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I've reviewed a little bit about those symptoms of autism and tried to give examples of how they can present
both in hearing children. I've talked about ways that they might overlap in some cases for children,
who are deaf and hard of hearing. Then I've tried to give examples of how to distinguish those symptoms
of autism from typical development for children, who are deaf and hard of hearing.
The classic example, that's actually used, is the symptom that's commonly on checklists or screeners
for autism is, "Does your child respond to their name?" We know that children, who have reduced hearing,
may not respond to their name. That might be perceived as a symptom of autism,
however, we know that that's due to their reduced hearing.
Having said that, we still get concerned for children with reduced hearing,
who aren't responding to their name, if we know that
they should have auditory access to their name.
The child, who is deaf and hard of hearing
who has enough access to spoken communication
that they can hear when somebody turns on
the Thomas the Tank Engine cartoon, or
calls Thomas in the other room, and they orient to that.
But when you walk in the room, they don't look up,
or when you call their name, they don't look up
even when they're not engaged in something
that's capturing their attention or in an environment
that doesn't have a lot of other noise that would prevent
them from being able to discriminate that.
Similarly for children, who are deaf and hard of hearing,
if they don't respond to other typical ways of getting
their attention that are culturally appropriate,
sometimes I've seen children, who are deaf
who have autism, and I can tap them on the knee,
or on the leg, or wave in front of their visual field
and they don't orient to that. If they're not orienting
to the ways that we expect children, who are deaf,
to orient, we get concerned about a possible
autism spectrum disorder.
The overall idea here is to think about autism as a model of atypical development, that children are acquiring
these skills in ways that differ, that are not just delayed but that are markedly unique and that differ from
typical developmental patterns. The other thing that I want to emphasize is at any time a child,
who has reduced hearing or who is hearing,
loses a developmental skill, for example, if they stop
signing or they stop using spoken words
that they've been using for several months
spontaneously in different contexts, if they stop making
eye contact and stop seeming socially engaged,
or if they lose motor skills that they were able to do
previously. In any of those situations they should be
referred for medical follow-up. The reason for this is
that those could be symptoms of autism.
In a percentage of children, who have autism,
we do see a developmental regression that often occurs
between 12 months and two years of age.
Anytime you see that loss, we want to refer
for a workup to consider the possibility of an autism
spectrum disorder, but the physician will likely
also consider other medical explanations
for that loss of skill. In the case of children,
who are deaf and hard of hearing, if they stop speaking.
Of course we also want to include their audiologists in that to consider, whether there's been a change
in their hearing. There are some subpopulations of children, who are deaf and hard of hearing
who might be more at risk for autism.
When we look at the general literature, we see that
some syndromes or medical differences are more
highly correlated with an autism spectrum disorder
than in the typical population. These are often very
tricky, diagnostic workups, just because a child
has one of these potential diagnoses,
doesn't necessarily mean that they will have autism.
It just means that they might be more at risk.
In the case of children who have CHARGE Syndrome, who have both visual and hearing differences,
they really require a very astute clinical workup for consideration of autism, if we're concerned
that their social behaviors differ markedly from our expectations of other children, who are Deafblind.
Some of the other potential differences, that might be associated with autism include children,
who have symptoms of cytomegalovirus or CMV
when they're born. In the general literature of children,
who are hearing, there has been an association between
CMV and autism. Anytime that there has been
something that has potentially disrupted or changed
brain development, that could put you at greater risk
for autism. In some situations in some cases
of prematurity, those children might be at higher risk for an autism spectrum disorder. There's been one
study, that showed a higher incidence of autism among individuals with Usher syndrome.
Again for children, who have both vision and hearing differences, we really have to make sure
that we're clinically distinguishing the expected impact of those differences on their social behavior
from a symptom of autism. Both a rule-in process, where we rule-in whether they have the behaviors
that might be symptomatic of autism, but also a rule-out process, where we consider whether there might be
other explanations for those same behaviors. Some of the common conditions that might mimic
an autism spectrum disorder include children
who have an intellectual disability,
whose social skills and communication skills lag
behind their same age peers.
