CHARGE syndrome: sensory integration problems

This page is about the sensory integration problems commonly experienced by children with CHARGE syndrome.

It explains what is meant by sensory integration and details the problems children with CHARGE experience as a result of all seven of their senses being affected.

It also covers ways of managing sensory integration problems, including occupational therapy assessment and techniques in the classroom.

On this page:

The senses

We use the following seven senses to function:

  1. Sight – the visual system.
  2. Hearing – the auditory system.
  3. Balance – the vestibular system.
  4. Taste – the gustatory system.
  5. Smell – the olfactory system.
  6. Touch – the tactile system.
  7. Pressure – the proprioceptive system.

Through each of our senses, we receive information, such as light, sound or smells. 

This information travels through the nervous system to the brain. 

The brain processes the information and sends instructions back through the nervous system to the body’s muscles so that we can perform certain tasks.

What is sensory integration?

Sensory integration means all of the above senses working together so that we can function properly in everyday life.

When any one of our senses doesn’t work properly, it may affect how one or more of the other senses receives and sends information to the brain for processing.

This is referred to as “sensory integration problems” or “sensory integration dysfunction” – a sort of traffic jam in the brain.

Children with CHARGE syndrome and sensory integration 

Children with CHARGE syndrome can have difficulty using all their senses together in a coordinated way.

This is because some senses overreact, while others underreact.

Senses overreacting is also known as “sensory defensiveness”. 

Sensory integration problems in children with CHARGE syndrome

Sight

In CHARGE syndrome, sight can be affected by different parts of the eye not forming fully. This is known as coloboma.

Read more about coloboma treatments and management.

Children with CHARGE may:

  • Cover their eyes due to photophobia – extreme sensitivity to light.
  • Hang upside down to see things. 
  • Struggle outside where there are fewer vertical markers.
  • Seek out of strong sensory input, e.g. spinning their body, sitting and spinning a toy, spinning twirling objects near their eyes, rocking, bouncing and hanging upside down over the edge of a sofa or bed.

Hearing

In CHARGE syndrome, hearing is affected by:

  • The cochlea (hollow, spiral-shaped bone in the inner ear) being different.
  • The cochlear nerve (the auditory/hearing nerve) being thin or missing entirely.

Read more here about CHARGE syndrome and hearing problems.

Children with CHARGE may:

Balance

In CHARGE syndrome, the balance (vestibular) system – three tiny, fluid-filled semicircular canals in the inner ear, activated by head movement – is affected because the semicircular canals may be missing.

Our balance system tells us whether we are upright or lying down, moving or standing still, and how fast or slow we are going. 

Children with CHARGE may:

  • Have low muscle tone. 
  • Struggle to stay upright.
  • Prefer to lie on their back or their side.
  • Need to prop up their chin. 
  • Experience delayed physical development, including being able to balance while sitting and when moving. 
  • Seek out strong sensory input, such as spinning their body, sitting and spinning a toy, spinning twirling objects near their eyes, rocking, bouncing and hanging upside down over the edge of a sofa or bed. 
  • Feel tired and have difficulty controlling their posture. 
  • Have a poor walking pattern, with feet turned out.
  • Have poor head posture. 
  • Experience difficulty organising all sensory information. 

Smell

In CHARGE syndrome, the sense of smell is affected by cranial nerve abnormalities and blocked nasal passages (choanal atresia/atresia choanae).

Our sense of smell plays an important role in the way memory affects preferences, choices and behaviour.

Children with CHARGE may experience difficulties in a number of ways due to lack of smell:

  • They can’t, for example, learn to relate a particular perfume to a specific person, like their mother or a carer. 
  • They can’t associate smells with other people, which can delay the development of social skills. 
  • They can’t associate smells with happy memories or experiences, such as bath-time. 
  • Food preferences depend greatly on our sense of smell, so they may choose to eat a limited diet of certain food colours, textures and flavours.
  • They don’t develop their sense of smell as a natural alarm system – bad smells mean there is something to avoid.

Children with CHARGE syndrome may also smear and smell their own faeces.

