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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).

Objectifs Dans le contexte de l’épidémie de SARS-CoV-2, les patients présentant des pathologies relevant de l’otoneurologie médicale ont pu être dissuadés de consulter, s’exposant ainsi à un risque de perte de chance. Ce guide de conseils de bonne pratique vise à définir l’apport de la téléconsultation à l’évaluation de l’adulte ou de l’enfant vertigineux, et à préciser les éléments recueillis à distance devant conduire à une prise en charge médicale rapide. Méthodes Ces conseils de bonne pratique reposent sur l’expérience des auteurs ainsi qu’une revue de la littérature. Le recensement des solutions de télémédecine adaptées à l’otoneurologie a été effectué sur la base d’une requête par moteur de recherche datant de mars 2020. Résultats La première évaluation clinique du patient vertigineux ne peut être réussie en téléconsultation que si les conditions suivantes sont réunies : prise de contact préalable pour vérifier la faisabilité de l’évaluation à distance, présence indispensable d’un aidant auprès du patient, possibilité de réaliser des enregistrements vidéos. Que ce soit chez l’adulte ou chez l’enfant, et de façon identique à l’évaluation présentielle, l’anamnèse permet d’apprécier le type, la durée, la fréquence et les circonstances de survenue du vertige. Lors de la téléconsultation peuvent être réalisés des tests d’oculomotricité, une recherche de nystagmus, une évaluation de la posture, des tests neurologiques simplifiés, la mise en évidence d’un vertige positionnel voire son traitement. Chez l’enfant doivent être recherchés en priorité des antécédents de surdité ou ophtalmologiques, un contexte fébrile ou traumatique, une otorrhée, des signes d’irritation méningée. Conclusion La télémédecine en otoneurologie trouve sa légitimité dans la fiabilité d’un bilan clinique, basé sur l’interrogatoire et quelques tests élémentaires, incitant à proposer des algorithmes décisionnels adaptés à la téléconsultation. Cette dernière comporte néanmoins des limites lors d’un premier épisode vertigineux en urgence, si bien qu’elle ne peut se substituer, dans un certain nombre de cas, à une consultation en présentiel. Elle est souvent adaptée pour le suivi de patients vertigineux sélectionnés par une évaluation présentielle initiale.

Objectives In the context of the SARS-CoV-2 pandemic, patients may have been dissuaded from seeking consultation, thus exposing themselves to a risk of loss of chance. This guide aims to define how teleconsultation can assist in assessing vertiginous adults or children, and to gather the information needed to provide quick medical care. Methods These recommendations rely on the authors’ experience as well as on literature. A survey on otoneurologic approach via telemedicine has been conducted based on a literature search until March 2020. Results The first clinical assessment of the vertiginous patient via teleconsultation can only be successful if the following conditions are met: initial contact to verify the feasibility of the assessment at a distance, the presence of a caregiver in order to assist the patient, the possibility of making video recordings. Medical history via telemedicine, as in a face-to-face assessment, allows to assess the characteristics, duration, frequency, and potential triggering factors of the vertigo, in both children and adults. During teleconsultation, the following tests can be carried out: oculomotricity evaluation, assessment of balance, simple neurological tests, checking for positional vertigo/nystagmus and, eventually to perform canalith-repositioning procedures. In children, the following should be searched for: history of hearing or visual impairment, a context of fever or trauma, otorrhea, signs of meningeal irritation. Conclusion The neurotologic telemedicine relies on the accuracy of the clinical assessment, which is based on history taking and a few simple tests, encouraging the development of a decision-making algorithm adapted for teleconsultation. However, the latter has its limitations during an emergency examination of a new patient presenting vertigo, and, at least in some cases, cannot replace a face-to-face consultation. Teleconsultation is often adapted for follow-up consultations of previously selected vertiginous patients during face-to-face assessment.

Resume Objectifs Durant l’epidemie de Covid-19, l’acces au diagnostic et aux soins des pathologies relevant de l’audiologie medicale a regresse en France, posant un risque de perte de chance pour le patient. Ce guide de conseils de bonne pratique vise a repertorier les solutions techniques existantes permettant d’examiner a distance le patient presentant une plainte en audiologie et a decrire leurs interets et, le cas echeant, leurs limites. Methodes Les conseils ont pu etre elabores a la fois a partir de l’experience clinique des experts medicaux ayant participe a la redaction du guide, et d’une revue extensive de la litterature portant sur les recommandations de pratique clinique en tele-audiologie. Les solutions de tele-audiometrie ont ete recensees sur la base d’une requete par moteur de recherche effectuee en avril 2020, avant verification de leur disponibilite sur le marche europeen. Resultats Des solutions de video-otoscopie permettent la tele-transmission d’images compatibles avec un diagnostic de qualite soit en se connectant via internet a une plate-forme de tele-soin, soit via un smartphone ou une tablette utilisant un systeme d’exploitation iOS ou Android. Selon les memes modalites, il est possible de realiser a distance un examen audiometrique tonal dans les regles de l’art, une audiometrie vocale dans le silence ou dans le bruit ainsi que des explorations fonctionnelles objectives de l’audition. Les examens cliniques et paracliniques peuvent etre consultes par le medecin de facon differee pour etre interpretes (tele-audiologie asynchrone). Ils peuvent aussi etre realises en temps reel chez un patient, a tout âge de la vie, a condition qu’un aidant puisse etre present durant l’installation des transducteurs ou la realisation de l’acoumetrie (tele-audiologie synchrone). Les solutions de teleaudiologie trouvent egalement une application dans la formation a distance des futurs professionnels de sante engages dans la prise en charge des pathologies de l’audition. Conclusion Dans la legislation francaise, la tele-otoscopie est un acte medical qui releve soit de la tele-expertise (avis asynchrone) soit d’un acte de teleconsultation (avis synchrone). L’evaluation subjective et objective de la fonction auditive du patient est aujourd’hui possible a distance a condition de respecter les precautions listees.

