University of Edinburgh

Supporting Deaf Pupils with Additional Learning Needs

Presented on Tuesday, 18 January 2011

CHARGE syndrome

Wendy McCracken


Multiple physical, sensory and behavioural anomalies

Every part of the auditory system may be affected

  • External ear anomalies
  • Middle ear anomalies, ossicular, chronic otitis media and chronic Eustachian tube dysfunction
  • Under-development of the cochlea, and vestibular structures (Mondini's dysplasia)
  • Reduced auditory nerve diameter
  • Prolonged latencies
  • Losses typically mixed with a very considerable conductive element
  • Prevalence of severe-profound is approximately 50%

CHARGE syndrome

60% of children will acquire symbolic language

Mechanics of speech may be affected by cranio-facial abnormalities, breathing problems, and clefts

Success in acquiring symbolic communication has been found to be relate to the ability to walk independently by the age of 3 years

Behavioural issues are common and may be linked to:

  • Multi-sensory impairments
  • Communication frustrations
  • Physical pain or discomfort

May be linked to cognitive impairment

Regulatory disorder, leading to problems with:

  • Sleep-wake cycle [respite care]
  • Hunger-satiety cycle
  • Ability to console themselves
  • Ability to plan their motor activity

Recent research suggests the presence of executive dysfunction, problems with:

  • Shifting attention
  • Initiating activity
  • Inhibiting activity
  • Sustaining activity

Functional communication is of central importance. Whatever system is put in place must be used at home as well as school.

Choosing a communication system

  • Must include family
  • Look at motor control-oro-motor and extremity
  • User preferences
  • Previous exposure
  • CONSIDER a communication passport
  • Practically usable
  • Ergonomically acceptable
  • Transportable
  • Flexible
  • Rate of use
  • Possibility of extending
  • User community
  • Socially acceptable