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National audience; Introduction. La presbyacousie correspond à la diminution physiologique de l’audition due à l’avancée en âge et commence bien avant la sixième décennie. Ces recommandations rappellent les principes du diagnostic précoce de la presbyacousie et les moyens de réhabilitation optimale dès l’apparition des premiers symptômes. Matériel et méthodes. Les recommandations sont basées sur une analyse systématique de la littérature réalisée par un groupe multidisciplinaire réunissant des médecins, des audiologistes et des audioprothésistes provenant de toute la France. Elles sont classées en grade A, B, C ou accord professionnel selon un niveau de preuve scientifique décroissant. Résultats. Le diagnostic de presbyacousie est plus difficile en début d’évolution, mais un certain nombre d’outils sont disponibles pour son diagnostic précoce et sa prise en charge en présentiel, voire à distance. Conclusion. En cas de profil clinique évocateur de presbyacousie chez un sujet jeune, surtout s’il y a plusieurs cas familiaux, il est recommandé de proposer une enquête génétique. Il est recommandé de réaliser l’audiométrie vocale dans le bruit en champ libre pour mesurer l’intelligibilité dans un environnement au plus proche de la réalité. Les questionnaires en supplément de l’audiométrie permettraient d’évaluer au mieux le handicap du patient. Il est recommandé de considérer que la réhabilitation auditive par prothèse auditive ou implant cochléaire peut ralentir ou prévenir le déclin cognitif. Une rééducation combinée auditive et cognitive devrait être proposée, quel que soit le temps écoulé depuis l’appareillage. Il est recommandé d’intégrer des programmes accessibles par smartphones, tablettes ou Internet intégrant différents domaines d’entraînement en complément des séances en présentiel.

National audience; IntroductionLa presbyacousie comme les maladies neurodégénératives apparaissent insidieusement avec l’âge. Enjeu majeur de santé publique, elles sont néanmoins sous diagnostiquées car la présence de l’une entrave l’objectivation de l’autre.ObjectifsIntégrer lors du bilan cognitif en hôpital de jour en centre mémoire un dépistage des troubles de la compréhension de la parole dans le bruit.MéthodesCent vingt-deux participants, dont 70 femmes, d’âge moyen 72,9 (60–87 ans) ont réalisé trois épreuves : questionnaire Hearing Handicap Inventory for Elderly-Screening (HHIES) mesure la gêne auditive ; test de compréhension de la parole dans le bruit (dispositif ambulatoire – Vocal Rapide dans le Bruit, VRB Leclercq et al., 2018) et échelle cognitive de Mattis. Des analyses corrélationnelles entre l’âge, le résultat du VRB, le score HHIE-S et à la Mattis ont été menées.RésultatsLes corrélations objectivent une association entre les capacités de compréhension de la parole dans le bruit et l’âge (r = 0,324, p < 0,001), la gêne auditive (r = 0,249, p = 0,006) et les troubles cognitifs globaux (r = −0,502, p < 0,001).DiscussionCes résultats confortent le lien établi entre l’âge et les capacités de compréhension de la parole dans le bruit. Ils objectivent un lien entre la plainte auditive et les capacités de compréhension de la parole dans le bruit et révèlent que ces capacités sont amoindries chez les personnes ayant des troubles cognitifs objectivés à l’échelle de la Mattis.ConclusionLe dépistage précoce d’un déficit auditif lors d’évaluations cognitives est indispensable puisque la surdité est le principal facteur modifiable de la démence.

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).

Dexamethasone-loaded silicone matrices offer an interesting potential as innovative drug delivery systems, e.g. for the treatment of inner ear diseases or for pacemakers. Generally, very long drug release periods are targeted: several years/decades. This renders the development and optimization of novel drug products cumbersome: experimental feedback on the impact of the device design is obtained very slowly. A better understanding of the underlying mass transport mechanisms can help facilitating research in this field. A variety of silicone films were prepared in this study, loaded with amorphous or crystalline dexamethasone. Different polymorphic drug forms were investigated, the film thickness was altered and the drug optionally partially/completely exchanged by much more water-soluble dexamethasone ‘phosphate’. Drug release studies in artificial perilymph, scanning electron microscopy, optical microscopy, differential scanning calorimetry, X-ray diffraction and Raman imaging were used to elucidate the physical states of the drugs and polymer, and of the systems’ structure as well as dynamic changes thereof upon exposure to the release medium. Dexamethasone particles were initially homogeneously distributed throughout the systems. The hydrophobicity of the matrix former very much limits the amounts of water penetrating into the system, resulting in only partial drug dissolution. The mobile drug molecules diffuse out into the surrounding environment, due to concentration gradients. Interestingly, Raman imaging revealed that even very thin silicone layers (

