Children with Special Needs

Question 1

What physical and cognitive conditions will add to the complexity of audiologic assessment?

Answer 1

Severe to profound hearing loss
Developmental delays
Physical challenges
Autism spectrum disorder
Attention deficit/hyperactivity disorder
Visual impairment
Functional hearing loss

Question 2

What are the 4 major etiologic classifications of congenital hearing loss?

Answer 2

Chromosomal origin
Genetic origin
Environmental teratogens
Low birth weight

Question 3

How many newborns who are deaf or hard of hearing have additional neurodevelopmental conditions?

Answer 3

25 to 50%

Question 4

Why are these children difficult to test?

Answer 4

Auditory response behavior may not be as predictable
Might not orient to sound
Might be hypersensitive tosound to the extent that they exhibit painful hearing
Might have a preoccupation with or agitation to sound
Cannot be conditioned to sound
Might demonstrate very poor test-retest reliability within a test session and between test sessions
May have problems with speech, responding to sound, and understanding the task

Question 5

What are some things to expect while testing this population?

Answer 5

Longer time to focus
Latency time
Consider other responses
May fatigue quicker
Unexpected reactions

Question 6

By appropriately controlling the test environment, can almost any child be tested using behavioral techniques?

Answer 6

Yes
If a child cannot be tested with behavioral test measures, the audiologist needs to take ownership for the inability to test and say “I was unable to test this child” rather than “This child is untestable”

Question 7

How does testing these individuals usually begin?

Answer 7

With electrophysiologic testing (ABR and OAE)
If this testing indicates no concern, and if parental and therapist observation does not indicate any concerns, additional testing might not be needed
If hearing is a concern, behavioral testing is critical

Question 8

Is the cross-check principle necessary to complete a hearing evaluation?

Answer 8

Yes

Question 9

Can observing the child in the waiting room provide clues of the child’s physical and developmental status?

Answer 9

Yes
Temperament of the child
Alertness of the child
Clues about the developmental status of the child (e.g., walking, talking).
Clues about the interaction/communication between the child and their family
Independence/shyness/fearfulness following introduction
Willingness of the child to engage/participate in conversation

Question 10

What are some rapport-building and anxiety-reducing strategies?

Answer 10

Introduce yourself to the child and family
Complementing the child on attire or toys
Ask about grade level or age
Introduce positive reinforcements/rewards that might be used during or at completion of assessment
Inform the child and family about expectations of what is to come during the assessment (ease anxiety and fears)
Allow the child to touch equipment

Question 11

Can BOA be useful with this population in conjunction with physiologic measures?

Answer 11

Yes

Question 12

What are three categories in which BOA testing results might be placed?

Answer 12

No observable response to sound
Responses only to high-intensity stimuli (70–80 dB HL)
Responses to relatively soft and comfortable stimuli (30–50 dB HL)

Question 13

What are some things to consider when doing BOA?

Answer 13

Remain unbiased when judging the presence or absence of a response
Enlist the assistance of a second audiologist
Reduce habituation by alternating between several differenttypes of stimuli
Enlist the parents’ assistance in determining a response

Question 14

What are three factors that should be considered when performing VRA?

Answer 14

Judging response behavior (might be more difficult, have second audiologist assist, increase probe trial and control trial duration)
Increasing attention and motivation (darken room, keep child in alert position, minimize distractors, vary auditory stimuli, use longer presentation of reinforcement)
Decreasing false responses (reshape responses, versatile midline distractors, lengthen interstimulus intervals)

Question 15

When performing CPA on this population, should you customize the task to match their physical and behavioral characteristics?

Answer 15

Yes

Question 16

What are some considerations for CPA?

Answer 16

Use tactile cues (can be helpful in teaching the task)
Practicing the task together several times
Consider using narrow-band noise or warbled tones (more interesting and novel)

Question 17

What are some things to consider when performing conventional audiometry on this population?

Answer 17

Several modifications can be made if needed
Remember there is a small testing window
Keep them motivated and attentive by intermittent social reinforcement and providing different response options

Question 18

What are some things to consider when performing speech audiometry on this population?