In the case of an intellectual disability, however,
we would expect children to acquire those
communicative and social skills at a rate consistent
with their development even though it's delayed
compared to other kids their age, different than
the atypical patterns of development, that we see
in autism. Some other things that you might look at
are speech and language disorder,
or communication disorder, ADHD, anxiety disorders,
mood disorders, and certain medical conditions
can have an impact on the acquisition of language and
can sometimes have an impact on the child's behavior.
Those could be things like children, who have seizure
disorders, that impact their language acquisition or use,
children, who have vision differences again can have
an impact on their acquisition of many of those early
social communication skills and their behavior.
Diagnosing autism among children, who are deaf and hard of hearing, you may be understanding by this point
is quite complex, and what contributes to that complexity is not just that some of the symptoms
might mimic symptoms of autism, might look similar to behaviors that we might typically see in children,
who are deaf and hearing, like not responding to their name or having a delayed theory of mind.
But that the ways that we typically assess an autism spectrum disorder are based on tools
that were not developed for children, who are deaf and hard of hearing.
They often include items that may be considered
unfair for children, who are deaf and hard of hearing,
either because the child's reduced hearing impacts
their access to the skill, like wondering whether
the child responds to their name or not.
Or the ways that we're assessing that look
different than how we would expect a child,
who's typically developing deaf and hard of hearing to act. Another complicating factor is that there are few
providers, who are trained both in deafness and
in autism and so that informs their perspective
in considering whether a child has an autism spectrum
disorder. They might mistake those overlapping
symptoms as being indicative of autism,
when they're really not, or they might fail to distinguish
those unique features of autism, and sometimes
we see providers, who are not familiar with deafness
attributing too much to their hearing loss rather than
really understanding the context of that child's behavior,
and how those behaviors differ from most children,
who are deaf and hard of hearing.
Again, there's the fact that some of the symptoms might
present differently in a sign language versus a spoken
language, and if you're not familiar with that, it would
make it difficult to assess. And because there are
few providers, who are trained in both deafness
and autism, there are few providers, who can provide
a direct assessment for children, who communicate in a sign modality. When you think about the symptoms
that we talked about that are unique language features in sign language, if you weren't familiar
with sign language, it would be very difficult to assess
for that via an interpreter. We also don't know how
having an interpreter in a room, when you're trying
to assess for that social interaction between
an evaluator and the child, might influence and shift
that dyad, and therefore influence the evaluator's
perspective of the child's social behavior.
Those are all things that significantly complicate the diagnostic process. Therefore, when assessing
for an autism spectrum disorder for a child, who is deaf or hard of hearing, best practice really is to refer
to a clinician who's familiar with deafness and autism,
who can communicate directly with a child.
If that's not available, I realize that in most places
in the country, it's not. It's really important to have
really good communication between the evaluator
assessing for autism with the child's other
professionals, who understand and know deafness,
their Teachers of the Deaf, their interpreter,
their speech language therapists, their audiologists,
other people, who have an expectation of what
to expect in terms of the child's acquisition of language
and their behavior.
It's also really important not to rely on any single
assessment tool for diagnosing autism.
Instead, we want to have multiple sources
of information. We'll talk a little bit more about this.
If you have to use an interpreter, again this is discouraged and not recommended.
But if an interpreter must be used, it's really important
to prepare them for what to look for. Those symptoms
of palm rotations, and pronoun avoidance, and asking
the interpreter to communicate to you,
if the child is using signs in ways that are unusual
compared to other children, who are deaf.
That can be very difficult to distinguish from
a communication disorder, but at least you'll
be preparing the interpreter to be looking
for those specific symptoms of autism.
It's also important for us as a field to better understand
the importance of that communication match
between the examiner and the child
during an assessment for autism. Right now, that's
something that we just don't totally understand.
I'm going to talk briefly about some of the tools that are used for assessing autism in the general population.
That is in the population of individuals, who are hearing. One thing that I want to caution people is that there are
many instruments that are out there, that can be used as screening tools or as diagnostic tools for autism.
