The Teaching Research
In This Issue
Cortical Visual Impairment: Guidelines and
Children with Usher Syndrome: Learning to Cope
through Work with Mentors
New Master's Degree Program in Severe
Disabilities with an Emphasis on Dual Sensory Impairment in Mississippi
A Model for Paraprofessional Training in
For Your Library
Conferences and Events
Cortical Visual Impairment: Guidelines and
Susan Edelman, Peggy Lashbrook, Annette Carey,
Diane Kelly, Ruth Ann King, Christine Roman-Lantzy, and Chigee Cloninger
Cortical visual impairment (CVI) has been
identified as the leading cause of visual impairment in children in the Western
world (Jan, Good, & Hoyt, 2004). As increasing numbers of children are
diagnosed with this condition, including many who are deafblind, the importance
of understanding CVI and its implications and the development of effective
educational approaches have become paramount. The purpose of this article is to
(a) share emerging definitions and descriptions of CVI, (b) present educational
considerations and specific guidelines being followed by the multistate CVI
Mentorship Project, and (c) propose questions for research on educational
approaches for children with CVI.
Definition and Characteristics
Cortical visual impairment has been defined for educational
purposes as "a neurological disorder, which results in unique visual responses
to people, educational materials, and to the environment" (American Printing
House for the Blind, 2004). Students are considered to have CVI if they have
specific visual or behavioral characteristics (see Table 1). Canadian pediatric
neurologist Dr. James Jan and his colleagues at British Columbia's Children's
Hospital have contributed significantly to the identification of these
characteristics (Jan & Groenveld, 1993). With new advances in medical
technology, more children with neurological damage are surviving, and many have
multiple and complex disabilities (Good, Jan, Burden, Skoczenski, & Candy,
2001). Dr. Jan and others have brought much needed attention to the visual
functioning of children with severe damage to specific areas of the central
nervous system and have provided distinctions between the acuity loss seen in
ocular impairment and the type of loss seen in CVI. CVI and ocular impairment
can co-exist (Huo, Burden, Hoyt, & Good, 1999), but strategies for
identifying, assessing, and serving children with ocular impairment alone do
not address the needs of children with CVI.
Before 1980, CVI was called "cortical blindness" and was believed
to be a complete lack of visual processing by a damaged brain (Roman, in
press). Little was understood about what a person with cortical blindness could
see, and interventions to address the loss of visual function did not exist.
Recent research about the way the brain functions and its ability to adapt have
resulted in new ways of understanding the visual behavior of children with CVI
(Huo et al., 1999; Giudice et al., 2002). Vision is processed throughout the
brain, and discoveries about neurological pathways and connections show that
improvement in visual function is possible (Roman, in press). An understanding
of these findings is essential for the development of sound educational
programs for children with CVI.
Characteristics of CVI
- Normal or minimally abnormal eye exam
- Difficulty with visual novelty (prefers to look at old
objects, not new, and lacks visual curiosity)
- Visually attends in near space only
- Difficulties with visual complexity/crowding
- Non-purposeful gaze/light gazing behaviors
- Distinct color preference
- Visual field deficits
- Visual latency (visual responses are slow, often
- Attraction to movement, especially rapid movements
- Absent or atypical visual reflexive responses (fails to blink
at threatening motions)
- Atypical visual motor behaviors
- Inefficient, highly variable visual sense
Reprinted with permission from American Printing
House for the Blind, Inc., 2004, http://www.aph.org/cvi/define
CVI Mentorship Project
Early identification of CVI and systematic educational strategies
that target its unique characteristics can enhance visual function and promote
educational progress and quality of life (Good et al., 2001). Improved vision
naturally increases opportunities for incidental learning, resulting in greater
personal control and independence for a child. If CVI is undiagnosed,
misdiagnosed, or diagnosed late, critical periods of learning may be missed.
This points to the need for the provision of services in a timely way. As a
group of interdisciplinary professionals who provide services to students with
vision impairment and multiple disabilities, including deafblindness, we have
identified the need for in-depth training related to screening, assessment,
program planning, and intervention for learners with CVI. We represent state
deaf-blind projects in Delaware, Maryland, Vermont, and West Virginia, and we
have committed to a five-year collaborative multistate training initiative
known as the CVI Mentorship Project. The purpose of the project is to pool
resources and consolidate efforts to provide training and mentorship
experiences to a small team of interdisciplinary professionals in each state.
