient possibles, leur point commun etant une alteration de la boucle de retrocontrole de l’organe de Corti. Actualites et points forts. – Il est fort probable que parmi les causes, une reactivation de virus neurotropes et/ou une ischemie cochleaire soient frequentes. Quelle que soit la cause, le traitement doit etre instaure en urgence et comprendre une corticotherapie a forte dose. Tout autre traitement n’a jamais prouve formellement son efficacite. On cherche ensuite, dans un second temps moins urgent, a eliminer une atteinte retrocochleaire, telle qu’une tumeur de l’angle pontocerebelleux, notamment chez le sujet jeune. Perspectives et projets. – Un des objectifs actuels est de pouvoir determiner les cas d’ischemie cochleaire de facon mini-invasive, notamment par velocimetrie laser doppler, afin d’optimiser le traitement. Sur le plan therapeutique, la protection acoustique precoce est benefique en cas d’ischemie cochleaire, au moins chez l’animal. Son efficacite en cas de surdite brusque, toute etiologie confondue, est en cours d’evaluation, dans un projet multicentrique.

The arteries and veins of the left vagus (VN) and left recurrent laryngeal (RLN) nerves from the thoracic inlet to the subaortic region are described following vascular casting with red colored latex in 6 adult fresh non-embalmed cadavers. In all specimens the anterior bronchoesophageal artery supplied at least one vessel to the VN and RLN in the subaortic region. For the RLN other arterial sources were arteries arising from the aortic arch in 1 specimen, the subclavian artery in 3 specimens, the first intercostal artery in 1 specimen, and the inferior thyroid artery in all specimens. For the VN other arterial sources were arteries arising from the aortic arch in 2 specimens and the inferior thyroid artery in 1 specimen. For both the VN and RLN the veins were located under the pleura and directed towards the internal thoracic vein anteriorly and the thoracic intercostal veins posteriorly. In conclusion, the inferior thyroid artery at the thoracic inlet for the RLN and the anterior bronchoesophageal artery are the more consistent vessels supplying the VN and RLN. Vascular damage occurring during mediastinal lymph node excision to the VN and RLN, especially in the subaortic region, may explain postoperative vocal fold paralysis.

Resumen Las vias auditivas centrales ascendentes se situan en el tronco cerebral (nucleos cocleares, complejo olivar superior), el mesencefalo (colliculus inferior), el talamo (cuerpo geniculado medial) y el cortex auditivo. Tambien existen vias eferentes y circuitos interneuronales que permiten, ligados a las vias ascendentes, realizar un analisis complejo del sonido, con fenomenos de convergencia y de divergencia de la informacion acustica, tratamientos de la senal en serie y en paralelo y una modulacion por retroaccion.

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 132 - N° 2 - p. 113-114

The effects of water temperature (Tw) on core body temperature (Tb) and foraging behaviour were studied in four free-ranging and one captive otter Lutra lutra in NE Scotland. The free-ranging animals were observed in and around two freshwater lochs, and measurements were made of Tw (2–16°C), Tb at the beginning and end of swimming bouts, lengths of swimming bouts, and dive times and intervals. Tb ranged from 35.9 to 40.4°C (mean 38.1°C). At the beginning of a period of activity and of a swimming bout. Tb rose significantly; during swimming it fell at a rate of 2.3°C/h, independent of Tw. Dive intervals were longer in colder waters, but this was probably due to other, seasonal environmental changes (perhaps water level). Tw had no significant effect on length of swimming bouts or on Tb. Although otters entered water with increased Tb, they exited when Tb was not significantly different from the mean resting Tb. The data suggest that otters maintain Tb irrespective of Tw, by increasing it before entering water, then either exiting at a given Tb, or at an earlier time determined by other environmental conditions.

Endolymphatic pressure measurement is of interest both to researchers in the physiology and pathophysiology of hearing and ENT physicians dealing with Menière's disease or similar conditions. It is generally agreed that endolymphatic hydrops is associated with Menière's disease and is accompanied by increased hydrostatic pressure. Endolymphatic pressure, however, cannot be measured precisely without endangering hearing, making the association between hydrops and increased endolymphatic pressure difficult to demonstrate. Several integrated in vivo models have been developed since the 1960s, but only a few allow measurement of endolymphatic hydrostatic pressure. Models associating measurement of hydrostatic pressure and endolymphatic potential and assessment of cochlear function are of value to elucidate the pathophysiology of endolymphatic hydrops. The present article presents the main types of models and discusses their respective interest.