Taste

In CHARGE syndrome, the sense of taste is affected by cranial nerve abnormalities.

Our sense of taste – which is also closely linked to our sense of smell – helps us to survive and provides us with essential information about bitter, salty, sweet and sour flavours. 

Taste is important for selecting foods and associating certain tastes with things that might be harmful or make us ill. 

In children with CHARGE syndrome, not having a sense of taste, along with no sense of smell, can lead to them choosing a limited diet of certain food colours, textures and flavours. 

Read more about eating and drinking difficulties commonly experienced by children with CHARGE syndrome.

Touch

In CHARGE syndrome, when both sight and hearing are affected, the sense of touch may become the main source of sensory information. 

Our sense of touch plays a major part in shaping our physical, mental and emotional behaviour, as well as body awareness and movement. 

Because children with CHARGE syndrome rely so much on touch, this can lead to overreaction in various ways:

  • Self-injury. 
  • Strong emotional reactions to being touched.
  • Strong behavioural reactions to being touched, including aggression.
  • High levels of stress and anxiety. 
  • Strong dislike of specific fabrics, clothing textures or tags on clothes
  • Dislike of daily grooming activities such as hair brushing, combing and washing, nail clipping, teeth cleaning and washing their face.
  • General dislike of getting dirty, especially their hands, feet and face.

Pressure

Our sense of pressure refers to the sensory information that we receive from our joints and muscles. 

This information tells us about the position, movement, force and direction needed for all kinds of activities, e.g. buttoning clothes, writing, screwing a lid on a jar or playing with a toy without breaking it.

Children with CHARGE syndrome may: 

  • Use excessive force. 
  • Appear fearful of or avoid movement.
  • Have an abnormally high pain threshold. 
  • Seek out strong sensory input through repetitive behaviours:
    • flapping arms, feet, hands, legs and fingers.
    • rapidly bending and straightening joints.
    • bending fingers into unusual positions.
    • flicking fingers.
    • moving fingers near the eyes.
    • putting hands, fingers or objects in their mouth.
    • prop their chin in their hand, with their elbow on the table.
    • shoulder shrugging.
    • craving deep pressure.
    • walking with feet turned out.
    • extra slaps or taps with their feet.
    • walking on tip-toes.
    • using their fists, wrists or knuckles to hit themselves on the face, head, chest or stomach.
    • lying on their back on the floor, on a bed, in a beanbag or on a sofa.

Managing sensory integration problems in children with CHARGE syndrome

To manage your child’s sensory integration problems, they should first be assessed by a suitably qualified occupational therapist. 

After assessment, the occupational therapist can devise a range of activities for your child’s particular sensory integration problems (sometimes referred to as a “sensory diet”).

These activities can help your child to feel calm, alert and organised more of the time and should include: 

  • Specific time-oriented activity routines. 
  • Adaptations and changes in routines and interactions with people. 
  • Changes to their environment or routines. 

In the classroom

In the classroom, this may include providing:

  • Ball chairs to work on.
  • A safe space for your child to hide when feeling overloaded.
  • Facilities for heavy work, such as swinging and jumping. 

This should help your child to focus more easily on learning without being so affected by the distractions surrounding them. 

If your child gets very tired because of their condition, they may:

  • Want to rest their head on the desk.
  • Have difficulty concentrating or focusing.
  • Behave in challenging ways.

If so, they should be provided with a quiet area away from the other children, where they can be quiet.

They may also benefit from some deep pressure or lying under a weighted blanket. 

Other useful approaches include:

  • Using a total communication approach.
  • Lessons that use the different senses. 
  • Alternating between active and passive activities through the day, which can help your child to better organise how they use their senses. 
  • Sensory breaks are essential to give your child time to refocus and organise all the sensory information they are constantly receiving. 
  • Specific techniques such as a Wilbarger protocol (brushing technique) for sensory defensiveness, the use of weighted items, vibration work and suspended equipment. 
  • Having consistent and predictable environmental supports. 
  • Working collaboratively with a qualified practitioner experienced in working with children with multisensory impairment.

Get support from Sense

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This content was last reviewed in April 2022. We’ll review it again next year.