By Year 5 of the project, teams will be prepared to train, mentor, and advocate
for service development related to CVI as needed within each state. Our
collaboration necessitated the examination and articulation of our beliefs
about working with children who have CVI and about how to mentor educators with
respect to CVI. Based on this, we have developed guidelines for working with
learners, working with families, and providing quality education. They serve as
a framework for the CVI Mentorship Project.
Guidelines for Working with Learners
- All children can learn.
- Children have a right to services that help them learn.
- Children make educational progress when given appropriate
- Appropriate instruction may improve visual functioning.
- Improvement in vision positively impacts a child's performance
in areas such as literacy, academics, mobility, and daily living.
Our collective experience has been that many children with CVI who
have multiple disabilities, intellectual challenges, or a perceived lack of
capacity for learning and literacy are less likely to have access to good
systematic instruction and services that meet their unique visual functioning
needs. These guidelines keep us focused on children's learning and progress and
the careful planning that is needed for appropriate vision support
Guidelines for Working with Families
- Families are valued as full members of the educational
- A family-centered approach is essential.
- Parents know their children best and provide valuable
- Parents are reliable and accurate reporters of their children's
vision-related behaviors (Roman, 1996).
- Professionals have knowledge about and are respectful of
families' cultural and ethnic characteristics.
- Realistic hope and shared optimism are important.
Families play a central role in our approach to children with CVI.
Their experiences, observations, and perspectives are valued and necessary and
provide the foundation for positive forward-thinking teamwork.
Guidelines for Quality Education for Children with CVI
- Careful and reliable assessment is the foundation for
- Systematic instruction and evaluation are essential.
- Assessment and instruction are conducted by personnel who are
trained and qualified to work with children who have CVI.
- Team collaboration, problem solving, and interdisciplinary
expertise are necessary for quality instruction.
- Evidence-based research drives practice.
- Education is delivered in natural settings as much as
- Environmental adaptations and accommodations that match
students' needs are used consistently.
- Delivery of related services is integrated across all
environments and activities.
These guidelines represent good teaching and quality education for
all children, including children with CVI. In our work, we find that the
assessment process, instructional methods, and evaluation must take into
account the unique characteristics of CVI for each child.
Carefully designed instruction and environmental adaptations help
a child progress toward resolution of CVI in three phases. During Phase I, the
focus is on building visual behaviors. Phase II involves integrating vision
into daily routines and activities. During Phase III, a child begins to develop
more typical visual functioning. (Roman, in press) During each phase the
emphasis is not on vision stimulation activities but rather on carefully
selected modifications and adaptations of environmental characteristics that
support the student's best visual functioning at the time. This "environmental
engineering" can enable a child to use his or her vision more effectively and
influence a child's capacity to make progress and benefit from structured and
incidental learning opportunities.
Careful consideration of a child's medical history and
neurological diagnosis is critically important. The field of medicine has
provided the constructs and clinical data to define CVI as a condition. It is
now the responsibility of educators to bring this valuable medical information
into the educational planning arena to create meaningful experiences and
positive educational outcomes for children.
Shared guidelines have served to focus our work, but we have
discovered there is still much to be learned about cortical visual impairment.
We propose the following research questions for consideration:
- What are the features of educational interventions that result
in improved visual functioning for children with CVI?
- Can the levels and phases of CVI as described by Roman (in
press) be validated? What are reliable assessment methods to identify CVI
- What are the unique professional skills necessary for educators
serving learners with CVI?
- Is an interdisciplinary approach to serving children with CVI
- What are the best training models for building a skilled
provider workforce to serve children with CVI?
Our hope is that these questions will promote a national dialogue
that draws upon expertise and knowledge from a variety of fields to improve our
knowledge about CVI and the effectiveness of services for children with CVI.
Ultimately, enhanced visual functioning for children and youth with CVI will
bring about a better quality of life for children and their families. For more
information about CVI, visit the Web site of the American Printing House for
the Blind at http://www.aph.org/cvi.
American Printing House for the Blind (2004). What is CVI?
Accessed April 6, 2006, at
Giudice, N., Madison, M. C., Costello, P., Zhuang, J. C., Legge,
G. E., Hu, X., & He, S. (2002). Tactile vision: Neuroimaging and
brain-reorganization in the blind; implications for learning and
adaptive-technology. In Technology and Persons with Disabilities Conference
Proceedings [On-line]. Accessed at
Good, W. V., Jan, J. E., Burden, S. K., Skoczenski, A., &
Candy, R. (2001). Recent advances in cortical visual impairment.