The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Meniere’s disease (MD). A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). MRI exploration was carried out on a 3-T Achieva® TX scanner. We analyzed the presence of the RWS, defined as a nodular FLAIR high signal in the round window (RW) and the presence of associated saccular hydrops. When this RWS was present, a temporal bone CT scan was performed and the RW was analyzed. Of the 164 patients with definite MD (85 patients) or probable MD (79 patients), we found the RWS in 18 (11%), and 17/18 were classified into the group of probable MD. All these 18 patients showed other MR sequences considered as normal, including heavily weighted T2 imaging. Among these 18 patients, the temporal bone CT examination presented a filling of the RW in 13 patients (72%) and no filling of the RW in 5 patients (28%). Seven patients were surgically managed confirming in vivo the PLF diagnosis. The RWS was associated with the presence of a saccular hydrops in 4 cases. Delayed postcontrast 3D-FLAIR may reveal perilymphatic fistulae in patients with probable Meniere’s disease using the round window sign. • MRI with delayed acquisition can detect perilymphatic fistulae with perfect sensitivity, based on the presence of the round window sign. • This visual sign is only visible on a 3D-FLAIR sequence. • 3D-FLAIR sequence with delayed acquisition is more sensitive than temporal bone CT scan examination in detecting PLF.

Objective: This study investigated the audiological and tinnitus outcomes of cochlear implantation (CI) in adults with single-sided deafness (SSD) and tinnitus. Study Design: Multicentered prospective, non-randomized intervention study. Setting: Six French CI centers. Patients: Twenty-six patients with SSD and incapacitating tinnitus (Tinnitus Handicap Inventory [THI] >58) underwent cochlear implantation. Interventions: First, CIs delivered only masking white noise stimulation for 1 month and then standard CI stimulation. Main Outcome Measures: Before and after CI surgery, patients completed the THI, Tinnitus Reaction Questionnaire (TRQ), Subjective Tinnitus Severity Scale (STSS), and two visual analogue scales quantifying tinnitus loudness and annoyance. Speech perception in spatialized noise was tested at 13 months. Results: The first month of white noise stimulation triggered a significant improvement in THI scores (72 ± 9 to 55 ± 20, p

Resume Objectif L’objectif etait d’evaluer les performances de localisation sonore spatiale azimutale dans differentes conditions pour l’optimisation d’un protocole de localisation sonore spatiale de routine. Materiel et methode Deux groupes de sujets sains ont ete testes de facon identique. Le premier groupe devait garder la tete fixe alors que le deuxieme groupe etait autorise a tourner la tete. La localisation sonore spatiale etait testee oreilles nues, puis avec un bouchon d’oreille droit (asymetrie auditive aigue) pour chacun des stimuli sonores suivants : bande etroite centree sur 250 Hz pulse, bandes etroites centrees sur 2000 Hz, 4000 Hz et 8000 Hz continus, wobule 4000 Hz continu, bruit blanc pulse et mot (« lac »). L’erreur quadratique moyenne ou RMS Error (Root Mean Square Error) permettait de calculer la performance de localisation. Resultats Tete fixe, la localisation sonore etait significativement perturbee apres bouchon d’oreille pour l’ensemble des stimuli (p Conclusion Le test realise chez un sujet tete fixe est plus sensible. L’utilisation d’un bruit en bande etroite centre sur 4000 Hz et continu explore la difference d’intensite interaurale. Un bruit en bande etroite centre sur 250 Hz et pulse permet d’explorer la difference de temps interaurale. Le test tete mobile, plus proche des conditions de vie reelle, est optimise avec un bruit en bande etroite centre sur 2000 Hz et continu.

Cochlear implants containing iridium platinum electrodes are used to transmit electrical signals into the inner ear of patients suffering from severe or profound deafness without valuable benefit from conventional hearing aids. However, their placement is invasive and can cause trauma as well as local inflammation, harming remaining hair cells or other inner ear cells. As foreign bodies, the implants also induce fibrosis, resulting in a less efficient conduction of the electrical signals and, thus, potentially decreased system performance. To overcome these obstacles, dexamethasone has recently been embedded in this type of implants: into the silicone matrices separating the metal electrodes (to avoid short circuits). It has been shown that the resulting drug release can be controlled over several years. Importantly, the dexamethasone does not only act against the immediate consequences of trauma, inflammation and fibrosis, it can also be expected to be beneficial for remaining hair cells in the long term. However, the reported amounts of drug released at “early” time points (during the first days/weeks) are relatively low and the in vivo efficacy in animal models was reported to be non-optimal. The aim of this study was to increase the initial “burst release” from the implants, adding a freely water-soluble salt of a phosphate ester of dexamethasone. The idea was to facilitate water penetration into the highly hydrophobic system and, thus, to promote drug dissolution and diffusion. This approach was efficient: Adding up to 10% dexamethasone sodium phosphate to the silicone matrices substantially increased the resulting drug release rate at early time points. This can be expected to improve drug action and implant functionality. But at elevated dexamethasone sodium phosphate loadings device swelling became important. Since the cochlea is a tiny and sensitive organ, a potential increase in implant dimensions over time must be limited. Hence, a balance has to be found between drug release and implant swelling., Graphical abstract Unlabelled Image

