ETIOLOGIES and DEAFBLINDNESS Dottie Snyder April 11, 2018 Vision and hearing 80% or more of learning occurs through vision All learning takes place through movement Dual sensory loss limits access to people, places, and things Impact on learning is HUGE Incidental learning is limited Distorted input visually and auditorially If no direct interaction, no engagement

Common Causes Over 50 etiologies that include vision and hearing loss Most common Usher syndrome and other genetic condition Rubella Syndrome Meningitis and similar illnesses Injury auto accident, falls Aging Congenital blindness with acquired hearing loss Kidney failure Prescription or illegal drugs

Loud noise Congenital hearing loss with acquired vision loss Macular degeneration Cataracts Diabetic neuropathy Glaucoma Tumors NC Annual Child Count Unknown/Undetermined most common entry Complications due to Prematurity (45) CHARGE Syndrome (28) Head/Traumatic Brain Injury (8) Usher Syndrome (8) Cytomegalovirus (CMV) (8) Hydrocephalus or Microcephalus

Hereditary Syndromes and Disorders CHARGE Syndrome 924 Down syndrome (Trisomy 21 syndrome) 305 Usher I syndrome

215 Stickler syndrome 133 Dandy Walker syndrome 111 Goldenhar syndrome

107 Cornelia de Lange 99 Usher II syndrome 72 Wolf-Hirschhorn syndrome (Trisomy 4p)

71 Trisomy 13 (Trisomy 13-15, Patau syndrome) 55 Pre Natal Congenital Complications Cytomegalovirus (CMV) Hydrocephaly Microcephaly Maternal Drug Use Congenital Rubella

Fetal Alcohol syndrome Congenital Toxoplasmosis Neonatal Herpes Simplex (HSV) OTHER: Pre-Natal/ Congenital Complications 292 213 208 77 38 20 11

10 510 Post Natal Non-Congenital Complications Asphyxia 199 Severe Head Injury 161

Meningitis 135 Tumors 83 Stroke 56

Encephalitis 49 Infections 32 Chemically Induced 13

Direct Trauma to the eye and/or ear 10 OTHER: Post-Natal/ Non-Congenital 399 Complication of Prematurity 1028

No Determination of Etiology 1814 What is an etiology? Study of etiology is ancient China, Greece, and Rome Etiology the study of causes Used in anthropology and physics Most commonly used in the medical world

Why is important to know the etiology? Important in treating the person Easier to treat when you know the cause Unknown/Undetermined Not sure of changes in health status Try a variety of instruction techniques to find what works Lack of informational literature Complications of

Prematurity General Information Born between 34 to 36 weeks The earlier the baby is born, the higher the risk of complications Birth weight can be a factor as well Complications determine length of stay in the neonatal intensive care unit (NICU) Risk of short term and/or long term complications Development of sensory system may be impacted Short Term Complications

Breathing lungs dont expand Heart low blood pressure, failure, murmur Brain bleeding (short term effect vs permanent damage) Temperature low core body temperature Blood anemia, jaundice Immune system - infections Long Term Complications Cerebral Palsy movement and muscle tone Learning developmentally delayed, learning disabilities Chronic health issues infections, feeding, and asthma Behavior and Psychological problems

Vision Retinopathy of Prematurity Hearing at risk for some hearing loss Prematurity and vision and hearing loss Impact of combined vision and hearing loss Loss of access to people, things, places, activities, Limited incidental learning Concept development delayed, distorted, or doesnt happen ensory System TACTILE

(Touch) VESTIBULAR (Balance and Motion) What it Does Communicates pleasure and pain Helps babies identify and recognize objects Helps babies explore their world Helps babies keep their balance and be ready to learn to sit, crawl and walk

Helps babies know where they are in space GUSTATORY (Taste) Encourages babies to explore their environment Helps later learning skills, such as eating and playing with toys OLFACTORY (Smell)