La surdité de transmission de l’enfant est un diagnostic diffi cile. Elle peut passer inaperçue pendant plusieurs années et pourtant son retentissement sur le développement langagier oral est possible à partir de 30 dB de perte auditive. Les étiologies sont nombreuses. Il est utile de les classer en fonction de l’âge d’une part et de l’aspect tympanique d’autre part. Chez le nourrisson, les atteintes malformatives prédominent. De deux à 6 ans l’otite séromuqueuse est de loin la cause la plus fréquente. Après 6 ans, on est plus souvent confrontés à des séquelles d’otites répétées ou d’otite séromuqueuse. L’aspect tympanique est très informatif, notamment chez l’enfant plus grand sans malformation apparente. Il permet de diagnostiquer rapidement les séquelles d’otite chronique, les aspects malformatifs tympaniques éventuels, ou au contraire d’évoquer une surdité de transmission à tympan normal.

Introduction La dysplasie fibreuse osseuse (DFO) est une maladie benigne sporadique congenitale rare (prevalence inferieure a 1/2000). L’atteinte peut etre monostotique ou polyostotique. L’anomalie moleculaire est une mutation somatique du gene GNAS codant pour la sous-unite α de la proteine G avec pour consequence un defaut de differentiation des osteoblastes, une proliferation fibreuse medullaire et une hyperactivite osteoclastique en partie liee a l’expression excessive de l’IL-6 dans les cellules mutees. Cette mutation affectant egalement d’autres types cellulaires, cette maladie peut etre associee a des manifestations cutanees (taches « cafe au lait ») et endocrines (puberte precoce, hyperthyroidie, acromegalie, syndrome de Cushing) dans le cadre du syndrome de Mac Cune-Albright, a un diabete phosphore ou encore a des myxomes dans le cas du syndrome de Mazabraud. Observations Nous rapportons les cas de quatre patients atteints de DFO crânio-faciales. 1re observation : femme de 71 ans. Antecedents : aucun. Symptomatologie : depuis 2 ans, instabilite a la marche avec, depuis 3 mois, quasi-cecite de l’œil droit. Examen ophtalmologique : AV 2e observation : femme de 24 ans. Antecedents : aucun. Symptomatologie : vertiges positionnels depuis 2 mois. Examen ophtalmologique : normal. Examen neurologique : normal. Scanner + IRM : lesion sphenoidale droite de contenu graisseux. Scintigraphie osseuse : pas d’autre lesion. Bilans phosphocalcique et endocrinien : normaux. Traitement et evolution : surveillance clinique et radiologique car lesion non responsable de la symptomatologie. 3e observation : homme de 51 ans. Antecedents : cirrhose virale et ethylique. Symptomatologie : cephalees frontales s’aggravant depuis 2 mois. Examen ophtalmologique : normal. Examen neurologique : normal. Scanner + IRM : lesion evocatrice de DFO des sinus frontal et sphenoidal gauches. Scintigraphie osseuse : pas d’autre lesion. Bilans phosphocalcique et endocrinien : normaux. Traitement et evolution : les perfusions d’AREDIA vont etre debutees prochainement. 4e observation : femme de 51 ans. Antecedents : aucun. Symptomatologie : cephalees frontales invalidantes depuis 2 ans. Examen ophtalmologique : normal. Examen neurologique : normal. Scanner + IRM : epaississement d’allure fibreuse frontal bilateral et ethmoidal. Scintigraphie osseuse : pas d’autre lesion. Bilans phosphocalcique et endocrinien : normaux. Traitement et evolution : disparition des cephalees des la 1re perfusion d’AREDIA. A un an, stabilite de la lesion au scanner. Espacement des perfusions a tous les 6 mois. Conclusion Devant des cephalees, des nevralgies, des atteintes sensorielles (vision, audition, equilibre, olfaction), des troubles fonctionnels (obstruction nasale, dilatation des voies lacrymales, trouble de l’occlusion des mâchoires), des complications infectieuses (sinusites, otites, mastoidites), il faut evoquer le diagnostic de DFO cranio-faciale et realiser un scanner du massif facial et du crâne complete d’une IRM pour le diagnostic differentiel de meningiome en plaque de la base. On observe schematiquement 3 formes : osteolytique, condensante, et moderement condensante ressemblant a l’os pagetique pour laquelle le diagnostic differentiel se fera par la constatation de la persistance de la lame corticale. La biopsie osseuse n’est pas systematique. Un traitement chirurgical est discute au cas par cas, en particulier en cas de complication nerveuse ou d’evolutivite des lesions. En cas de douleur resistante aux traitements habituels, l’utilisation de biphosphonates est recommandee. Chez les patients non repondeurs, plusieurs cas cliniques ont montre le succes d’un anticorps monoclonal dirige contre le recepteur de l’IL-6, le tocilizumab. Des etudes, comme l’etude randomisee TOCIDYS, sont necessaire pour faire la preuve de cette efficacite.