PURPOSE The purpose of this study was to describe the MRI characteristics of intralabyrinthine schwannoma (ILS) on post contrast three-dimensional (3D) fluid-attenuation-inversion-recovery (FLAIR) images obtained four hours after intravenous administration of a gadolinium-based contrast agent (4h-3D-FLAIR). MATERIALS AND METHODS This IRB-approved retrospective multi-center study included patients presenting with typical ILS from January 2016 to October 2020. All medical charts were systematically collected. All MRI examinations, including 4h-3D-FLAIR images, were reviewed by two board-certified neuroradiologists. Main outcome measures were location, signal intensity and associated anomalies of ILS. RESULTS Twenty-seven out of 8730 patients (0.31%) referred for the investigation of a cochleovestibular disorder had a final diagnosis of ILS. There were 13 men and 14 women with a mean age of 52 ± 17 (SD) years (age range: 20-86 years). The most common clinical presentation was unilateral progressive sensorineural hearing loss (16/27; 59%). All ILS were unilateral and 15 (15/27; 55%) were intracochlear. All ILS presented as a hypointense filling defect within the labyrinth on T2-weighted images that enhanced on post-contrast T1-weighted images. On 4h-3D-FLAIR images, all ILS presented as a hypointense filling defect, associated with diffuse perilymphatic hyperintensity. Two patients (2/27; 7%) presented with ipsilateral endolymphatic hydrops. CONCLUSION ILS displays consistent features on post-contrast 4h-3D-FLAIR images. ILS should not be confused with endolymphatic hydrops and requires a systematic analysis of the corresponding T2-weighted images.

International audience; Our objective was to identify a sensitive marker of disease progression in Friedreich’s ataxia. We prospectively evaluated speech, voice, and oromotor function in 40 patients at two timepoints. The mean disease duration was 20.8 ± 9.8 years and mean SARA score 23.7 ± 8.6 at baseline. Oral motor mobility, assessed by a combination of movements of the face, eyes, cheeks, lips, and tongue, decreased significantly after 1 year (P < 0.0001). The standardized response mean over 12 months was considered as large for oral mobility (1.26) but small for SARA (0.12). Oral mobility could therefore be a sensitive marker in therapeutic trials.

International audience; Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls ( P = 0.001); half were previously unknown (AF < 0.000002). MYO6 , MYO7A , PTPRQ , and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1 N321I/+ mice, carrying the TMC1 :p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

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.

Resume Introduction Consensus de la SFORL concernant les bonnes pratiques de la realisation d’un examen audiometrique chez l’adulte et l’enfant. Methodes Un groupe de travail multidisciplinaire a effectue une revue de la litterature. Des recommandations ont ete erigees, relues par un groupe de lecture independant du groupe de travail et finalisees lors d’une reunion de synthese. Resultats Il est recommande de realiser les examens audiometriques dans un environnement sonore maitrise (

Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions. The Abbreviated Profile of Hearing Aid Benefit (APHAB) inventory was evaluated preoperatively and at 6 and 12 months postoperatively. Results No difference was found between pre- and postoperative tests for disyllabic and monosyllabic words. For sentences in quiet and noisy environments, a difference between pre- and postoperative performance was present at 1 year ( P = .002 and P = .02, respectively). In a noisy environment, a difference was present at 6 and 12 months postoperatively as compared with the preoperative value (mean ± SD: 6 months: 42% ± 7.1% vs 61% ± 6.5%, P = .016). A significant improvement in the APHAB score was found at 6 and 12 months postimplantation (Friedman's 2-way analysis of variance by ranks, P < .001). The number of years of hearing deprivation of the deaf ear was not correlated with performance. Conclusion When incapacitating fluctuating hearing loss occurs in patients presenting a contralateral deaf ear, a cochlear implant is indicated in the latter ear, significantly improving performance in noisy conditions and allowing a better quality of communication to be achieved.

The impact of hearing loss and of auditory rehabilitation (hearing aid, cochlear implant) on quality of life is a crucial issue. Commonly used questionnaires to assess quality of life in these patients (Nijmegen, APHAB, GBI) are time-consuming, difficult for patients to fill out, and show poor sensitivity to small improvements or deterioration. The objective of the present study was to validate a dedicated quality of life scale for hearing-impaired adults with or without auditory rehabilitation.ERSA (Évaluation du Retentissement de la Surdité chez l'Adulte: Evaluation of the Impact of Hearing Loss in Adults) is a self-administered questionnaire. It is divided into 4 domains, each comprising 5 questions graded from 1 to 10. The questions are simple, and formulated so patients will answer according to how they feel at the actual time of the session. Test-retest reliability was measured in 38 patients. Internal coherence and validity against the APHAB questionnaire as gold standard and in relation to hearing performance were measured in 122 patients at auditory assessment. Sensitivity to change in hearing was measured in 36 cochlear implant patients, before and 6 or 12 months after implantation.Test-retest reliability was very satisfactory (ρ=0.88). Internal coherence was good for all questions. External validity, comparing ERSA to APHAB scores in the same non-implanted hearing-impaired patients, was good (ρ=0.52). Additionally, ERSA scores correlated with hearing performance in adverse conditions (monosyllabic words: ρ=0.22; sentences in noise: ρ=0.19). In patients tested before and after cochlear implantation, improvement in hearing performance in silence and in noise correlated with an improvement in ERSA score (ρ=0.37 to 0.59, depending on the test), but not to GBI score.The ERSA questionnaire is easy and quick to use, reliable, and sensitive to change in hearing performance after cochlear implantation.

Resume Objectifs L’evaluation de l’impact de la surdite et de sa rehabilitation (aides auditives, implant cochleaire) sur la qualite de vie est fondamentale. Les questionnaires couramment utilises (Nijmegen, Aphab, GBI) sont longs, compliques a remplir et peu sensibles aux faibles ameliorations/deteriorations. L’objectif de cette etude est de valider une echelle de qualite de vie destinee aux patients adultes presentant une perte auditive qu’elle soit rehabilitee ou non. Materiel et methodes Le questionnaire d’evaluation du retentissement de la surdite chez l’adulte (Ersa) est un questionnaire auto-administre, divise en quatre sous-domaines comprenant chacun cinq questions notees de 1 a 10. Les questions sont simples et formulees de facon a ce que le patient reponde a l’instant present. La fiabilite test-retest d’Ersa a ete mesuree chez 38 patients. La coherence interne et la validite par rapport au questionnaire Aphab et aux performances auditives ont ete mesurees chez 122 patients lors du bilan de surdite. La sensibilite au changement a ete mesuree chez 36 patients avant et 6 a 12 mois apres l’implantation cochleaire. Resultats La fiabilite du questionnaire Ersa (test/retest) est tres satisfaisante (ρ = 0,88). La coherence interne est bonne pour l’ensemble des questions. La validite de structure externe, etudiee en comparant, chez les patients sourds non implantes, les scores Ersa a ceux du questionnaire Aphab, est bonne (ρ = 0,52). Le score Ersa est correle aux performances auditives dans des situations d’ecoute difficiles (mots monosyllabiques : ρ = 0,22 ; phrases dans le bruit : ρ = 0,19). Chez les patients testes avant et apres implantation, l’amelioration des performances auditives dans le silence et dans le bruit est correlee a l’amelioration du score Ersa (ρ = 0,37 a 0,59 selon les tests), mais pas au score GBI. Conclusion Le questionnaire Ersa est un outil facile, rapide d’utilisation, fiable et sensible au changement des performances auditives apres une implantation cochleaire.

