Objectives.:The current settings of cochlear implants (CIs) do not respect specific cochlear tonotopy, resulting in tonotopic mismatch and potentially decreased auditory performance. The development of new implant settings adapted to individual tonotopy, called anatomybased fitting (ABF), could improve the auditory information coding, particularly by making surrounding sounds more recognizable. This study aimed to evaluate whether ABF allows better environmental sound recognition in new CI users compared to the conventional setting (CS); and to investigate the effect of ABF on speech recognition in quiet and noise. Methods.:A prospective, randomized, double-blind, two-period cross-over study in 17 new CI users was performed between March 2022 and August 2024. Adult subjects were recruited from candidates selected for cochlear implantation within a single French university hospital. Newly implanted adult recipients of a MED-EL cochlear implant with an electrode array insertion angle greater than 540° were eligible. Subjects were randomized to receive either ABF or CS for 6 weeks, then switched to the other setting for the same duration. Audiometric testing, including Environmental Sound Identification Test (Test d'Identification des Sons de l'Environnement, TISE), speech audiometry in quiet using Fournier lists, and speech audiometry in noise (Vocale Rapide dans le Bruit, VRB) was performed at 6 and 12 weeks. Results.:Sixteen subjects (mean age 60 years [SD=15.1]) were analyzed. ABF showed a significant improvement in the TISE score (mean effect (ME)=1.2, 95% confidence interval (CI95%)=[0.3;2.0], standardized effect size (SES)=0.97, p=0.016). Speech recognition in quiet was statistically better with CS (ME=3.6, CI95%=[1.8;5.3], SES=1.4, p=0.001) while no statistically significant difference was found for speech recognition in noise (CI95%=[- 2.8;0.1], p=0.08). Conclusion.:In newly implanted cochlear implant users, ABF improved environmental sound recognition, suggesting improved perceived sound naturalness in the first weeks after implantation. This benefit did not extend to speech recognition in quiet and remains inconclusive in noise due to floor effect.

Inhibition of the biosynthesis of bacterial heptoses opens novel perspectives for antimicrobial therapies. The enzyme GmhA responsible for the first committed biosynthetic step catalyzes the conversion of sedoheptulose 7-phosphate into d-glycero-d-manno-heptose 7-phosphate and harbors a Zn2+ ion in the active site. A series of phosphoryl- and phosphonyl-substituted derivatives featuring a hydroxamate moiety were designed and prepared from suitably protected ribose or hexose derivatives. High-resolution crystal structures of GmhA complexed to two N-formyl hydroxamate inhibitors confirmed the binding interactions to a central Zn2+ ion coordination site. Some of these compounds were found to be nanomolar inhibitors of GmhA. While devoid of HepG2 cytotoxicity and antibacterial activity of their own, they demonstrated in vitro lipopolysaccharide heptosylation inhibition in Enterobacteriaceae as well as the potentiation of erythromycin and rifampicin in a wild-type Escherichia coli strain. These inhibitors pave the way for a novel treatment of Gram-negative infections.

LEDGF/p75 (LEDGF) main cellular cofactor of HIV-1 integrase (IN), acts as tethering factor to target integration of HIV in actively transcribed genes. Recently a class of IN inhibitors based on inhibition of LEDGF-IN interaction has been developed. We describe here a new series of IN-LEDGF allosteric inhibitors (INLAIs), with potent anti-HIV-1 activity in the one-digit nanomolar range. These compounds inhibited IN-LEDGF interaction while enhancing IN-IN aberrant multimerization by allosteric mechanism. Compounds of this series were fully active on HIV-1 mutants resistant to IN strand transfer inhibitors (INSTIs) and other class of anti-HIV drugs, confirming that they belong to a new class of antiretrovirals. These compounds displayed most potent antiretroviral activity at post-integration due to aberrant IN polymerization responsible of infectivity defect of viral particles produced. INLAI-resistant mutants were selected by dose escalation method and the most detrimental mutation found was T174I. The impact of these resistance mutations was analyzed by fold shift EC50 with regard to wild type virus and the replication capacity of mutated viruses were determined. Crystal structure of BDM-2, the lead compound of this series in complex with IN catalytic core domain (CCD) was elucidated. No antagonism was observed between BDM-2 and a panel of 16 antiretroviral drugs from different classes. In conclusion, the overall virologic profile of BDM-2 warrants the recently completed single ascending dose phase I trial (ClinicalTrials.gov Id: NCT03634085) and supports further clinical investigation for potential use in combination with other antiretroviral drugs. Author summary Integrase-LEDGF allosteric inhibitors are a new class of antiretrovirals recently developed that target the interaction of HIV-1 Integrase with its cellular cofactor LEDGF/p75 required for HIV-1 integration in actively transcribed genes. The great interest of developing such new class of antiretroviral is to add to the anti-HIV drug arsenal compounds that are fully active on resistant viruses to all other classes of drugs currently used in clinic. However, none of these inhibitors are currently in clinical use or in late clinical trials. Here we describe a series of highly potent Integrase allosteric inhibitors that can be considered as precursors of a new class of antiretroviral drugs, with fully conserved activity on viruses resistant to currently used anti-HIV drugs, and a lead compound that has no antagonism with a large panel of present anti-HIV drugs and that was successfully validated in a phase I clinical trial.