One of the most well-developed senses in newborn babies Helps with recognition and discovery Plays an important role in feeding AUDITORY (Hearing) VISUAL (Sight) Important for attention and learning

Helps babies know about whats going on around them and anticipate certain events Is basic to the development of spoken language 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 What Might Happen

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

Exploring things by mouth is part of typical infan development and babies who have negative experiences may be less interested in doing thi which can affect their overall development. Babies demonstrate positive and negative reactions to certain smells very early. Early exposure to strong smells causes negative memories. Triggering these memories may cause negative reactions, even into adulthood. Babies exposed to high noise levels may find it difficult to filter out background noises and pay attention to whats important. This may cause

sleeping or attention problems in some children Hearing problems may also exist. Babies born early are at risk for vision problem These problems may come from damage to the underdeveloped eyes or from certain conditions that affect premature babies. Some may not be detected until later when a child appears clums is afraid of certain activities or has trouble playing with toys that require a lot of concentration. Retinopathy of Prematurity (ROP)

Underdeveloped blood vessels bleeding blood vessels leak scarring tissue build-up retinal detachment retina detaches from underlying membrane Possible effect on vision

decreased visual acuity severe myopia (near sighted) field loss Strabismus glaucoma Vision Visual acuity ability to distinguish object details and shape at specified distances (20 feet for distance, 16 inches near vision) Severe myopia inability to see at distances Corrective lenses or contact lenses High lighting

Minimal glare Field loss ability to use indirect vision to see to the side, upper, and lower fields Strabismus eyes do not focus on an object at the same time Esophoria one or both eyes turn inward

Esotropia crossed eyes Exophoria one or both eyes turn outward Hypertropia one or both eyes turn upward Hypophoria one or both eyes turn downward Prisim glasses Eccecntric viewing

Use one eye for distance, one eye for near tasks Eye strain, blurring of print, loss of place when reading Preferential seating Source: Horizontal Refraction Glasses Bed Prism - Eyewear Traders Glaucoma increased eye pressure

flucuating vision peripheral loss poor night vision light sensitivity

near vision problems decreased contrast sensitivity headache red eyes dizziness cloudy cornea degeneration of optic disc Adaptations/Considerations Sunglasses Adjustable lighting No glare

Good contrast Magnifiers Closed-circuit television (CCTV) Be aware of fluctuation in vision performance Be aware of pain Travel assistance in unfamiliar areas Communication Receptive language - understand language before they speak (comprehension) Expressive language ability to speak May talk late

The Premature child goes to school Initially, may be all about health care depending on gestation Early Intervention and sensory stimulation critical OT, Speech/Language therapists, stimulating environments at home and EI settings May lag behind through elementary school On-going therapy and intervention Work with child on strengths and weaknesses, build confidence Tutoring and teacher/parent contact May catch up in middle school, especially if focused on a goal

RESOURCES ROP Premature Birth mptoms-causes/syc-20376730 Levack, N. (2001) Low Vision A Resource Guide with Adaptations for Students with Visual Impairments, 2nd Edition, Texas School for the Blind and Visually Impaired Being Born Early Means More than You Think Hear See Hope Resources - ROP

LookingAhead/OverviewofLearningandEducationinthePrematureBaby/Pages/ Back-in-the-Classroom.aspx USHER SYNDROME Usher Syndrome Is genetic with a hearing and vision loss Vision loss Retinitis pigmentosa Retina deteriorates Diagnosed by an ophthalmologist or an optometrist Hearing loss Sensorineural

Newborn screening identifies infants with hearing loss Estimated that 1-6,000 to 1 -25,000 have US Usually normal intelligence Usher Syndrome Type I Born with severe to profound hearing loss Poor balance walk around 15 months Night blindness as early as age 5 Visual field loss age 10, usually less than 20 by mid 20s Central vision progressive loss, cataracts

Prognosis varies, may have light perception Type I Implications RP may not be diagnosed until early teens Many children attend schools for the deaf ASL is usually the primary communication mode Cochlear Implants may help Many consider themselves members of the deaf community Acceptance is individual Usher Syndrome Type 2 Hearing loss at birth, may be mild

