Strategies for Young Children with Dual Sensory Disabilities: The Sooner the Better

For Families and Service Providers - Birth-to-3 and Preschool

Section 1: Recognizing and Understanding Infants/Toddlers with Deaf-blindness

A. What Is Deaf-Blindness?

B. Identifying Deaf-Blindness: Red Flags and Risk Factors

C. Implications of Prematurity and/or Medical Complications

D. Impacts of Deafness, Visual Impairment and Deaf-blindness

E. How Do Infants with Deaf-Blindness Learn?

Handouts:

1. NCDB Risk Factors for Combined Vision and Hearing Loss

2. Vision and Hearing – A List of Who’s Who

3. Combined Vision and Hearing Loss: Implications of Prematurity

4. NCDB – Welcome to My Home

WSDS/Deaf-Blind Project: 1-800-572-7000 (or) 425-917-7827 www.wsdsonline.org

Who are Young Children with Deaf-Blindness? Combinations of Vision and Hearing Loss

Degree of Vision Loss

Typical 20/20*

Acuity 20/70 – 20/200

Peripheral Fields <20o

Acuity 20/200 –20/400

Acuity 20/400 –20/1000

Light Perception

Only/No Vision

Cortical Visual Impairment

(CVI)

0 – 15 dB HL (Typical Hearing Level)

15 – 25 dB HL (Minimal)**

At Risk – Consult with WSDS/Deaf-Blind Project

Young children with dual sensory loss as a result of congenital infections, hereditary syndromes, and post-natal complications are at risk for compromised access to learning.

26 – 40 dB HL (Mild)

Eligible for Services – Refer to WSDS/Deaf-Blind Project

Infants/toddlers and preschoolers with more severe hearing levels and vision impairment will experience difficulty accessing spoken language,

visual language, and the auditory/visual world around them. Accommodations must be made in order to provide adequate access to

learning—concept development, communication, and language.

Go to www.wsdsonline.org and click REQUEST SUPPORT (upper right corner)

41 – 55 dB HL (Moderate)

56 – 70 dB HL (Moderately Severe)

71 – 90 dB HL (Severe)

> 91 dB HL (Profound)

Auditory Neuropathy Spectrum Disorder (ANSD)

Central Auditory Processing Disorder (CAPD)

*A child with a vision loss in one eye and normal vision in the other may not qualify for educational services from a Teacher of the Visually Impaired (TVI). **A child in the early years of language learning with hearing levels between 15 – 25 dB is eligible for services because of the impact on early spoken language acquisition, whereas an adult with hearing levels from 0 – 25 dB HL is considered to have normal hearing.

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Risk Factors for Combined Vision and Hearing Loss

Prenatal Conditions

Maternal infection

o Rubella

o Cytomegalovirus (CMV)

o Toxoplasmosis

o Herpes

o Syphilis

Prenatal infant exposure to drugs or alcohol

Birth Complications & Postnatal Conditions

Preterm birth

Low Apgar scores

Jaundice requiring transfusion

Mechanical ventilation longer than 5 days

Low birth weight (2500 grams or less)

Retinopathy of prematurity (ROP)

Meningitis

Encephalitis

Intraventricular Hemorrhage (IVH)

Hydrocephalus/hydrocephaly

Microcephalus/microcephaly

Periventricular Leukomalacia (PVL)

Central Nervous System disorders (e.g. cerebral palsy)

Asphyxia

Genetic Syndromes or Disorders

Partial list includes:

Alstrom Syndrome

CHARGE Syndrome

Cockayne Syndrome

Cornelia de Lange Syndrome

Down Syndrome

Marshall Syndrome

Norrie Disease

Peroxisomal Biogenesis Disorder (IRD, NADL and Zellweger Syndrome)

Stickler Syndrome

Treacher-Collins Syndrome

Trisomy 13, 18

Usher Syndrome

Waardenburg Syndrome

Additional Conditions

Brain tumors or malformations

Accident or abuse

o Direct trauma to the eye and ear

o Severe head injury

o Shaken Baby Syndrome

o Near drowning event

Family history of both vision and hearing loss

Multiple disabilities

Original handout presented at 2013 EHDI National Meeting

info@nationaldb.org nationaldb.org

The contents of this website were developed under a grant from the U.S. Department of

Education #H326T130013. However, those contents do not necessarily represent the

policy of the Teaching Research Institute, nor the US Department of Education, and you

should not assume endorsement by the Federal Government. Project Officer, Jo Ann

McCann.

