Research on Challenging Behavior in Children and Adults Who Are Deaf-Blind - Bibliography

by DB-LINK on Feb 1, 2013
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This is a partial list of materials on this topic available from the DB-LINK Catalog Database.  If you have additional questions, please contact us via email:

Updated 05/2015

Assessment and Treatment of Stereotype in an Individual with Cornelia de Lange Syndrome and Deafblindness--Lanovaz, Marc J.;Rapp, John T.;Maciw, Isabella. Journal of Intellectual and Developmental Disability, Vol. 39, #4, 2014, pp. 375-380. (2014) Individuals with Cornelia de Lange syndrome often engage in mouthing, tapping and other repetitive behaviors.  This research study aimed to identify if the researchers could reduce the behaviors of an individual without use of punishment.  They found noncontingent access to edible items reduced mouthing but the use of tactile stimuli did not. Combining noncontingent access to tactile items with differential reinforcement reduced mouthing and tapping while increasing appropriate behavior.  More research is necessary to reproduce the findings.  

Behavioral Cusps: A Person-Centered Concept for Establishing Pivotal Individual, Family, and Community Behaviors and Repertoires --Smith, Garnett J.; McDougall, Dennis; Edelen-Smith, Patricia. Focus on Autism and Other Developmental Disabilities, Vol. 21, No. 4, Winter 2006, 223-229. (2006) Cumulative-hierarchical learning (CHL) and behavior, a premise first introduced by Staats in 1975, describes how higher-level behavioral patterns and structures can emerge from interactions among a set of lower-level actions. Proponents of CHL emphasize the importance of pivotal response interventions, behavior repertoires, generative learning, and the development of behavioral cusps, the human equivalent of the computer world's killer application, or "killer app" (Wikipedia, n.d.). Rosales-Ruiz and Baer (1997) defined a behavioral cusp as an entry point for pivotal behavioral change that, once initiated, so profoundly alters, displaces, or transforms one's behavioral repertoire that it renders preexisting behavioral repertoires obsolete. In this article, we demonstrate how behavioral cusps can be used to construct CHL behaviors and repertoires of persons with autism and other pervasive developmental disorders within and across five pivotal behavioral elements. We also describe how behavioral cusps could be used to improve the quality of the collaborative conversations during person-centered planning sessions.

Behavioral Phenotype --Hartshorne, Timothy S. The ASHA Leader, October 17, 2006, p. 41. (2006) Very brief article highlighting some of the research aimed at describing a behavioral phenotype for CHARGE Syndrome. Recent research supports the presence of executive dysfunction, or problems with shifting, initiating, inhibiting, or sustaining actions based on prefrontal cortex activity. Other areas being explored include the presence of a regulatory disorder making it difficult for the child to regulate complex processes such as their sleep-wake cycles, hunger-satiety cycle, manage their emotions, etc. Research has also found indications of significant sleep disorders in well over half of these children which can have a significant impact on behavior.

The Behavioral Phenotype in CHARGE: The "B" in CHARGE --Hartshorne, Tim. Costa Mesa, CA: 8th International CHARGE Syndrome Conference, July 26-29, 2007, Costa Mesa, CA. (2007) PowerPoint Presentation. Offers a brief history of development of information about behavior and CHARGE, personal history and steps in developing a working model of a behavioral phenotype. Steps include: understanding the syndrome, observing children, review research, understand the impact of sensory deficits, parenting styles, impact of early medical issues, cognitive ability, etc. Offers a first draft of a behavioral phenotype, benefits of having one, possible intervention plans and considerations for the IEP.

Behavioral profiles and symptoms of autism in CHARGE syndrome: preliminary Canadian epidemiological data --Smith IM; Nichols SL; Issekutz K; Blake K. --Canadian Paediatric Surveillance Program. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 248-256. (2005) Individuals with CHARGE syndrome were identified through the Canadian Pediatric Surveillance Program (CPSP). From this population-based cohort (n=78), we present data on developmental and behavioral characteristics for the first 13 individuals (eight males, five females) for whom assessments are complete. Standardized parent questionnaires on development and behavior were followed by a structured telephone interview, with a specific emphasis on symptoms of autistic spectrum disorder (ASD). Preliminary results confirm that individuals with CHARGE syndrome have relatively low adaptive behavior skills, motor impairments being particularly significant. Most individuals did not present with significant behavior problems; however, evidence of ASD symptoms was judged to be moderate to strong in six of the ten children who were above the age of 4-5 years. Results are discussed with reference to the challenges inherent in the diagnosis of autism in individuals with sensory impairments, and to the implications for understanding the etiology of CHARGE syndrome and of ASD. Copyright (c) 2005 Wiley-Liss, Inc. Available on the web:

Behavioral Risk Factors And Dual Sensory Impairment Final Report --Su, Ya-ping; Brennan, Mark. --Arlene R. Gordon Research Institute, Lighthouse International. Gerontological Society of America, Annual Scientific Meeting, November 2000, Boston, MA. (2003) There is an emerging body of literature on the consequences of vision and/or hearing impairment. But relatively little is known about the behavioral risk factors for incurring such sensory impairments. The lack of established empirical relationships between behavioral risk factors and sensory impairments has seriously limited health promotion and disease prevention efforts. This study, funded by the M.C. Adams Charitable Trust, examines the concurrent relationship between sensory loss and behavioral risk factors, i.e., body mass index, exercise, smoking, drinking alcohol. Results from binary probit regression models indicate that light alcohol consumption was protective of vision, hearing and dual sensory impairments. However, either current or past smoking increased the risk of sensory impairment in later life. Higher levels of vigorous physical activities also increased the risk of sensory impairment while light physical activity decreases the risk. Obesity was associated with hearing and dual sensory loss. Implications for health promotion and disease prevention are discussed.

Behavioural Phenotypes in CHARGE Syndrome: A Study of Behavioural Aspects in People with CHARGE Syndrome --Hoevenaars-van den Boom, Marcella, A.A.; Vervload, Mathijs P.J.; Admiraal, Ronadl J. C. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation describes the aim of a study of the existence of a pattern of behavioural characteristics in people with CHARGE syndrome.

Can specific deficits in executive functioning explain the behavioral characteristics of CHARGE syndrome: a case study --Nicholas J. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 300-305. (2005) I present the case of a 12-year-old girl diagnosed with CHARGE syndrome. As an infant she had shown typical characteristics of CHARGE. Neuroimaging revealed asymmetrical ventriculomegaly. Both her teacher and parents reported academic and psychosocial problems. Neuropsychological assessment revealed significant executive dysfunction, including attention difficulties. We discuss the possible association between executive deficits and school and psychosocial problems. This case also highlights the importance of evaluating executive function in children with CHARGE, which is particularly useful in efficiently identifying needs for direct intervention. Copyright (c) 2005 Wiley-Liss, Inc. Available on the web:

Challenging Behaviour in an Adult Male With Congenital Deaf-Blindness: Analysis and Intervention --Jacobsen, Karl; Bjerkan, Bertil; Sorlie, Randi. SCANDINAVIAN JOURNAL OF DISABILITY RESEARCH, vol. 11, #3, pp. 209-220. (2009)People with severe congenital disabilities have been assessed on negatives, on what they do not have. Skill training and education of these missing abilities have been the major focus for the habilitation since emergence of the normalization ideology in the 1960s. Developmental theories and movements like quality of life and positive psychology have changed focus from training and education to well-being and other internal states in people with disabilities. This article describes how challenging behaviour vanished in a 28-year-old deaf-blind man when developmental theory was applied as the framework for his habilitation. Emotional processing and initiatives increased and the man became easier to understand for the staff. The special case of a deaf-blind man illustrates how simple a focus on internal states may slip and be exchanged for intervention dominated by demands and training. The article discusses whether the framework employed in the present intervention should be present in all kind of habilitation.

Challenging Behaviors in CHARGE Syndrome: Psychiatric and behavioral approaches to assessment and treatment --Wachtell, Lee Elizabeth; Kahng, Sung Woo. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005) Copy of powerpoint presentation. Authors review research about sensory deprivation and attachment and compare the resulting behaviors to the behavioral phenotype in CHARGE syndrome. Treatment options discussed emphasize touch and communication.