Language delay or speech irregularity may be first sign Balance normal Night blindness teens to 20s Visual field loss late teens, slow progression Central vision late teens through early adulthood Prognosis varies, fair vision in daylight through 20s to middle age or later, possible only light perception around age 60+ Type II Implications US may not be apparent or suspected at birth Hearing loss at birth through early childhood may be very mild Hearing aids may be beneficial, especially from diagnosis to late

middle age RP may not be diagnosed until mid-teens or later Acceptance may be more difficult life changes Finding support and information may be challenging for the family and individual Usher Syndrome Type III Normal or near normal hearing at birth Hearing loss noticeable in the teens Significant hearing loss to deafness by late adulthood Vision may be normal Blind spots in the late teens to early adulthood

Legally blind in mid-adulthood Onset varies from person to person Audiograms Audiogram Type I Check copyright Type II Audiogram Type III

Retinitis Pigmentosa (RP) Retina is affected Degeneration of photoreceptor cells in the retina Rods light sensitive receptor cell that works in low light Cones light sensitive receptor providing sharp visual acuity and color discrimination As receptors die, vision is lost RP Symptoms Difficulty seeing at night Difficulty seeing in low light

Loss of peripheral vision May take many years to progress to blindness When it is not Ushers Syndrome Possible to have RP and bilateral hearing loss and NOT US Alstrom Syndrome Bardet-Biedel Syndrome Cockayne Syndrome Refsum Syndrome Flynn-Aird Syndrome Sometimes US is incorrectly diagnosed Retinal specialist can verify

Usher Screening- Back in the day . . . . Student questionnaire Low contrast Cone adaptation Balance Field Hand Disc Perimeter Students who passed Legitimate Vision was fine, no Usher Syndrome Knew how to answer questions, perform tasks

Laid-back evaluator Unqualified evaluator Young adults (aged 11 - 21) with Ushers Syndrome Students who failed US screening Vision problems (acuity) Didnt understand the directions Evaluator didnt understand the directions Wanted to go with staff for eye appointment Classroom implications Lighting good lighting with minimal glare

Seating Non-cluttered background Avoid excessive movement Windows behind student Easy access to areas in the classroom Keep drawers and doors closed

Environment Boards clean, high contrast marker Neutral colors Travel - Inside Travel obstacles, hazards, familiar, and unfamiliar Arrangement of furniture Trailing rails Keep drawers and cupboard doors closed Contrast wall, floors, furniture Textures/Cues stairs, classrooms

Steps Color contrast, textures Glare Travel - Outside Night travel Lighted pathways Tactual guide strips Adequate lighting on buildings name, doors, numbers Placement of planters and other potential hazards Placement of informational signs (speed, do not enter) Use of human or dog guide

Label the rooms Print 2 inches high Color Contrast Placement on the wall by the door. Consistent Braille Consider raised numbers and letters Materials Learning Media Assessment (LMA) Print or braille? Print contrast contrast

Size font font Tactile skill development size size Individual Materials vs Universal Design Graphs or charts Tests Assistive Technology

Low vision aids FM system TESTS to identify RP Elctroretinogram (ERG) The definitive test for RP, however, is the the electroretinogram (ERG), which has been found to be 95 percent accurate. The test must be administered by an ophthalmologist. An ERG measures the elecricity given off by nerve impulses in the retina of the eye. The patient sits in a dark room until the eyes adjust to the darkness. One eye is then patched and the other fitted with a special contact lens. The test, which is painless, measures the electrical response of the uncovered

eye as it is exposed to flashing lights. Electronystagmography (ENG) This test is considered the gold standard for diagnosing ear disorders affecting one ear at a time. This test is used to determine whether or not an individuals dizziness or vertigo is caused by inner ear disease. This test involves carefully measuring the eyes involuntary movements while the individuals balance is stimulated in a variety of ways. The test is composed of four separate parts: (1) the calibration test, which will assess rapid eye movements, (2) the tracking test, which will assess the eyes movements as it tracks a visual target, (3) the positional test,