Developmental Medicine & Child Neurology, 43, 5660.
Huo, R., Burden, S., Hoyt, C. S., & Good, W. V. (1999).
Chronic cortical visual impairment in children: Aetiology, prognosis, and
associated neurological deficits. British Journal of Ophthalmology, 83,
Jan, J. E., Good, W. V., & Hoyt, C. S. (2004). An
international classification of neurological visual disorders in children.
Accessed at http://www.aph.org/cvi/articles/jan_1.html
Jan, J. E., & Groenveld, M. (1993). Visual behaviors and
adaptations associated with cortical and ocular impairment in children.
Journal of Visual Impairment and Blindness, 87, 101105.
Roman, C. A. (in press). Cortical visual impairment:
Identification, assessment and intervention. New York: AFB Press.
Roman, C. A. (1996). Validation of an interview instrument to
identify behaviors characteristic of cortical visual impairment in infants.
Ann Arbor: University of Michigan.
Children with Usher Syndrome: Learning to Cope
through Work with Mentors
Elias Kabakov and Debbie Toubi
The Center for
Deaf-Blind Persons, Israel
The Center for Deaf-Blind Persons of the
Beth David Institute in Israel has developed an innovative program for children
with Usher Syndrome. Through work with mentors, children learn about their
diagnosis and receive exposure to a successful coping model. The program is
open to every child with Usher Syndrome in the entire country, including Jews,
Muslims, and Christians. It aims to serve 40 children a year.
Based on more than a decade of experience working with deaf-blind
individuals and inspired by the book Usher Syndrome in the School Setting
(Miner & Cioffi, 1996), we have concluded that children with Usher Syndrome
need early contact with adults who also have Usher Syndrome. The children can
easily identify with these adults, and interaction enables them to learn about
progressive vision loss in a positive setting, unlike cases we have seen of
children who become depressed by doctors who provide misleading information and
make fatalistic statements such as "You're going blind." We have found that
children are inspired by contact with young, vibrant, and successful persons
with Usher Syndrome who are enjoying an active life.
This program, which is the first of its kind in Israel, took shape
under the professional and personal guidance of Yael Halevi. Yael, who has
Usher Syndrome herself, holds a BA in Literature and is a certified movement
therapist. The program begins by identifying children between the ages of 10
and 21 and simultaneously recruiting and training young adults with Usher
Syndrome to become mentors. Requirements for mentors include a certain level of
maturity and life experience, good communication skills, high motivation, and
an open and accepting attitude toward their deaf-blindness. The mentors
participate in a mandatory training program to learn how to teach the children
to function with Usher Syndrome at work and leisure and how to set an example
of success despite obstacles. In addition to the initial training, the mentors
all receive weekly guidance and learn how to approach sensitive issues that the
children may raise, such as the desire for a driver's license or when and how
to tell friends that they have Usher Syndrome.
Over the course of a year, the mentors meet with the children
weeklyindividually or in small groups. Through this structured contact,
the children learn very gradually about Usher Syndrome through the presentation
of facts and personal examples. They gain skills and knowledge to help them
broaden their horizons and be willing to accept help from others. They also
learn to come to terms emotionally with their physical limitations. This
contact is critical to future success in that it helps children gain a
foundation of independence, self-esteem, and confidence. As they progress, they
tend to become more open to learning mobility skills and alternative
A double-empowerment process takes place during this program. The
children learn useful skills and coping strategies, and the mentors gain
valuable work experience that may lead to new career opportunities. The mentor
training they participate in also provides them with a valuable support group.
Most have never before participated in a similar group situation.
Early mentoring offers action and the hope of success as opposed
to passivity and a feeling of impending failure. This program benefits not only
the deaf-blind community, but also Israeli society as a whole. Instead of
supporting these children when they become adults, it gains members of society
who make valuable contributions.
The Center for Deaf-Blind Persons provides comprehensive
rehabilitation, educational, and social services for the deaf-blind population
in Israel. We continually develop and run a wide range of innovative programs
and strive to enable deaf-blind individuals to live full and independent lives,
to as great a degree as possible. The Center encourages all people with
deaf-blindness to learn about their disability and helps them to cope and play
an active role in their own rehabilitation and that of others.
For more information about this or other programs, please contact
Elias Kabakov, professional director, Center for Deaf-Blind Persons, by mail at
P.O.B. 9259 Tel Aviv, 61092 ISRAEL, by fax at +972-3-6316419, or by e-mail at
Miner, I., & Cioffi, J. (1996). Usher Syndrome in the
school setting. Sands Point, NY: Helen Keller National Center.