Background: A prospective longitudinal multicentre study was conducted to assess the one-year postsurgical hearing preservation profile of the EVOTM electrode array. Methods: Fifteen adults presenting indications of electro-acoustic stimulation (pure-tone audiometry (PTA) thresholds ≤70 dB below 750 Hz) were implanted with the EVO™ electrode array. Hearing thresholds were collected at five time-points from CI activation to twelve months (12M) after activation. Hearing thresholds and hearing preservation profiles (HEARRING group classification) were assessed. Results: All subjects had measurable hearing thresholds at follow-up. No case of complete loss of hearing or minimal hearing preservation was reported at any time point. At activation (Nact = 15), five participants had complete hearing preservation, and ten participants had partial hearing preservation. At the 12M time point (N12m = 6), three participants had complete hearing preservation, and three participants had partial hearing preservation. Mean hearing loss at activation was 11 dB for full range PTA and 25 dB for PTAs low-frequency (125–500 Hz). Conclusions: This study provides the first longitudinal follow-up on associated hearing profiles to the EVO™ electrode array, which are comparable to the literature. However, other studies on larger populations should be performed.

Cochlear implant is the method of choice for the rehabilitation of severe to profound sensorineural hearing loss. The study of the tissue response to cochlear implantation and the prevention of post-cochlear-implant damages are areas of interest in hearing protection research. The objective was to assess the efficacy of dexamethasone-eluting electrode array on endo canal fibrosis formation by three-dimensional immunofluorescence analysis in implanted Mongolian gerbil cochlea. Two trials were conducted after surgery using Mongolian gerbil implanted with dexamethasone-eluting or non-eluting intracochlear electrode arrays. The animals were then euthanised 10 weeks after implantation. The cochleae were prepared (electrode array in place) according to a 29-day protocol with immunofluorescent labelling and tissue clearing. The acquisition was carried out using light-sheet microscopy. Imaris software was then used for three-dimensional analysis of the cochleae and quantification of the fibrotic volume. The analysis of 12 cochleae showed a significantly different mean volume of fibrosis (2.16 × 108 μm3 ± 0.15 in the dexamethasone eluting group versus 3.17 × 108 μm3 ± 0.54 in the non-eluting group) (p = 0.004). The cochlear implant used as a corticosteroid delivery system appears to be an encouraging device for the protection of the inner ear against fibrosis induced by implantation. Three-dimensional analysis of the cochlea by light-sheet microscopy was suitable for studying post-implantation tissue damage.

Objectives The present study aimed to determine normal SNR values per age group for the 50% speech reception threshold in noise (SNR Loss) on the VRB (Vocale Rapide dans le Bruit: rapid speech in noise) test. Material and Methods Two hundred patients underwent pure-tone threshold and VRB speech-in-noise audiometry. Six ages groups were distinguished: 20–30, 30–40, 40–50, 50–60, 60–70 and > 70 years. All subjects had normal hearing for age according to ISO 7029. SNR Loss was measured according to age group. Results Mean SNR Loss ranged from −0.37 dB in the youngest age group (20–30 years) to +6.84 dB in the oldest (> 70 years). Range and interquartile range increased with age: 3.66 and 1.49 dB respectively for 20–30 year-olds; 6 and 3.5 dB for > 70 year-olds. Linear regression between SNR Loss and age showed a coefficient R2 of 0.83. Conclusion The present study reports SNR Loss values per age group in normal-hearing subjects (ISO 7029), confirming that SNR Loss increases with age. Scatter also increased with age, suggesting that other age-related factors combine with inner-ear aging to impair hearing in noise.

La Société française de médecine d’urgence (SFMU) a recommandé la création d’une échelle spécifique, unique au niveau national, pour le triage des patients à l’accueil d’une structure d’urgences, prenant en compte les spécificités de l’adulte et de l’enfant. La commission de l’évaluation et de la qualité de la SFMU a créé, à l’instar des échelles de tri internationales, une échelle de tri avec cinq niveaux de priorité croissante (tris 5 à 1, du moins urgent au plus urgent) auxquels correspondent des motifs de recours aux soins de complexité/sévérité croissante. Le tri 3 a été subdivisé en deux groupes pour distinguer (et prioriser) les patients qui ont au moins une comorbidité en rapport avec le motif de recours aux soins ou qui sont adressés par un médecin (3A) des autres patients (3B). L’échelle de tri FRENCH (FRench Emergency Nurses Classification in Hospital) a donc six niveaux de priorité. À chaque niveau de tri correspondent des motifs de recours aux soins fréquents en médecine d’urgence, des modulateurs de tri, une répartition rationnelle des circuits patients et un délai maximum d’attente avant prise en charge médicale, après évaluation par l’infirmier(ière) d’accueil. Une première évaluation de la FRENCH a montré qu’elle répondait aux objectifs du triage en facilitant le repérage de l’urgence complexe/sévère de façon fiable et reproductible. De nouvelles évaluations sont nécessaires dans d’autres structures d’urgences pour confirmer sa performance et favoriser son évolution.