which will measure dizziness depending on the posture of the head, and (4) the caloric test, which will measure the ears response to varying temperatures of water run through the ear in small tubes. Visual Field Test Measures side vision using a machine called a Goldman Perimeter. It will indicate the field of vision (normal field=180 degrees). Psycho-Physical Test Indicates which colors, if any, an individual can distinguish and the amount of contrast needed to see. Dark Adaptation Test Determines an individual's ability to see in the dark and how long it

takes to adjust to the dark. Your Teacher of the Visually Impaired can help provide all the accommodations and moderations needed to make the classroom and all educational activities accessible. Communication Speech Use of FM or other devices Be aware of potential to miss words Sign Language

Within visual field Tactile Interpreter Use of touch cues throughout the day (Haptics) The Teacher of the Deaf and Hard of Hearing can help you determine and create the most useful communication mode.

Adjustment - Family Has the family made the child aware of the diagnosis? Acceptance by the parents, by the child Grieving process both child and parents Denial/Isolation

Anger Bargaining Depression Acceptance Counseling family and child Adjustment - student Different from peers Acceptance of support services O&M, braille, career goals Depression Isolation

Major life changes hobbies, friendships, spontaneity, independence, freedom, driving Change in communication mode Deaf culture accepted or no? Time Deaf-Blind Standard Time

Adequately explain the material in preferred language mode Use of touch cues by one to one staff Examine materials Ask questions regarding instruction Time to process all the information Time to produce the answers Assignment adjustment half of the questions Resources Ushers Syndrome w-about-usher5.html Ashley Benton, NC Deaf/ Deaf-Blind Coordinator [email protected] Tip Sheet %20Students%20with%20Usher%20Syndrome.pdf Cytomegalovirus (CMV) General Information Affects people of all ages

By age 40, over half of the adults have been affected It stays in the body forever and can reactivate Most people show no symptoms CMV transmission Blood transfusions Organ transplants Respiratory droplets Saliva Sexual contact Urine Tears

Most people come into contact with CMV in their lifetime Babies One of 200 babies are born with congenital CMV infection Only one of five will be sick or have long term health problems The virus passes from the mother to the infant Signs and Symptoms At birth Premature

Small Small head Seizures Organ problems liver, lungs, and spleen Signs and Symptoms Long term Hearing loss Vision loss Intellectual disability Small head size Muscle weakness Seizures

Treatment Antiviral medications for infant showing signs and symptoms at birth Regular hearing checks Regular Pediatrician follow-up Educational Consideration Hearing Vision Intellectual disability Seizures Support services Physical Therapy, TVI, Occupational Therapy,

Teacher of the deaf/HH CMV in North Carolina 7 of 8 have Intellectual Disability Five have orthopedic complications 8 of 8 have communication limitations 4 of 8 have complex medical needs CMV Resources es/syc-20355358 CEUs To be eligible for the 1.0 CEU you must Attend all 5 webinars Respond to questions If you have questions please contact me QUESTIONS In your opinion, what are some advantages and disadvantages in having a child labelled with an etiology or etiologies? Do you believe that the NC census accurately represents the number

of children with a) ROP, b) Usher Syndrome and c)CMV? Choose one of the three etiologies above and briefly explain why you believe that the census is accurate/inaccurate. Choose a child that you currently work with. Do you what his/her etiology is? How does knowledge of the etiology drive your services or what other factors do you use to determine how to best serve the child? EVALUATION Please take a minute to respond to the evaluation: 4- High Quality/Relevance 3-Good

2- Fair 1Poor The presenter was knowledgeable on the topic The webinar content will be useful to me in working with students I have reconsidered the abilities of students who are DB based on the webinar The webinar was an effective method of providing the information One hour was the right amount of time for this topic Comments: Submit answers and evaluation to: [email protected]

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