A List of Who’s Who

Professionals in the Field of Visual Impairment

Ophthalmologist: a medical doctor (M.D.) who specializes in comprehensive eye care and provides examinations, diagnosis, ad treatment for a variety of

eye disorders. Ophthalmologists are skilled in all facets of eye care, from prescribing eyeglasses or contact lenses to performing intricate eye surgery. Some ophthalmologists receive special training in pediatric ophthalmology.

Optometrist: a doctor or optometry (O.D.), but not a medical doctor. Optometrists are licensed to examine, diagnose, and manage various visual problems and eye diseases, and are specially trained to test vision in order to prescribe eyeglasses or contact lenses. They do not perform eye surgeries.

Some optometrists receive special training in pediatric optometry.

Optician: a technician who fills the prescriptions for eyeglasses and contact

lenses. Opticians fit and adjust eyeglasses.

Teacher of the Visually Impaired (TVI): a teacher who is specially trained and

credentialed to address the learning needs of students who are blind or visually impaired. TVIs may provide direct instruction to learners or consultation to the learner’s instructional team members. Some TVIs have specific training and credentials to provide early intervention services.

Orientation and Mobility (O&M) Specialist: a credentialed instructor who is specially trained to teach individuals who are blind or visually impaired to develop the skills needed to move safely in the environment. O&M specialists

may provide direct instruction to the learner or consultation to the learner’s instructional team members.

Professionals in the Field of Deafness

Audiologist: a healthcare professional with a degree in audiology (the study of hearing and hearing disorders) and who is concerned with the identification,

assessment, and treatment of hearing impairments and disorders of the vestibular system (balance system).

Hearing Aid Dealer/Dispenser: a salesperson authorized by the state to fit and sell hearing aids. A hearing aid dealer/dispenser is not an audiologist and does not have a college degree related to hearing loss.

Otolaryngologist (ENT): a physician specializing in diseases and treatment of the ear, nose, and throat.

(continued)

A List of Who’s Who 2

Otologist: a physician trained in otolaryngology who specializes in diseases and treatment of the ear.

Speech-Language Therapist/Pathologist: a certified professional who has been trained to assess, diagnose, and treat speech and language problems.

Educational Interpreter: a specialized professional whose role as an interpreter is to facilitate communication between a student who is deaf or hard of hearing and others.

Teacher of the Deaf/Hard of Hearing (TOD): a teacher who is specially trained and credentialed to address the learning needs of students who are deaf or hard of hearing. TODs may provide direct instruction to learners or consultation to the learner’s instructional team members. Some TODs have specific training

and credentials to provide early intervention services.

Professionals in the Field of Deaf-Blindness

Deaf-Blind Consultant: a representative of your State Deaf-Blind Program who has special training and experience to consult with early intervention and school teams on the unique needs of children and youth with deaf-blindness (or, combined visual impairment and hearing loss). These people might be certified teachers of the deaf-blind, or teachers of the deaf, teachers of the blind, or

from other related fields.

Deaf-Blind Intervener: a person (typically a paraeducator) who: (1) has received

specialized training and skills in deaf-blindness; (2) works consistently one-on-one with a child or youth who is deaf-blind; and (3) serves as a member of a child’s educational team.

AND, All of the Above Under Visual Impairment and Deafness

Prematurity Statistics for the U.S.• Everyeightsecondsababyisborninthe

U.S.

• Aboutoneineightbabiesisbornpreterm(lessthan37weeksgestation)

• Everytwominutesalowbirthweightbabyisborn(lessthanfiveandone-halfpounds).

