G. Le Maner-Idrissi

Abstract: Objectives With more children receiving cochlear implants during infancy, there is a need for validated assessments of pre-verbal and early verbal auditory skills. The LittlEARS® Auditory Questionnaire is presented here as the first module of the LittlEARS® test battery. The LittlEARS® Auditory Questionnaire was developed and piloted to assess the auditory behaviour of normal hearing children and hearing impaired children who receive a cochlear implant or hearing aid prior to 24 months of age. This paper presents results from two studies: one validating the LittlEARS® Auditory Questionnaire on children with normal hearing who are German speaking and a second validating the norm curves found after adaptation and administration of the questionnaire to children with normal hearing in 15 different languages. Methods Scores from a group of 218 German and Austrian children with normal hearing between 5 days and 24 months of age were used to create a norm curve. The questionnaire was adapted from the German original into English and then 15 other languages to date. Regression curves were found based on parental responses from 3309 normal hearing infants and toddlers. Curves for each language were compared to the original German validation curve. Results The results of the first study were a norm curve which reflects the age-dependence of auditory behaviour, reliability and homogeneity as a measure of auditory behaviour, and calculations of expected and critical values as a function of age. Results of the second study show that the regression curves found for all the adapted languages are essentially equal to the German norm curve, as no statistically significant differences were found. Conclusions The LittlEARS® Auditory Questionnaire is a valid, language-independent tool for assessing the early auditory behaviour of infants and toddlers with normal hearing. The results of this study suggest that the LittlEARS® Auditory Questionnaire could also be very useful for documenting children's progress with their current amplification, providing evidence of the need for implantation, or highlighting the need for follow-up in other developmental areas.

Abstract: Resume Introduction Le questionnaire EAS ( Buss & Plomin, 1984 ) permet la mesure de quatre dimensions heritables et relativement stables du temperament : Activite, Emotivite, Sociabilite et Timidite. Objectif Une traduction francaise de cet outil a fait l’objet d’une etude de validation chez des enfants âges de six a 12 ans ( Gasman et al., 2002 ), notre objectif est d’etendre le processus de validation a un groupe d’enfants plus jeunes. Methode Le temperament de 350 enfants âges entre deux et cinq ans et de 290 enfants âges entre six et neuf ans a ete evalue a l’aide de deux questionnaires : l’EAS et le DOTS-R (versions parent). Resultats Des analyses factorielles confirmatoires multi-groupes ont permis de verifier l’invariance de mesure et l’invariance structurale de l’EAS (modele en quatre facteurs) sur les deux echantillons et ont montre que les scores d’echelles constituent de bonnes approximations des scores factoriels. Par ailleurs, si nous n’avons pas observe de differences significatives liees au sexe dans les scores de temperament, les enfants les plus âges sont en moyenne moins actifs et plus emotifs que les enfants les plus jeunes. Enfin, la coherence interne et la validite concordante de chaque echelle se sont revelees satisfaisantes. Conclusion L’EAS (version parent) peut donc etre utilise des l’âge de deux ans, facilitant ainsi l’etude du developpement du temperament dans l’enfance.

Abstract: Le but de ce travail est de decrire comment les meres de jumeaux se representent precocement la relation entre les deux enfants et d'examiner s'il existe des influences mutuelles entre les perceptions des meres, leurs comportements et attitudes educatives vis-a-vis de la gemellite et le debut du developpement verbal des enfants. Les donnees portent sur 32 familles de jumeaux (monozygotes, dizygotes de meme sexe et dizygotes de sexe different), rencontrees deux ans apres la naissance pour un entretien semi-structure avec la mere. L'entretien a ete analyse a l'aide d'une grille composee de deux echelles : l'echelle des attitudes et pratiques educatives des meres relatives a la differenciation (degre de similitude/difference et personnalisation des vetements et des jouets des enfants); l'echelle des representations maternelles du lien gemellaire (description de la structure du lien entre les enfants selon cinq dimensions : complicite, rivalite, relations agonistiques, dominance et personnalite). L'evaluation du developpement verbal a ete realisee au moyen d'une adaptation du Brunet-Lezine. Les resultats sont presentes en mettant en relation les indicateurs de developpement verbal, la typologie des meres relative a leurs attitudes et pratiques envers la gemellite et celle relative a leurs representations du lien fraternel gemellaire. Ils sont egalement examines en fonction du type de gemellite.