OBJECTIVE To determine whether cervical vestibular evoked myogenic potentials (cVEMPs) are predictive of hearing preservation in patients undergoing vestibular schwannoma removal through middle fossa craniotomy approach. STUDY DESIGN Retrospective case study. SETTING Tertiary referral center. PATIENTS Eighteen patients who underwent a middle fossa craniotomy for vestibular schwannoma (stage I or II of Koos classification) with attempted hearing preservation from January 2008 to February 2016 were retrospectively reviewed. INTERVENTION Pre-surgical cVEMPs test, videonystagmography (caloric test), and magnetic resonance imaging (MRI) as well as a pre- and post-surgical audiometry test. MAIN OUTCOME MEASURES cVEMPs parameters including amplitude asymmetry ratio (AR), P13, and N23 latencies and peak-to-peak amplitude between P13 and N23 waves were calculated. Hearing data were classified according to the AAO-HNS hearing classes. The nerve of origin of the tumor was specified during surgery and the largest tumor diameter was measure on MRI axial plane on T2-CISS weighed images. RESULTS Preoperative amplitude asymmetry ratio was lower (n = 15, Mann-Whitney U test, p

The Oticon Medical Neuro cochlear implant system includes the modes Opti Omni and Speech Omni, the latter providing beamforming (i.e., directional selectivity) in the high frequencies. Two studies compared sentence identification scores of adult cochlear implant users with Opti Omni and Speech Omni. In Study 1, a double-blind longitudinal crossover study, 12 new users trialed Opti Omni or Speech Omni (random allocation) for three months, and their sentence identification in quiet and noise (+10 dB signal-to-noise ratio) with the trialed mode were measured. The same procedure was repeated for the second mode. In Study 2, a single-blind study, 11 experienced users performed a speech identification task in quiet and at relative signal-to-noise ratios ranging from -3 to +18 dB with Opti Omni and Speech Omni. The Study 1 scores in quiet and in noise were significantly better with Speech Omni than with Opti Omni. Study 2 scores were significantly better with Speech Omni than with Opti Omni at +6 and +9 dB signal-to-noise ratios. Beamforming in the high frequencies, as implemented in Speech Omni, leads to improved speech identification in medium levels of background noise, where cochlear implant users spend most of their day. Funding Agencies|Oticon Medical; Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior (Coordination for the Improvement of Higher Education Personnel) from the Ministry of Education of Brazil

Introduction: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. Methods: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. Results: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the “CI” arm versus “observation” arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). Conclusion: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.

International audience; Aim: The incidence of type 1 diabetes is increasing, and more people are going to live many years with the disease. Quality of life might become the most challenging long-term complication. The JUBILE study describes the quality of life of people living with type 1 diabetes for more than 40 years. Methods: Patients were recruited from 35 French regional or university hospitals: patients and physicians completed questionnaires, validated by the Delphi method. From 1200 questionnaires circulated, 808 patients and their physicians returned questionnaires. Results: The duration of type 1 diabetes was 49 ± 6 years (mean±SD), age at diagnosis 15 ± 10 years, HbA1c 7.4 ± 0.9% [58 ± 10 mmol/mol] and 52% were men. Macrovascular disease was present in 32%, 46% had no or only mild non proliferative retinopathy. Insulin pumps were used by 25% and insulin pen/syringe users injected 3.9 ± 2.1 times per day. Blood glucose was self monitored at least five times per day by 67% of patients. Men had 1.8 ± 1.2 children, women 1.4 ± 1.0. More than half (55%) of this population was working, 38% had a university degree. Patients still had a busy life, going out (59%), eating out (82%), playing sports (38%) and travelling (66%). No differences appeared based on age, duration of diabetes, demography or social features. Conclusions: Living a long and pleasant life is possible with type 1 diabetes. Diabetes does not prevent people from having children, working at highly qualified jobs, travelling abroad: a message of hope that is comforting for patients, their family, relatives and the medical teams.

Purpose For cochlear implant users, the ability to use the telephone is often seen as an important landmark during rehabilitation and an indicator of cochlear implant benefit. The goal of this study was to develop a short questionnaire exploring the ability to use the telephone in cochlear implant users, named Telislife, and test it in a group of experienced users. Method This prospective multicenter study was based on the completion of self-administrated questionnaires. The Telislife includes 20 items using a 5-point Likert scale for answers. Speech recognition scores were obtained with monosyllabic word lists at 70 dB HL. Quality of life was evaluated with the Nijmegen Cochlear Implant Questionnaire. This study included 55 adult patients wearing a cochlear implant for over 1 year. Results The Telislife questionnaire showed excellent reliability (Cronbach's α = .91). A significant correlation was found between Telislife scores and Nijmegen Cochlear Implant Questionnaire scores ( r = .69, p < .001) and speech recognition scores ( r = .35, p = .007). Conclusion Given significant correlations between Telislife scores and both speech recognition and quality of life and given its short form, the Telislife questionnaire appears to be a reliable tool to evaluate cochlear implant outcomes in clinical practice. Supplemental Material https://doi.org/10.23641/asha.13322873

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.

Objectives: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. Design: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. Setting: Seven tertiary university hospitals. Participants: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. Main outcome measures: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). Results: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. Conclusions: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.

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.

