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International audience; Interactions among plants have been long recognized as a major force driving plant community dynamics and crop yield. Surprisingly, our knowledge of the ecological genetics associated with variation of plant-plant interactions remains limited. In this opinion article by scientists from complementary disciplines, the international PLANTCOM network identified four timely questions to foster a better understanding of the mechanisms mediating plant assemblages. We propose that by identifying the key relationships among phenotypic traits involved in plant-plant interactions and the underlying adaptive genetic and molecular pathways, while considering environmental fluctuations at diverse spatial and time scales, we can improve predictions of genotype-by-genotype-by-environment interactions and modeling of productive and stable plant assemblages in wild habitats and crop fields.

Aim: To synthesize published evidence regarding hearing impairment diagnosis and interventions in infants with or at high risk for cerebral palsy in the first year after birth. Method: Nine databases were searched for MeSH terms up to February 2020. Included studies were published in English, enrolled infants with or at high risk for cerebral palsy, and addressed hearing evaluation/rehabilitation within the first year after birth. Quality of evidence was evaluated using RTI Item Bank and QUADAS-2. Results: Eighteen articles met inclusion criteria. Quality of the evidence ranged from low to high, revealing variability in diagnostic assessment methodologies and adherence to diagnostic schedules. Concerns for bias included lack of recognition of cerebral palsy effects and etiologies on functional hearing assessment methods and results. Two interventions (hearing aid and cochlear implantation) were identified; however, reported use was inconsistent. Interpretation: Hearing screening in infants with or at high risk for cerebral palsy requires evaluation of the entire auditory pathway preferentially using comprehensive electrophysiological panels of assessments. For infants with perinatal neural insults, pediatric neurologists are uniquely positioned to recommend adherence to systematic surveillance and comprehensive audiology assessments, regardless of comorbidities and motor impairments.

Background Two non-invasive ventilation strategies, Bubble Continuous Positive Airway Pressure (Bubble CPAP) and standard nasal Continuous Positive Airway Pressure (nasal-CPAP), are commonly used to treat preterm infants in the NICU, differentially impacting their auditory environment and contributing to noise exposure above recommended safe levels. Aim To compare differences in sound exposure for preterm infants receiving two types of non-invasive CPAP. Method Prospective observational study of 108 preterm infants, receiving either Bubble-CPAP or nasal-CPAP, using repeated measures of sound exposure in single-patient rooms and semi-private bays. Results Analyses with repeated measures demonstrated that both types of CPAP increase noise levels above the background of the NICU, and that non-invasive nasal-CPAP produces higher noise pollution compared to Bubble CPAP, regardless of the room type. Conclusion While CPAP is a necessary treatment for many preterm infants, cumulative noise exposure in extremely preterm infants may also be a care consideration.

Plasticity of plant architecture is a promising lever to increase crop resilience to biotic and abiotic damage. Among the main drivers of its regulation are the spectral signals which occur via photomorphogenesis processes. In particular, branching, one of the yield components, is responsive to photosynthetic photon flux density (PPFD) and to red to far-red ratio (R:FR), both signals whose effects are tricky to decorrelate in the field. Here, we developed a device consisting of far-red light emitting diode (LED) rings. It can reduce the R:FR ratio to 0.14 in the vicinity of an organ without changing the PPFD in outdoor high irradiance fluctuating conditions, which is a breakthrough as LEDs have been mostly used in non-fluctuant controlled conditions at low irradiance over short periods of time. Applied at the base of rapeseed stems during the whole bolting-reproductive phase, LightCue induced an expected significant inhibitory effect on two basal targeted axillary buds and a strong unexpected stimulatory effect on the overall plant aerial architecture. It increased shoot/root ratio while not modifying the carbon balance. LightCue therefore represents a promising device for progress in the understanding of light signal regulation in the field.

Introduction In young children, EEG data acquisition during stimulation tasks is difficult due to anxiety, movement and behaviorally-related interruptions, especially in those with disabilities. New method We used standardized music therapy (MT) protocols with and without acclimatization, during and prior to time-locked EEG with a published tactile testing protocol. Our prospective study leveraged a larger trial in children with/without cerebral palsy aged 7–27 months. Group1 received no preparation, Group2 received 15-minute MT prior to the EEG session, Group3 received the same as Group2 plus a rubber cap for home practice. All groups received MT procedural support during the EEG session. Sessions were stopped/started to acquire a full dataset. Trials were reviewed using a two-step artifact detection strategy by specialists masked to group allocation. Results 64 patients were included, 20 each in Groups 2 and 3, and 24 in Group1. Average age was 16.08 ± 6.33 months. All (100%) of children had data of sufficient quality and quantity for outcomes measurement without a second testing visit. There were no differences in useable trials by procedural group, disability status, age or stimulus condition. EEG recording time was shorter in Group3 vs. 1 (p = 0.02) and more patients in Group1 required repeat trials compared to Groups2 and 3 (p = 0.04 for both). Comparison with old method Our new methods resulted in no attrition from data loss, an improvement compared to published similar studies with data loss 30–55%. Acclimatization had minimal effects. Conclusion In children under 3, MT protocols result in high rates of EEG data acquisition, decrease behaviorally-related interruptions and session acquisition time. This method is successful for typically developing children and those with cerebral palsy.