Abstract: Resume La strategie nationale « Autisme au sein des troubles neurodeveloppementaux » preconise la mise en place de dispositifs de soutien et d’accompagnement pour les familles. En effet, les familles d’un enfant presentant un handicap, et plus particulierement un trouble du spectre de l’autisme (TSA), doivent regulierement s’adapter au trouble et a ses manifestations. Le concept de qualite de vie permet d’interroger les difficultes des familles du point de vue des parents et des fratries. Peu de travaux ont cependant evalue la qualite de vie des freres et sœurs, leurs besoins et les effets des accompagnements parfois mis en place. Nous avons realise une revue de la litterature sur la qualite de vie des fratries d’enfants presentant un TSA (bases de donnees : PubMed, PsycINFO et Google Scholar). Nous avons recense 53 etudes publiees entre 1993 et 2019. Les recherches font apparaitre des resultats tres contrastes quant a l’impact du TSA sur la qualite de vie des fratries. Nous presentons ici les resultats, un tiers des etudes concluant a une absence d’impact, un autre tiers observant des effets globalement negatifs, le tiers restant faisant apparaitre des effets mixtes, negatifs et positifs. Les recherches soulignent l’effet particulier des difficultes comportementales des enfants avec un TSA sur le vecu de ses freres et sœurs. Les etudes presentent egalement des differences methodologiques importantes, avec des approches qualitatives ou quantitatives, des differences dans le mode de recueil de donnees et le choix de la dimension comparative. Au plan theorique, les concepts retenus pour mesurer le bien-etre des fratries sont differents selon les etudes. La plupart s’interesse surtout a l’ajustement social et comportemental des fratries, qui n’est qu’un aspect de la qualite de vie.

Abstract: Genetic factors, the most common etiology in severe to profound hearing loss, are one of the key determinants of Cochlear Implantation (CI) and Electric Acoustic Stimulation (EAS) outcomes. Satisfactory auditory performance after receiving a CI/EAS in patients with certain deafness gene mutations indicates that genetic testing would be helpful in predicting CI/EAS outcomes and deciding treatment choices. However, because of the extreme genetic heterogeneity of deafness, clinical application of genetic information still entails difficulties. Target exon sequencing using massively parallel DNA sequencing is a new powerful strategy to discover rare causative genes in Mendelian disorders such as deafness. We used massive sequencing of the exons of 58 target candidate genes to analyze 8 (4 early-onset, 4 late-onset) Japanese CI/EAS patients, who did not have mutations in commonly found genes including GJB2, SLC26A4, or mitochondrial 1555A>G or 3243A>G mutations. We successfully identified four rare causative mutations in the MYO15A, TECTA, TMPRSS3, and ACTG1 genes in four patients who showed relatively good auditory performance with CI including EAS, suggesting that genetic testing may be able to predict the performance after implantation.

Abstract: This study proposed and evaluated a guideline for outcome evaluation for infants and children with hearing loss who wear hearing aids. The University of Western Ontario Pediatric Audiological Monitoring Protocol (UWO PedAMP) was developed following a critical review of pediatric outcome evaluation tools and was systematically examined by the Network of Pediatric Audiologists of Canada. It consists of tools to gather clinical process outcomes as well as functional caregiver reports. The UWO PedAMP was administered to a clinical population of infants and children with hearing aids. Sixty-eight children were administered the functional outcome evaluation tools (i.e., caregiver reports) a total of 133 times. Clinical process outcomes of hearing aid verification (e.g., real-ear-to-coupler difference) revealed typical aided audibility (e.g., Speech Intelligibility Index). Results for the LittlEARS(®) questionnaire revealed that typically developing children with hearing loss who wear hearing aids are meeting auditory development milestones. Children with mild to moderate comorbidities displayed typical auditory development during the 1st year of life after which development began to decline. Children with complex factors related to hearing aid use had lower scores on the LittlEARS, but auditory development was in parallel to norms. Parents' Evaluation of Aural/Oral Performance (PEACH) results indicated no age effect on scoring for children above 2 years of age; however, the effect of degree of hearing loss was significant. This work provides clinicians with a systematic, evidence-based outcome evaluation protocol to implement as part of a complete pediatric hearing aid fitting.

