Scottish Sensory Centre

Working with children with Cytomegalovirus, Auditory Neuropath Spectrum Disorders or Autistic Spectrum disorders

Presented on Monday 2 November 2009

Autism and Communication

With thanks: Sandra Driver, Coordinator /Principal Speech and Language Therapist Guys and St Thomas NHS Foundation Trust Cochlear Implant Team; Geoff Evans Options Group

Overview

• Autistic spectrum disorder
  How is it diagnosed?
  What impact does it have Deafness and ASD Differences and commonalities
• What about CI and ASD?

The four most prevalent conditions to consider in the differential diagnosis of children with inadequate language development are hearing loss, learning difficulties, language disorder and autism. These diagnoses are not mutually exclusive ... Diagnosis is particularly difficult in children in whom two of more of these conditions co-exist. (Jure, 1991)

Notes: I just wanted to highlight the complexity of a dual diagnosis

Since each of these disorders (Deafness and autism) has a profound effect on the development of language and communication, their joint effects are likely to be cumulative and devastating. Early identification of both disorders is essential in order to provide appropriate habilitation and language learning when intervention is known to be most effective. (Ruben and Rapin 1998)

Notes: The effect is also significant on the family and their expectations of a CI. Most families go ahead with a CI hoping for spoken language and speech - which is a very achievable outcome for the majority of early implanted deaf children however this is not realistic for the majority of deaf /autistic children. So goals will be different. (will go into outcomes in more detail later)

Prevalence deafness & ASD

• 0.1% congenital permanent deafness
• 0.4-1.6% ASD
• 61/1150 in Jure study = 5.3% dual diagnosis

What we Think we Know About Autism Spectrum Disorders

Causes of Autism Spectrum Disorders

• Strong evidence of biological basis
• Evidence of a genetic link, possibly a number of genes interacting together.
• General Pre-disposition
• Possibly a number of causes all effecting the same part of the brain
• Links with other disorders

Notes:

Stress cause still not known but strong indicators for genetic predisposition.
2001 Human Genome Project found indicators that certain genes may increase susceptibility to autism.
Origins of autism are believed to be organic rather than psychological.
Much of the current research concentrates on the physical dysfunction of the brain, connected to its development at the time of birth or in early infancy.
You may wish to discuss the social model of disability, that is, people being disabled by their environment rather than their disability.
Possible triggers include; (Gillberg 1990,Gillburg and Coleman 1992 ) Viral infections such as rubella, encephalitis; Exposure to environmental chemicals such as lead. Genetic factors such as Fragile X Syndrome.

The Behavioural Characteristics of Children with Autism

There are no medical tests for diagnosing Autism Spectrum Disorders.Autism is diagnosed through the existence of a number of common behaviours which include the following:

• Displays indifference
• Joins in only if adult insists and assists
• One-sided interaction
• Indictes needs by using an adult's hand
• Lack of creative pretend play
• Handles or spins objects
• Echolalic speech
• Does not play with other children
• Talks incessantly about one subject
• Variety is not the spice of life
• Eye contact lacking or inappropriate
• Idiosyncratic mannerisms
• Inappropriate laughing or giggling
• But some can do some things very well.

Notes: Some of the ways in which autism shows itself in the individuals we support Discuss the possible function of each behaviour and illustrate from your experience (Trainer to emphasise again that this is purely illustrative - not all individuals will have these characteristics; and that behavioural characteristics may also change over time with experience, intervention and maturity). Echolalia Instant and Delayed - Echo phrases used to communicate feelings and emotional states,Talk about echo behaviour Get participants to identify when they may behave in similar ways discuss function of behaviour for them apply to those with ASD.

Triad of Impairments

Notes: Triad of impairments Wing and Gould Camberwell study 1979, Fundamentally unchanged. Some development in our understanding of imagination. Not so much a lack of imagination more difficulties with its appropriate use. Sensory processing difficulties seen as part of core difficulties of autism.

ASD: A developmental spectrum disorder - Triad

Deafness Impairment of hearing

Consequences:

• Depending on age diagnosed
• Hearing aid/CI management
• Good language input

Impairment of Communication with ASD

Areas of impairments

• Apparent lack of desire to communicate
• No meaningful verbal language
• Echolalic and repetitive communication
• Communication confined to expression of needs
• Literal use and understanding of Language

Notes: Stress the idea of a non-fixed range
50 to 60% no meaningful language
2 types of echolalia instant and delayed eg, good morning John-good morning john
Al types of communication eg, sign

Areas of Impairment of Communication cont.