Resume Objectifs Les auteurs exposent les recommandations de la Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou (SFORL) concernant la strategie diagnostique et therapeutique dans la maladie de Meniere. Methodes Un groupe de travail multidisciplinaire a ete charge d’effectuer une revue de la litterature scientifique sur la thematique etudiee. Les recommandations ont ete redigees, puis relues par un groupe de lecture independant du groupe de travail. Les recommandations proposees ont ete classees en accord avec le guide d’analyse de la litterature et de gradation des recommandations, publie par l’ANAES (janvier 2000). Resultats Le diagnostic de la maladie de Meniere est pose devant l’association des quatre plaintes cliniques classiques et en l’absence d’une autre cause decelable par IRM. En cas de tableau clinique incomplet, il est recommande de realiser les tests d’explorations audio-vestibulaires objectifs. La prise en charge therapeutique comporte des traitements medicaux et chirurgicaux, conservateurs ou destructeurs de la fonction vestibulaire. La prise en charge medicale repose sur l’hygiene de vie, la betahistine, les diuretiques et l’injection transtympanique de corticoides et/ou de gentamicine. Les principaux moyens chirurgicaux disponibles sont par ordre d’agressivite la chirurgie du sac endolymphatique, la neurotomie vestibulaire et la labyrinthectomie.

Dominant optic atrophy is a blinding disease due to the degeneration of the retinal ganglion cells, the axons of which form the optic nerves. In most cases, the disease is caused by mutations in OPA1, a gene encoding a mitochondrial large GTPase involved in cristae structure and mitochondrial network fusion. Using exome sequencing, we identified dominant mutations in DNM1L on chromosome 12p11.21 in three large families with isolated optic atrophy, including the two families that defined the OPA5 locus on chromosome 19q12.1-13.1, the existence of which is denied by the present study. Analyses of patient fibroblasts revealed physiological abundance and homo-polymerization of DNM1L, forming aggregates in the cytoplasm and on highly tubulated mitochondrial network, whereas neither structural difference of the peroxisome network, nor alteration of the respiratory machinery was noticed. Fluorescence microscopy of wild-type mouse retina disclosed a strong DNM1L expression in the ganglion cell layer and axons, and comparison between 3-month-old wild-type and Dnm1l+/- mice revealed increased mitochondrial length in retinal ganglion cell soma and axon, but no degeneration. Thus, our results disclose that in addition to OPA1, OPA3, MFN2, AFG3L2 and SPG7, dominant mutations in DNM1L jeopardize the integrity of the optic nerve, suggesting that alterations of the opposing forces governing mitochondrial fusion and fission, similarly affect retinal ganglion cell survival.

Objective To evaluate the efficacy of portable Meniett low-pressure pulse generator (Medtronic Xomed, Jacksonville, FL) in Meniere disease. Study Design Randomized, double-blind, placebo-controlled, multicenter trial carried out in 17 academic medical centers. Methods One hundred twenty-nine adults presenting Meniere disease (American Academy of Otolaryngology–Head and Neck Surgery criteria) not controlled by conventional medical treatment were included. The protocol included three phases: 1) placement of a transtympanic tube and evaluation of its effect (if resolution of symptoms, the patient was excluded); 2) randomization: 6-weeks treatment with Meniett (Medtronic Xomed) or placebo device; 3) removal of the device and 6-week follow-up period. The evaluation criteria were the number of vertigo episodes (at least 20 minutes with a 12-hour free interval) and the impact on daily life as assessed by self-questionnaires. Results Ninety-seven patients passed to the second phase of the study: 49 and 48 patients received the Meniett (Medtronic Xomed) or the placebo device, respectively. In the placebo group, the number of vertigo episodes decreased from 4.3 ± 0.6 (mean ± standard error of the mean) during the first phase to 2.6 ± 0.5 after 6 weeks of treatment, and to 1.8 ± 0.8 after the removal of the device. Similar results were observed in the Meniett device (Medtronic Xomed) group: 3.2 ± 0.4 episodes during the first phase, 2.5 ± after 6 weeks of Meniett device (Medtronic Xomed) treatment, and 1.5 ± 0.2 after the third phase. Conclusion An improvement of symptoms was evidenced in all patients, with no difference between the Meniett (Medtronic Xomed) and the placebo device groups. The decrease in the number of vertigo episodes could be explained by an effect of the medical care. Level of Evidence 1b. Laryngoscope, 2016 127:470–475, 2017

Objectives The present study assessed the interest of a contralateral hearing aid (HA) in adult cochlear implant (CI) bearers. Material and methods The study recruited 10 French-speaking adult HA bearers with postlingual bilateral hearing loss, fitted for at least 2 years with a unilateral CI after loss of benefit from HA in one ear but continuing to use their contralateral HA: 4 male, 6 female; mean age, 58 years. All had regularly used bilateral HAs prior to CI. Audiometric assessment comprised: (1) individual ear hearing assessment on pure-tone audiometry and speech discrimination; and (2) free-field testing without aid, with CI only, with HA only and with CI plus HA, on pure-tone audiometry and speech discrimination with quiet background and on speech discrimination in noise. Results Speech discrimination was significantly improved in the bimodal condition (CI plus HA) as compared to CI alone, on all tests. In quiet, discrimination for disyllabic words was > 50% in 7 cases with HA alone, in 2 cases with CI alone and in 1 case in with HA + CI. Under 0 dB signal-to-noise ratio, discrimination was > 50% in 1 case with HA alone, in 3 cases with CI alone and in 6 cases with HA + CI. Conclusion The present results showed benefit in auditory perception in quiet and in noise with bimodal stimulation. When there is residual hearing in the non-implanted ear, a HA should be fitted; and in progressive bilateral hearing loss, CI should be suggested when HA benefit decreases in one ear.