New Master's Degree Program in Severe Disabilities
with an Emphasis on Dual Sensory Impairment in Mississippi
Mississippi Deaf-Blind Project
In spring 2004, there was a new opportunity
for teachers and service providers in Mississippi who work with children and
adults who are deaf-blind. The University of Southern Mississippi (USM), in
collaboration with Texas Tech University (TTU), has developed a master's degree
program in severe disabilities with an emphasis on dual sensory impairment. The
program accepts individuals who have an undergraduate degree in education or a
related field and at least two years experience working with individuals with
multiple disabilities. Graduating students are obligated to provide two years
of service in Mississippi after completing the coursework. The program aims to
train up to 60 people over a 5-year period to fulfill a long-felt and
completely unmet need in the state of Mississippi. Funding for the program is
provided through a grant awarded by the U.S. Department of Education. It covers
students' tuition, books, travel, and child-care expenses.
The program's focus is helping students to learn and implement the
Competencies for Teachers of Learners Who Are Deafblind set forth by the
Perkins National Deafblind Training Project (McLetchie & Riggio). Most of
the courses are provided on-line. Other formats include CD-ROMs, videotapes,
teleconferencing, videoconferencing, and e-mail. Students are required to be on
the USM campus for one day each semester for scheduled face-to-face meetings
with instructors. The program includes six courses provided by TTU and three
courses provided by USM. The TTU courses are:
- Programs and Services for Students with Dual Sensory
- Physical Aspects Affecting Students with Visual Impairments and
- Basic Orientation and Mobility Skills for Students with Visual
and Multiple Disabilities
- Physical Aspects of Hearing for Students with Dual Sensory
- Methods and Materials for Teaching Students with Dual Sensory
- Communication and Child Centered Assessment for Students with
The USM courses are:
- Administration and Disability Law Applied Research
- Transitioning to Adult Services
In addition, a class on assistive technology is available through
a contract with Mississippi State University.
Two observation experiences are also requiredone in-state
and one out-of-state. These provide opportunities for students to observe
experienced teachers in different settings. The observations typically last
from one to two weeks. In-state observation experiences are facilitated by the
Mississippi Deaf-Blind Project, which is housed at USM. Out-of-state
observation sites have included Perkins School for the Blind, Texas School for
the Blind and Visually Impaired, the Helen Keller National Center, the Arkansas
School for the Blind, and a public school system in Florida. Additional sites
are being added. Dr. Linda McDowell, director of the Mississippi Deaf-Blind
Project, has worked diligently to secure sites that provide valuable
experiences for students.
In December 2005, the program successfully graduated its first
group of 15 students. A second group of 13 students is scheduled to graduate in
December 2006, and a third group of 11 students, who began the program in
January 2006, will graduate in December 2007. The application process for a
fourth group is currently underway. At this time, the program is only available
to students in Mississippi. However, as interest in the program has increased,
discussion has begun about the possibility of accepting out-of-state students
in the future.
Feedback from the first group of students has been positive. One
particular benefit that many have mentioned is how helpful it is to have a
statewide network of colleagues with expertise in deaf-blindness to call on for
support. Following are some additional student comments:
The information I learned that specifically focused on
individuals who are deaf-blind could easily be adapted to fit individuals with
The program increased my advocacy skills for individuals who
The program benefitted me not only in the area of
deaf-blindness but in the field of special education in general. I have a much
deeper understanding of the laws that protect all special education
The experience was positive and encouraging, because it
forced me to see possibilities in each and every person who is deaf-blind. I
also feel that I now have a support system in the state.
Six students in the first group of graduating students were
directly affected by Hurricane Katrina. Some did not have power or Internet
access for up to two months. Other class members were able to help them by
providing support and access to class notes, assignments, and materials.
The master's degree program is a dream come true for the many
people who recognized a need for more trained teachers and service providers in
Mississippi and who worked hard to create and implement the program: Linda
McDowell, Ph.D. (USM), director of the Mississippi Deaf-Blind Project and
author of the grant that secured funding for the program; Liz Grantham (USM),
administrative assistant to Dr. McDowell; Janet Salek (USM), instructor;
Roseanna Davidson, Ed.D. (TTU), instructor; Effie Laman (TTU), research
instructor; Esther Lucy (TTU), administrative assistant to Roseanna Davidson;
and the graduating class of 2005.