INTRODUCTION Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. RESULTS The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its management in face-to-face or even distance learning. CONCLUSION In case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. It is recommended to perform free-field speech audiometry in noise to measure intelligibility in an environment as close as possible to reality. Questionnaires can be used in addition to audiometry to best assess the patient's disability. It is recommended that hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time elapsed since the fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, integrating different training domains in addition to face-to-face sessions.

Transtympanic administration is used clinically for the injection of gentamicin and/or corticosteroids. This atraumatic route is based on passive diffusion through the round window membrane (RWM). The main limitation of this method is related to the clearance through the Eustachian tube, making the concentration of the therapeutic agent at the intracochlear level uncertain and limited. Moreover, this technique remains unsuitable for molecules of high molecular weight or in the case of gene therapies. The purpose was to study a new technique of intracochlear administration in an atraumatic, direct and controlled manner by laser-assisted bioprinting (LAB). LAB was used to deliver dexamethasone phosphate with thermosensitive hydrogel on the mouse RWM. After validation of the regularity and homogeneity of the pattern, the diffusion in vivo of the dexamethasone into the perilymph after LAB has been confirmed by ELISA. Auditory function measurements showed no hearing impairment suggesting that bioprinting does not induce significant cochlear damage. Hence, the present proof of concept study introduces a promising approach for inner ear drug delivery.

HIV-1 integrase-LEDGF allosteric inhibitors (INLAIs) share the binding site on the viral protein with the host factor LEDGF/p75. These small molecules act as molecular glues promoting hyper-multimerization of HIV-1 IN protein to severely perturb maturation of viral particles. Herein, we describe a new series of INLAIs based on a benzene scaffold that display antiviral activity in the single digit nanomolar range. Akin to other compounds of this class, the INLAIs predominantly inhibit the late stages of HIV-1 replication. A series of high-resolution crystal structures revealed how these small molecules engage the catalytic core and the C-terminal domains of HIV-1 IN. No antagonism was observed between our lead INLAI compound BDM-2 and a panel of 16 clinical antiretrovirals. Moreover, we show that compounds retained high antiviral activity against HIV-1 variants resistant to IN strand transfer inhibitors and other classes of antiretroviral drugs. The virologic profile of BDM-2 and the recently completed single ascending dose phase I trial (ClinicalTrials.gov identifier: NCT03634085) warrant further clinical investigation for use in combination with other antiretroviral drugs. Moreover, our results suggest routes for further improvement of this emerging drug class.

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). (c) 2021 Elsevier Masson SAS. All rights reserved.

