Communicative deficits associated with maladaptive behavior in individuals with deafness and special needs

BackgroundAt least one in three individuals who are prelingually deaf has special needs, most commonly due to intellectual disabilities. The scant literature on challenging behavior in this population, however, suggests high rates of prevalence and an important need to better understand the contributing factors.AimWe sought to analyze the prevalence of maladaptive behavior and its association with intellectual functioning, adaptive skills, language skills, and social communication in a population of adults with deafness and special needs.MethodsParticipants were 61 individuals from three therapeutic living communities established for people with deafness and special needs. The participants had a mean age of 54.7 years, 64% were male. Intellectual functioning was measured with two versions of the Snijders–Oomen Non-verbal Intelligence Scale. The Vineland-II Scales were used to assess adaptive and maladaptive behavior. Language skills were measured with instruments specifically adapted for this population, including the Reynell Developmental Language Comprehension Scale, the comprehension scale of the Child Development Inventory, and the Profile of Multiple Language Proficiencies. Due to high correlations between instruments, a composite language score was used. A specific questionnaire to measure social communication in adults with intellectual disabilities was also utilized.ResultsThe mean nonverbal developmental reference age was 6.5 years, whereas the equivalent for the language measures was about 3.5 years. The prevalence rate of elevated maladaptive behavior was 41% (v-scale score ≥18) and 18% of the participants had a clinically significant score (v-scale score ≥21). Regression analyses showed that only language and social communication skills were significantly associated with maladaptive behavior, while intellectual functioning and adaptive skills were not.ConclusionThese findings emphasize the importance of the constant promotion of communicative skills, as those people with better language and social communication skills demonstrate lower levels of maladaptive behavior

Research gaps in the neurodevelopmental assessment of children with complex congenital heart defects: a scoping review

BackgroundChildren with congenital heart defects (CHD) are at risk for a range of developmental disabilities that challenge cognition, executive functioning, self-regulation, communication, social-emotional functioning, and motor skills. Ongoing developmental surveillance is therefore key to maximizing neurodevelopmental outcome opportunities. It is crucial that the measures used cover the spectrum of neurodevelopmental domains relevant to capturing possible predictors and malleable factors of child development.ObjectivesThis work aimed to synthesize the literature on neurodevelopmental measures and the corresponding developmental domains assessed in children aged 1−8 years with complex CHD.MethodsPubMed was searched for terms relating to psycho-social, cognitive and linguistic-communicative outcomes in children with CHD. 1,380 papers with a focus on complex CHD that reported neurodevelopmental assessments were identified; ultimately, data from 78 articles that used standardized neurodevelopmental assessment tools were extracted.ResultsThirty-nine (50%) of these excluded children with syndromes, and 9 (12%) excluded children with disorders of intellectual development. 10% of the studies were longitudinal. The neurodevelopmental domains addressed by the methods used were: 53% cognition, 16% psychosocial functioning, 18% language/communication/speech production, and 13% motor development-associated constructs.ConclusionsData on social communication, expressive and receptive language, speech motor, and motor function are underrepresented. There is a lack of research into everyday use of language and into measures assessing language and communication early in life. Overall, longitudinal studies are required that include communication measures and their interrelations with other developmental domains

ANIMAQU - eine computerbasierte Befragung als Möglichkeit zur Erfassung besonderer Zielgruppen: ein Beispiel einer Anwendung bei gehörlosen Menschen