International audience; Importance Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine.Objective To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL.Design, Setting, and Participants This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019.Main Outcomes and Measures A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting.Results In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement).Conclusions and Relevance These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

Objective: This study evaluated the outcomes of the Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor. Design: Neuro One users were upgraded to Neuro 2. Monosyllabic word identification was evaluated in adults with Neuro One after ≥5 months, with Neuro 2 at upgrade, and with Neuro 2 after 3 months. Self-reported listening ability, satisfaction, and usability were measured in adults and children. Study sample: Participants were 44 adults and 26 children. Results: Speech identification scores in quiet and noise were 58% and 45% with Neuro One and 67% and 55% with Neuro 2 after 3 months, respectively. Hearing impairment duration and number of active electrodes significantly predicted speech identification in noise with Neuro 2. Significantly higher questionnaire ratings were obtained for Neuro 2 than Neuro One regarding listening ability in complex listening situations, comfort and music, as well as nine aspects of satisfaction and usability. Conclusion: This study demonstrates the clinical superiority of the Neuro 2 sound processor over Neuro One in terms of speech identification in quiet and in noise and reported patient benefit and satisfaction. Given the study design, sources of improvement may include factors unrelated to the sound processor itself.

Introduction Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center. Materials and methods All recent articles concerning tumors other than vestibular schwannoma in NF2 disease were reviewed, using PubMed databases. Results Intracranial meningiomas occur in 50% of NF2 patients, and are often multiple. Surgery remains the main treatment and should be performed in cases of growing tumors. The role of antiangiogenic therapy is currently under evaluation and the role of radiosurgery still remains to be defined in NF2 disease. Spinal tumors occur in about half of NF2 patients. Surgery should be discussed when radiological tumor progression is demonstrated, even if spinal tumors are asymptomatic, in order to preserve neurological function and good quality of life. As regards lower cranial nerve schwannomas, radiosurgery appears to be a more appropriate treatment for growing tumor with a small volume in order to avoid post-operative complications, especially swallowing disorders. Facial nerve schwannomas may appear, on MRI, like vestibular schwannomas. The diagnosis should be suspected when the facial palsy is an early symptom during cerebello-pontine tumor progression. Trigeminal schwannomas are frequent in NF2 disease and fortunately they are often asymptomatic. Among major neurofibromatosis types, peripheral nerve sheath schwannomas are only present in patients with NF2 disease and schwannomatosis. Surgical resection is required when the cutaneous schwannomas is painful or when tumor progression is observed and causes symptoms. Conclusion Tumors other than vestibular schwannoma are also associated with a poor prognosis in NF2 patients. Surgery remains the main treatment in most cases. Each treatment decision in NF2 disease requires a complete evaluation of all cranial and spinal locations of the disease in order to establish surgical priorities and strategies.

International audience; Objective: To perform a long-term evaluation of the localisation capabilities in the horizontal plane of single-sided deaf patients fitted with a BAHA device.Design: Single-centre retrospective study.Participants: Twenty-one adults with single-sided deafness (SSD) with normal hearing in the contralateral ear (pure tone average 90%) rehabilitated with a Cochlear BAHA device from 2003 to 2012 on the deaf side over a median follow-up of 8 years.Outcome measures: The task used in this paper is a sound localisation identification task with a set-up of seven loudspeakers on a semi-circular array at 30-degree intervals performed at three periods: before BAHA, initially and at last follow-up. Our main criterion of judgement was the root-mean-square (RMS) localisation error. In addition, the Bern Benefit in Single-Sided Deafness Questionnaire (BBSS) was administered.Results: The mean RMS localisation error was initially estimated at 64° without any rehabilitation (for a chance level RMS estimated at 81°). Initially, with the BAHA device, the RMS localisation error dropped to 51°. At the last follow-up evaluation, a significant decrease at 23° was noted. Concerning the Bern Questionnaire, 19% of the patients (n = 4) did not report any change (score of 0), 33% (n = 7) are satisfied (score of +1 or +2) and 48% (n = 10) are very satisfied with the BAHA device (score better than +3).Conclusion: Improvement of sound localisation in the horizontal plane for some SSD patients is likely related to altered processing of monaural spectral cues. The time needed to learn to use the azimuth-dependent spectral cues takes time. Long-term follow-up should be considered for studies investigating sound localisation performance.

Objective We aimed to evaluate the outcome of patients who underwent salvage microsurgery for vestibular schwannoma (VS) that failed primary Gammaknife radiosurgery (GKS). Patients and Methods Among the 1098 patients who received GKS for the treatment of VS in our center between January 2004 and December 2012, the follow-up was organized in our institution for 290 patients who lived in our recruitment area. Tumor progression was noted in 23 patients. A salvage microsurgical resection was performed in 11 patients, who were included in our study. Grading of facial function was done according to the House & Brackman scale. Results The mean age at diagnosis was 50.2 years (19–68 years) and the mean follow-up was 9.4 years (4–13 years). The mean dose was 11.8 Gy (11–12 Gy) and the mean volume was 922 mm3 (208–2500 mm3). The mean period between GKS and diagnosis of tumor progression was 32 months (18–72 months). Concerning salvage microsurgery, complete resection was obtained in 8 patients. Small residual tumor on the facial nerve was deliberately left in 3 patients and no tumor progression was noted with a mean follow-up of 26 months. At last follow-up, facial nerve function was grade 1 in 4 patients, grade 2 in 3 patients, grade 3 in 1 patient and grade 4 in 3 patients. Conclusion Salvage surgery of recurrent vestibular schwannoma after failed initial GKS remains a good treatment. However, facial nerve preservation is more challenging in this case and small tumor remnant could be sometimes deliberately left.