• Everyyearnearly4,500babiesarebornweighinglessthanonepound.

• Prematurityisoneoftheleadingcausesofdeafblindnessnationally.

• InIndiana,ComplicationsofPrematurityistheleadingcauseofcombinedvisionandhearingloss.

Combined Vision and Hearing Loss: Implications of Prematurity

A full-term baby is expected to be delivered at 40 weeks gestation. Babies born at 36 weeks gestational age or sooner are considered to be born preterm (or premature). Over the last decade the rate of preterm births in the United States has increased by 15 percent. By late 2008, the rate of premature births had reached 12.7% or approximately one in every eight births (March of Dimes, 2008).

Due to advances in medical technologies, there has been a corresponding rise in the survival rate of these infants, as well as an increased survival rate of low birthweight babies (typically caused by being born preterm or as a multiple birth). Today, it is not uncommon for infants born younger than 28 weeks gestational age (about 3 months early) to survive. More than 90% of preterm babies weighing 800 grams or more (a little less than two pounds) survive (Kids Health, 2009). This increase in the survival of very young and/or very low birthweight babies has significant impact on infant

INDBS Fact SheetsThe Sooner the Better

development and the development of a child’s sensory systems is at particular risk.

Developmental Effects of PrematurityIn typical prenatal sensory development, each sensory system begins to develop at a particular time and in a particular sequence. In addition, each sensory system completes its own unique developmental sequence, assuring that the system will be mature and operational at birth. An infant’s developing sensory systems are extremely vulnerable and any compromise can have long-lasting implications.

In the womb, a baby spends his or her time curled up, cozy and warm in the dark, listening to mother’s heartbeat and occasional muted sounds from the outside world. This environment is perfectly designed to provide just the right space, temperature, and security for the developing fetus. During the early weeks the infant’s tactile system (touch, temperature, pressure, pain), vestibular system (balance and motion), gustatory system (taste) and olfactory system (smell) develop rapidly. These systems are fully functional by week 21. The auditory and visual systems are the most complex of the sensory systems and take longest to develop. An infant’s auditory system (hearing) is fully functional at week 24, however, an infant’s ability to distinguish and process sounds is not complete until after birth. Visual alertness is present in infants by week 36, but development of the visual system continues for three to four months following birth. As all this sensory development is taking place, the infant’s nervous system also is developing at rapid rate, forming thousands of

Being Born Early Could Mean More than You Think . . .

Reported Vision and Hearing Loss in Children Identified as Deafblind

Vision Loss

17% totally blind or light perception only 24% legally blind21% low vision17% cortical vision impairment21% other

essential nerve cell connections. When a baby is born prematurely, his or her nervous system is immature and disorganized. It simply isn’t ready to handle all of the sensory messages bombarding the baby after birth (Biel, L, 2009).

An early delivery and subsequent time spent in the hospital may cause a baby’s sensory systems to develop more slowly or differently than they typically would, resulting in differences in how the brain and nervous system are wired. These neurological differences may result in sensory

Sensory System What it Does What Might Happen

Tactile(Touch)

• Communicates pleasure and pain• Helps babies identify and

recognize objects• Helps babies explore their world

This system is well developed even in very premature babies, so the entire body is very sensitive. Some babies become oversensitive to being touched as a result of all of the handling and touching involved with their hospital stay.

Vestibular (Balance and

Motion)

• Helps babies keep their balance and be ready to learn to sit, crawl and walk

• Helps babies know where they are in space

Increased early stimulation to this system can sometimes cause babies to be extra fussy or have difficulty settling down. It may also cause toddlers to move in unusual ways or respond in unusual ways to different types of movements (swinging, climbing, etc.).

Gustatory(Taste)

• Encourages babies to explore their environment

• Helps later learning skills, such as eating and playing with toys

Exploring things by mouth is part of typical infant development and babies who have negative experiences may be less interested in doing this, which can affect their overall development.