Resume Objectifs L’objectif de cette etude etait de montrer l’interet et les benefices obtenus par l’utilisation d’une aide auditive controlaterale chez des adultes porteurs d’un implant cochleaire (IC). Materiel et methodes L’etude a porte sur une population d’adultes implantes cochleaires unilateraux, pour une surdite post-linguale bilaterale, avec perte du benefice prothetique d’un cote et poursuite de l’utilisation d’une aide auditive (AA) controlaterale. Il s’agissait de 10 adultes francophones, 4 hommes et 6 femmes, dont la moyenne d’âge etait de 58 ans. Ils etaient implantes depuis 2 ans au moins. Tous les patients utilisaient regulierement des AA bilaterales anterieurement a l’implantation cochleaire. L’evaluation audiometrique a comporte : (1) une evaluation de l’audition residuelle oreilles separees (tonale et vocale), (2) des tests en champ libre : « oreilles nues », IC seul, AA seule et IC et AA, en tonale, vocale dans le calme et vocale dans le bruit. Resultats Les resultats obtenus ont montre que l’intelligibilite etait significativement superieure en condition bimodale, IC et AA, par rapport a ceux obtenus avec l’IC seul, dans tous les tests realises. Dans le calme, les resultats ont montre une intelligibilite qui etait superieure a 50 % pour des mots dissyllabiques, dans 7 cas avec la seule AA, 2 cas avec l’IC et un cas en condition bimodale : IC et AA. Les tests realises avec un rapport signal/bruit a 0 dB ont montre un niveau de comprehension superieur a 50 %, pour un patient avec uniquement son AA, pour 3 d’entre eux avec l’IC et pour 6 en condition bimodale. Conclusion Les resultats obtenus ont montre l’interet du port de l’AA controlaterale a l’IC en termes d’amelioration de la perception auditive dans le calme et le bruit. En cas d’audition residuelle controlaterale a l’IC, une AA doit etre proposee ; et dans les surdites bilaterales progressives, l’IC doit etre proposee des que le patient perd le benefice de l’AA d’un des cotes.

Comment on :Mutations in DNM1L, as in OPA1, result in dominant optic atrophy despite opposite effects on mitochondrial fusion and fission. [Brain. 2017]The expanding neurological phenotype of DNM1L-related disorders. [Brain. 2018]; International audience; Letter to the editor

International audience; INTRODUCTION: French Society of ENT (SFORL) good practice guidelines for audiometric examination in adults and children. METHODS: A multidisciplinary working group performed a review of the scientific literature. Guidelines were drawn up, reviewed by an independent reading group, and finalized in a consensus meeting. RESULTS: Audiometry should be performed in an acoustically controlled environment (\textless30dBA); audiometer calibration should be regularly checked; and patient-specific masking rules should be systematically applied. It should be ensured that masking is not overmasking. Adult pure-tone audiometry data should be interpreted taking account of clinical data, speech audiometry and impedancemetry. In case of discrepancies between clinical and pure-tone and speech audiometry data, objective auditory tests should be perform. In children aged 2 years or younger, subjective audiometry should be associated to behavioral audiometry adapted to the child's age. In suspected hearing impairment, behavioral audiometry should be systematically supplemented by objective hearing tests to determine and confirm the level and type of hearing impairment.

Relations between sensory functions and Alzheimer's disease are still under-explored. To understand them better, the Fondation Médéric Alzheimer has brought together a multi-disciplinary expert group. Aristote's five senses must be enhanced by today's knowledge of proprioception, motor cognition and pain perception. When cognition breaks down, the person with dementia perceives the world around her with her sensory experience, yet is unable to integrate all this information to understand the context. The treatment of multiple sensory inputs by the brain is closely linked to cognitive processes. Sensory deficits reduce considerably the autonomy of people with dementia in their daily life and their relations with others, increase their social isolation and the risk of accidents. Professionals involved with neurodegenerative diseases remain poorly aware of sensory deficits, which can bias the results of cognitive tests. However, there are simple tools to detect these deficits, notably for vision, hearing and balance disorders, which can be corrected. Many interventions for cognitive rehabilitation or quality of life improvement are based on sensory functions. The environment of people with dementia must be adapted to become understandable, comfortable, safe and eventually therapeutic.

Objectives The authors present the guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society ( Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou : SFORL) for diagnostic and therapeutic strategy in Meniere's disease. Methods A work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, then read over by an editorial group independent of the work group. The guidelines were graded according to the literature analysis and recommendations grading guide published by the French National Agency for Accreditation and Evaluation in Health (January 2000). Results Meniere's disease is diagnosed in the presence of the association of four classical clinical items and after eliminating differential diagnoses on MRI. In case of partial presentation, objective audiovestibular tests are recommended. Therapy comprises medical treatment and surgery, either conservative or sacrificing vestibular function. Medical treatment is based on lifestyle improvement, betahistine, diuretics or transtympanic injection of corticosteroids or gentamicin. The main surgical treatments, in order of increasing aggressiveness, are endolymphatic sac surgery, vestibular neurotomy and labyrinthectomy.

OBJECTIVE The relationship between hearing loss and cognitive function has already been established. The objective of our study was to determine whether the two short cognitive tests, COgnitive Disorders EXamination (CODEX) and Montreal Cognitive Assessment (MoCA), could be used in daily clinical practice to detect cognitive impairment, and its changes after cochlear implantation. PATIENTS Eighteen patients with severe to profound postlingual progressive hearing loss (mean age ± SEM: 64 ± 3.5 yr; range, 23-83 yr) were tested before, and 12 months after cochlear implantation, with adapted visual presentation of CODEX and MoCA tests. Auditory performance was tested under best-aided conditions in quiet and noise. RESULTS Twelve months after cochlear implantation, hearing performance had clearly improved (paired t tests, p

Hearing loss and visual impairment in childhood have mostly genetic origins, some of them being related to sensorial neuronal defects. Here, we report on eight subjects from four independent families affected by auditory neuropathy and optic atrophy. Whole-exome sequencing revealed biallelic mutations in FDXR in affected subjects of each family. FDXR encodes the mitochondrial ferredoxin reductase, the sole human ferredoxin reductase implicated in the biosynthesis of iron-sulfur clusters (ISCs) and in heme formation. ISC proteins are involved in enzymatic catalysis, gene expression, and DNA replication and repair. We observed deregulated iron homeostasis in FDXR mutant fibroblasts and indirect evidence of mitochondrial iron overload. Functional complementation in a yeast strain in which ARH1, the human FDXR ortholog, was deleted established the pathogenicity of these mutations. These data highlight the wide clinical heterogeneity of mitochondrial disorders related to ISC synthesis.

Aim of the study To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic. Patients The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation. Results Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition. Conclusions ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.