Mutations in the CHD7 gene, encoding for the chromodomain helicase DNA-binding protein 7, are found in approximately 60% of individuals with CHARGE syndrome (coloboma, heart defects, choanal atresia, retarded growth and development, genital hypoplasia, ear abnormalities and/or hearing loss). Herein, we present a clinical case of a 14-year-old male presenting for evaluation of poor growth and pubertal delay highlighting the diagnostic challenges of CHARGE syndrome. The patient was born full term and underwent surgery at 5 days of life for bilateral choanal atresia. Developmental milestones were normally achieved. At age 14 his height and weight were -2.04 and -1.74 standard deviation score respectively. He had anosmia as well as prepubertal testes and micropenis (4 cm×1 cm). The biological profile showed low basal serum testosterone and gonadotropins (testosterone, 0.2 nmol/L; luteinizing hormone, 0.5 U/L; follicle-stimulating hormone, 1.3 U/L), and otherwise normal pituitary function and normal imaging of the hypothalamic-pituitary area. The constellation of choanal atresia, anosmia, mild dysmorphic features, micropenis and delayed puberty were suggestive of CHARGE syndrome. Targeted genetic testing of CHD7 was performed revealing a de novo heterozygous CHD7 mutation (c.4234T>G [p.Tyr1412Asp]). Further paraclinical investigations confirmed CHARGE syndrome. Despite the presence of suggestive features, CHARGE syndrome remained undiagnosed in this patient until adolescence. Genetic testing helps clarify the phenotypic and genotypic spectrum to facilitate diagnosis, thus promoting optimal follow-up, treatment, and appropriate genetic counselling.

Resume Dans un contexte de perpetuelle evolution des traitements, l’acces a l’innovation therapeutique est un enjeu majeur pour les patients et pour les differents acteurs impliques dans les procedures de mise a disposition des produits de sante. Les revolutions en matiere de medecine genomique et personnalisee, d’intelligence artificielle et de biotechnologies vont transformer la medecine de demain et l’organisation de notre systeme de sante. Il est ainsi fondamental que la France se prepare a ces mutations et accompagne le developpement de ses entreprises dans ces nouveaux domaines. Le recent Conseil strategique des industries de sante lance par Matignon permet de proposer un arsenal reglementaire propice a l’implementation et la diffusion des innovations therapeutiques. Dans le present atelier, nous declinons un certain nombre de propositions, notre approche se veut pragmatique avec un souci permanent d’acces securise des patients aux medicaments et de simplification des procedures reglementaires. Ceci a ete realise grâce a la participation a cette table ronde de la plupart des acteurs impliques (industriels, Agence nationale de securite du medicament et des produits de sante [ANSM], Haute Autorite de sante [HAS], Institut national du cancer [INCa], Les entreprises du medicament [LEEM], Hopitaux, observatoire du medicament, des dispositifs medicaux et de l’innovation therapeutique…). Les principales propositions tendent a favoriser l’implementation d’essais cliniques sur notre territoire en particulier les phases precoces, un acces plus large aux innovations en favorisant les « early access programs », et en mettant en place des autorisations temporaires d’utilisation pour les extensions d’indication de medicaments deja disponibles qui permettent de prescrire un medicament meme si ce dernier a une autorisation de mise sur le marche (AMM) dans une autre indication. De plus, nous proposons un remboursement dont le maintien serait conditionne par la levee des incertitudes initiales, permettant ainsi une mise a disposition sur la base de donnees preliminaires et prometteuses dans les maladies graves ou le besoin medical n’est pas couvert. Ce mecanisme impliquera une reevaluation a la lumiere de donnees consolidees issues des essais cliniques et/ou de donnees de vie reelle. Enfin, pour mener au mieux ces evaluations, en vue de l’acces ou de la prise en charge, nous proposons la mise en place de conventions de partenariat agences sanitaires/hopitaux afin de favoriser l’emergence d’experts de terrains, l’idee etant de privilegier une expertise ascendante au plus proche des besoins ressentis des patients et de la vie reelle.

In a context of perpetual evolution of treatments, access to therapeutic innovation is a major challenge for patients and the various players involved in the procedures of access to medicines. The revolutions in genomic and personalized medicine, artificial intelligence and biotechnology will transform the medicine of tomorrow and the organization of our health system. It is therefore fundamental that France prepares for these changes and supports the development of its companies in these new areas. The recent "Conseil stratégique des industries de santé" launched by Matignon makes it possible to propose a regulatory arsenal conducive to the implementation and diffusion of therapeutic innovations. In this workshop, we present a number of proposals, our approach having remained pragmatic with a permanent concern to be effective in the short term for the patients and to simplify the procedures as much as possible. This was achieved thanks to the participation in this workshop of most of the players involved (industrial companies, "Agence nationale de sécurité du médicament et des produits de santé", "Haute Autorité de santé", "Institut national du cancer", "Les entreprises du médicament", hospitals, "Observatoire du médicament, des dispositifs médicaux et de l'innovation thérapeutique"…). The main proposals tend to favor the implementation of clinical trials on our territory, especially the early phases, a wider access to innovations by favoring early access programs and setting up a process called "autorisation temporaire d'utilisation d'extension" (ATUext) that make it possible to prescribe a medicinal product even if the latter has a marketing authorisation in another indication. In addition, we propose a conditional reimbursement that will be available based on preliminary data but will require re-evaluation based on consolidated data from clinical trials and/or real-life data. Finally, in order to better carry out these assessments, with a view to access or care, we propose the establishment of partnership agreements with health agencies/hospitals in order to encourage the emergence of field experts, in order to prioritize an ascending expertise closer to patients' needs and to real life.