Some of them have better statistical reliability and validity than other instruments,
and so even though none of these tools were developed for children, who are deaf and hard of hearing,
you want to at least start with the instruments that have been shown to have better reliability and validity
in the general population. And then try to interpret the results based on an understanding of what autism
can look like among children, who are deaf and hard of hearing.
One of the tools that is commonly used in screening
for an autism spectrum disorder in very young children
about 16 months to 30 months is called
the Modified Checklist for Autism in Toddlers
and there's a revised version available.
It's available. It's in Public Domain. It's available online
at the website, that you see here.
It includes a follow-up interview and again knowing how
autism can present in children, who are deaf and
hard of hearing, will be helpful to you in considering
those follow-up questions. There are other tools.
They are considered screeners like
the Autism Spectrum Screening Questionnaire,
which is for children, who are ages 6 to 17.
Once there has been an established concern for whether a child might specifically have autism
versus another developmental difference, there are some other screeners that have been developed
to look at that. To look more closely, one of them
that's commonly used in schools is called
the Childhood Autism Rating Scale.
It's now in the second edition. That's for children
ages 2 and up. This is an instrument that uses
clinician ratings based on that clinician's observations
and parent report. What this means, is that it shouldn't
just be distributed to anybody to complete
and must be completed by an evaluator,
who has a solid understanding of autism
and to try to use this tool effectively with a child,
who's deaf and hard of hearing means of that clinician
must have a solid understanding not just of autism,
but of how it could present in children who are deaf.
There are other parent report instruments that are commonly used as screening tools for an autism
spectrum disorder. One of the most common ones is called the Social Communication Questionnaire.
This is for children who are over age 4, and who have
developmental abilities over age 2.
This is a questionnaire where parents read questions,
that are based on a diagnostic interview called
the Autism Diagnostic Interview Revised,
that specifically asks some questions about autism
symptoms, and they indicate "Yes" or "No", and then you
calculate a total score. If that total score is over
a clinical cutoff of 15, then the individual would be
referred for a full evaluation. It's available in English
and Spanish versions. Again, looking at the items
of the Social Communication Questionnaire.
Some of the items are not necessarily fair or valid
for children, who are deaf and hard of hearing,
or have to be interpreted within a context, and what
we've seen clinically is that in many samples of children,
who are deaf who have been assessed
for an autism spectrum disorder, parents often rate
their children, who have autism as having more
of these symptoms than typically developing children
who are deaf and hard of hearing, but still is falling
below that clinical cutoff that's used as a guideline
for autism referral for hearing children.
What this means is, if you use this instrument
with children, who are deaf and hard of hearing,
first of all, you really have to interpret the results
cautiously, but secondly even if the child's score
technically does not exceed that clinical cutoff,
if you have concerns about these red flags
for autism, that we've talked about today,
they should still be referred for a full evaluation.
Another tool that's commonly used
is the Social Responsiveness Scale 2nd Edition,
and that's for children ages 2 through adulthood.
There are parent and teacher rating scales for this.
This instrument measures behaviors that might be
associated with an autism spectrum disorder,
but it can also capture developmental differences
that might be attributed to another developmental
difference, that affects social communication
and social understanding.
Another tool that is commonly used in an assessment
for an autism spectrum disorder is a clinical interview
that should be given by a professional trained in autism
and trained specifically on the tool called
the Autism Diagnostic Interview-Revised.
This is an interview with family members or caregivers
or parents of a child or individual that specifically
assesses for those symptoms of an autism spectrum
disorder, that then yields scores that are mapped
onto an algorithm. Again this is an instrument that has
some value in assessing for a possible autism
spectrum disorder, but has to be administered and
interpreted with an understanding of how deafness
affects the developmental trajectory of a child,
and how that's distinct from an autism presentation.
In general for many of the screeners and the tools that we have that were developed for children,
who are deaf and hard of hearing, even though we might expect on a surface review of the instrument
for a child who's deaf to score as possibly having an autism spectrum disorder.
Our clinical samples are showing that even when kids are given a diagnosis of autism, who are deaf
and hard of hearing, their parents often don't endorse enough symptoms to meet that clinical cutoff.