International audience; Most cochlear implantations are unilateral. To explore the benefits of a binaural cochlear implant, we used water-labelled oxygen-15 positron emission tomography. Relative cerebral blood flow was measured in a binaural implant group (n = 11), while the subjects were passively listening to human voice sounds, environmental sounds non-voice or silence. Binaural auditory stimulation in the cochlear implant group bilaterally activated the temporal voice areas, whereas monaural cochlear implant stimulation only activated the left temporal voice area. Direct comparison of the binaural and the monaural cochlear implant stimulation condition revealed an additional right temporal activation during voice processing in the binaural condition and the activation of a right fronto-parietal cortical network during sound processing that has been implicated in attention. These findings provide evidence that a bilateral cochlear implant stimulation enhanced the spectral cues associated with sound perception and improved brain processing of voice stimuli in the right temporal region when compared to a monaural cochlear implant stimulation. Moreover, the recruitment of sensory attention resources in a right fronto-parietal network allowed patients with bilateral cochlear implant stimulation to enhance their sound discrimination, whereas the same patients with only one cochlear implant stimulation had more auditory perception difficulties.

Los sintomas otorrinolaringologicos (ORL) que se observan durante la gestacion suelen ser benignos. En ocasiones son indicativos de una enfermedad especifica. Los mas habituales se relacionan de forma directa o indirecta con las modificaciones hormonales que se producen en el embarazo. Por ejemplo, la obstruccion nasal es frecuente y obliga a buscar una causa especifica de rinitis (alergica, infecciosa, etc.) antes de establecer el diagnostico de rinitis hormonal. Los otros sintomas son diversos: manifestaciones ORL del reflujo gastroesofagico, trastornos del gusto, etcetera. Las afectaciones auditivas son de varios tipos: disfuncion de la trompa de Eustaquio, acufenos e incluso problemas auditivos con hipoacusia moderada. Las relaciones entre la otosclerosis y su evolucion son motivo de controversia. Por ultimo, las afectaciones neurosensoriales (sordera brusca, paralisis facial) obligan a realizar un estudio etiologico en busca de una enfermedad, en especial de tipo tumoral.

Cochlear implant in adults is a procedure, dedicated to rehabilitate severe to profound hearing loss. Because of technological progresses and their applications for signal strategies, new devices can improve hearing, even in noise conditions. Binaural stimulation, cochlear implant and hearing aid or bilateral cochlear implants are the best opportunities to access to better level of comprehension in all conditions and space localisation. By now minimally invasive surgery is possible to preserve residual hearing and use a double stimulation modality for the same ear: electrical for high frequencies and acoustic for low frequencies. In several conditions, cochlear implant is not possible due to cochlear nerve tumour or major malformations of the inner ear. In these cases, a brainstem implantation can be considered. Clinical data demonstrate that improvement in daily communication, for both cochlear and brainstem implants, is correlated with cerebral activation of auditory cortex.

Our objective is to prospectively report very early complications and outcomes of cerebellopontine angle (CPA) surgery. Between January and August 2007, 72 patients were operated on by different transpetrosal approaches in a tertiary referral center. During preoperative assessment, facial nerve function (House-Brackmann grading system), the presence of vertigo or tinnitus and caloric test results were recorded and correlated with complications and symptoms occurring daily from day (D) 1 to D 8. The overall number of complications did not differ from those of former retrospective studies; nevertheless, the prospective feature of this study prompts several comments. Even slight (grade II) preoperative facial impairments increased the risk of severe postoperative facial dysfunction. Keratitis was frequent (42%) even in patients with normal facial function. Thrombo-embolic complications only occurred after long air-travel (≥5 h). Preoperative caloric test status was predictive of postoperative vestibular disturbance occurrence. With respect to the activity recovery; younger patients (40 years old) displayed faster central compensations than the older (60 years old) patients. This study highlights several features that may be used for preoperative patient counseling and complication management. In particular, the practitioner has to pay attention to even minor preoperative clinical signs of facial dysfunction to properly inform the patient of facial outcome. Routine ophthalmologic evaluation should be practiced, even when facial function is normal or subnormal.1b.

OBJECTIVE To evaluate the auditory brainstem implant (ABI) performances in neurofibromatosis type 2 (NF2) and non-NF2 patients. STUDY DESIGN Retrospective cohort study. SETTING Tertiary referral center. PATIENTS Between 1996 and 2006, 31 adult patients (mean age, 41 yr; range, 17-65 yr) were implanted with a 21-electrode Nucleus device (Cochlear Inc., Lane Cove, Australia). The population comprised 23 NF2, 3 postmeningitis bilateral ossified cochleas, 3 solitary vestibular schwannomas on the only hearing ear, 1 inner ear malformation, and 1 bilateral cochlear destruction by otosclerosis. INTERVENTION Auditory brainstem implant was placed through a translabyrinthine or a retrosigmoid approach. MAIN OUTCOME MEASURES Auditory brainstem implant was evaluated by open-set words and sentences in sound, vision, and sound-plus-vision modes. RESULTS In NF2 patients, 16 (70%) were daily users of their implants. In these patients, the open-set dissyllabic word recognition was 36 +/- 6.0 % for vision-only mode, 33 +/- 6.5 % for sound-only mode, and 65 +/- 8.0 % in vision-plus-sound mode with a high interindividual variation. Negative prognostic factors were long duration of total hearing loss (>10 yr), low number of active electrodes (

Objective To evaluate the functional results of otosclerosis surgery using diode laser. Study design Retrospective cohort analysis. Patients One hundred seven patients operated on for otosclerosis with a diode laser (119 ears, all primary cases) and 141 patients operated on with a conventional technique (141 ears, all primary cases). Revision cases using the diode laser were also described. Methods Preoperative tomographic computed scan findings and intraoperative observations were collected. Pure-tone and vocal audiometry was performed preoperatively and postoperatively (at 3 mo and 1 yr). Results In the laser group, the air-bone gap was 29 +/- 0.8 dB (n= 112) preoperatively and 9 +/- 0.6 dB (n = 58) at 1 year. Air conduction was improved by 22 +/- 1.7 dB at 1 year (n = 58). In the conventional group, the air-bone gap was 32 +/- 0.9 dB (n=127) preoperatively and 10 +/- 0.6 dB (n = 127) at 1 year. Air conduction was improved by 25 +/- 1.1 dB (n = 127) at 1 year. No difference of hearing gain was observed between the 2 groups at 1 year. A decreased rate of footplate fracture was observed with the diode laser (3.6%) compared with the conventional technique (21.3%). Conclusion Diode laser is a reliable and safe device for otosclerosis surgery. The functional results were similar to those reported in other series.