Ce document présente les fondamentaux de l’audiométrie vocale dans le bruit, les considérations générales nécessaires à sa réalisation ainsi que les critères de choix parmi les tests disponibles en français en fonction des besoins des professionnels. 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. Nous avons identifié 8 tests d’audiométrie vocale dans le bruit utilisables en France. Pour être complète, l’évaluation du statut auditif d’un sujet nécessite que soit testée sa compréhension de la parole dans le bruit. L’examen doit débuter par un minimum de deux mesures familiarisant le sujet avec la procédure de passation. Dans le cadre du diagnostic initial, les procédures adaptatives visant à établir le seuil des 50 % d’intelligibilité dans le bruit (SIB50) sont à privilégier pour obtenir une mesure rapide et standardisée de la perception de la parole dans le bruit. Lorsque l’on souhaite mesurer la compréhension de la parole de la façon la plus écologique possible, les tests à base de phrases, les bruits multi-locuteurs et la diffusion en champ libre sont à privilégier. Le gain prothétique est évalué exclusivement en champ libre. Seule cette modalité permet d’évaluer l’apport de la binauralité et de mesurer la perception dans le bruit dans un environnement au plus proche de la réalité. Afin d’éviter les interférences acoustiques en champ libre, l’emploi d’un minimum de cinq haut-parleurs est recommandé, notamment pour l’évaluation de l’efficacité des microphones directionnels, d’un appareillage CROS permettant de restituer au niveau de l’oreille fonctionnelle les sons captés au niveau de l’oreille lésée, ou d’une adaptation bimodale (c’est-à-dire lorsque deux conditions d’écoute permettent l’audition, par exemple : aide auditive pour une oreille, implant cochléaire pour l’autre).

Le questionnaire HHIE-S (Hearing Handicap Inventory for the Elderly – Screening) est largement utilisé dans le dépistage des troubles auditifs liés à l’âge. Notre objectif principal était d’en valider une version française. Notre objectif secondaire était d’en déterminer un score seuil pertinent pour une indication d’appareillage auditif. La traduction française du HHIE-S était élaborée selon la méthode d’adaptation culturelle des questionnaires médicaux. Une étude observationnelle de cohorte a évalué la pertinence du questionnaire (10 questions, score entre 0 et 40) chez des sujets âgés de 60 ans minimum, en situation de dépistage, par rapport à leurs résultats audiométriques (audiométrie tonale, vocale dans le silence et dans le bruit). La surdité était définie par une perte tonale moyenne > 20 dB sur les fréquences 500, 1000, 2000 et 4000 Hz sur 1 ou 2 oreilles. L’étude a inclus 294 participants (âge moyen = 67 ± 6 ans). La prévalence de la surdité était de 34,7 %. La cohérence interne du questionnaire était élevée (indice alpha de Cronbach = 0,84). Pour un score seuil de 8/40, le questionnaire présentait une sensibilité de 80,4 %, une spécificité de 85,4 %, une valeur prédictive positive de 74,5 % et une valeur prédictive négative de 89,1 %. Une indication théorique d’appareillage auditif concernait 73 participants (24,8 %). Un score > 16/40 permettait de les détecter avec une efficacité maximale (88,4 %). Cette étude permet la validation d’une version française du HHIE-S en précisant ses caractéristiques chez des sujets âgés de plus de 60 ans. Cet outil pourrait être utile dans le cadre d’un programme de dépistage de la surdité liée à l’âge dans la population française.

Le « Musical Ear Syndrome » est un phénomène rare se définissant comme la perception de sensations auditives musicales ne correspondant pas à un stimulus extérieur. Il semble être plus fréquent chez les patients sourds profonds. Notre objectif était d’évaluer sa prévalence, ses caractéristiques principales et ses facteurs de risque de survenue chez des patients implantés cochléaires. Étude de prévalence rétrospective chez des patients (adultes en 2020) implantés cochléaires (entre 1993 et 2019) analysant la présence et les caractéristiques des sensations auditives musicales. Sur 358 patients, 118 (= 33 %) ont, ou ont perçu, des sensations auditives musicales : 71 (19,8 %) avant l’implantation cochléaire, 100 (28 %) après et 53 (14,8 %) avant et après. Elles étaient principalement brèves, bien tolérées, de type « musique instrumentale » et se répétaient plusieurs fois par jour. Treize patients (11 %) les trouvaient insoutenables. La fatigue était retrouvée comme facteur déclenchant chez 40 patients (33,9 %). Les caractéristiques personnelles ou médicales des patients, le type d’implantation, la marque d’implant, l’étiologie de la surdité ou la présence d’acouphènes n’étaient pas des facteurs de risque de développer ce phénomène. Cependant, les patients percevant des sensations auditives musicales étaient significativement plus jeunes (56 ± 17,4 ans versus 61,9 ± 17,9 ans ; p = 0,0009). Malgré ce phénomène, les patients étaient satisfaits du fonctionnement de l’implant. Sa présence n’influençait pas les performances auditives subjectives avec l’implant. La prévalence des sensations auditives musicales est élevée chez les implantés cochléaires, surtout les plus jeunes, rendant essentielle une meilleure connaissance du phénomène.