Answer 18

Select the appropriate speech perception measures that match the developmental level of the child and their vocabulary level

Question 19

When are SRT and SAT used for this population?

Answer 19

Can be obtained with a variety of stimulus words or phrases to gain the child’s attention and cooperation
The signal selected can be delivered in repetition until response is obtained
Test trial duration should not exceed 5sec

Question 20

What must we consider when trying to obtain SRTs for this population?

Answer 20

Must consider the child’s familiarity with the words being considered and the ability to repeat the word
Use pointing tasks, game tasks, or classic repetition games`
Ask child to point to body parts or a parent’s body parts (e.g., “show me your nose,” “eyes,” “hair,” “fingers,” “toes,” “shoes”)

Question 21

When is physiologic testing required for this population?

Answer 21

If they are not able to participate in behavioral testing
Might be lethargic, hyperactive, combative, tactilely defensive, or unwilling to sit quietly
Might be unwilling or unable to comply with instructions and unable to cooperate for a sufficient length of time for test completion

Question 22

What are several modified strategies that can easily be incorporated to maximize patient compliance?

Answer 22

Choosing appropriate audiologic tests and deciding on the appropriate order of test presentation are essential to a successful outcome

Question 23

How are acoustic impedance measures useful?

Answer 23

Useful in the interpretation of other components in the audiologic test battery
Carefully observe the child (is he ready, calm, crying?) and tailor your approach accordingly
Enlist assistance from the parent
This could range from verbal reassurance to gentle restraint of the child
Enlist a second audiologist to provide positive comments or visual distractions such as bubbles
Select screening mode when possible rather than the diagnostic mode

Question 24

How are OAEs helpful?

Answer 24

Expand the pediatric audiology test battery by providing a physiologic means of assessing pre-neural auditory function
Enlist parental support in holding the child on their lap and hold the child’s hands as necessary
Place younger children in a highchair.
Allow the child to quietly play with objects of distraction
Have a small DVD player available and allow the child to watch a cartoon or children’s video without sound
Consider your protocol selection

Question 25

Can children with complex developmental conditions often present challenges to the interpretation of ABR and ASSR findings?

Answer 25

Yes
Several studies have reported delayed conduction times in the ABR of children with autism
Children with hydrocephalus can have elevated or absent ABR thresholds
The ABR in children with Down syndrome reveals Wave V latency and amplitude differences at reduced intensity levels when compared to normal developing controls. However, at greater stimulus levels (e.g., 60 dB nHL) latency of the ABR is shorter than matched control subjects
Children with ANSD

Question 26

For children who are neurologically, cognitively, and/or behaviorally involved, should the interpretation of ABR waveforms be done with caution?

Answer 26

Yes
Findings should be considered as part of a test before developing a definitive diagnosis of the child’s hearing status

Question 27

How do you facilitate electrophysiologic recordings in this population?

Answer 27

Request that the child arrive sleep
Request that the bottle-fed or nursing child arrive hungry
Reduce stimulation in the room
Make the setting more comfortable; e.g., recliner, rocking chair…
Allow the parent to bring any items that comfort the child
Sedation might be required in some cases but might pose risks for some children

Question 28

How can you customize a hearing assessment?

Answer 28

Consideration must be given to any physical or cognitive limitations that could affect the assessment procedures
Audiologic test protocols must be, by necessity, flexible to accommodate individual child differences and preferences
Adapting test parameters might be necessary to match the physical and behavioral characteristics of the children under test

Question 29

Can children with severe to profound hearing loss who are developmentally normal be taught auditory test tasks in the same way as their normal hearing peers?

Answer 29

Yes
Except it may take more than the usual number of presentations for the child to learn to respond to the conditioning task
May not be able to be conditioned with the same noise level (do the same thing but make things louder)

Question 30

Is it important to not give visual cues for children with severe to profound loss?

Answer 30

Yes
They are usually very visually alert

Question 31

What transducer should you use for a young child with a severe to profound hearing loss?