To test whether infant-directed foreign language active learning would specifically increase speech sound differentiation to the intervention language while not decreasing differentiation in response to English.Pilot randomized controlled trial of stable infants born preterm in the newborn intensive care unit with normal auditory brainstem responses, whose parents spoke only English and had no musical training or familial hearing abnormality. Assignment was to 1 of 3 groups: passive exposure to English infant-directed lullabies and readings (English-enrichment, control group) and contingent exposure by active sucking on a sensor-equipped pacifier to either infant-directed French lullabies and readings (English environment, French-contingent learning group) or infant-directed Mandarin lullabies and readings (English environment, Chinese-contingent learning group). The main outcome measures were preintervention and postintervention event-related potentials (ERPs) in response to standardized speech syllables in each language.Forty-one subjects completed the study, including 15 in the English-enrichment control group and 13 each in the French-contingent and Chinese-contingent groups. The median gestational age at birth was 34 weeks (IQR, 8.75 weeks); postmenstrual age at intervention ranged from 36 to 46 weeks and was similar across the 3 groups. Postintervention mean ERP amplitude to pairs of English speech sounds did not differ across the 3 groups; however, ERP amplitude in French sound differentiation was greater in the French-contingent group than in the Chinese-contingent or English-enrichment groups, and ERP amplitude greater in Chinese sound differentiation was greater in the Chinese-contingent group compared with the other 2 groups.Contingent infant-directed foreign language exposure increased speech sound differentiation specific to the intervention language and did not decrease differentiation in response to English.ClinicalTrials.gov: NCT03232931.

Atypical cries have been identified in infants with neurological dysfunction. The aim of this study was to conduct a systematic review and meta-analysis to appraise existing evidence for associations between acoustic cry characteristics and neurological dysfunction in infants aged 18 months or less.PubMed/MEDLINE, PsycINFO, CINAHL, and Embase were searched for original, peer-reviewed studies published in English reporting cry variables in infants aged 18 months or less with or at risk of neurological dysfunction. Studies without a nonneurologically impaired control sample were excluded. Pooled effect sizes were estimated using standardized mean difference (SMD) and odds ratio (OR). IFrom March 2018 to February 2019, 28,294 studies were retrieved. Eight were meta-analyzed. Infants with or at risk of neurological dysfunction exhibited higher mean (SMD = 0.11 [95% confidence interval, 0.00 to 0.23]) and minimum (SMD = 0.93 [0.64 to 1.23]) fundamental frequency; higher odds of hyperphonation (OR = 13.17 [1.05 to 165.87]), biphonation (OR = 10.62 [1.53 to 73.59]), rise-fall-rise melodies (OR = 4.66 [1.16 to 18.66]), and flat melodies (OR = 4.47 [1.27 to 15.68]); and lower odds of fall-rise-fall melodies (OR = 0.21 [0.05 to 0.83]).Infants with underlying neuropathology have unique cries characterized by higher fundamental frequency, dysphonation, and atypical melodies, although study heterogeneity and imprecision of effect size estimates limited our interpretation. Assessment of acoustic cry characteristics offers the potential for noninvasive, rapid, point-of-care screening for neurologically high-risk infants.

International audience; Maintaining seed yield under low N inputs is a major issue for breeding, which requires thoroughly exploiting the genetic diversity of processes related to Nitrogen Use Efficiency (NUE). However, dynamic analysis of processes underlying genotypic variations in NUE in response to N availability from sowing to harvest are scarce, particularly at the whole-plant scale. This study aimed to dynamically decipher the contributions of Nitrogen Uptake Efficiency (NUpE) and Nitrogen Utilization Efficiency (NUtE) to NUE and to identify traits underlying NUpE genetic variability throughout the growth cycle of rapeseed. Three experiments were conducted under field-like conditions to evaluate seven genotypes under two N conditions. We developed NUE_DM (ratio of total plant biomass to the amount of N available) as a new proxy of NUE at harvest, valid to discriminate genotypes from the end of inflorescence emergence, and N conditions as early as the beginning of stem elongation. During autumn growth, NUpE explained up to 100% of variations in NUE_DM, validating the major role of NUpE in NUE shaping. During this period, under low N conditions, up to 53% of the plant nitrogen was absorbed and NUpE genetic variability resulted not from differences in Specific N Uptake but in fine-root growth. NUtE mainly contributed to NUE_DM genotypic variation during the reproductive phase under high-N conditions, but NUpE contribution still accounted for 50–75% after flowering. Our study highlights for the first time NUpE and fine-root growth as important processes to optimize NUE, which opens new prospects for breeding.

International audience

Multisensory processes include the capacity to combine information from the different senses, often improving stimulus representations and behavior. The extent to which multisensory processes are an innate capacity or instead require experience with environmental stimuli remains debated. We addressed this knowledge gap by studying multisensory processes in prematurely born and full-term infants. We recorded 128-channel event-related potentials (ERPs) from a cohort of 55 full-term and 61 preterm neonates (at an equivalent gestational age) in response to auditory, somatosensory, and combined auditory-somatosensory multisensory stimuli. Data were analyzed within an electrical neuroimaging framework, involving unsupervised topographic clustering of the ERP data. Multisensory processing in full-term infants was characterized by a simple linear summation of responses to auditory and somatosensory stimuli alone, which furthermore shared common ERP topographic features. We refer to the ERP topography observed in full-term infants as “typical infantile processing” (TIP). In stark contrast, preterm infants exhibited non-linear responses and topographies less-often characterized by TIP; there were distinct patterns of ERP topographies to multisensory and summed unisensory conditions. We further observed that the better TIP characterized an infant’s ERPs, independently of prematurity, the more typical was the score on the Infant/Toddler Sensory Profile (ITSP) at 12 months of age and the less likely was the child to the show internalizing tendencies at 24 months of age. Collectively, these results highlight striking differences in the brain’s responses to multisensory stimuli in children born prematurely; differences that relate to later sensory and internalizing functions. Electronic supplementary material The online version of this article (10.1007/s10548-020-00791-4) contains supplementary material, which is available to authorized users.