'Die Anwendung einer schriftlichen standardisierten Befragung ist an bestimmte Voraussetzungen der interviewten Personen gebunden. Insbesondere stellt ausreichende Lesekompetenz eine zentrale Anwendungsvoraussetzung dar. Für gehörlose Menschen - d.h. Mitglieder der Gehörlosenkultur - ist die Gebärdensprache die eindeutig bevorzugte Kommunikationsform. Die Lesekompetenz liegt im Durchschnitt unter der der Normalbevölkerung und weist eine große Variationsbreite auf. Es wird daher eine computergestützte Erhebungsform präsentiert, welche die selbstadministrierte Befragung dieser Zielgruppe ermöglicht.' (Autorenreferat)'Standardized paper-and-pencil interviews are not suitable for administration with every population or with every individual within a population. Respondents require to be literate, that is, able to read the language used for the interview. Severely hearing impaired people who have been deaf from an early age generally use sign language to communicate and their level of reading ability in the national language is generally lower than of the population at large and may also vary greatly among the deaf population. The paper describes a computer-assisted form of administration which allows these deaf respondents to self-complete an interview.' (author's abstract)

Different Binding Properties and Function of CXXC Zinc Finger Domains in Dnmt1 and Tet1

Several mammalian proteins involved in chromatin and DNA modification contain CXXC zinc finger domains. We compared the structure and function of the CXXC domains in the DNA methyltransferase Dnmt1 and the methylcytosine dioxygenase Tet1. Sequence alignment showed that both CXXC domains have a very similar framework but differ in the central tip region. Based on the known structure of a similar MLL1 domain we developed homology models and designed expression constructs for the isolated CXXC domains of Dnmt1 and Tet1 accordingly. We show that the CXXC domain of Tet1 has no DNA binding activity and is dispensable for catalytic activity in vivo. In contrast, the CXXC domain of Dnmt1 selectively binds DNA substrates containing unmethylated CpG sites. Surprisingly, a Dnmt1 mutant construct lacking the CXXC domain formed covalent complexes with cytosine bases both in vitro and in vivo and rescued DNA methylation patterns in dnmt1−/− embryonic stem cells (ESCs) just as efficiently as wild type Dnmt1. Interestingly, neither wild type nor ΔCXXC Dnmt1 re-methylated imprinted CpG sites of the H19a promoter in dnmt1−/− ESCs, arguing against a role of the CXXC domain in restraining Dnmt1 methyltransferase activity on unmethylated CpG sites

Health care access among deaf people: Table 1

Access to health care without barriers is a clearly defined right of people with disabilities as stated by the UN Convention on the Rights of People with Disabilities. The present study reviews literature from 2000 to 2015 on access to health care for deaf people and reveals significant challenges in communication with health providers and gaps in global health knowledge for deaf people including those with even higher risk of marginalization. Examples of approaches to improve access to health care, such as providing powerful and visually accessible communication through the use of sign language, the implementation of important communication technologies, and cultural awareness trainings for health professionals are discussed. Programs that raise health knowledge in Deaf communities and models of primary health care centers for deaf people are also presented. Published documents can empower deaf people to realize their right to enjoy the highest attainable standard of health

Special Issue “Early Intervention for Hearing Loss in Children: Drafting from Theory to Clinical Practice”

Globally, around 34 million children are affected by disabling hearing loss [...

Multidimensional Family-Centred Early Intervention in Children with Hearing Loss: A Conceptual Model

At least two per thousand newborns are affected by hearing loss, with up to 40% with an additional disability. Early identification by universal newborn hearing screening and early intervention services are available in many countries around the world, with limited data on their effectiveness and a lack of knowledge about specific intervention-related determinants of child and family outcomes. This concept paper aimed to better understand the mechanisms by which multi-dimensional family-centred early intervention influences child outcomes, through parent behaviour, targeted by intervention by a review of the literature, primarily in the field of childhood hearing loss, supplemented by research findings on physiological and atypical child development. We present a conceptual model of influences of multi-disciplinary family-centred early intervention on family coping/functioning and parent–child interaction, with effects on child psycho-social and cognitive outcomes. Social communication and language skills are postulated as mediators between parent–child interaction and non-verbal child outcomes. Multi-disciplinary networks of professionals trained in family-centred practice and the evaluation of existing services, with respect to best practice guidelines for family-centred early intervention, are recommended. There is a need for longitudinal epidemiological studies, including specific intervention measures, family behaviours and multidimensional child outcomes