We have to use these tools very cautiously. However, having said that, if you have an understanding
both of deafness and autism, the tools can still have some clinical use in gathering information.
But children should be referred for further evaluation, even if their score does not exceed the clinical cutoff,
if there are concerns about an autism spectrum disorder.
We don't know much about, and we haven't established a clinically agreed upon best practice for assessment
of autism among children, who are deaf
and hard of hearing. But in a hearing population
the gold standard, what's called the "Gold Standard"
in assessing for autism, is considered
the Autism Diagnostic Interview Revised, or a similar
developmental and clinical interview, that again
assesses specifically for those symptoms of autism
as well as those rule-outs, those diagnostic rule-outs,
and those potential associated features that
we talked about earlier. In addition to that,
part of the "Gold Standard" is use of a tool called
the Autism Diagnostic Observation Schedule.
We are now in the Second Edition of that and finally
clinician's judgement. Again I want to emphasize that,
no tool in and of itself, should ever be used
in diagnosing autism.
It's not a hard and fast steady rule how a child scores
on a particular tool. Instead it's a cumulative
information that's gathered through this process.
I can think of many situations and especially for children, who are deaf and hard of hearing, where they may
or may not score on one of the tools that we use for an autism spectrum disorder.
But clinically the cumulative data suggests a different diagnostic conclusion.
There are some efforts underway in Great Britain to validate many of these tools for use with individuals,
who are deaf and hard of hearing, and we hope to learn from that research to be able to apply it
to other sign languages outside of British Sign Language, like adaptations for American Sign Language.
Even though we just talked about the caveats to using these assessments for diagnosing autism
with children, who are deaf and hard of hearing, in an unpublished survey that was distributed to professionals
trained in deafness by Dr. Shield and myself, when we asked them how they assessed for an autism spectrum
disorder among children, who are deaf and hard of hearing, the Autism Diagnostic Observation Schedule,
the ADOS-2, was described as the most commonly used instrument. Having said that, there was a lot
of variability in the training of the individuals, who are using the instrument and how it was being used
and modified for children, who are deaf and hard of hearing. There are definitely challenges
to using the ADOS-2 with children, who are deaf and hard of hearing. In fact, if you read
the manual, it indicates that the instrument is not recommended for use with individuals
with vision or hearing loss. One of the biggest challenges is that in
the Autism Diagnostic Observation Schedule, signs are
calculated as equivalent to gestures. Rather than
perceiving signs to be equivalent to spoken language.
The signs, if this tool is used in a standardized way,
are considered equivalent to gestures.
Currently, there's no agreed-upon or research-based understanding of how to use the ADOS.
However, in my clinical experience, there's value to using it, if you come to the table
with an understanding both of deafness and autism. Here's an example of why the tool can be challenging
when used with children, who are deaf and hard of hearing, and why we particularly need to avoid
using the scoring algorithms that were developed for hearing children with children, who are deaf
and hard of hearing. One of the scoring items on the ADOS is,
"How does the child respond to the examiner or the parent calling the child's name?"
We've already talked about how this can be impacted by a child's reduced hearing.
Another item on the scoring algorithm is the intonation of the child's vocalizations or verbalizations.
We know that sometimes children, who are deaf and hard of hearing have speech
that differs from how hearing children produce speech in some situations.
We want to be cautious that evaluators, who are not familiar with how the child's hearing impacts
their spoken language to make sure that they're not overly attributing those differences to symptoms
of autism rather than understanding, that as related to their hearing.
This one's particularly tricky in the ADOS-2 scoring algorithms. We look at whether a child is using
facial expressions that are varied, that matched with their affects, and that they direct
those facial expressions to others. A child with autism might smile about something, but direct that smile
inward, rather than orienting it to someone else, or they might show a limited range of facial expressions.