PurposeWe sought to critically analyze and evaluate published evidence regarding feasibility and clinical potential for predicting neurodevelopmental outcomes of the frequency-following responses (FFRs) to speech recordings in neonates (birth to 28 days).MethodA systematic search of MeSH terms in the Cumulative Index to Nursing and Allied HealthLiterature, Embase, Google Scholar, Ovid Medline (R) and E-Pub Ahead of Print, In-Process & Other Non-Indexed Citations and Daily, Web of Science, SCOPUS, COCHRANE Library, and ClinicalTrials.gov was performed. Manual review of all items identified in the search was performed by two independent reviewers. Articles were evaluated based on the level of methodological quality and evidence according to the RTI item bank.ResultsSeven articles met inclusion criteria. None of the included studies reported neurodevelopmental outcomes past 3 months of age. Quality of the evidence ranged from moderate to high. Protocol variations were frequent.ConclusionsBased on this systematic review, the FFR to speech can capture both temporal and spectral acoustic features in neonates. It can accurately be recorded in a fast and easy manner at the infant's bedside. However, at this time, further studies are needed to identify and validate which FFR features could be incorporated as an addition to standard evaluation of infant sound processing evaluation in subcortico-cortical networks. This review identifies the need for further research focused on identifying specific features of the neonatal FFRs, those with predictive value for early childhood outcomes to help guide targeted early speech and hearing interventions.

International audience

Epigenetic variations are involved in the control of plant developmental processes and participate in shaping phenotypic plasticity to the environment. Intense breeding has eroded genetic diversity, and epigenetic diversity now emerge as a new source of phenotypic variations to improve adaptation to changing environments and ensure the yield and quality of crops. Here, we review how the characterization of the stability and heritability of epigenetic variations is required to drive breeding strategies, which can be assisted by process-based models. We propose future directions to hasten the elucidation of complex epigenetic regulatory networks that should help crop modelers to take epigenetic modifications into account and assist breeding strategies for specific agronomical traits.

International audience; Plants represent the main source of organic carbon in soils. While inputs of carbon from the aerial parts are easy to measure, the difficulty in quantifying inputs from roots has prevented us to estimate the actual potential of soil carbon sequestration associated to each type of organic matters released by the roots. Besides the decay of root tissues, root systems have been shown to release organic carbon by various mechanisms, e.g. exudation of soluble compounds, mucilage secretion, and cells sloughing. Such rhizodeposition processes may consume 5% to 15% of the total amount of carbon photosynthetically fixed by the plant, and can generate an input of carbon to the soil ranging from 0.5 to 5 tC ha-1 yr-1. Because of this large range and the uncertainties associated to rhizodeposition mechanisms, current models of soil organic matter dynamics poorly assess the actual sequestration potential offered by plants. We performed a meta-analysis of the literature data generated over the last 60 years in order to assess which rhizodeposition processes quantitatively prevail, and what is the maximal amount of carbon inputs that can be expected from an entire growing root system, depending on plant species, growth stage, soil properties, and environmental conditions. According to our current dataset, the exudation of soluble sugars represents the major flow of organic carbon into the rhizosphere in most conditions, but other rhizodeposition processes such as mucilage secretion and cells sloughing can locally become as important in terms of carbon release. Following the presentation of these results, the consequences of these emissions on the actual soil carbon sequestration potential will be discussed based on our current understanding of carbon use efficiency and priming effect.

International audience

National audience

National audience; Être capable de caractériser la diversité génétique du système racinaire est un enjeu réelpour la sélection, et notamment pour proposer des variétés multi-performantes, adaptées auxnouveaux agro-écosystèmes au sein desquels les interactions plante-sol jouent un rôle majeur, du faitdes multiples fonctions du système racinaire. Cependant les méthodes directes de phénotypage auchamp du système racinaire sont rarement compatibles avec la caractérisation d’un grand nombred’accessions. De ce fait, le système racinaire est souvent caractérisé à des stades précoces et dans desconditions contrôlées, ne permettant pas toujours une bonne prédiction des phénotypes en conditionsagronomiques. Ici nous présentons une méthode complémentaire, le phénotypage assisté par modèle,basée sur le phénotypage de variables “processus”, estimées à partir du modèle ARCHISIMPLE etpermettant d’accéder aux mécanismes contrôlant la mise en place de l’architecture racinaire. La miseen oeuvre de cette approche novatrice pour la caractérisation des systèmes racinaires est ensuiteillustrée à travers l’analyse de la diversité génétique de l’architecture racinaire du colza. Nousmontrons comment ces paramètres du modèle constituent des traits de sélection efficaces,permettant de proposer des idéotypes adaptés à des systèmes de culture à faibles niveaux d’intrantsazotés, qui optimisent l’exploration du sol et l’absorption de l’azote.