Abstract: Objectives: Progress has been made in recent years in the provision of amplification and early intervention for children who are hard of hearing. However, children who use hearing aids (HAs) may have inconsistent access to their auditory environment due to limitations in speech audibility through their HAs or limited HA use. The effects of variability in children’s auditory experience on parent-reported auditory skills questionnaires and on speech recognition in quiet and in noise were examined for a large group of children who were followed as part of the Outcomes of Children with Hearing Loss study. Design: Parent ratings on auditory development questionnaires and children’s speech recognition were assessed for 306 children who are hard of hearing. Children ranged in age from 12 months to 9 years. Three questionnaires involving parent ratings of auditory skill development and behavior were used, including the LittlEARS Auditory Questionnaire, Parents Evaluation of Oral/Aural Performance in Children rating scale, and an adaptation of the Speech, Spatial, and Qualities of Hearing scale. Speech recognition in quiet was assessed using the Open- and Closed-Set Test, Early Speech Perception test, Lexical Neighborhood Test, and Phonetically Balanced Kindergarten word lists. Speech recognition in noise was assessed using the Computer-Assisted Speech Perception Assessment. Children who are hard of hearing were compared with peers with normal hearing matched for age, maternal educational level, and nonverbal intelligence. The effects of aided audibility, HA use, and language ability on parent responses to auditory development questionnaires and on children’s speech recognition were also examined. Results: Children who are hard of hearing had poorer performance than peers with normal hearing on parent ratings of auditory skills and had poorer speech recognition. Significant individual variability among children who are hard of hearing was observed. Children with greater aided audibility through their HAs, more hours of HA use, and better language abilities generally had higher parent ratings of auditory skills and better speech-recognition abilities in quiet and in noise than peers with less audibility, more limited HA use, or poorer language abilities. In addition to the auditory and language factors that were predictive for speech recognition in quiet, phonological working memory was also a positive predictor for word recognition abilities in noise. Conclusions: Children who are hard of hearing continue to experience delays in auditory skill development and speech-recognition abilities compared with peers with normal hearing. However, significant improvements in these domains have occurred in comparison to similar data reported before the adoption of universal newborn hearing screening and early intervention programs for children who are hard of hearing. Increasing the audibility of speech has a direct positive effect on auditory skill development and speech-recognition abilities and also may enhance these skills by improving language abilities in children who are hard of hearing. Greater number of hours of HA use also had a significant positive impact on parent ratings of auditory skills and children’s speech recognition.

Abstract: Objective This study had two aims: (1) to document the auditory and lexical development of children who are deaf and received the first cochlear implant (CI) by the age of 16 months and the second CI by the age of 31 months and (2) to compare these children's results with those of children with normal hearing (NH) Methods This longitudinal study included five children with NH and five with sensorineural deafness All children of the second group were observed for 36 months after the first fitting of the device (cochlear implant) The auditory development of the CI group was documented every 3 months up to the age of two years in hearing age and chronological age and for the NH group in chronological age The language development of each NH child was assessed at 12, 18, 24 and 36 months of chronological age Children with CIs were examined at the same age intervals at chronological and hearing age Results In both groups, children showed individual patterns of auditory and language development The children with CIs developed differently in the amount of receptive and expressive vocabulary compared with the NH control group Three children in the CI group needed almost 6 months to make gains in speech development that were consistent with what would be expected for their chronological age Overall, the receptive and expressive development in all children of the implanted group increased with their hearing age Conclusion These results indicate that early identification and early implantation is advisable to give children with sensorineural hearing loss a realistic chance to develop satisfactory expressive and receptive vocabulary and also to develop stable phonological, morphological and syntactical skills for school life On the basis of these longitudinal data, we will be able to develop new diagnostic tools that enable clinicians to assess child's progress in hearing and speech development

Abstract: Background and Aims Newborn hearing screening and early intervention for congenital hearing loss have created a need for tools assessing the hearing development of very young children. A multidisciplinary evaluation of children's development is now becoming standard in clinical practice, though not many reliable diagnostic instruments exist. For this reason, the LittlEARS ® Auditory Questionnaire (LEAQ) was created to assess the auditory skills of a growing population of infants and toddlers who receive hearing instruments. The LEAQ relies on parent report, which has been shown to be a reliable way of assessing child development. Results with this tool in a group of children who received very early cochlear implantation are presented. Methods The LEAQ is the first module of the LittlEARS ® comprehensive test battery for children under the age of two who have normal hearing (NH), cochlear implants (CIs) or hearing aids (HAs). The LEAQ is a parent questionnaire comprised of 35 “yes/no” questions which can be completed by parents in less than 10 min. Sixty-three children who received unilateral CIs at a young age were assessed longitudinally and their performance was compared to that of a NH group. Results All CI children reached the maximum possible score on the LEAQ on average by 22 months of hearing age, i.e. 38 months of chronological age. In comparison, the NH group reached the maximum score by 24 months of age demonstrating that auditory skills of CI children often develop quicker than those of NH children. In the two comparison groups of children aged (a) younger and older than 12 months, and (b) between 6–9 and 21–24 months at first fitting, the early implanted children reached the highest scores faster than the later implanted children. Furthermore, three children with additional needs were tested. They showed slower growth over time but also received benefits from early implantation. Conclusions The LEAQ is a quick and effective tool for assessing auditory skills of very young children with or without hearing loss. In our study, the auditory skills of children with CI progressed very quickly after implantation and were comparable with those of NH peers.