CHARGE Syndrome: Relations Between Behavioral Characteristics and Medical Conditions --Vervloed, Mathijs; Hoevenaars-van den Boom, Marella; Knoors, Harry; van Ravenswaaij, Conny; Admiraal, Ronald. American Journal of Medical Genetics, 2006, 140A:851-862. (2006) The behaviors and medical problems in 27 persons with CHARGE syndrome were studied, because it was hypothesized that their behavior might be partly dependent on the heterogeneous medical status. With the exception of more tics, cardiac surgery was associated with positive behaviors: less withdrawn behavior, better mood, and a more easy temperament. Tube feeding was also related to positive behavior, since participants with a history of tube feeding showed less intense behavior. Cerebral deficits were associated with three problem behaviors: more intense and withdrawn behavior and a worse mood. Deafblindness was associated with developmental delays in expressive and overall communication level, and recurrent middle ear infections correlated with delays in written language. Of all medical conditions, only the presence or absence of heart defects and cardiac surgery could differentiate between the participants with regard to the number of behavioral problems. Participants with heart surgery especially, had less behavior problems. The number of operations and hospitalizations was not associated with behavior, but the total length of the hospitalizations was. Long hospital stays were associated with less problem behavior, especially internalizing behaviors. Cerebral and heart problems did not result in longer hospital stays, whereas esophageal reflux did. Age effects were reflected in older participants, who showed more internalizing problems. Heart surgery and hospitalization may be protective factors, but the protection might not be the actual surgery or hospital stay, as there may be other variables that are the actual cause, such as reduced vitality or altered parent child interactions after heart surgery. The study could not confirm a significant association between medical conditions and autism found in previous studies. © 2006 Wiley-Liss, Inc. Publisher's web site:

CHARGE syndrome: 2005. --Carey JC. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 227. (2005) Editorial.The discovery in 2004 of mutations in the gene, CDH7 in 10 of 17 individuals with CHARGE establishes it as a syndrome. This makes the timing of this special issue on CHARGE even more important. These articles are particularly unique for this journal in their detailed coverage of the behavior phenotype but this is an important theme for medical geneticists involved in the management of individuals with this syndrome. Available on the web:

CHARGE syndrome: developmental and behavioral data --Souriau J; Gimenes M; Blouin C; Benbrik I; Benbrik E; Churakowskyi A; Churakowskyi B. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 278-281. (2005) Report on research conducted in France. Focus on the behavioral and developmental issues related to CHARGE. A questionnaire to be completed by families was developed and completed by 71 families. Results are discussed. Although this is only preliminary research, it does offer a description of behaviors that can be observed in a significant number of children with CHARGE. Further research is needed. Available on the web:

Charge Syndrome: Dual Sensory Impairment, Cognitive Development and Anxiety --Reau, Charlotte; Blouin, Corinne; Tap, Christine. DBI REVIEW, #33, January-June 2006, pp. 24-25. (2006) The three authors are psychologists working in France. Christine is also Headteacher of CESSA, a school for deafblind children. This is the second article of a series of three which discuss methodology, cognitive development and the first clinical observations about anxiety. This is the second article and focuses on the first cognitive results.

Charge Syndrome: Dual Sensory Impairment, Cognitive Development and Anxiety --Reau, Charlotte; Blouin, Corinne; Tap, Christine. DBI REVIEW, #39, January-June 2007, pp. 34-35. (2007) The three authors are psychologists working in France. Christine is also Headteacher of CESSA, a school for deafblind children. This is the third article of a series of three which discuss methodology, cognitive development and the first clinical observations about anxiety. In this article they discuss their clinical observations which are centered around the expression of language, the nature of anguish in their expression, the loss of objects and tactile sensitivity in place of spoken language.

Emerging Evidence from Single-Subject Research in the Field of Deaf-Blindness --Parker, Amy T.; Davidson, Roseanna; Banda, Devender R. JVIB, November 2007, Volume 101, Number 11, pp. 690-700. (2007) Professionals in the field of deaf-blindness are challenged to use instructional practices that have been tested using experimental methodology. Single-subject design has been examined as a form of research that assists in substantiating practice. In a review of the literature, the authors identified 54 single-subject studies from 1969 to 2006 that provided emerging evidence for practitioners. Publisher's web site:

Executive Function in Charge Syndrome --Hartshorne, Timothy S.; Nicholas, Jude; Grialou, Tina L.; Russ, Joanna M. Child Neuropsychology; Aug 2007, Vol. 13 Issue 4, p333-344. (2007) This study addressed the presence of executive dysfunction in children with CHARGE syndrome, a genetic disorder with multiple physical anomalies and severe challenging behaviors. Ninety-eight children were included in the study. More than half received clinically significant scores on the Behavior Rating Inventory of Executive Function (BRIEF; Gioia et al., 2000) scales of Shift, Monitor, and the Behavioral Regulation Index, with additional high scores on Inhibit and the Global Executive Composite. Associations were found with the age the child first walked, scores on the Autism Behavior Checklist (ABC; Krug et al., 1993), and being classified as deafblind. Difficulties with making transitions and flexible problem solving, monitoring their work and their effect on others, and acting on impulse, may be related to the behavioral difficulties exhibited by children with CHARGE. Interventions targeting improved self-regulation may help to manage this challenging behavior. Publisher's web site:

Mental and Behavioral Disorders Among People With Congenital Deafblindness --Dammeyer, Jesper. RESEARCH IN DEVELOPMENTAL DISABILITIES, vol. 32, #2, March-April 2011, pp. 571-575. (2011)This article reports the results of a study of the prevalence of mental and behavioral disorders among 95 congenitally deaf-blind adults in Denmark. Seventy-four percent were found to have a mental and/or behavioral diagnosis. Mental retardation was found among 34% and psychosis among 13%. The article concludes with a discussion of factors involved in diagnosing these types of conditions in individuals with deaf-blindness.

Parent and Teacher Perceptions of Challenging Behaviorin their Children and Students with CHARGE Syndrome --Sheriff, Lorna Kathleen. Texas Tech University. (2012) A dissertation in special education submitted to the graduate faculty of Texas Tech University in partial fulfillment of the requirements for the degree of doctor of education. Available on the web:

Reducing challenging behaviors and fostering efficient learning of children with CHARGE syndrome --van Dijk JP; de Kort A. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 273-277. (2005) Letter. Describes a single-subject case study. Author states that if the challenging behaviors exhibited by individuals with CHARGE are to be effectively reduced, intervention must take into account and address the underlying causes and the consequences of the behaviors. Concludes that careful, detailed observation and assessment can elucidate many causes of these behaviors. Carefully, coordinated, consistent management plans to control the environment and alter reactions to behaviors can be a major step towards reducing the frequency and intensity of these disruptive behaviors. The result can create an environment more conducive to social interaction and learning for these children. Available on the web:

Relationship Between Parental Contingent-Responsiveness and Attachment Outcomes --Kassow, Danielle Z.; Dunst, Carl. J. Research and Training Center on Early Childhood Development. BRIDGES: Practice-Based Research Syntheses, vol. 2, #4, November 2004, pp. 1-17. (2004) This research synthesis is the relationship between parental contingent-responsiveness and attachment outcomes was examined in 13 studies including 962 infants and their parents. Parental contingent-responsiveness refers to parental behavior emitted in response to child behavior that functions as a reinforcement influencing child behavior outcomes. Parental contingent-responsiveness was assessed prior to the child reaching 8 months of age, and child attachment was assessed between 12 to 15 months of age. Findings indicated a discernable relationship between parental contingent-responsiveness and attachment outcomes for young children. The results suggest that early parental contingent-responsiveness is an important determinant of later secure child attachment.

Repetitive behaviors in CHARGE syndrome: differential diagnosis and treatment options --Bernstein V; Denno LS. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 232-239. (2005) Twenty-nine students with CHARGE syndrome were evaluated using the Compulsive Behavior Checklist (CBC). Most of the students obtained a high score for repetitive behaviors, averaging 11.5 repetitive behaviors per student. Most students' repetitive behaviors significantly interfered with their daily routine. Thirty-four percent of the students responded to redirection with aggression toward themselves or others. Many of these behaviors do not respond well to traditional behavioral techniques. Through observation, definitions, and using functional analysis of behavior, repetitive behaviors were assigned to one of four categories. Each category has different treatment options. A case study of an adolescent girl with CHARGE syndrome who has profound deafness, colobomas, and moderate cognitive delay is presented. She displayed severe behavior challenges and significant medical issues. The course of treatment is outlined over 5 years. Behavior management, medical management, and psychiatric management were used in regulating her behavior, medical issues, and anxiety disorder. Close coordination between disciplines and excellent family involvement led to a very positive outcome. The student, now 19 years old, has made progress in her educational program and has been able to experience more community integration. Copyright (c) 2005 Wiley-Liss, Inc. Available on the web:

Review and Evaluation of Research on the Deaf-blind from Perceptual, Communicative, Social and Rehabilitative Perspectives --Ronnberg, Jerker; Borg, Erik. SCANDINAVIAN AUDIOLOGY, #30, 2001, pp. 67-77. (2001) This paper reviews research on deaf-blind individuals, primarily from behavioral and communicative points of view. Inclusion in the population of deaf-blind is qualified by describing a variety of subgroups and genetically based syndromes associated with deaf-blindness. Sensory assessment procedures - based primarily on residual capacities - are appraised. Non-sensory, alternative classificatory schemes and procedures are presented and the results from behavior modification procedures used for correcting maladaptive behaviors are summarized. Methods for communicating tactilely are described and evaluated. Attention is also drawn to some suggestions regarding learning of alphabetic codes and sign acquisition. Finally, suggestions for future research are proposed.

Review of Research Projects --Hefner, Meg, M.S. CHARGE ACCOUNTS, vol. 15, #2, Summer 2005, p. 5. (2005) This is a brief one page status report on research projects on the topic of CHARGE Association. Some are already approved by the CHARGE Research Committee; some are approved by others; and some are pending/future possibilities. Available on the web:

Role of the Emotional Brain Webcast --van Dijk, Jan. Watertown, MA: Perkins School for the Blind. (2011) Dr. Jan van Dijk presents his research and ideas related to the brain, the limbic system and the impact on teaching and learning for students who are blind with additional disabilities including deafblindness. The chapters in this webcast are: 1. Introduction, 2. Limbic System, 3. Stress, 4. Mirror Neurons, 5. Challenging Behavior, 6. Evidence Based Practice. Publisher's web site:

Supporting High Quality Interactions with Students Who are Deafblind --Axelrod, Craig. Austin, TX: Texas School for the Blind and Visually Impaired. (2006) This paper begins with a summary of current research on interactions with children who are deaf-blind, focusing primarily on research conducted by Janssen, Riksen-Walraven, and van Dijk. Topics include interactive challenges, the impact of adult-dominated interactions, consequences of disharmonious interactions, and an educator-oriented intervention. The second part of the paper describes an interaction training program developed at the Texas School for the Blind and Visually Impaired developed to help educational staff improve their interactions with children who are deaf-blind. This paper updates and combines two articles previously published in See/Hear. Available in Spanish. Available on the web:

Teaching Social/Communicative Interventions to Youth with Disabilities --Alwell, Morgen; Cobb, Brian. Charlotte , NC: National Secondary Transition Technical Assistance Center (NSTTAC) What Works Transition Research Synthesis. (2007) This practice-based systematic review summarizes “scientifically-based” research studies that have been produced in the past two decades from three distinct perspectives: (a) interventions designed to build social and/or communicative skills, (b) transition or transition-related outcomes, and (c) samples of secondary-aged youth with disabilities. This review adds to existing theory in the area of transition in two important ways. First, we have only included studies that combine the use of a transition-related treatment and measurement of one or more transition-related outcomes exclusively (or in large part) for secondary aged youth with identified disabilities, and the effects of the treatment must have been measured and reported in such a way that an effect size could be calculated (except, of course, with studies using qualitative designs). None of the previous reviews on these topics constrained their reviews to this focus on measured effects across research design types, disability categories, and social and communicative skills with both broad and specific perspectives, for secondary youth with identified disabilities. Second, we also required every study included in this review to meet minimum standards of internal and external validity. Our rubric was adapted from early design work completed by meta-analysts and systematic review experts at both the Evidence for Policy and Practice Information and Co-ordinating Centre (EPPI-Centre) at the University of London, and at the What Works Clearinghouse (WWC) in the U. S. Department’s Institute of Educational Sciences. Some studies reviewed include subjects who are deaf-blind.

Use of Anticipatory Cues to Reduce Dependence on Physical Prompts by an Adolescent with Multiple Disabilities --Lancioni, Giulio E.; O’Reilly, Mark F.; Campodonico, Francesca; Serenelli, Silvia. SCANDINAVIAN JOURNAL OF BEHAVIOUR THERAPY, vol. 29, #1, pp. 43–47. (2000) Anticipatory cues (vibratory stimulation activated through remote control) were used in this single-subject design study to help an adolescent with deaf-blindness and multiple disabilities to reduce his dependence on the caregiver’s physical prompts. The results show that anticipatory cues were effective in helping the adolescent perform practical daily responses independently of physical prompts. Towards the end of the study, the adolescent could also perform increasingly independently of the anticipatory cues. Implications of the findings are discussed. 

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