• Makes factual comments often irrelevant to the social situation
• Requires extra time for processing information
• Talks incessantly regardless of response from others
• Hasgood vocabulary but is unable to use it for communication
• Displays distortion of language rules

Notes: eg; group talking about football, indiviudal with autism suddenly says :do you know Mount Everest is the highest mountain in the world?"
eg; fist computer game compared to modern one, old one needed to make far more connections there for took far longer. Displays distortion of language rule, you instead of I

What People with Autism Say

I had had trouble differentiating between literal and implied meanings. My parents had asked me, do you want a cookie, enough times that I must have concluded that my new name was 'you'. So the next time the hunger pangs hit, I declared "you want a cookie"
Vern Nicholson

What Individuals With Asperger's Syndrome Say;

My mind is completely visual and spatial work such as drawing is easy. I taught myself drafting in six months. I have designed big steel and concrete cattle facilities, but remembering a phone number or adding up numbers in my head is still difficult. I have to write them down. Every piece of information I have memorised is visual. If I had to remember, an abstract conceptI see the page of the book or the notes in my mind and I read information from it.
Grandin (1984)

1. Typical Social Development/Interaction

2. Typical Communication Development

3. Typical development of flexible/abstract thought and TOM

Differential Diagnosis deaf and ASD similarities summary

• Limited types of communicative intent in the early years (deaf children of hearing parents more likely to direct or request than to comment or ask questions) similar to ASD and unlike normally developing children who develop a wider range or intent.
• Delayed Theory on Mind and abstract concepts/idioms etc.
• Child may be controlling of environment, like routine, be independent/ resourceful in getting things for themselves.

Differential Diagnosis deafness or deafness and ASD differences

More evident in the area of the triad - Social Communication and repetitive/ritualistic behaviour

• ASD May not smile at you when you smile at them (normal 4-6 months) and even in 2 month olds (primary intersubjectivity can be lacking 'sharing a moment').
• ASD may be quiet and easy babies - do not seek attention (0-6mnths). Deaf babies are vocal and seek attention with whole body movements and reassured by the presence of a person.
• Although deaf children do not show entirely normal range of skills in communicative intent with a linguistic focus due to possible language delay (so minimal labelling, pointing out of interest but are needs led) they do develop joint attention which ASD may not.
• ASD may be more interested in moving objects not faces. (Deaf children very visually aware and do have interest in people (normally well established by 12 months).
• ASD possibly poor joint attention - Ability to be tuned in to follow the eye gaze, point of others or point with index finger themselves to show something of interest and engage back with the communicative partner (Rollins et al 1998) (normally established by 18 months to two years.
• ASD possible overreaction to certain environments, textures, sensory sensitivities (HAids?). Not obvious to adult what the trigger was.
• May sign of use speech but not always directed at a person (just labelling, echolalia, rehearsal).
• Show ritualistic/repetitive behaviours and lack of imaginative play.

Social Interaction: Areas of Difficulty

• Socially isolated but not necessarily aware of it
• Lack of awareness of other people
• Lack of interest in other people
• Unaware of social requirements in terms of Location (private public) Who they are talking to Timing (when people are busy)
• Making friends
• Wanting interaction but not knowing how to initiate it, maintain it , and bring it to an end.
• Interacts but not always appropriately eg; too close, or bursts into giggles.
• May find the pressures of social interaction distressing knowing what interests others.

I don't feel any need for social interaction, left to my own devices, I am never bored or lonely. Paula Johnston

Flexibility of Thought and Behaviour (Imagination)

• Follows rigid routines without variation
• Finding change difficult
• Special interests that can become all - absorbing
• Difficulty working out what comes next
• Difficulties transferring skills from one setting to another
• Difficulties adjusting behaviour according to the situation
• May have difficulties with the creativity of peer play

The use of visual cues may help children with Asperger's Syndrome

• Learn more quickly
• Reduce aggression or self injury
• Decrease anxiety
• Understand their world better
• Gain greater levels of independence
• Follow instructions
• Complete complex tasks

Part day symbolic schedule

Makaton Symbols (register, work, shops)

Working top to bottom. Puts card in pocket / turns over card

Student matches cards on table to work baskets.  'Left to right' system  showing  finished box.