Resume La decouverte de l'insuline il y a juste 100 ans a transforme une maladie aigue rapidement mortelle en une pathologie de longue duree, vehiculant son fardeau de complications chroniques. Detection, prevention et prise en charge de ces dernieres se sont heureusement considerablement ameliorees. La cohorte nationale francaise Jubile de 808 diabetiques de type 1 de 50 annees de duree ou plus a ete analysee a l'aide de questionnaires portant sur les principales caracteristiques clinico-biologiques, mais aussi sur le style et la qualite de vie. Les patients, imparfaitement controles avec une hemoglobine glyquee (HbA1c) a 7,4 ± 0,9 %, presentent neanmoins peu de complications microangiopathiques, presque la moitie sont indemnes d'atteinte retinienne significative et 32 % seulement ont une atteinte macrovasculaire a un âge moyen de 64 ans. Ils ont pratique une autosurveillance glycemique intense, plus de 4 fois par jour pour les 4/5. Un peu plus souvent citadins, ils ne fument pas, vivent le plus souvent en couple et ont presque autant d'enfants que la moyenne nationale. Plus du 1/3 ont fait des etudes superieures, ils ne partent pas en retraite anticipee. Quant a la vie socioculturelle, elle est intense : sorties, voyages, distractions, sports. Seule l'etude Jubile a regarde la qualite de vie des diabetiques de type 1 de duree extreme. A l'encontre du discours encore trop repandu de vie forcement ecourtee et gâchee, elle apporte un message d'espoir reconfortant pour les patients, parents et proches, comme pour les equipes de soignants impliquees.

International audience; Aim: To evaluate azimuthal sound-source localization performance under different conditions, with a view to optimizing a routine sound localization protocol.Material and method: Two groups of healthy, normal-hearing subjects were tested identically, except that one had to keep their head still while the other was allowed to turn it. Sound localization was tested without and then with a right ear plug (acute auditory asymmetry) for each of the following sound stimuli: pulsed narrow-band centered on 250Hz, continuous narrowband centered on 2000Hz, 4000Hz and 8000Hz, continuous 4000Hz warble, pulsed white noise, and word ("lac" (lake)). Root mean square error was used to calculate sound-source localization accuracy.Results: With fixed head, localization was significantly disturbed by the earplug for all stimuli (P