L’otite séromuqueuse (OSM) de l’enfant est une pathologie fréquente souvent mal comprise par les parents. La qualité et la lisibilité de l’information sur l’internet semblent très variables. L’objectif principal était de mesurer la qualité et la lisibilité des sites internet à propos de l’OSM en France. Une recherche avancée sur le moteur de recherche Google « Otite séromuqueuse OR Otite séreuse » était réalisée. La qualité de l’information était déterminée par le score DISCERN. La lisibilité était mesurée par les tests de Flesch Reading Ease Scoring (FRES), Flesch-Kincaid Grade Level (FKGL), SMOG, et le graphique de Fry. La médiane et l’écart type (DS) étaient calculés. Une corrélation entre qualité et lisibilité notée r était recherchée par un test de Spearman. Les 10 premiers sites répondant aux critères d’inclusion et d’exclusion ont été analysés. Un site avait été mis à jour depuis moins d’un an. La médiane du score DISCERN était 49/80 (DS = 13,7). Les médianes des scores FRES, FKGL et SMOG étaient respectivement 46/100 (DS = 9,5), 11 (DS = 1,7) et 12 (DS = 1,5). Six sites avaient un score de Fry > 12. Un site présentait une qualité élevée. Un site présentait une lisibilité accessible pour le public cible selon FRES et FKGL. L’analyse suggère une diminution de la lisibilité selon SMOG, quand la qualité augmente : r = 0,72 (p = 0,024). Trois sites suivaient les dernières recommandations scientifiques. La qualité et la lisibilité de l’information disponible sur internet à propos de l’OSM sont hétérogènes ; lorsque les critères de qualités sont bons, le contenu semble trop difficile à comprendre pour les parents.

Introduction: Childhood otitis media with effusion (OME) is a frequent disease often misunderstood by parents. Information on the Internet is of variable quality and readability. The aim of this study was to measure the quality and readability of French websites related to OME. Material and methods: An advanced Google search was conducted using the terms "Otite sero-muqueuse OR Otite sereuse". Quality was assessed on DISCERN criteria. Readability was assessed using Flesch Reading Ease Scoring (FRES), Flesch-Kincaid Grade Level (FKGL), the Simple Measure of Gobbledygook (SMOG) and a Fry graph. Medians and standard deviations were calculated. Correlation between quality and readability was assessed on Spearman r coefficient. Results: The first ten websites meeting inclusion and exclusion criteria were evaluated. One had been updated during the last 12 months. Median DISCERN score was 49 +/- 13.7/80. Median FRES score was 46 +/- 9.5/100. Median USA grade-level estimated by FKGL and SMOG respectively was 11 +/- 1.7 and 12 +/- 1.5. Six websites had Fry score > 12. One website showed high quality. One had a readability score in the target range (below 9th grade reading level (age 14-15)) according to FRES and FKGL. A suggestive correlation was found between lower SMOG readability and higher quality: r = 0.72 (P = 0.024). Three websites followed the most recent scientific guidelines. Conclusion: Online information about OME was of variable quality and readability. Good quality information tended to be less easily understandable by parents. (c) 2021 Elsevier Masson SAS. All rights reserved.

INTRODUCTION:The HHIE-S (Hearing Handicap Inventory for the Elderly - Screening) is widely used for hearing-loss disorder in the elderly. The main objective of the present study was to validate a French version. The secondary objective was to determinate a cut-off score as indication for hearing rehabilitation. METHODS:We translated the HHIE-S into French, respecting the cross-cultural adaptation process for medical questionnaires. An observational study assessed the translation (10 questions, scored from 0 to 40) used for screening purposes in a prospective cohort, aged ≥60 years, with comparison to pure tone, speech-in-silence and speech-in-noise audiometry. Subjects were considered hearing-impaired if the pure-tone average at 500, 1,000, 2,000 and 4,000 Hz was >20 dB HL in one or both ears. RESULTS:We tested 294 subjects (mean age =67±6 years). Hearing loss prevalence was 34.7 %. Cronbach's alpha (test reliability) was high (0.84). Taking HHIE-S score >8/40 as cut-off defining hearing loss, sensitivity was 80.4%, specificity 85.4 %, positive predictive value 74.5 % and negative predictive value 89.1 %. Seventy-three subjects (24.8 %) had theoretic indications for hearing aids, optimally detected by HHIE-S score >16/40 (88,4 %). CONCLUSION:Our study validated the French version of the HHIE-S. This tool could be useful in screening for age-induced hearing loss in the elderly French population.