Full day written schedule

Sensory Processing Difficulties:Sounds

• I was frightened of the vacuum cleaner, the food mixer and the liquidiser because they sound about five times as loud as they actually were.
• The bus started with a clap of thunder, the engine sounded four times as loud as normal and I had my hand in my ears for most of the journey.
• While I was typing the capital I in the last sentence the dog scratched and her collar jangled so I heard a crashing painful noise.
• Another trick my ears played was to change the volume of sounds around me. Sometimes when other kids speak to me I could scarcely hear them and sometimes they sounded like bullets.
  (White and White)

Visual perception

• I used to hate small shops because my eyesight used to make them look as if they were ever smaller than they actually were.
• I also remember one Christmas when I got a new bike for a present.It was yellow, I would not look at it extra red was added to the colour making it like orange, and it blurred upward making it look like it was on fire.

What people with Autism say

When I look someone straight in the eyes, particularly someone I am not familiar with, the feeling is so uncomfortable that I can't really describe it. First of all, I feel that their eyes are burning me, and I feel as if I am looking into the face of an alien.

Sometimes it is hard to concentrate on looking and listening at the same time. People are hard enough to understand as their words are often so very cryptic, but when their faces are moving around, and their eyebrows keep raising and falling, and their eyes getting wider and squinting, I can not fathom all that in one go, so to be honest I don't even try.
Luke Jackson (2002)

Tactile Problems

My sense of touch can be overly sensitive, sharp as a pin and to be touched can be 'shocking' as though being jolted. (Donna Williams)

Most people adapt to the feeling of different types of clothing in a few minutes, it takes me three or four days to fully adapt to new ones.

The nerve endings on my skin were super-sensitive.

Stimuli that were insignificant to most people, were like Chinese water torture.

When people hugged me I stiffened and pulled away to avoid the engulfing tidal wave of stimulation. (Temple Grandin)

Deafness and ASD - Contraindications to CI

• Severe aversion to H Aids (need to establish H Aid use first if going to consider CI)
• The older the child and more entrenched in their stereotypical behaviours
• Expectations and understanding of family (CI must not be a barrier to the child getting appropriate intervention and choice of communication methodology support)

Parents in both deafness and autism quickly find themselves in mired in bitter disagreements, whether between sign and speech advocates, or between believers in developmental versus behavioural approaches to autism. (Beals, 2004)

Must be a recognition that ASD and its impact on joint attention underpins all social, communication and language development and thus is potentially the primary disability.

Research and Outcomes of children with deafness and ASD

Limited:

Two published research papers

1. Hearing Loss and Autism

2. CI and Autism

St Thomas outcomes

Jure et Al (1991) - General Outcomes

A clear relationship between the severity of ASD and severity of cognitive deficit No clear relationship between severity of HL and sociability.

Jure - Language outcomes

Spoken Language

• 2 with mild to moderate HL used speech
• 3 others (not profound) used single spoken words/simple phrases
• 41 (89%) no speech

Sign Language

• 27 had some sign language tuition
• 7 marginally adequate
• 5 echolalia
• 6 did not sign (2 severe ASD/ 4 moderate ASD)
• No child was a fluent signer

Evident that it is the degree of ASD not HL that effected ability to sign not HL

Interestingly those deaf ASD children with echolalia is sign mirror hearing

ASD children who are reported to be echolalia in speech. (suggests a degree of attention and memory but poor comprehension, but is a starting point to develop linguistic skills)

Research - Outcomes ASD and CI

Research - Donaldson et al (2004) Children included CI/ASD (age 3-16yr)

Mais (5 children) Average 72%

• 6/7 gestures/sign
• 1/7 word approximations

St Thomas: Children included (2.5-14 yrs)

MAIS (5 children) Average 71% (45-100%) with deterioration seen

• 5/9 use sign
• 2/9 use SSE
• 2/9 use PECs
• CAP Maximum 5/6
• SIR All between 1-3 ratings (severity of ASD appears to correlate)

Educational Placement: 2-Special Sch/3 Sch for deaf/4 HIU

Device use varied (not related to degree of ASD)

Donaldson et al

• Parental Survey - Suggested changes in responsiveness to sound, interest in music, vocalisations and eye contact, responsiveness to requests (not all of these are definitely as a result of CI, could be developmental or result of therapy)
• 5/6 parents would recommend a CI to another family
• Children diagnosed with ASD post CI-Parents reported their child to have done worse than those diagnosed pre-CI. (Importance of pre CI diagnosis for expectations and appropriate intervention)

Notes: So how does our centre compare with others? Michigan University CI centre Mais scores-same average as are communication modes and speech outcomes.

National Autistic Society

• www.nas.org.uk

Where next

• Working with children with Cytomegalovirus