Objectives To validate a French-language version of the spatial hearing questionnaire (SHQ), including investigating its internal structure using cluster analysis and exploring its construct validity on a large population of hearing-impaired (HI) and normal-hearing (NH) subjects, and to compare the SHQ with the speech, spatial, and qualities of hearing scale (SSQ) in the same population. Design The SHQ was translated in accordance with the principles of the Universalist Model of cross-cultural adaptation of patient-reported outcome instruments. The SSQ and SHQ were then presented in a counterbalanced order, in a self-report mode, in a population of 230 HI subjects (mean age = 54 years and pure-tone audiometry [PTA] on the better ear = 28 dB HL) and 100 NH subjects (mean age = 21 years). The SHQ feasibility, readability, and psychometric properties were systematically investigated using reliability indices, cluster, and factor analyses and multiregression analyses. SHQ characteristics were compared both to different literature data obtained with different language versions and to the SSQ scores obtained in the same population. Results Internal validity was high and very good reproducibility of scores and intersubject variability were obtained across the 24 items between the English and French SHQ for NH subjects. Factor and cluster analyses concurred in identifying five correlated factors, corresponding to several SHQ subscales: (1) speech in noise (corresponding to SHQ subscales 7 and 8), (2) localization of voice sounds from behind, (3) speech in quiet (corresponding to SHQ subscale 1), (4) localization of everyday sounds, and (5) localization of voices and music (corresponding to parts of the SHQ localization subscale). Correlations between SSQ subscales and SHQ factors identified the greatest correlations between SHQ factors 2, 4, and 5 and SSQ spatial subscales, whereas SHQ factor 1 had the greatest correlation with SSQ_speech. SHQ and SSQ scores were similar, whether in NH subjects (8.5 versus 8.4) or in HI subjects (6.6 for both), sharing more than 80% of variance. The SHQ localization subscale gave similar scores as the SSQ spatial subscale, sharing more than 75% of variance. Construct validity identified better ear PTA and PTA asymmetry as the two main predictors of SHQ scores, to a degree similar to that seen for the SSQ. The SHQ was shorter, easier to read and less sensitive to the number of years of formal education than the SSQ, but this came at a cost of ecological validity, which was rated higher for the SSQ than for the SHQ. Conclusions A comparison of factor analysis outcomes among the English, Dutch, and French versions of the SHQ confirmed good conceptual equivalence across languages and robustness of the SHQ for use in international settings. In addition, SHQ and SSQ scores showed remarkable similarities, suggesting the possibility of extrapolating the results from one questionnaire to the other. Although the SHQ was originally designed in a population of cochlear implant patients, the present results show that its usefulness could easily be extended to noncochlear-implanted, HI subjects.

To identify and quantify sources of variability in scores on the speech, spatial, and qualities of hearing scale (SSQ) and its short forms among normal-hearing and hearing-impaired subjects using a French-language version of the SSQ.Multi-regression analyses of SSQ scores were performed using age, gender, years of education, hearing loss, and hearing-loss asymmetry as predictors. Similar analyses were performed for each subscale (Speech, Spatial, and Qualities), for several SSQ short forms, and for differences in subscale scores.One hundred normal-hearing subjects (NHS) and 230 hearing-impaired subjects (HIS).Hearing loss in the better ear and hearing-loss asymmetry were the two main predictors of scores on the overall SSQ, the three main subscales, and the SSQ short forms. The greatest difference between the NHS and HIS was observed for the Speech subscale, and the NHS showed scores well below the maximum of 10. An age effect was observed mostly on the Speech subscale items, and the number of years of education had a significant influence on several Spatial and Qualities subscale items.Strong similarities between SSQ scores obtained across different populations and languages, and between SSQ and short forms, underline their potential international use.

Objectives To evaluate the relation between ventilation tube insertion, otitis media with effusion duration and otologic outcomes in unilateral cleft lip and/or cleft palate children from infancy to teenage age. Design and population Retrospective longitudinal charts review of patients from the multidisciplinary cleft team of the University Hospital of Lausanne over a 30-year period. 146 charts from consecutive patients with non-syndromic unilateral cleft lip and/or cleft palate who were born between January 1986 and January 2003 were included. Results The earlier in life a cleft child experience his first otitis media with effusion (OME), the worse his long-term hearing will be. Along with the age of onset of OME, we disclosed an influence of the duration of OME without ventilation tube (VT) insertion on short and long-term hearing outcomes. Different patterns were observed between cleft palate (CP) and cleft lip palate children (CLP), with a higher incidence of otitis media with effusion for the CLP group than the CP group. Direct positive relationship between VT insertion and hearing were disclosed and evaluation of long-term complications did not reveal significant relation with VT insertion. Of note, OME in CLP children led to a higher rate (but not statistically significant) of chronic ear complications than in the CP group, that may indicate more persistent OME or different adverse effect on the middle ear mucosa between CP and CLP children. Conclusions Individualized counseling should take into account different factors such as the type of cleft, the age of onset of OME and duration of OME, keeping in mind the adverse effect of persistent middle ear fluid. In the present report, results prone an early ventilation tube insertion to prevent short and long-term injury to the middle ear homeostasis, hearing loss and related issues.