McLetchie, B. A. B., & Riggio, R. (no date). Competencies
for teachers of learners who are deafblind. Watertown, MA: Perkins School
for the Blind.
Services for Individuals Who Are Deaf-Blind Must
be Lifelong William and Laura Hull South Carolina
Like many parents of children who are
deaf-blind and have multiple disabilities, we have for years been fighting for
appropriate services for our son. This began with a struggle to get appropriate
educational and related services during his school years and has continued now
that he is an adult. Socialization, daily activities, stimulation, overall
care, and housing fall on us, the parents, as we ourselves are aging. This
problem must be addressed. Parents and professionals need to work together to
develop programs and approaches that meet the needs of individuals who are
deaf-blind throughout their lives.
Our son's life began with stormy events. At 3 pounds 12 ounces, he
struggled to survive. He had a soft cleft palate, chronic respiratory problems,
four adult-size hernias, rectal prolapse, seizures, and problems nursing. He
spent the first three years of his life in and out of pediatric intensive care
units at various hospitals. At 3 months of age he had a gastric tube inserted
into his stomach for tube feedings and was tube fed until age 7. It was
determined by age 2 that he had severe hearing loss. At age 3 he was diagnosed
with a rare genetic syndrome and received glasses and hearing aids. By age 7 he
was found to have severe cataracts requiring surgical removal, and at age 15 he
had laser retina surgery to save his sight. Unfortunately, he was declared
legally blind by age 16.
During his school years, we had to battle for suitable programs,
even to the extent of going through due process. The lack of appropriate
services and another student's dangerous behavior forced us to remove our son
from public school at age 17. We established our own home teaching program and
used two hospitals for occupational and speech therapies. Our state's
deaf-blind project coordinator provided counseling and support and worked with
our son's speech and occupational therapists. Her services were invaluable
because she believed in our son. We saw his sign language vocabulary increase
significantly. After several months in our home program, it was determined that
he knew 175 to 200 signs expressively and about 500 receptively. Now, at age
25, he knows approximately 275 to 300 expressively and 500 to 600 receptively.
The deaf-blind project coordinator's services and encouragement inspired all of
those who worked with our son to believe in him and his achievements.
The struggle to obtain adequate services recurred after our son
turned 21. People with disabilities, especially deaf-blindness, often have
limited or no local individualized programs available. Some people with
disabilities do find jobs, but most are limited to sheltered workshops.
Unfortunately, there are too few qualified service providers because most are
not trained to deal with people who are multiply disabled, deaf-blind, and
nonverbal. At one disabilities day program we viewed, we found that no one knew
sign language or knew how to communicate with people with communication
problems, let alone deaf-blindness. We observed participants with disabilities
sitting around watching TV, sleeping on desks, or with nothing to do, and we
saw only a few working. We knew this program would not be productive or
enhancing for our son so he remained with us. To assist him with socialization,
we helped a group of disabled individuals to organize their own club known as
People First. The members are in charge, run the meetings for the most part,
and choose goals and objectives. This club is going strong after seven
Finding and retaining qualified service providers is extremely
difficult. In 2000, our son began receiving services from a community support
program that provides assistance to individuals with disabilities at home and
in the community. The program provides rehabilitation support specialists to
assist with daily activities like self-help skills, communication, shopping,
and eating out. The experience began fairly well with a support specialist who
knew sign language, but this person left to return to a full-time job at the
end of the summer. The program had repeated difficulty locating and retaining
support specialists who knew sign language or were able to learn to communicate
with our son. Over a 5-year period we had four different support specialists,
and between each there were extended periods of time (once for as long as 27
months) when our son had no one to work with him. He was recently terminated
from the rehabilitation program because no one could be found to work with him.
Presently, we are considering looking again at the day program we saw earlier,
because new grant monies are bringing about some positive changes there. We
hope this works out because our son needs space, socialization, and time away
from us. After fighting for years for services, we need a break too.
A New Approach
Fortunately, there is hope with a new model approach on the
horizon to help those with deaf-blindness. It is the intervener model, for
which there is a national task force. Interveners are trained individuals who
work with children who are deaf-blind and provide support in areas such as
communication, daily care, and mobility. The trained intervener serves as a
deaf-blind person's eyes and ears and facilitates communication. Interveners
are a must for school-age children because a deaf-blind child's educational
advancement is at stake. It is just as important to use the intervener model
with many adults who are deaf-blind. We must support a national movement for
the provision of trained intervener services throughout life because all
children one day reach the magic age of 21. No one should be cut off from their
life and the environment around them. The intervener model is a good example of
a better way to help people with deaf-blindness and multiple disabilities. To
bring about positive changes, parents, guardians, and professionals must band
together to support this new model approach and its task force.