Introduction: Musical Ear Syndrome (MES) is an uncommon phenomenon described as the perception of auditory musical sensations not corresponding to any external stimulus. It seems to be more frequent in case of profound hearing loss. Our objective was to evaluate prevalence, characteristics and risk factors in a population of cochlear implant patients. Methods: A retrospective study was conducted in cochlear implant patients, who were adult (> 18 years) in 2020 and underwent cochlear implantation between 1993 and 2019. We analyzed the presence and characteristics of MES. Results: 118 of the 358 patients (33%) perceived or had perceived auditory musical sensations: 71 (19.8%) before, 100 (28%) after, and 53 (14.8%) both before and after implantation. The musical auditory sensations were usually short and well-tolerated, resembling instrumental music, and occurring several times a day. Thirteen patients (11%) considered them intolerable. Fatigue was a triggering factor in 40 patients (33.9%). Personal and medical characteristics, type of implantation, make of implant, etiology and tinnitus did not emerge as risk factors. On the other hand, MES+ patients were significatively younger (56 +/- 17.4 years versus 61.9 +/- 17.9 years; P = 0.0009). Despite the phenomenon, patients were satisfied with implant functioning and subjective auditory performance was not affected. Conclusion: Prevalence of Musical Ear Syndrome was high in cochlear implant patients, and especially in younger subjects. It is essential to improve knowledge of this phenomenon. (c) 2020 Elsevier Masson SAS. All rights reserved.

The authors of these research declare that there are no conflicts of interest associated with this publication. The data can be shared upon request.

Penicillin-binding proteins (PBPs) are the targets of the β-lactams, the most successful class of antibiotics ever developed against bacterial infections. Unfortunately, the worldwide and rapid spread of large spectrum β-lactam resistance genes such as carbapenemases is detrimental to the use of antibiotics in this class. New potent PBP inhibitors are needed, especially compounds that resist β-lactamase hydrolysis. Here we describe the structure of the E. coli PBP2 in its Apo form and upon its reaction with 2 diazabicyclo derivatives, avibactam and CPD4, a new potent PBP2 inhibitor. Examination of these structures shows that unlike avibactam, CPD4 can perform a hydrophobic stacking on Trp370 in the active site of E. coli PBP2. This result, together with sequence analysis, homology modeling, and SAR, allows us to propose CPD4 as potential starting scaffold to develop molecules active against a broad range of bacterial species at the top of the WHO priority list.

Recently, a new class of HIV-1 integrase (IN) inhibitors with a dual mode of action, called IN-LEDGF/p75 allosteric inhibitors (INLAIs), was described. Designed to interfere with the IN-LEDGF/p75 interaction during viral integration, unexpectedly, their major impact was on virus maturation. This activity has been linked to induction of aberrant IN multimerization, whereas inhibition of the IN-LEDGF/p75 interaction accounts for weaker antiretroviral effect at integration. Because these dual activities result from INLAI binding to IN at a single binding site, we expected that these activities co-evolved together, driven by the affinity for IN. Using an original INLAI, MUT-A, and its activity on an Ala-125 (A125) IN variant, we found that these two activities on A125-IN can be fully dissociated: MUT-A-induced IN multimerization and the formation of eccentric condensates in viral particles, which are responsible for inhibition of virus maturation, were lost, whereas inhibition of the IN-LEDGF/p75 interaction and consequently integration was fully retained. Hence, the mere binding of INLAI to A125 IN is insufficient to promote the conformational changes of IN required for aberrant multimerization. By analyzing the X-ray structures of MUT-A bound to the IN catalytic core domain (CCD) with or without the Ala-125 polymorphism, we discovered that the loss of IN multimerization is due to stabilization of the A125-IN variant CCD dimer, highlighting the importance of the CCD dimerization energy for IN multimerization. Our study reveals that affinity for the LEDGF/p75-binding pocket is not sufficient to induce INLAI-dependent IN multimerization and the associated inhibition of viral maturation.