Answer 31

For very young children, testing may begin in soundfield but if a child shows no aversion to otoscopy or tympanometry, it may be easy to begin with insert earphones
Begin with low frequency stimuli
If not responding to an auditory stimulus, use a tactile stimulus

Question 32

Do some children with developmental delays have structural deformities of the ear?

Answer 32

Yes
They may also have significant ME disease
It is critical to know the cognitive age in order to select the appropriate test protocol

Question 33

What are some special testing considerations for testing a child with a developmental delay?

Answer 33

Positioning
Timing of test stimulus presentation (calibrate your speed and rhythm to them)
Response reliability and interpretation
Don’t forget control trial (don’t present and see if they respond)

Question 34

What is intellectual disability?

Answer 34

Also a developmental delay
Includes impairments of general mental abilities that impact adaptive functioning
Almost 10% of children with hearing loss also have intellectual disabilities

Question 35

What are the two areas in which children with intellectual disability usually have limitations?

Answer 35

Intellectual functioning
Adaptive behaviors

Question 36

What is intellectual disability characterized by?

Answer 36

Impaired cognitive functioning
Below-average intelligence
Lack of skills necessary for day-to-day living

Question 37

What are some special testing considerations for testing children with intellectual disabilities?

Answer 37

VRA is effective and can be used with infants as young as 6 months cognitive developmental age
However, children with Down syndrome require a cognitive developmental age of 10 to 12 months to successfully participate in a VRA procedure
Some children with intellectual disability may not have developed auditory localization ability
Tend to respond to thresholds 10 to 25 dB poorer than what was confirmed with ABR
Use tactile-auditory conditioning procedure: pairing auditory stimulus with a tactile stimulus
Demonstrate the play task to the patient (CPA)
Include control trials
Monitor ME status bc of higher incidence of CHL and abnormal tymp (do BC whenever possible)

Question 38

What are the three types of cerebral palsy?

Answer 38

Spastic - high muscle tone producing stiff and difficult movement
Athetoid - producing involuntary and uncontrollable movement
Ataxic - low muscle tone producing a disturbed sense of balance, disturbed position in space, and general uncoordinated movement

Question 39

Can children with motor disabilities find it difficult to respond to sound?

Answer 39

Yes
May find it difficult to orient to sound due to physical limitations

Question 40

What are some VRA modifications for children with physical disabilities?

Answer 40

Use an infant seat to provide additional head support
If head turn is difficult, alternative responses such as localizing to the sound stimuli with their eyes as opposed to head turns can be accepted

Question 41

What are some CPA modifications for children with physical disabilities?

Answer 41

Select toys that are easy enough for the child to manipulate; fine motor skills vs. gross motor skills
For older children partial hand raising, or even just a head nod can be considered as acceptable responses

Question 42

Should you limit speech tests for children with physical disabilities to closed set tests?

Answer 42

Yes

Question 43

What are some ABR considerations for children with physical disabilities?

Answer 43

Sedation may be required when conducting ABR with individuals who have CP in an attempt to relax their head and neck or to reduce extraneous muscle movements, thus reducing myogenic artifact
If the physical disability has a neuromotor component, physiological measures might be affected (Yilmaz et al., 2001) resulting in an abnormality that could be misinterpreted as indicative of hearing loss

Question 44

Are children with ASD known to lag behind on language milestones?

Answer 44

Yes
They will likely be referred to audiologists for hearing assessments as part of the developmental evaluation to rule out hearing loss as the cause of language delay (no increased risk of hearing loss)

Question 45

Do children with ASD exhibit abnormal responses to sounds?

Answer 45

Yes
May ignore sounds
May appear overly sensitive to sound

Question 46

During behavior testing, do children with ASD (who has normal hearing) have elevated thresholds and are less reliable?

Answer 46

Yes
Children with ASD who have hearing loss are diagnosed, on average, almost 1 year later than those without hearing loss

Question 47

What are some testing modifications for ASD patients?