Olfactory(Smell)

• One of the most well-developed senses in newborn babies

• Helps with recognition and discovery

• Plays an important role in feeding

Babies demonstrate positive and negative reactions to certain smells very early. Early exposure to strong smells can cause negative memories. Triggering these memories may cause negative reactions, even into adulthood.

Auditory(Hearing)

• Important for attention and learning• Helps babies know about what’s

going on around them and • anticipate certain events• Is basic to the development of

spoken language

Babies exposed to high noise levels may find it difficult to filter out background noises and pay attention to what’s important. This may cause sleeping or attention problems in some children. Hearing problems may also exist.

Visual(Sight)

• Most complex of all the sensory systems

• Continues to develop for 3-4 months (even in full-term babies)

• Has a strong connection to the system of touch

• Works with the vestibular system to help babies maintain their balance

• Very important for all kinds of learning

Babies born early are at risk for vision problems. These problems may come from damage to their underdeveloped eyes or from certain conditions that affect premature babies. Some may not be detected until later when a child appears clumsy, is afraid of certain activities or has trouble playing with toys that require a lot of concentration.

Purvis, B. (2005).

problems. Even if there is nothing physically wrong the obvious components of a baby’s sensory systems (e.g. eyes, mouth, ears, nose), premature babies often experience sensory integration issues. This is especially true of the youngest and smallest “preemies.”

Premature infants have many special needs that make their

care different from that of full-term infants. Their tiny bodies must deal with a constant barrage of sights, sounds, smells and activities that their senses are not yet mature enough to handle, so they often begin their lives in an Intensive Care Nursery (ICN) or NICU (Neonatal Intensive Care Unit). Intensive care nurseries are designed to provide an atmosphere that limits stress to the infant and meets the basic needs of warmth, nutrition, and protection to assure proper growth and development (Kids Health, 2009). While most NICUs do their best to minimize overstimulation, an intensive care nursery is an unnatural environment that is very different from the warm, dark, constant environment of a mother’s womb.

Since no two babies are alike, there is no way to predict how preterm birth and the NICU experience may affect individual babies. There is a wide range of sensory issues that could result. The chart on the left provides an overview of sensory development and some of these effects.

Vision & Hearing Losses in PrematurityAlthough most premature babies grow to be normal healthy children, some will have a disability. The chance for any particular premature baby to have an ongoing disability depends upon several factors. In general, the most premature and lowest birth weight babies are at highest risk for ongoing problems. In addition, those babies who have had specific complications of prematurity are also at high risk (About Kids Health, 2009). It is in this group of high risk infants that we find many cases of combined vision and hearing loss.

Premature infants are particularly prone to hearing loss. This is due to multisystem illness and the increased use of medications such as antibiotics and diuretics that can be harmful to the

auditory system (Batshaw, 2007). The

underdeveloped blood vessels in the inner ear

may rupture interrupting the flow of blood to the inner ear. This results in the destruction of

hair cells and contributes to a sensorineural hearing loss (Edwards, Goehl & Gordon, 1992). Hearing loss may or may not be diagnosed early on, so it is critical that early intervention providers be alert to signs and behaviors associated with hearing loss and assist families to follow-up with appropriate examinations.

The eyes of premature infants are especially vulnerable to injury after birth. A serious complication is called retinopathy of prematurity (ROP), which is abnormal growth of the blood vessels in an infant’s eye. The blood vessels in the retina stop growing normally and begin to branch excessively. This causes the development of scar tissue which may eventually pull the retina off the inner wall of the eye. (Edwards, Goehl & Gordon, 1992).