Rare and fairly unknown, Arnold Chiari deformity is defined by the abnormally low position of the cerebellar tonsils that engage through the foramen magnum. Its association with the triad of tinnitus-hypoacousia-vertigo causes an authentic Meniere’s disease worth discussing. We report an unusual association of Arnold Chiari deformity with Meniere’s disease. A 46-years-old patient was diagnosed with left Meniere’s disease in 1994 on the classical diagnostic triad and the mode of progression: rotatory vertigo evolving by iterative crises; Intermittent buzzing tinnitus; left perception deafness with notion of wadded left ear. For a decade (1994 to 2015), he has been put under hygieno-dietary measures, vestibular re-education and medical treatment. The evolution was marked by the worsening of vertigo becoming progressively incapacitating, as well as an aggravation of the left deafness and persistence of tinnitus. Audiometry highlighted a severe endocochlear left perception deafness. The videonystagmography revealed a well-compensated left vestibular deficit. Cerebral magnetic resonance imaging (MRI) revealed a cerebellar tonsils’ ptosis through the foramen magnum. The diagnosis of Arnold-Chiari deformity associated with Meniere’s disease was then retained. The patient received a surgical left labyrinthectomy in 2015. The clinical course was uneventful, marked by the disappearance of vertigo twenty four months later. Association of Meniere’s disease and Arnold Chiari deformity is rare and must be diagnosed. These two pathologies being manifested by peripheral vertigo, a meticulous clinical and Para clinical examination is necessary to guide the diagnosis. Cerebrospinal fluid flow and pressure anomaly due to Arnold Chiari malformation can truly impact labyrinthine physiology, which explains the correlation between these two entities.

OBJECTIVES:To analyze long-term cognitive status and function after cochlear implantation in profoundly deaf individuals. DESIGN:Prospective observational longitudinal study. SETTING:Ten academic medical centers referent for cochlear implantation. PARTICIPANTS:Individuals aged 65 and older who qualified for cochlear implantation (N=70). MEASUREMENTS:Cognitive tests were administered before cochlear implantation and 1 and 5 or more years after cochlear implantation. Evaluation consisted of 6 tests assessing attention, memory, orientation, executive function, mental flexibility, and fluency. Cognitive status was determined as normal, mild cognitive impairment (MCI), or dementia. Speech perception in quiet and noisy conditions was assessed using disyllabic words, and quality of life was assessed using the Nijmegen Cochlear Implant Questionnaire. RESULTS:Mean follow-up was 6.8 years (range 5.5-8.5 years). Speech perception scores and quality of life remained stable from 1 to 7 years after cochlear implantation. Of 31 participants (45%) with MCI before cochlear implantation, 2 (6%) developed dementia during follow-up, 19 (61%) remained stable, and 10 (32%) returned to normal cognition. None of the 38 with normal cognition developed dementia during follow-up, although 12 (32%) developed MCI. CONCLUSION:MCI is highly prevalent in older adults with profound hearing loss. Nevertheless, we observed a low rate of progression to dementia, and cognitive function improved in some individuals with MCI at baseline. These results highlight that cochlear implantation should be strongly considered in profoundly deaf individuals, even those with MCI, who may have a specific subtype of MCI, with a possible positive effect of hearing rehabilitation on neurocognitive functioning.

Osteomas of the middle ear are rare benign tumors. Their consequences and symptoms are due to their specific location, such as the promontory or the epitympanum and their contact with the facial nerve, the semicircular canal, the ossicles, and the oval or round windows. We report a very unusual case of middle ear osteoma (MEO) in a 23-year-old male patient causing a right mixed hearing loss by contacting and overwhelming the incus and stapes. The lesion was also closely attached to the tympanic portion of the fallopian canal. Since the stapes was not clearly visible behind the lesion, careful observation was preferred to surgery owing to the high risk of inner ear damage and facial palsy with removal of the lesion. MEOs are rarely situated at this critical site. Regular clinical and computerized tomography monitoring is warranted to check their growth. This case also supports the etiological theory of chronic middle ear inflammation causing osteomas.