Auditory brainstem responses (ABRs) associated with other audio-vestibular examinations and a thorough clinical examination should allow detection of the majority of cerebellopontine angle (CPA) lesions (99.2-100%).The increasing quality of MRI in the detection of CPA lesions, and the reports of false negative ABRs have raised issues concerning the value of ABR in the diagnosis and preoperative assessment of CPA lesions. The aim of this work was to assess the value of the ABR in the diagnosis of vestibular schwannomas (VS) and other CPA lesions.This retrospective study included 676 solitary VS (548 operated on and 128 followed up) and 70 other CPA tumours (72% meningiomas, 11% cholesteatomas, 3% ependymomas, 15% miscellaneous) managed between 1990 and 2001. All patients underwent clinical examination, audiometry, ABR, vestibular caloric tests and MRI.ABRs were normal in 4.8% of VS. Association of normal ABR, vestibular caloric tests and audiometry (AAO-HNS class A) represented only 0.7% of VS. In other CPA lesions, ABR were normal in 15% of cases and the association of the three above-mentioned examinations was encountered in 10%. However, in the latter cases the clinical examination showed an abnormality in all cases.

Objective: To describe and analyze a series of intracochlear schwannomas. Methods: From 1987 to 2005, 19 patients with schwannomas involving the cochlea were included in this series. Clinical, audiovestibular, and imaging data concerning initial and follow-up visits were collected. Results: The mean age was 54 years (range, 25-71 yr). The series comprised 10 women and 9 men, 18 solitary tumors and 1 neurofibromatosis Type 2 patient. The mean diagnosis delay was 11 years (range, 1-31 yr). At diagnosis, a total or profound hearing loss (Class D of American Academy of Otolaryngology-Head and Neck Surgery classification) was noted in 17 patients (89%), and a severe loss (Class C) was reported in 2 patients (11%). Facial paresis was reported in 2 patients (11%; Grades 2 and 4 of House and Brackmann classification). Magnetic resonance imaging showed an involvement of the posterior labyrinth in 8 patients (37%), an extension of the schwannoma to the internal auditory meatus in 8 patients (37%), and a cerebellopontine angle extension in 7 patients (32%). In 11 patients (58%), the schwannoma was removed through a transotic route. In the 8 remaining patients, a watch-and-rescan policy was decided. The postoperative course was uneventful. Postoperative follow-up period was 27 months (range, 6-88 mo). Postoperative facial function was assessed as Grade 1 in 7 patients, as Grade 2 in 4, and as Grade 4 in 1 patient. Conclusion: The diagnosis of intracochlear schwannomas can be difficult on magnetic resonance imaging. The diagnosis should be considered in all unilateral hearing losses.

Objective Vertigo and dizziness, which are major symptoms of diseases affecting the vestibular system, drastically impair patients' health-related quality of life (QoL). Patient's perspectives are thus essential to symptom assessment. We sought to make a critical review of published questionnaires measuring vertigo or dizziness, and/or their impact on QoL. Methods Twenty-nine articles reporting the validation or use in clinical trials of vertigo- or dizziness-specific questionnaires were identified over the 1991–2004 period, and reviewed using a methodological and a Patient-Reported Outcomes specific checklist. Questionnaires were classified into three categories according to content: QoL (or handicap), mixed (assessing both symptoms and QoL), and symptom questionnaires. Results Four QoL, three mixed questionnaires, two symptoms, and one Meniere's disease-specific questionnaire were identified. QoL questionnaire validation was usually not complete. The structural validity of the Dizziness HandicapInventory is not established, although this questionnaire is considered to be the reference questionnaire in the QoL domain. Moreover, QoL questionnaires were not very specific to vertigo or dizziness. Similarly, the Vertigo Handicap Questionnaire appeared to have the most pertinent content, but its validation remains to be completed. Mixed questionnaires have the same imperfections. The Vertigo, Dizziness, Imbalance (VDI) Questionnaire had the best validation score from the checklist, but its responsiveness appears to be weak. Regarding symptom questionnaires, the European Evaluation of Vertigo questionnaire evaluated the five major symptoms of vestibular syndrome satisfactorily. Conclusion The present literature review failed to find any relevant and validated questionnaire assessing the impact of vertigo or dizziness on QoL.

Resume Objectifs Le but de cette etude est de presenter les principes de l'implant auditif du tronc cerebral (ITC) et d'evaluer le benefice apporte par cette technique innovante dans differentes indications. Materiel et methodes Les indications de l'ITC sont : la neurofibromatose de type 2 (NF2), les ossifications cochleaires totales bilaterales, les associations de schwannome vestibulaire a une surdite controlaterale et les malformations cochleaires majeures et aplasies du nerf auditif. Le bilan preoperatoire comporte une evaluation clinique, orthophonique, radiologique, et psychologique. La voie d'abord chirurgicale est retrosigmoide ou translabyrinthique. L'evaluation du benefice apporte en termes de perception auditive est realisee avec ou sans le support de la lecture labiale, pour des mots et phrases sans contexte. Resultats Les resultats montrent que, chez les patients NF2, le meilleur benefice fonctionnel auditif est obtenu dans les cas de schwannome vestibulaire de taille moyenne, avec une privation auditive du cote implantee breve ou nulle. Les facteurs de mauvais pronostic sont une privation auditive prolongee, un diametre cisternal superieur a 30 mm, une difficulte a la mise en place du porte-electrodes en peroperatoire et l'activation de moins de dix electrodes avec des sensations auditives. Dans les cas de surdite totale postmeningitique avec ossification cochleaire totale, le resultat obtenu est tout a fait comparable a celui des implants cochleaires dans cette indication (surdite postmeningitique sans ou avec ossification partielle). Il en est de meme pour les autres indications non tumorales (hors NF2). Conclusion Ces resultats demontrent que l'ITC apporte un benefice fonctionnel chez les patients NF2, ce d'autant que la privation auditive est breve et que le volume tumoral est limite. En cas d'ossification cochleaire postmeningitique, le resultat est comparable a celui d'un implant cochleaire, pour des cochlees non ou partiellement ossifiees.

Background. A consensus conference recommended empirical antibiotic therapy for all patients with postoperative meningitis and treatment withdrawal after 48 or 72 h if cerebrospinal fluid culture results are negative. However, this approach is not universally accepted and has not been assessed in clinical trials. Methods. We performed a cohort study of all patients who received a diagnosis of postoperative meningitis from January 1998 through May 2005 in a teaching hospital. From January 1998 through September 2003 (control period), guidelines were lacking or were not implemented. From October 2003 through May 2005 (interventional period), all patients received a predefined intravenous antibiotic therapy that was discontinued on the third day if the meningitis was considered aseptic. Clinical outcome and duration of antibiotic therapy were analyzed for each patient. Results. Seventy-five episodes of postoperative meningitis (21 cases of bacterial meningitis and 54 cases of aseptic meningitis) were investigated. Patients with aseptic meningitis received antibiotic treatment for a mean ± standard deviation duration of 11 ± 5 days during the control period and 3.5 ± 2 days during the intervention period (P = .001). The duration of antibiotic treatment for bacterial meningitis was not significantly different between the 2 periods. All episodes of bacterial and aseptic meningitis were cured, and complications were rare during both periods. Conclusions. Stopping antibiotic treatment after 3 days is effective and safe for patients with postoperative meningitis whose cerebrospinal fluid culture results are negative.