In the most serious stages of ROP, there may be partial or complete retinal detachment. While one eye may be more damaged than the other, ROP affects both eyes. About 7% of babies weighing 1,250 grams (2.75 pounds) or less at birth develop ROP, and the resulting damage may range from mild (the need for glasses) to severe (blindness). The cause of ROP in premature infants is unknown, although it is known that a lower birth weight and gestational age increases the incidence and

CommonComplicationsofPrematurity

• IntraventricularHemorrhageorBleedingintheBrain

• AsphyxiaorLackofOxygentotheBrain• ApneaorRespiratory“Pauses”lasting15-20

seconds• SevereJaundice• HypoglycemiaorLowBloodSugarLevels• SevereInfections• PeriventricularLeukomalaciaorLesionsinor• InjurytotheWhiteMatterintheBrain• SensorineuralHearingImpairment• RetinopathyofPrematurityorAbnormalitiesin

RetinalVascularDevelopment• Long-TermUseofVentilationLeadingto

BronchopulmonaryDysplasia(BPD)orChronicLungDisease

Batshaw, M., Pellegrino, L. & Roizen., N. (2007).

• Currently in the US, approximately 1 out of every 8 babies is born with low birth weight or preterm.

• Prematurity alters the development of all sensory systems and is a leading cause of a variety of sensory issues.

• Vision and hearing are the most complex sensory systems and are at high risk of being affected by preterm birth.

• In Indiana, prematurity is the leading cause of combined vision and hearing loss.

severity of ROP. It was previously thought that too much oxygen (from excessive oxygen therapy) was the primary problem; however, further research has shown that oxygen levels (either too low or too high) play only a contributing factor.

It is important to remember that babies born early are at risk for vision problems even if they are not diagnosed with ROP or other eye conditions. Such problems may not be detected until later when a child appeqrs clumsy, is afraid of certain activities or has trouble playing with toys that require a lot of concentraion. It is essential that early intervention providers be aware of what to look for and work with families to address concerns whenever they arise.

Batshaw, M., Pellegrino, L. & Roizen, N. (2007). Children with Disabilities, 6th Edition (pp. 111-121). Baltimore, MD: Paul H. Brookes Publishing Co.

Biel, L. “Sensory Integration Problems in Preemies.” Premature Baby-Premature Child. 2009. 15 Sept. 2009 <http://www.prematurity.org/child/sensory-integration-preemie.html>

Purvis, B. (in press). The sooner the better: Effective strategies for identifying infants and young children with combined vision and hearing loss. National Consortium on Deaf-Blindness.

Edwards, L., Goehl, K. & Gordon, L. (1992). Profiles: Individuals with Deaf-Blindness. Terre Haute, IN: Indiana Deafblind Services Project, Indiana State University.

March of Dimes (2008). Maternal, Infant, and Child Health in the United States. Washington, DC: March of Dimes.

Roan, S. “Booster Shots.” Los Angeles Times 12 November 2008.

White, H. “Physical and Sensory Effects.” About Kids Health. 2009. 15 Sept. 2009 <http://www.aboutkidshealth.ca>

For additional resources and information about deafblindness, go to www.nationaldb.org.

References/Resources

Key Points to Remember

Families and educators of infants, toddlers and children who are deafblind need to have access to training and support. Each state has a federally funded technical assistance project specifically designed to improve educational services, provide opportunities for training, and to help support families of children who are deafblind. For more information or to request services in Indiana contact:

Indiana Deafblind Services ProjectBlumberg Center, COE/University Hall

401 N. 7th St, Room 009WTerre Haute, IN 47809

www.indbservices.org

Lisa Poff, Program CoordinatorLisa.Poff@indstate.edu

TA, Training, & Support

The purpose of the Indiana Deafblind Services Project’s (INDBS) The Sooner the Better Fact Sheets is to increase knowledge of early intervention and early childhood education personnel, families, medical and community agency personnel about factors related to combined vision and hearing loss in young children.

WELCOME to

MY HOME

Hi. Welcome to my home. I think. I mean, maybe you’re welcome. I’m not sure yet. When I get to know you,

I’ll know for sure. My child has a disability and I need help to do all the things he needs done. So I need you.

He needs you too, because he gets worn out and bored with me and sometimes dislikes me about as much as I

sometimes dislike him (Please don’t start making judgments about me – we just got started. It’s just that I’m

honest, and as much as he’s the sole reason for my existence, there are times when both of us wear thin).