Editor's note: The National Intervener
Task Force is an informal network of professionals and parents interested in
issues related to the training and use of interveners. The focus of the task
force so far has been on interveners in educational and early intervention
settings, but expanding the focus to adults who are deaf-blind is possible if
there is interest and individuals willing to participate and work on these
issues. For more information, contact Linda Alsop, SKI-HI Institute, Utah State
University (phone: 435-797-5598; e-mail: firstname.lastname@example.org).
A Model for Paraprofessional Training in
SKI-HI Institute, Utah State
The use of paraprofessionals with children
and youth who are deafblind continues to increase. The Individuals with
Disabilities Improvement Education Act of 2004 (IDEA) acknowledged and
strengthened the role of the paraprofessional in the provision of special
education and related services. It clearly states that paraprofessionals and
assistants who are properly trained and supervised may assist in the provision
of special education and related services to children with disabilities. The No
Child Left Behind Act mandates the availability of qualified staff for all
students. Paraprofessionals who work with children and youth who are deafblind
not only need training and specialized skills, but they must also meet state
standards for paraprofessionals. Currently, there are no preservice training
programs in the United States designed specifically for paraprofessionals in
deafblindness that also interface with state paraprofessional standards.
The Consortium for National Paraprofessional Training in
Deafblindness is addressing this lack through a model for distance education.
Program components include:
- Offering three semesters (nine credit hours) of on-line
coursework in deafblindness that utilize the best practices in
distance-education instructional strategies;
- Field testing the three courses at three community colleges in
Utah, Massachusetts, and Texas, and at one 2-year program of study in
- Embedding the three courses as electives into existing programs
of study at each site, so that students can take the three courses in
deafblindness alone or as part of an associate degree program;
- Ensuring that the competencies for the coursework interface
with each state's standards for paraprofessionals; and
- Coordinating with state deaf-blind projects to involve them in
recruitment and practicum efforts.
The coursework is scheduled to begin in the fall of 2006 and will
be offered at the following sites: Salt Lake Community College, Utah; Mount
Wachusett Community College, Massachusetts:; The University of Arkansas at
Little Rock; and South Plains College, Texas.
The project is funded by the U.S. Department of Education's Fund
for the Improvement of Postsecondary Education (FIPSE). For additional
information, contact: Linda Alsop, SKI-HI Institute, Utah State University,
Logan, UT 84322-6500, Phone: 435-797-5598, E-mail:
of touch, gesture,
a turn of the head,
with the reach
of a hand,
CHARGE Development in Adolescence
Timothy S. Hartshorne
Interest in CHARGE Syndrome has increased dramatically since it
was initially identified. Although the first reported cases were published in
1979, knowledge of the condition grew slowly. Only a few articles were
published each year until 1985, when there were 6. By 2005, there were more
than 30. Prior to the identification of a gene in 2004, diagnosis relied on the
identification of characteristic features of the syndrome including coloboma
(missing piece of the eye), choanal atresia (blocked nasal passages), cranial
nerve abnormalities (particularly those involved with hearing, swallowing,
balance, and the face), and the distinctive CHARGE-shaped ear (Blake et al.,
1998). Heart, kidney, hand, abdominal, and spinal problems are less common
features. A difficulty with this type of diagnosis is that the features vary in
their presence or absence, and when present, in their severity. A heart defect,
for example, may be very minor, or it may be extremely complex and life
Children diagnosed from the late 1980s to the early 1990s were
elementary school age or younger and typically had severe characteristics.
Fewer older children and adults were identified at that time, probably because
they happened to be less severely affected. In earlier decades, individuals
with serious, less treatable characteristics would not have been likely to
survive. As a result, knowledge of the natural history of CHARGE had to develop
along with the children identified in the 1980s and 1990s, so many of the less
common features were only identified as the population aged.