Resume Introduction Les osteomyelites de la base du crâne sont des infections rares mais severes et de diagnostic parfois difficile, compliquant habituellement une otite externe necrotique. Le but de cette etude clinique etait d'en preciser les caracteristiques cliniques et paracliniques, ainsi que les modalites therapeutiques et evolutives. Methodes Etude d'une cohorte de cinq patients evalues et suivis a l'hopital Beaujon entre 2004 et 2005. Resultats Les cinq patients etaient âges en moyenne de 75 ans (63–89). Quatre d'entre eux etaient diabetiques avec des antecedents plus ou moins recents d'otite externe. Tous presentaient des symptomes associant a des degres divers des cephalees et l'atteinte d'un ou plusieurs nerfs crâniens, en particulier, le nerf facial. Le diagnostic a ete etabli a partir des donnees de l'IRM qui montraient l'atteinte de la medullaire osseuse avec disparition de son hypersignal T1 spontane. Le germe en cause etait Pseudomonas aeruginosa. Un patient est decede de complications neurologiques liees a l'extension de l'osteomyelite. Les quatre autres ont presente une evolution clinique favorable, avec persistance d'une atteinte faciale sequellaire dans deux cas. Cette infection etait rattachee dans quatre cas au traitement inadapte de l'otite externe necrotique initiale du fait d'une antibiotherapie inefficace et/ou trop breve. Conclusions Le diagnostic d'osteomyelite de la base du crâne est evoque, chez un patient presentant une otite externe necrotique, devant l'apparition de cephalees et de l'atteinte d'un ou plusieurs nerfs crâniens. Il est etabli grâce a une evaluation IRM comportant des sequences en saturation de graisse. Cette pathologie devrait etre evitee grâce a la prise en charge adaptee des otites externes necrotiques.

International audience; IMPORTANCE: The association between hearing impairment and cognitive decline has been established; however, the effect of cochlear implantation on cognition in profoundly deaf elderly patients is not known. OBJECTIVE: To analyze the relationship between cognitive function and hearing restoration with a cochlear implant in elderly patients. DESIGN, SETTING, AND PARTICIPANTS: Prospective longitudinal study performed in 10 tertiary referral centers between September 1, 2006, and June 30, 2009. The participants included 94 patients aged 65 to 85 years with profound, postlingual hearing loss who were evaluated before, 6 months after, and 12 months after cochlear implantation. INTERVENTIONS: Cochlear implantation and aural rehabilitation program. MAIN OUTCOMES AND MEASURES: Speech perception was measured using disyllabic word recognition tests in quiet and in noise settings. Cognitive function was assessed using a battery of 6 tests evaluating attention, memory, orientation, executive function, mental flexibility, and fluency (Mini-Mental State Examination, 5-word test, clock-drawing test, verbal fluency test, d2 test of attention, and Trail Making test parts A and B). Quality of life and depression were evaluated using the Nijmegen Cochlear Implant Questionnaire and the Geriatric Depression Scale-4. RESULTS: Cochlear implantation led to improvements in speech perception in quiet and in noise (at 6 months: in quiet, 42% score increase [95% CI, 35%-49%; P \textless .001]; in noise, at signal to noise ratio [SNR] +15 dB, 44% [95% CI, 36%-52%, P \textless .001], at SNR +10 dB, 37% [95% CI 30%-44%; P \textless .001], and at SNR +5 dB, 27% [95% CI, 20%-33%; P \textless .001]), quality of life, and Geriatric Depression Scale-4 scores (76% of patients gave responses indicating no depression at 12 months after implantation vs 59% before implantation; P = .02). Before cochlear implantation, 44% of the patients (40 of 91) had abnormal scores on 2 or 3 of 6 cognition tests. One year after implant, 81% of the subgroup (30 of 37) showed improved global cognitive function (no or 1 abnormal test score). Improved mean scores in all cognitive domains were observed as early as 6 months after cochlear implantation. Cognitive performance remained stable in the remaining 19% of the participants (7 of 37). Among patients with the best cognitive performance before implantation (ie, no or 1 abnormal cognitive test score), 24% (12 of 50) displayed a slight decline in cognitive performance. Multivariate analysis to examine the association between cognitive abilities before implantation and the variability in cochlear implant outcomes demonstrated a significant effect only between long-term memory and speech perception in noise at 12 months (SNR +15 dB, P = .01; SNR +10 dB, P \textless .001; and SNR +5 dB, P = .02). CONCLUSIONS AND RELEVANCE: Rehabilitation of hearing communication through cochlear implantation in elderly patients results in improvements in speech perception and cognitive abilities and positively influences their social activity and quality of life. Further research is needed to assess the long-term effect of cochlear implantation on cognitive decline.

Les effets des nuisances sonores sur l’organisme interessent principalement le systeme auditif, mais d’autres atteintes sont possibles. Leur expression est differente d’une personne a l’autre, en raison d’une sensibilite interindividuelle variable. Si les effets d’une exposition unique sont facilement diagnostiques en raison d’une forte expression clinique, il n’en est pas de meme des expositions repetees. En effet, ces atteintes sont volontiers latentes. Leurs consequences a long terme sur la fonction auditive justifient leur depistage precoce. Celui-ci est plus ou moins efficace selon que les nuisances sonores sont rencontrees dans un cadre professionnel ou de loisirs. Quel que soit le type d’atteinte, l’evaluation diagnostique et pronostique justifie un examen ORL specialise ainsi qu’une audiometrie, completes, si necessaire, par d’autres investigations fonctionnelles ou radiologiques. En matiere d’exposition aux nuisances sonores sur le lieu de travail, la legislation a recemment evolue tant en matiere de mesures de protection que de reconnaissance du handicap auditif rattache a cette exposition au cours de l’activite professionnelle. Les modalites therapeutiques reposent essentiellement sur la prevention. Dans certaines circonstances, telles que les traumatismes sonores aigus, un traitement d’urgence est parfois propose. Les developpements les plus recents se font dans la direction de therapeutiques in situ , delivrees le plus pres possible de l’oreille interne atteinte.