Hearing loss has adverse developmental, cognitive and social impacts on individual’s lives. To improve its diagnosis and remediation outcomes, development of objective hearing methods offer new ways of optimizing care strategy. Complementary to the classical click-evoked brainstem and cortical responses, interest grows regarding the speech auditory brainstem responses, whose components, namely the onset and frequency following responses, are proposed as biomarkers of speech encoding at the brainstem level. To improve the understanding of auditory processing in the human brainstem and its effect on cortical processing, we used a multimodal set-up and recorded brainstem and cortical potentials in response to click stimuli and speech stimuli presented at different stimulation intensities in normal hearing adults. We hypothesized that even though click- and onset responses of the speechauditory brainstem responses share some similarities; their underlying mechanisms are in some point distinct. We also presumed that using a noninvasive method we could assess the generators underlying click, onset and frequency following responses and the time wise influence of brainstem encoding on cortical processing of click and speech stimuli. Results showed evidence regarding mechanisms underlying onset response and frequency following response components at the brainstem level. A direct time wise relationship between subcortical encoding and cortical encoding was revealed: cortical activity in the left cortex was related to the onset response latency at the brainstem level. These results highlight the potential use of new methods in speech processing electroencephalographic studies and provide qualitative and topographical data regarding subcortical and cortical auditory processing network.

Hypothesis Investigation of differential protein expression will provide clues to pathophysiology in otosclerosis. Background Otosclerosis is a bone remodeling disorder limited to the endochondral layer of the otic capsule within the temporal bone. Some authors have suggested an inflammatory etiology for otosclerosis resulting from persistent measles virus infection involving the otic capsule. Despite numerous genetic studies, implication of candidate genes in the otosclerotic process remains elusive. We employed liquid chromatography-mass spectrometry (LC-MS) analysis on formalin-fixed celloidin-embedded temporal bone tissues for postmortem investigation of otosclerosis. Methods Proteomic analysis was performed using human temporal bones from a patient with severe otosclerosis and a control temporal bone. Sections were dissected under microscopy to remove otosclerotic lesions and normal otic capsule for proteomic analysis. Tandem 2D chromatography mass spectrometry was employed. Data analysis and peptide matching to FASTA human databases was done using SEQUEST and proteome discoverer software. Results TGFβ1 was identified in otosclerosis but not in the normal control temporal bone specimen. Aside from TGFβ1, many proteins and predicted cDNA-encoded proteins were observed, with implications in cell death and/or proliferation pathways, suggesting a possible role in otosclerotic bone remodeling. Immunostaining using TGFβ1 monoclonal revealed marked staining of the spongiotic otosclerotic lesions. Conclusions Mechanisms involved in cochlear extension of otosclerosis are still unclear, but the implication of TGFβ1 is supported by the present proteomic data and immunostaining results. The established role of TGFβ1 in the chondrogenesis process supports the theory of a reaction targeting the globulae interossei within the otic capsule.

A retropharyngeal abscess is a collection of pus located behind the posterior pharyngeal wall. Main symptoms include torticoli, pyrexia and odynodysphagia. It can be secondary to upper airway infections, pharyngeal penetrating trauma or idiopathic. Complications are rare, but may be life-threatening due to airway obstruction or infection's spread to the surrounding structures. Surgery is conducted in case of abscess2 cm on CT scan images, complication or worsening of the symptoms. This article presents a clinical case with literature review.L’abcès rétropharyngé (ARP) est une collection de pus située en arrière de la paroi pharyngée postérieure. Les symptômes d’alerte sont la présence d’un torticolis fébrile et d’une odyno-dysphagie. L’origine en est le plus souvent une infection des voies aériennes supérieures (VAS, 45% des cas en moyenne), un traumatisme pharyngé, mais parfois aucune cause évidente n’est retrouvée. Les complications sont rares, mais potentiellement graves en raison du risque d’obstruction des VAS ou de l’extension de l’infection aux structures avoisinantes. En cas d’abcès avéré, un drainage chirurgical est proposé d’emblée en cas de taille2 cm sur la tomodensitométrie (grand axe), en cas de complication ou d’évolution secondairement défavorable. Cet article présente un cas clinique avec revue de la littérature.

Velopharyngeal insufficiency (VPI) represents an incomplete closure between the soft palate and the posterior pharyngeal wall. Its etiology can be anatomical (cleft palate), neurologic, or iatrogenic (after adenoidectomy). The evaluation of a VPI begins with a through speech and language assessment and can be complemented by instrumental investigations. VPI treatment relies on its early identification, followed by a specific speech therapy management. Surgery is performed in case of no improvement with speech therapy or in case of an anatomical defect not allowing the child to improve. IVP management requires a multidisciplinary team.L’insuffisance vélo-pharyngée (IVP) désigne un défaut d’occlusion entre le voile du palais et la paroi postérieure du pharynx. Son étiologie peut être d’ordres anatomique (fente palatine), neurologique ou iatrogène (adénoïdectomie). L’évaluation de l’IVP débute par un bilan phoniatrique et orthophonique détaillé et peut être complétée par des explorations instrumentales. Son traitement repose sur une identification précoce puis une prise en charge et un suivi orthophonique spécifiques. Une chirurgie est réalisée en cas d’absence d’amélioration ou de défaut anatomique ne permettant pas à l’enfant de progresser. L’IVP est une pathologie intéressant plus particulièrement l’enfant et sa prise en charge se fait par une équipe multidisciplinaire.