The first large group of children to be diagnosed as infants is
now in late adolescence or early adulthood, therefore, our knowledge of
adolescent development for persons with CHARGE is extremely limited. A number
of questions have been raised, but there are few answers, and appropriate
treatment protocols have not been identified. We know that puberty is usually
delayed, but we don't know the extent of the delay or whether there are useful
and appropriate treatments. We know that many children develop osteoporosis,
but we don't know how early this occurs or whether it can be prevented. We
suspect that medical complications emerge during adolescence, but we're not
sure what or how common they are. Behavioral challenges are common in children
with CHARGE, but we don't know if challenging behaviors increase, decrease, or
otherwise change during adolescence.
To better understand these issues, five experts have joined
together for a research initiative: Kim Blake, a clinical pediatrician from
Dalhousie University in Nova Scotia; Meg Hefner, a genetic counselor at St.
Louis University Hospital; Jeremy Kirk, an endocrinologist from Birmingham
Children's Hospital in England; George Williams, a pediatrician from Sydney,
Australia; and myself, a psychologist from Central Michigan University. We are
currently conducting a pilot study of 32 children, ages 9 to 21, from the U.S.,
Canada, England, New Zealand, and Australia. It is a complicated study because
in addition to more psychological measures, such as adaptive behavior,
executive function (cognitive skills that control and regulate thought and
action), and activity levels, it involves medical testing and reports. For
example, we are requesting blood work to look at pituitary functioning and
measures of bone age and density. The information obtained is likely to be of
vital importance in the medical, educational, and psychological management of
adolescents with CHARGE. After completion of this study, we hope to conduct a
Blake, K. D., Davenport, S. L., Hall B. D., Hefner, M. A., Pagon,
R. A., Williams, M. S., Lin, A. E., & Graham, J. M. (1998). CHARGE
association: An update and review for the primary pediatrician. Clinical
Request for Feedback on Assessment
Oregon Health & Science
Oregon Health & Science University is in the third year of a
grant from the U.S. Department of Education to study and validate methods to
assess the communication and cognitive skills of 2- to 8-year-old children who
are deafblind. We are seeking feedback on assessment instruments from
professionals and parents.
We would like feedback from professionals who have administered
any of the following to children age 2 to 8 who are deafblind (within the past
5 years): Callier-Azusa (Stillman); Carolina Developmental Profile (Harbin
& Bourland); Hawaii Early Learning Profile (HELP) (Parks); INSITE (Morgan
& Watkins); Oregon Project (Anderson, Boigon, & Davis); and Vineland
(Sparrow, Balla, & Cicchetti).
We would like feedback from parents who have access to the results
of any of the following instruments used within the past 2 years to evaluate
their 2- to 15-year-old children: Callier-Azusa (Stillman); Carolina
Developmental Profile (Harbin & Bourland); Communication Matrix (Rowland);
Dimensions of Communication (Mar & Sall); Hawaii Early Learning Profile
(HELP) (Parks); Home Talk (Numerous authors, OSEP Project); Infused Skills
Assessment (Hauser & Hagood); INSITE (Morgan & Watkins); Oregon Project
(Anderson, Boigon, & Davis); School Inventory of Problem Solving Skills
(Rowland & Schweigert); and Vineland (Sparrow, Balla, & Cicchetti).
Professionals will be asked to fill out a form to evaluate the
instrument and will be offered a $20.00 gift certificate for their efforts.
Parents will be asked to provide copies of assessment results, copies of their
child's latest IEP or IFSP, and will be asked to evaluate the instrument. They
will be offered a check or gift certificate in the amount of $50.00 for their
efforts. For more information, contact: Charity Rowland, Ph.D., Project
Director, Oregon Health & Science University, Phone: 503-494-2263, E-mail:
If you have information that you would like to include in
"Research Update," contact:
Teaching Research Institute
Monmouth, OR 97361
For Your Library
Tactile Learning Strategies: Interacting with Children Who Have
Visual Impairments and Multiple Disabilities (Video or DVD62
Deborah Chen & June Downing New York: AFB Press, 2006.
Using narration, interviews, and the detailed experiences of
children and their families, this video illustrates strategies to help children
who are visually impaired and have multiple disabilities learn through touch.
English and Spanish versions are on the same tape. Available from AFB Press.
Phone: 800-232-3044. E-mail: email@example.com. Web:
2006 AccessWorld® Guide to Assistive Technology
AFB Press, 2006.
This guide provides detailed profiles of over 200 products and
also includes a comprehensive list of objective product evaluations previously
published in AccessWorld, AFB's technology magazine Available from AFB Press.