What gets tricky about this, is we have to distinguish that function, and how do we distinguish that
from those ASL grammatical features? In a study conducted by Dr. Shield and myself of his sample
of deaf children of deaf adults, who were previously given an autism diagnosis,
we found that there were significant differences in how they scored on the ADOS depending on whether
we strictly adhered to the standardized guidelines
or whether we modified the ADOS in a clinical manner,
in a manner consistent with an understanding
of sign language and applying the tool
to use with children, who are deaf and hard of hearing.
In that study on the ADOS-2, there were significant discrepancies between how the children scored
on an ADOS module that was clinically decided upon based on understanding deafness
or one per standardized instructions. Children, who had significant and obvious symptoms of autism,
were likely to score within the autism range regardless of what module was given to them.
However, there was much more variability for children who had more subtle symptoms of autism.
The fluent or native signers were most likely to be misdiagnosed, if the ADOS-2 was used according
to standardized procedures. This was because it generally resulted in administering a module 1,
which for hearing children is given to the minimally verbal children, and an ADOS would definitely
in many cases under identify autism among those children. Therefore, it's important to consider
that use of the ADOS-2 is very complex
and requires both clinical judgement and knowledge
of both autism and deafness, in order to
appropriately select the module appropriate
for that child's communication abilities, as well as,
knowing how to modify the administration
for children, who are deaf, as well as, how to interpret the results.
Generally speaking, use of the standardized algorithms
at this point is not appropriate. Having said this,
again, if you have understanding of both deafness and autism, the tool can be useful to you in gathering
information to assist with that differential diagnosis. We really need to better understand what modifications
to these tools are appropriate, and we have to recognize
that the materials may not be as deaf friendly.
For example, the materials include musical toys
or their scoring based on how the children play
with figurines, and that can look very differently
for children, who are deaf who are using sign language,
than hearing children.
Several items are also problematic because
of the nature of sign language.
There's a task that asks the child to show you
and tell you how to perform a task, but in giving those
directions you give away the task due to the iconic
nature of some of the signs involved. It's also important
to recognize that even if we modify the tool to make it accessible for children, who are deaf
and hard of hearing, we may not be capturing everything that we need to in assessing
for an autism spectrum disorder among children, who are deaf and hard of hearing.
We wouldn't be capturing, for example with this tool,
whether the child is appropriately using those ASL
grammatical facial features, that are really important,
and that we know might present differently among
children, who are deaf with autism than among typically developing deaf children.
We've talked about some of the tools that can be used as part of the assessment process
for diagnosing autism among children, who are deaf and hard of hearing.
I want to highlight that again the specific autism tools that we talked about in and of themselves
are not sufficient for diagnosing autism.
Assessment of autism must include a comprehensive
developmental history that specifically looks
at those early symptoms of autism, as well as,
possible comorbid conditions or other explanations
of the child's behavior. It should include a medical
history, because again in the big picture of things,
we know that some of those medical symptoms
can impact the child's functioning. It should include
a cognitive or developmental assessment
and a measure of adaptive functioning,
because it's important for us when we're considering
whether a child has autism, we're really looking at gaps
between their developmental abilities and their social
skills specifically. We have to know where
their developmental abilities lie.
In children with an autism spectrum disorder, we often also see gaps between their cognitive abilities
and their adaptive functioning. Again these are components that can impact the child's quality
of life, so they must be included in the assessment.
It should include observations of the child's behavior
across different settings, and a multidisciplinary
evaluation really is ideal to have multiple perspectives
about the components of development that
might be impacting the child's behavior.
Specifically in assessment of autism for children, who are deaf and hard of hearing,
in addition to those multiple sources of information, it's really best practice to refer to a clinician,
who's familiar with both deafness and autism.
Again if that's not available, consult with professionals,
who want to establish consultation amongst
professionals, who understand deafness
and those who understand autism.
I've included some resources that might be helpful in understanding how autism can present among children,
who are deaf and hard of hearing, as well as, a reference list for additional information.
Today we reviewed considerations in assessment of an autism spectrum disorder
among children who are deaf and hard of hearing.
Look for future broadcasts regarding interventions
for autism with children, who are deaf and
hard of hearing, as well as, educational considerations.
For questions, I refer you to the resources that are provided
and my contact information that is on the screen.