International audience; No abstract available

Objecive The study aims to evaluate the potential impact of the revision of the thesaurus used by ED physicians to code medical diagnoses, on the syndromic indicators used daily to achieve the detection objective of the French syndromic surveillance system. Introduction As part of the French syndromic surveillance system SurSaUD®, the French Public Health Agency (Sante publique France) collects daily data from the emergency department (ED) network OSCOUR® [1]. The system aims to timely identify, follow and assess the health impact of unusual or seasonal events on emergency medical activity. Individual ED data contain demographic (age, gender, residence zip code), administrative (dates of attendances and discharge, ED, etc.) and medical information (chief complaint, main and associated medical diagnoses, severity). Medical diagnoses are encoded using the ICD10 classification. Then syndromic groups are built based on these ICD10 codes for ensuring syndromic surveillance in routine. Even if ICD10 is recommended on the national guidelines for coding ED attendances, this thesaurus offers a too large variety of codes. Particularly, it includes lots of diseases that may never be observed or confirmed in ED. This variety let selection of the appropriate codes difficult for physicians in a reactive use and could discourage them to code diagnoses. In order to encourage appropriate and reactive coding practice, we decided in 2017 to produce a new diagnoses thesaurus with a limited list of ICD10 codes. Then a committee of medical and epidemiological experts was created by the Federation of regional emergency observatories (FedORU), to propose an operational thesaurus that includes relevant codes for both ED in a daily routine practice and syndromic surveillance. Methods The committee has met 10 times since 2017. Since it would have been hard to work on the complete ICD10 list, the work was based on a more limited thesaurus already used by part of French ED. Only codes, which were pertinent regarding ED activity and interest for public health alert, have been considered. The main principles that have guided the selection were to 1) keep codes related to diagnoses that physicians are able to diagnose on a clinical basis or with rapid diagnostic tests, 2) remove diagnoses providing redundant information regarding other variables (such as circumstantial information) and 3) ensure that a substitution code was kept when a removed code was frequently used or was of interest for syndromic surveillance. Among the 86 syndromic groups defined on the basis of a list of ICD10 codes selected in the complete thesaurus, 34 are daily analyzed by Sante publique France for outbreak detection and early assessment of public health events. Those 34 syndromic groups have been recalculated by considering the revised thesaurus on a three-year period (from 2015 to 2017) at national level. In order to measure the potential impact of the revised thesaurus on the syndromic groups, we have considered three evaluation measures: 1. the proportion of ICD10 codes deleted (removal rate) from the initial definition of each syndromic group, due to the limitation of the thesaurus (calculated for the 86 syndromic groups); 2. the mean difference in the daily number of attendances between the initial and the new versions of each syndromic group (calculated for the 34 syndromic groups); 3. the linear correlation coefficient between the daily numbers of attendances of the initial and the new version of each syndromic group, in order to assess if the daily fluctuations of the new syndromic group are similar to those of the initial syndromic group (calculated for the 34 syndromic groups). Results Among the 86 syndromic groups, 75 (85%) have been impacted by the revised thesaurus, which implied codes removal. Among those 75 syndromic groups, the number of ICD10 codes included in their definition has been reduced by 71% on average. This removal rate varied between 17% and 100%. Syndromic groups including initially more than 100 codes have been the most concerned by a limitation of the number of ICD10 codes. Among the 34 syndromic groups daily analyzed for outbreak detection, 32 have been impacted by code removal with a mean removal rate of 68% (0%-97%). On average, 77% of daily attendances have been retained by the new version of syndromic groups, varying from 15% to 100%. Only 3 syndromic groups have kept less than 60% of attendances: Decrease of well-being (36%), Conjunctivitis (32%) and Hypothermia (15%). On average, the correlation coefficient has been of 0.96, varying from 0.57 to 1. The lowest values have been observed for the same three syndromic groups listed above: Decrease of well-being (0.57), Conjunctivitis (0.91) and Hypothermia (0.59). 18 among the 34 syndromic groups had a correlation coefficient higher than 0.99. Conclusions The study showed that most of the syndromic groups were impacted by the revised thesaurus, which resulted in a removal of about two thirds of the ICD10 codes usually considered in daily surveillance. However, more than three quarters of attendances were still retained in the new syndromic groups. This new thesaurus was conceived to rationalize the number of diagnoses codes but a substitution code was systematically proposed to replace removed codes. Those results highlighted that a large number of codes included in the complete ICD10 thesaurus were rarely used and that the most frequent codes were kept in the revised thesaurus version. However, this study showed that a few syndromic groups were strongly impacted by the revised thesaurus and can suffer of reduced performances to detect unusual variations. Based on those results, a second round of exploration of specific parts of the complete ICD10 thesaurus will be necessary to adapt either syndromic groups or the revised thesaurus. Even if the number of attendances may be reduced due to the removal of ICD10 codes, temporal variations remain similar for the majority of syndromic groups. Syndromic surveillance system does not aim to provide exhaustive quantification of attendances for a pathology, but aims to be able to detect expected or unusual public health variations. These evaluation results correspond to the worst-case scenario assuming that ED physicians will not modify their encoding habits by using the substitution codes but keep using their current thesaurus. However, we expect that this new and simplified version will facilitate diagnosis encoding task and lead toward a better diagnosis encoding rate. Once this new thesaurus will be widely used, we can expect a substantial improvement of the quality of ED medical data and then of syndromic surveillance results. Finally, this study enhances the importance that both data providers and epidemiologists in charge of syndromic surveillance work closely, in order to improve system in shared objectives. References [1] Fouillet A, Bousquet V, Pontais I, Gallay A, Caserio-Schonemann C. The French emergency department OSCOUR network: evaluation after a 10-year existence. Online J Public Health Inform. 2015; 7(1): e74.

A new type of advanced drug delivery systems is proposed: Miniaturized implants, which can be placed into tiny holes drilled into (or close to) the oval window. They consist of two parts: 1) A cylinder, which is inserted into the hole crossing the oval window. The cylinder (being longer than the depth of the hole) is partly located within the inner ear and surrounded by perilymph. This provides direct access to the target site, and at the same time assures implant fixation. 2) A cuboid, which is located in the middle ear, serving as a drug reservoir. One side of the cuboid is in direct contact with the oval window. Drug release into the cochlea occurs by diffusion through the cylindrical part of the Ear Cubes and by diffusion from the cuboid into and through the oval window. High precision molds were used to prepare two differently sized Ear Cubes by injection molding. The miniaturized implants were based on silicone and loaded with different amounts of dexamethasone (10 to 30 % w/w). The systems were thoroughly characterized before and upon exposure to artificial perilymph at 37°C. Importantly, drug release can effectively be controlled and sustained during long time periods (up to several years). Furthermore, the implants did not swell or erode to a noteworthy extent during the observation period. Drug diffusion through the polymeric matrix, together with limited dexamethasone solubility effects, seem to control the resulting drug release kinetics, which can roughly be estimated using mathematical equations derived from Fick's second law. Importantly, the proposed Ear Cubes are likely to provide much more reliable local long term drug delivery to the inner ear compared to liquid or semi-solid dosage forms administered into the middle ear, due to a more secured fixation. Furthermore, they require less invasive surgeries and can accommodate higher drug amounts compared to intracochlear implants. Thus, they offer the potential to open up new horizons for innovative therapeutic strategies to treat inner ear diseases and disorders.