Answer 47

Test set up must be well controlled!
Seat the child in a position that does not permit her to walk away easily from the test situation
Minimize physical contact with those who have tactile sensitivities; test in the soundfield
Transitions are often difficult for individuals with ASD
When possible, escort the patient to the testing area immediately rather than keeping him or her in the waiting area
Identify cognitive age before selecting a test protocol
Best to avoid speech stimuli at least initially
Select an appropriate distraction toy that will keep a child interested but not too involved to tune out auditory stimuli
Present stimuli at low levels and increase intensity gradually
Use loud stimulus for those children who ignore sound and almost appear “deaf”

Question 48

How should you choose a behavioral test for ASD children?

Answer 48

If VRA is used, consider minimizing the impact of the reinforcement by turning off the animation (if a lighted, animated toy is used) or using a video reinforcement
Tangible-reinforcement operant conditioning audiometry (TROCA) is noted to be particularly effective with children having cognitive or behavioral (e.g., ASD) disorders

Question 49

Can you do ABR as an objective test at ASD?

Answer 49

You can
Individuals with ASD are known to be difficult to sedate with currently available pediatric sedating agents and are at risk for seizures while under sedation
Difficulties with this may delay diagnosis

Question 50

Are children with ADHD difficult to test?

Answer 50

Yes, it may take longer than expected
They have a great deal of energy
They have a difficult time attending and sitting still

Question 51

What are some testing modifications for children with ADHD?

Answer 51

Organize test room carefully and use a structured test environment
Seat the child in a highchair or at a table with the chair pulled in close to encourage him to stay seated with his feet firmly placed on the floor to reduce fidgeting
Remind the child more often to attend to the stimulus
Change toys frequently to keep interest
Take small breaks if the child becomes bored
Jumping jacks
Walk to the water fountain

Question 52

What are some possible etiologies for children with vision and hearing deficits?

Answer 52

Syndromes such as CHARGE syndrome, Usher syndrome…
Congenital prenatal infections (e.g., rubella, toxoplasmosis, herpes, CMV)
Postnatal causes of vision and hearing deficits (e.g., meningitis, asphyxia, stroke)

Question 53

What are some test considerations for testing children with visual impairment?

Answer 53

Let the patient explore the test environment for a short period of time or until the patient appears to be comfortable
Allow the patient to examine the equipment (e.g., otoscope, ear- phones) tactilely (they explore with their hands)
Auditory responsiveness may be compromised by their lack of curiosity; they may not turn toward the source of sound for a VRA procedure

Question 54

Can children with visual impairment at the three-year-old level and higher perform CPA?

Answer 54

Yes
As long as the toy doesn’t require difficult physical manipulation

Question 55

What are some special considerations for children with vision loss?

Answer 55

Move reinforcement closer to patient if they have close vision
If not sufficient, darken the test room and use a bright flashlight
If the child does not have sufficient vision to see the bright light, pair the auditory stimulus with a vibrotactile stimulus

Question 56

Do children between the ages of 8 to 12 demonstrate functional hearing loss occasionally?

Answer 56

Yes

Question 57

When should we suspect functional hearing loss?

Answer 57

Test results do not agree with the child’s ability to communicate
Tests indicate elevated thresholds with normal OAEs
Speech recognition thresholds are much better or worse than pure tone thresholds
Responses to speech stimuli are unusual
Test results are not repeatable
Unmasked bone conduction thresholds are much poorer in one ear than in the other

Question 58

What should you do when you suspect functional hearing loss?

Answer 58

Suggest that there may be something wrong with the equipment, “There must be something wrong with this equipment. It is making it seem that you have much worse hearing than I know you have. Let’s go into a different test room and try again”
Tell the child that “the first tests we did (OAEs and reflexes) tell me that you can hear soft sounds, so please make sure you respond when I play the soft sounds too”
If still not successful, use a portable audiometer and have the child seated next to you so that you can make eye contact
If the child’s responses still are not providing accurate results, have him count the beeps or use a yes/no response (find out where no disappears)