University of Edinburgh

'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.


  • 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!


  • 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.


  • 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


  • 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


  • 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


  • 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]


  • 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!