'Deaf Plus': a look at the learning implications of additional difficulties
Friday 24 February 2006
Jackie Grigor
Our Aims
- To identify conditions which co-exist with a child’s hearing loss;
- To describe and quantify their impact on the child’s ability to learn and live a fulfilled life.
Background
- Paediatrician – child development; advice to education re placement
- Paediatric role in audiology department – looking at "whole child" [development] – included looking for cause of deafness
- Discussions with Marian and Mary Brennan on impact of "additional difficulties" on learning
- Medical model – or not!
Descriptions
- Co-incidental – eg; asthma, eczema, diabetes, epilepsy; also autism, speech and language disorders, learning difficulties
- Common underlying cause –extreme prematurity or congenital rubella
- Genetic syndrome – recognised pattern of conditions of which deafness is just one manifestation.
Causes of hearing impairment
1. Genetic – in genes or chromosomes
2. Non-genetic – due to external or environmental influences
Non-genetic causes of deafness
- Infection - rubella, cytomegalovirus, meningitis
- Lack of oxygen [associated with time on ventilator]
- High levels of jaundice
- Drugs [ototoxic] – aminoglycosides
- Trauma – head injury
- Tumours – acoustic neuroma
- Noise
Genetic causes
- Non-syndromal
- autosomal recessive
- autosomal dominant
- x-linked
- Syndromal – affecting eyes, face, skull, external ears, skin, hair, neck, heart, kidneys, skeleton and fingernails
Some syndromes
- Treacher Collins syndrome
- Alports syndrome
- Branchio-oto-renal syndrome
- JLN syndrome [Jervell-Lange-Neilson]
- Waardenburg syndrome
- Downs syndrome
- Ushers syndrome
Other thoughts
- Some conditions are not present at birth but develop later.
- Even if condition present at birth there may be no obvious signs to identify the condition.
- Babies develop – and conditions evolve over time.
Steps in identifying co-existing conditions 1.
- at time of diagnosis – paediatrician to take clinical history, family history, carry out full physical and developmental examination;
- gather together all information on child;
- arrange appropriate tests to be carried out; referral to radiologist, ophthalmologist, geneticist.
Next Steps 2.
- Identify any cause/underlying condition(s);
- share information with parents;
- and with audiologists, teachers and speech
and language therapists
identify likely prognosis – in broad terms ; - monitor child’s health and development over time – and note progress, or lack of.
Next steps 3.
- if co-existing condition identified and thought to have impact on child's learning – or;
- if later unexplained slow progress or developmental delay.
- Contact educational psychologist – for help in defining child's difficulties.
Difficulties in identification
- Lack of awareness/knowledge of conditions associated with deafness among doctors.
- Lack of knowledge regarding hearing loss and its effect on speech and language acquisition – among all professionals.
- Difficulty in accurate assessment of cognitive [and other] functions in deaf and HI children [in speech or sign].
Other difficulties!
- Majority of areas do not have a named paediatrician with responsibility for HI children.
- Most areas do not look for cause of hearing loss – or other conditions.
- Little monitoring of HI children's progress.
- No routine link-up with geneticist, etc.
- Very little sharing of information.
But also
- Lack of shared clear definitions – among all professionals – all using words in differing ways.
- Very poor communication among professionals; either absence or in unintelligible terminology.
- Often unclear information given to parents.
John
- Medical condition identified at 16 months
- Hearing tested [known to be associated]
- Moderate loss – adequately aided
- Examined by paediatrician – noted to be feeling his way round – referred ophth.
- Severe visual impairment – optic atrophy
- Difficulty in accessing curriculum
Catherine
- Moderate loss discovered 9 months
- Aided well – seemed to make progress with speech and language
- Age 2 years progress poor – speech deteriorating ?progressive loss
- Hearing test - profound loss but difficult to test…….
- Diagnosis of autism - learning and communication difficulties
Jonathon
- Alports syndrome
- Mild hearing loss – first noted at primary school- slowly progressed; no need for aids
- Progressive kidney failure, beginning in late teens – inevitable progress to dialysis and transplant
- Very little loss of schooling
David
- Early diagnosis profound hearing loss
- First language Sign - whole (hearing) family signed with him from early age
- Special school – severe behaviour problems reported age 9 years
- Bright boy, in class of 5, others all with learning difficulties; 4 teachers in 1 year [none as good signers]
Amy
- Profound hearing loss at 8 months
- Large, chaotic family
- Hearing aids very inconsistently worn
- No apparent learning difficulties
- School for the Deaf from age 3 years
- Family failed to learn Sign language
- Age 15, poor educational attainments, isolated with severe behaviour and emotional problems
Impact on Learning
- Hard to describe– and to quantify
- Condition may have a severe impact on health but little impact on learning
- Condition may only become severe in teens or even later [that is, after leaving school]
- Cumulative effect of 2 or more conditions
- Can be modified by family, education and social environment.
In Conclusion
- Great difficulty in identifying additional or associated conditions.
- No common terminology, shared between health and education – and parents.
- Great difficulty in defining or quantifying the impact of any additional condition on a child’s learning.
- So – look critically at all figures!