Objectives/Hypothesis To determine the long-term growth rate of vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2) patients based on volumetric measurements. Study Design Retrospective review. Methods We retrospectively reviewed magnetic resonance imaging (MRI) scans acquired from patients with NF2 from 1999 to 2013. Patients with an annual radiologic follow-up over at least 3 consecutive years were included. Volumetric VS growth was prospectively measured using a three-dimensional imaging workstation and through manual contouring of the lesion. Time to tumor progression was assessed according to the Response Evaluation in Neurofibromatosis and Schwannomatosis Tumor Measurement Group. Results Eighteen patients presenting with a total of 26 VSs were included. The mean age at diagnosis was 26.1 years (range, 7 to 49 years). One hundred five MRI scans were analyzed during a median radiological follow-up of 5.6 years (range, 3 to 12 years). The annual volume and diameter growth rates were respectively 218.3 mm3/yr (range, −10 to 1,250 mm3/yr) and 0.9 mm/yr (range, −0.5 to 4.5 mm/yr). Time to tumor progression was 3 years (median survival). There was a weak correlation between volumetric and linear measurements (P < .0001, linear regression, n = 26, r2 = 0.58). Among the 26 VSs, 76.9% (20/26) showed progression (VS growth more than 20%), 19.2% were stable, and 3.9% (1/26) exhibited shrinkage (spontaneous regression of more than 20% of the initial volume). Conclusions This study helps to assess the long-term growth profile of VS in a population of NF2 patients with untreated VS. These data could help to better acknowledge VS natural growth history. Level of Evidence 4 Laryngoscope, 2016

Understanding speech in noise is a major challenge for most hearing-impaired subjects, with or without hearing aids. To overcome the weaknesses of French-language speech-in-noise tests, we developed a new instrument, with a balanced mix of difficulty of the speech material.The speech material comprised 127 sentences taken from the "Marginal Benefit from Acoustic Amplification" (MBAA) corpus, each including 3 keywords. The noise was created using the "onde vocale globale" (global vocal wave: OVG), described by Dodelé. The 127 speech/noise pairs were recorded individually after root-mean-square equalization. The first experiment, on 10 normal-hearing adults, determined the signal-to-noise ratio (SNR) associated with 50% correct keyword identification in each sentence (SNR-50), using an ascending method with noise level set at 73dB SPL. Relative levels between sentences and noise were then adjusted sentence by sentence to achieve an SNR-50 of 0dB. The second experiment, with 12 normal-hearing adults, validated the equalization of sentence difficulty.Mean SNR-50 was -6.64dB (σ=1.47). Mean adjusted SNR-50 was 0.08dB (σ=0.55). Mean psychometric curve slope was 19.3%/dB, with low standard deviations, testifying to the sensitivity of the speech material.The VRB (vocale rapide dans le bruit: rapid speech in noise) test is based on sentences from the MBAA corpus with background noise based on the OVG at different signal-to-noise ratios. The test is feasible and able to detect slight variations in speech-in-noise performance between subjects.

A new type of miniaturized implants for local controlled drug delivery to the inner ear is proposed: Hybrid Ear Cubes. They are composed of two main parts: (i) a cylinder, which is placed into a tiny hole (0.4 mm) drilled into (or close to) the oval (or round) window, and (ii) a cuboid, which is placed into the middle ear. The drug is released at a pre-programmed rate into the perilymph: (i) via the cylindrical part of the implant, which is in direct contact with this liquid, and (ii) via diffusion from the cuboid through the oval/round window. Importantly, the cylindrical part assures a reliable fixation of the drug delivery system at the site of administration. Furthermore, the cuboid provides a relatively "large" drug reservoir, without expulsing perilymph from the cochlea. The required surgery is minimized compared to the placement of an intracochlear implant. In contrast to previously proposed Ear Cubes, which are mono-block systems, Hybrid Ear Cubes consist of two halves, which can: (i) be loaded with different drugs, (ii) be loaded with the same drug at different concentrations, and/or (iii) be based on two different matrix formers. This offers a substantially increased formulation flexibility. Different types of silicone-based Hybrid Ear Cubes were prepared, loaded with 10% dexamethasone in one half and 0-60% dexamethasone in the other half. Importantly, tiny drug crystals were homogeneously distributed throughout the respective implant halves. The observed drug release rates were very low (e.g.,0.5% after 2 months), which can be attributed to the type of drug and silicone as well as to the very small surface area exposed to the release medium. Importantly, no noteworthy implant swelling was observed.

Pendred syndrome is an autosomal recessive familial disorder, defined as a sensorineural deafness coinciding with a goiter related to an iodine organification defect. It constitutes 4 to 10 % of all causes of familial congenital hearing loss. It is now well established that this condition was caused by mutations in the SLC26A4 gene which codes for pendrin, a protein involved in the transport of anions (I-, Cl, HCO3-), particularly in apical iodine efflux in thyroid cells and chloride (Cl-|HCO3-) at the cochlear level. A current issue is to determine factors that distinguish Pendred syndrome from other varieties of isolated deafness also including enlargement of the vestibular aqueduct, but without evidence of thyroid expression. A prospective investigation of these 2 situations, conducted in Lille, leads us to redefine diagnostic criteria and assessment methods, and to discuss genetic or environmental factors contributing to the phenotypes.