The congenital cholesteatoma is a rare benign tumor whose diagnosis is mainly made during childhood. Otoscopic examination shows a white retrotympanic collection with or without hearing loss. In case of a late diagnosis, the disease will spread to the surrounding structures, leading to ossicules destruction, facial palsy, sensorineural hearing loss and vestibular dysfunction. Surgery is the therapeutic gold-standard, with different techniques chosen based on the cholesteatoma's extension. Patient should benefit from a long term medical and radiological (MRI) follow-up.Le cholestéatome congénital est une tumeur bénigne rare mais dont la fréquence augmente de par une optimisation du diagnostic, celui-ci étant de plus en plus précoce. Le diagnostic est souvent posé devant une masse blanchâtre rétrotympanique visible en otoscopie associée ou non à un trouble auditif. En cas de diagnostic tardif, l’extension de la maladie peut engendrer de graves complications nerveuses (faciales), auditives et vestibulaires. Sa prise en charge est chirurgicale, le type de technique étant guidé par le stade d’extension du cholestéatome. Le suivi post-thérapeutique va bénéficier de l’apport de l’IRM et se déroulera sur le moyen et long terme.

Little is known about middle and inner ear development during the second and third parts of human fetal life. Using ultra-high resolution Microcomputed Tomography coupled with bone histology, we performed the first quantitative middle and inner ear ossification/mineralization evaluation of fetuses between 17 and 39 weeks of gestational age. We show distinct ossification paces between ossicles, with a belated development of the stapes. A complete cochlear bony covering is observed within the time-frame of the onset of hearing, whereas distinct time courses of ossification for semicircular canal envelopes are observed in relation to the start of vestibular functions. The study evidences a spatio-temporal relationship between middle and inner ear structure development and the onset of hearing and balance, critical senses for the fetal adaptation to birth.

Resume Buts Comparer les resultats obtenus dans la chirurgie du cholesteatome en technique fermee (TF), par l’utilisation de cartilage ou d’une PORP en hydroxylapatite (HA) positionne sur la tete de l’etrier et analyser l’influence de l’ablation du marteau et celle du renforcement total du tympan avec du cartilage. Patients et methodes Etude retrospective de 128 cas (99 adultes, 29 enfants) operes entre 2003 et 2012 d’un cholesteatome en TF ayant beneficie de la mise en place de cartilage (90 cas) ou d’une PORP (38 cas) sur la tete de l’etrier. Les resultats audiometriques ont ete analyses selon les criteres du BIAP (Bureau international d’audiophonologie) et ont fait l’objet d’une comparaison statistique. Toutes les ossiculoplasties ont ete effectuees lors du 1 er temps operatoire ; seuls 39 % des patients ont ete repris chirurgicalement. Le marteau a ete conserve dans 79 cas et sacrifie dans 49 cas. Resultats Avec 2 ans de recul, le Rinne residuel moyen etait de 16,8 dB dans le groupe cartilage (gain de 7,6 dB ; p = 0,001) et de 15,8 dB dans le groupe PORP(gain de 8,5 dB ; p = 0,002). Le Rinne etait inferieur a 20 dB dans 67,6 % des cas du groupe cartilage et 70,4 % du groupe PORP. Aucune difference statistique n’a ete retrouvee entre les 2 techniques. Il n’a pas ete note de difference significative lorsque le marteau avait ete conserve ou sacrifie aussi bien dans le groupe cartilage que dans le groupe PORP. Conclusion Ces resultats confirment l’interet de l’utilisation du cartilage place sur la tete de l’etrier comme technique d’ossiculoplastie dans les cholesteatomes operes en TF, donnant des resultats comparables a ceux obtenus par l’utilisation d’une PORP. L’ablation du marteau n’a pas entraine de diminution de la qualite des resultats auditifs obtenus. Le renforcement total de la membrane tympanique par du cartilage a semble diminuer la frequence des recidives des cholesteatomes (8,5 %).

Le role de la longueur des telomeres a ete largement etudie en cancerologie et dans plusieurs pathologies regroupees sous le terme de « telomeropathies » mais reste meconnu dans le developpement embryonnaire et la survenue de malformations congenitales. Plusieurs etudes ont montre un raccourcissement des telomeres dans les villosites choriales en cas de retard de croissance intra-uterin mais a ce jour, une seule etude a decrit l’association de telomeres courts avec des malformations congenitales [1] . Dans notre etude nous avons mesure la longueur des telomeres par PCR quantitative (Light cycler 480, technique SYBR Green I), a partir d’ADN extrait en direct sur des prelevements de liquide amniotique (n = 55) et de villosites choriales (n = 32) de fœtus « sains », hypotrophes ( [2] mais contrairement a ce qui avait ete decrit dans trois autres etudes [3] , [4] , [5] .