Hearing rehabilitation by cochlear implantation is not always possible in case of total ossification after pneumococcal meningitis. We report 3 cases of postmeningitis profound hearing loss with total cochlear ossification in adults who underwent auditory brainstem implantation (Nucleus 22®, Cochlear Inc., Lane Cove, Australia) between 1999 and 2004. The postoperative follow-up period ranged from 1 to 6 years. Eleven to 15 out of 22 electrodes were activated. All patients had significant speech discrimination in the sound-only mode and an enhanced lip-reading performance with the implant. Auditory brainstem implants are an efficient means of auditory rehabilitation and may be considered in selected cases of bilateral profound hearing loss with the impossibility of cochlear implantation.

Objectifs Le but de cette etude etait d’analyser de facon retrospective les performances, les facteurs predictifs et les complications de l’implant cochleaire dans une population de sujets adultes presentant une surdite post-linguale. Methodes Entre 1990 et 2003, 134 patients adultes ont ete implantes, de facon unilaterale chez 129 d’entre eux et bilaterale chez 5 d’entre eux. Le benefice auditif a 6, 12, 24 et 36 mois a ete analyse ainsi que les performances a 12 mois en fonction de l’etiologie, de l’âge, de la lecture labiale et de la strategie de codage. Resultats L’implant cochleaire apporte un benefice auditif important des 6 mois postoperatoire par comparaison aux performances avant l’implantation (p Conclusion L’implant cochleaire apporte un benefice important et rapide, avec une morbidite tres faible, quels que soient l’âge des sujets, l’etiologie et la qualite de la lecture labiale.

Objective To report the first case of spontaneous intratumoral and brainstem hemorrhage in a patient with a vestibular schwannoma. Study design Case report and review of the literature. Setting University-based, tertiary referral center. Patient A 73-year-old woman with a vestibular schwannoma, anti-vitamin K overdose, and arterial hypertension experienced a rapid onset of headache, facial palsy, diplopia, and hoarseness. Interventions The patient was admitted to the intensive care unit and treated with vitamin K and corticoids. Main outcome measures Clinical and radiologic findings. Results At admission, the magnetic resonance imaging and computed tomographic scans showed an intratumoral hemorrhage. The patient entered a coma 24 hours after the onset of the symptoms. At that time, the computed tomographic scan demonstrated a massive intratumoral and brainstem hemorrhage. The patient died 3 days later. Conclusion Intratumoral, subarachnoid, and brainstem hemorrhages can occur in patients with vestibular schwannoma. Oral anticoagulant therapy is a risk factor for tumor-related hemorrhage.

Resume Le diagnostic de surdite etant etabli et sa severite precisee, il est possible de proposer, cas par cas, une rehabilitation auditive qui permettra de retrouver une comprehension satisfaisante. Schematiquement, deux elements interviennent : le siege de l’atteinte sur les voies auditives et son intensite. Les pathologies de l’oreille moyenne (otite chronique, otospongiose…) sont habituellement a l’origine d’atteintes de la chaine des osselets ; ces derniers peuvent etre remplaces au cours d’interventions chirurgicales. Les atteintes de l’oreille interne et des voies auditives necessitent initialement l’utilisation d’une aide auditive (audioprothese). Les modeles numeriques les plus recents permettent une adaptation precise au niveau de surdite. Dans les atteintes plus severes, une prothese peut etre placee chirurgicalement au niveau de la chaine des osselets : il s’agit d’implants d’oreille moyenne. Quand la surdite est severe ou totale, la cochlee etant trop alteree, l’audition peut etre rehabilitee par un implant cochleaire : un faisceau d’electrodes est introduit dans l’oreille interne. Enfin, le passage transcrânien des vibrations sonores est utilise par le biais de protheses a ancrage osseux, dans les atteintes severes de l’oreille moyenne, avec une oreille interne « normale », et dans les surdites severes ou totales unilaterales.

Objectif Le but de ce travail est de rapporter un cas d’hemorragie labyrinthique, qui est une etiologie rare de surdite brusque, chez un patient sous traitement anticoagulant. Materiel et methode Un patient de 73 ans, traite par antivitamine K, a ete adresse dans le service pour une surdite brusque et un syndrome vertigineux. Le bilan clinique a montre une surdite totale gauche et une areflexie homolaterale non compensee. Il existait un surdosage en AVK (INR = 7,5). Resultats L’imagerie par resonance magnetique (IRM) a mis en evidence un hypersignal spontane de la cochlee et du vestibule gauches en sequence T1 sans injection de gadolinium. En sequence T2 FLAIR, il existait un hypersignal franc. Le diagnostic d’hemorragie labyrinthique a ete alors retenu. Les caracteristiques du signal T1 sans et avec injection de Gadolinium, T2 permettent d’eliminer les autres diagnostics : labyrinthite, schwannome ou lipome intra-labyrinthique. L’evolution a ete marquee par une regression des troubles de l’equilibre sans recuperation auditive. Conclusion Le diagnostic d’hemorragie labyrinthique doit etre evoque chez un patient traite par anticoagulant, presentant une surdite brusque associee a des vertiges. L’IRM permet le diagnostic.

A high rate of deterioration in hearing function and the loss of patient compliance during conservative management should be taken into account when considering hearing preservation strategies for patients with vestibular schwannoma (VS). To compare conservative management with surgery for solitary small VS. Among 693 patients followed up for VS between 1991 and 2002, 114 (16%) intracanalicular VSs (stage 1) and 302 (44%) VSs measuring15 mm in the cerebellopontine angle (stage 2) were included in this study. Initially, surgery was performed in 305 (73%) cases (50 stage 1, 255 stage 2) and 111 (27%) were managed conservatively (64 stage 1, 54 stage 2) by means of annual MRI scans and audiometry. Conservative management was chosen in patients aged60 years and in those who refused surgery. In this subgroup, the mean follow-up period was 33 months (range 6-111 months). In the conservative management group, 47% of VSs showed significant growth, 47% were stable and 6% showed regression. Seventeen patients (15%) were operated on secondarily for tumour growth and 1 (1%) was irradiated for tumour growth and because surgery was contraindicated. Deterioration of hearing function byor =1 class was observed in 56% of cases, 34% of patients were initially in hearing class D and only 10% showed stable hearing function. Of the conservative management group, 17% were lost during follow-up. After surgery, grade 1 or 2 facial function was obtained in 86% of cases. Following hearing preservation attempts (n=137), 54% of patients were in hearing classes A-C.