Phone: 800-232-3044. E-mail: firstname.lastname@example.org. Web:
The Intervenor Professional Network (Web site)
The Intervenor Professional Network is an international Internet
resource on deafblind communication strategies. It is intended to promote
communication between parents, intervenors, paraprofessionals, and anyone
working with individuals who are congenitally deafblind, for the purpose of
exchanging information and strategies. The site will promote communication by
providing a forum, chat room, and comment driven blogs that allow visitors to
communicate with each other locally and internationally. For more information,
contact Niall Brown at email@example.com.
Conferences and Events
Educating Students with Deafblindness:
Conference (featuring Jan van Dijk)
July 2126, 2006
Dr. Jan van Dijk is a world renowned educator from the Netherlands
who has pioneered teaching approaches for individuals who are deaf-blind. This
national conference is sponsored by the North Carolina Deaf-Blind Project, the
NC Department of Public Instruction Exceptional Children Division, and Western
Carolina University. The main topics will address behaviors, family dynamics,
social relationships, literacy, and technology for students who are deaf-blind
and will also include a panel of experts presenting information on innovative
educational practices from around the world. Early Bird conference registration
deadline is May 29, 2006. REGISTER EARLY!
For more information go to
or contact Chris Jones, Coordinator, North Carolina State Deaf-Blind Project.
Phone: 919-807-3991. E-mail: firstname.lastname@example.org.
For more information contact Chris Jones, Coordinator, North
Carolina State Deaf-Blind Project. Phone: 919-807-3991. E-mail:
Colorado Summer Institute on
August 79, 2006
The theme is "Links to School and Life Success: Communication and
Literacy for Children with Deafblindness and Other Significant Support Needs."
The featured speakers are Susan DeCaluwe and Lisa Jacobs, educational
consultants for the New England Center Deafblind Project at Perkins School for
the Blind. The Institute is primarily for Colorado families and service
providers, but participants from other states may be welcome if space is
available. For more information contact Tanni Anthony, Colorado Department of
Education. Phone: 303-866-6681. TTY: 303-860-7060. E-mail:
HKNC 2006 Senior Adult Program
Helen Keller National Center (HKNC) in Sands Point, New York,
announces its 2006 Senior Adult Training Program. Senior adults, age 55 or
older, have a unique opportunity to learn adaptive skills related to loss of
vision and hearing, obtain resource information, develop coping strategies,
learn about technology for independent living, and have fun with other senior
adults experiencing combined vision and hearing loss. There will be two 2-week
- September 1022, for individuals who are blind or visually
impaired and hard of hearing and who do not use sign language;
- October 113, for individuals who are blind or visually
impaired and deaf and who use sign language as their primary means of
For more information, contact Paige Berry, coordinator, HKNC
Senior Adult Program, at email@example.com, or visit the HKNC
Web site at http://www.hknc.org and click on
"Senior Adult Program."
7th Canadian Conference on
2006 September 2124, 2006
The Canadian Deafblind and Rubella Association has chosen
"Celebrating Potential" as the theme for this conference. For further
information contact Gitta Schwartz, Suite 201-1100 Concordia Ave., Winnipeg,
Manitoba, Canada R2K 4B8. Phone: 204-949-3733. E-mail:
Usher Syndrome and Related Disorders
October 46, 2006
This meeting will bring together scientists from different
disciplines in order to facilitate collaboration and encourage communication
between researchers. The format of the meeting will be a series of round tables
with international experts on various aspects of Usher syndrome. See the
following web site for more information:
or email William Kimberling at firstname.lastname@example.org
New Screening Tool for Usher Syndrome
Dr. William Kimberling at Boys Town National Research Hospital has
announced a new tool to screen for Usher Syndrome. USMChip is a microchip that
can test a saliva sample for all known types of Usher Syndrome (currently,
there are eight) by checking for approximately 420 known DNA mutations. The
success rate is expected to be between 65% and 75% and should improve over time
as new mutations are identified and included on the chip. The cost of the test
is $200 per person, which makes it an ideal tool for screening. Previously,
testing was only available through a process known as sequencing, which could
cost thousands of dollars and required DNA obtained from a blood sample. The
National Center for the Study and Treatment of Usher Syndrome welcomes new
participants interested in screening for Usher Syndrome. This involves
purchasing the chip and sending a saliva sample by mail. Once tested, results
will be returned in 2 to 4 months. Interested persons should contact Dr.
William J. Kimberling, Ph.D.
Center for the Study and Treatment
of Usher Syndrome
Boys Town National Research Hospital
555 N. 30th
Omaha, NE 68131
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