Resume Objectifs Les auteurs analysent la pertinence diagnostique predictive de la ponction cytologique et de l’analyse histopathologique extemporanee chez des patients adultes operes d’une pathologie nodulaire de la glande thyroide. Patients et methodes Pour chacune des pieces operatoires, le meme medecin anatomopathologiste a effectue une analyse macroscopique, une etude cytologique puis un examen histopathologique extemporane. Les resultats de la cytologie ont ete exprimes en trois groupes : cytologie benigne, maligne ou suspecte avec atypies. L’examen extemporane a ete donne en trois categories : lesion benigne, maligne ou suspecte car ne possedant pas tous les criteres de malignite. Resultats Sur 202 patients inclus, 166 sont des femmes (82 %). La moyenne d’âge est de 51 ans. Vingt-deux pour cent des femmes et 25 % des hommes de la population sont porteurs d’un carcinome thyroidien sur l’histopathologie definitive. La cytologie montre 85 % de lesions benignes, 9 % de lesions malignes et 6 % de cellules suspectes ou atypiques. Sa specificite est de plus de 99 % ; sa sensibilite en incluant et excluant les microcarcinomes est respectivement de 36 % et 48 %. Sa pertinence diagnostique est de 84 et 89 % en excluant les microcarcinomes. L’histopathologie extemporanee revele 85 % de lesions benignes, 13 % de lesions malignes et 2 % de lesions suspectes. Sa specificite est de plus de 99 % ; sa sensibilite en incluant et excluant les microcarcinomes est de 56 et 68 %. Sa pertinence diagnostique est de 89 et de 90 % en excluant les microcarcinomes. La pertinence diagnostique de l’association des deux examens est de 94 % en excluant les microcarcinomes. Conclusion La cytologie et l’histopathologie extemporanee ont une pertinence predictive diagnostique comparable. L’examen extemporane demande par l’operateur depend non seulement de la cytologie preoperatoire, surtout lorsqu’elle est de nature suspecte, voire indeterminee, mais aussi de ses constatations macroscopiques chirurgicales.

The purpose of the study was to determine the relationships between the extracranial glossopharyngeal (IX) nerve and the muscles of the styloid diaphragm. In humans, the IX nerve is a hidden retrostyloid nerve which plays a critical role notably in swallowing and has to be preserved during infratemporal fossa and parapharyngeal spaces surgical procedures. In ten adult heads from cadavers (20 sides) fixed in formalin, dissection of the extracranial IX nerve was performed under operating microscope with special attention given to the relationships between this nerve and the styloid muscles of the styloid diaphragm. The three styloid muscles delimit three triangular intermuscular intervals which were each thoroughly explored. Different osseous landmarks were investigated for easy nerve location. The styloid process (SP) is the main superior osseous landmark for the three muscles of the styloid diaphragm. The stylohyoid muscle (SHM) is anteromedially located to the posterior belly of the digastric muscle. The styloglossus muscle (SGM) is medial and anterior to the SHM. The stylopharyngeal muscle (SPM) is the most vertical and medial of the three styloid muscles. It courses from the medial surface of the SP in a deep plane hidden between the SHM and the SGM. The extracranial IX nerve turns around the SPM superiorly with a vertical segment posterior to the SPM and inferiorly with a horizontal segment lateral to the SPM. The meeting point of the two segments of the IX nerve is about 10 mm anteriorly located from the transverse process of the atlas. The external carotid artery and some of its branches lie in contact with the lateral side of the IX nerve. Such relationships between the extracranial IX nerve, the styloid muscles and the transverse process of the atlas should be appreciated by clinician who treats patients with stylohyoid complex syndromes and by the surgeon for the parapharyngeal spaces approach.

Resume La parathyroidectomie ciblee endoscopique sans insufflation de gaz apparait comme un geste peu invasif pour la decouverte et l’exerese d’un adenome parathyroidien lors des hyperparathyroidies primaires sporadiques. Ce geste technique regle est tributaire de la qualite de l’imagerie preoperatoire avec une echographie cervicale et une scintigraphie au Sestamibi. La mise en place d’un « harpon » echo-guide preoperatoire, l’analyse rapide de l’hormone parathyroidienne pendant l’intervention permettent d’optimiser le geste. Les echecs de cette chirurgie ciblee varient entre 1,7 % et 4 % des operes.

Resume Buts Rapporter les resultats obtenus chez des patients porteurs d’une tympanosclerose a tympan ferme bloquant l’articulation stapedo-vestibulaire, operes d’une ouverture labyrinthique et en discuter l’opportunite. Patients et methodes Etude retrospective de 28 cas de tympanosclerose bloquant l’articulation stapedo-vestibulaire operes entre 1982 et 2010. L’ouverture labyrinthique a ete realisee a l’aide d’un laser CO2 et le retablissement de l’effet columellaire effectue soit par platinectomie interposition et prothese Total Ossicular Replacement Prothesis (TORP) soit par stapedotomie avec mise en place d’un piston accroche sur la longue apophyse de l’enclume (LAE) ou le manche du marteau (MM). Resultats En postoperatoire (deux mois a six mois), la mise en place d’une TORP sur platinectomie interposition a procure une amelioration du Rinne moyen de 18 dB et la mise en place d’un piston accroche a la LAE ou au MM place dans un orifice de stapedotomie de 14 dB. Avec trois ans de recul, les benefices se sont maintenus avec la premiere technique mais se sont averes en revanche non statistiquement significatifs avec la seconde. Aucune cophose ni labyrinthisation superieure a 20 dB n’est a deplorer. Conclusion La realisation d’une ouverture labyrinthique apporte un benefice auditif immediat aux patients porteurs de tympanosclerose bloquant l’articulation stapedo-vestibulaire avec les deux techniques utilisees. A plus long terme, les resultats se maintiennent avec la technique de platinectomie interposition TORP ; le gain auditif est en revanche non statistiquement significatif a long terme avec la technique de stapedotomie du fait d’un nombre de cas trop reduit ; seule une etude comportant un nombre plus important de patients permettrait d’affirmer la superiorite de la premiere technique sur la seconde. Cette ouverture labyrinthique qui ne comporte qu’un faible risque de labyrinthisation et n’a engendre aucune cophose ne doit cependant etre realisee qu’en cas de surdite de transmission bilaterale importante, a tympan ferme, chez des patients refusant une prise en charge prothetique et avertis du risque d’aggravation auditive.