Your agency sent you here. I called for help, but I don’t get a choice of who comes into my home and into my

life. You come at your convenience, usually between 9am and 3pm Monday through Friday. I’m on my own

evenings and weekends, when my other children tug at me and want me and feel slighted and offended and

I feel stretched to my limit. You call and tell me you’re coming Tuesday morning so I put the stack of

unanswered mail and the unpaid bills in the cabinet with the cereal bowls, race dirty and clean clothes up and

down the stairs, shove toys and unmated shoes in closets and under beds, and run the gauntlet with Fantastic

to get fingerprints off everything, and then you call and tell me you have to cancel because of a meeting. Oh

sure, I understand – Yes, that’s fine – Friday afternoon? Well, I was going to try to go to the library and maybe

take a nap. What? Oh, that’s the only day you have? Well, sure, I know it’s important that you come. And we

really need your help. Fine – Friday at 1:30. We’ll be here.

My husband resents people coming in and out of our home. He says he feels as though he is living in a goldfish

bowl. He says getting help means sacrificing our privacy and spontaneity. He can’t scratch his stomach as he

walks down the hall in his shorts anymore. Now he has to have clothes on and suck in his gut and put on

company manners. And he really hates it after you leave because sometimes I cry because I feel inadequate

and stupid and foolish and just plain wrong. Sometimes you make me feel this way when you act suspicious of

what goes on when you’re not here and try to trip me up when we’re talking to find out if I’m really doing the

goals and objectives – or if I’m just taking the money and fudging the paperwork. Sometimes it’s nothing you

say or do – it’s just that your perfectness unsettles my motherness.

Sometimes when you are great I feel threatened, and because of others who came before you, I feel judged

and talked about, as though you have met with others and have developed a plan to implement on me.

I can’t always tell when you’re real, but my son can. So I watch him. If he responds

and welcomes you, then I set aside my needs and cares and let you have everything

I have, including my son. I have to trust you because he trusts you and looks forward

to your step on the porch.

What? Oh good grief! I forgot your paperwork again! Wait, I know it’s here somewhere. I was working on it

last week just after the hot water heater burst and right before my husband and came home laid off. Wait—I

think I wrote on the back when the bank called about the deposit to cover the overdraft. Yeah—I found them!

Right behind the peanut butter—wait—I’ll just wipe them off a bit.

You know, I used to be normal. I used to have control over my life, my time, my home. Have a child with a

disability turned my life upside down. My priorities changed.

What I would settle for changed. What I would ask for changed. All that changed because my child needs

things and people and ideas and funding. So my life consists of meetings, regulations, documentation and

paperwork, social workers and agency people, policies and procedure manuals and administrative decisions,

delays and rumors of delays in checks, people not showing up when needed, people quitting, and people

showing up when they’re not needed.

Please don’t judge me, and I’ll try not to judge you. You see, in the long run, if I don’t measure up I am still his

mother. So we are stuck with each other and will make the best of it. Help me to grow. Help me to become

better. Accept me as a person – not some perfect saint. I really DO know my child better than anyone else, so

help me express that and put it to best use. Walk with me a ways, not to judge me, but to understand my role

within the heart of my family. Give me tools and words and people that, like pieces in a jigsaw puzzle, interlock

to allow for my strengths to compensate for my shortcomings.

Please don’t push me past my endurance, because if you do, you’ll see me at my worst; short-tempered,

impatient, inflexible and emotional. I’m no good to my son then, either. Each one of us has that fine line. I try

to recognize when I’m approaching my line, and usually that’s when I’m most cranky and complaining to you.

Please realize that one facet of me is the tired b _ _ _ _, just as real and acceptable as the superwoman who

overcomes unbelievable obstacles. There are sunny days and then there are thunderstorms – all part of a

temperate climate.

Well, anyway – HI. Welcome to my home. I think.

Author(s) Unknown

From B. Purvis, National Consortium on Deaf-Blindness, 3/2008