Causes of deafblindness

Listen to this article

Some of the most common causes of deafblindness are Rubella, CHARGE syndrome, Usher syndrome and ageing.

Learn more about Rubella and deafblindness

Learn more about CHARGE syndrome and deafblindness

Learn more about Usher syndrome and deafblindness

Learn more about Ageing and deafblindness

Rubella

Rubella is a mild but infectious viral disease characterised by a rash which starts on the face and then spreads along the rest of the body. Also called German measles, in most cases rubella is relatively harmless with all symptoms disappearing after a week or so and leaving the patient with life-long immunity.

However in pregnant women, rubella can cause severe birth defects or miscarriage if contracted in the first trimester. If a pregnant woman contracts rubella, the virus can infect her foetus. Such an infection is especially dangerous in the first four months of pregnancy, as it can cause damage to the foetus’ developing organs. Although any part of the body can be affected, the eyes and ears seem to be especially susceptible to damage from a rubella infection.

Babies born with Congenital Rubella Syndrome (CRS) can be affected differently. Some are only mildly affected while others have significant disability.

Some of the problems associated with CRS include sensorineural hearing loss, visual problems such as cataract, inflammation of the retina (retinopathy), nystagmus, small eyes (microphthalmia), and occasionally optic atrophy, corneal haze, and glaucoma. Babies born with CRS may also experience hearing, neurological, or growth problems, and other disabling conditions. In later life some people may also experience glaucoma and detached retina.

CHARGE syndrome

CHARGE syndrome is a recognisable genetic pattern of birth defects which occurs in an estimated one in every nine to 10,000 births worldwide. It’s an extremely complex syndrome, involving extensive medical and physical difficulties that are different from child to child. In most cases, there is no history of CHARGE syndrome or any other similar conditions in a family.

Babies with CHARGE syndrome are often born with life-threatening birth defects, including complex heart defects and breathing problems. They spend months in the hospital and undergo many surgeries and other treatments. Swallowing and breathing problems make life difficult even when they come home.

Most have hearing loss, vision loss, and balance problems which delay their development and communication. All are likely to need medical and educational intervention for many years.

Despite these seemingly insurmountable obstacles, children with CHARGE syndrome often far surpass medical, physical, educational, and social expectations.

Continued research is needed to help us understand the medical and developmental challenges faced by people with CHARGE. Better understanding will lead the way to interventions, therapies and education strategies which can help people with CHARGE syndrome thrive.

Usher syndrome

Usher syndrome is the most common genetic cause of combined vision and hearing impairment and deafblindness. People with Usher syndrome often have partial or total hearing loss and a gradual vision loss caused by retinitis pigmentosa. Some people with Usher syndrome also experience problems with their balance.

The syndrome affects people differently. Some people’s vision starts to change when they are children, other people’s vision doesn’t change until much later.

A person with Usher syndrome often knows they are deaf or hard of hearing from early life. Later in life, they find out that they have retinitis pigmentosa when their eyesight starts to change, and they have trouble with night blindness and ‘tunnel vision’.

Usher syndrome is categorised into three broad groups according to the type and severity of symptoms – Types 1, 2 and 3. Type 1 and Type 2 account for approximately five per cent of all children born with a hearing impairment. There is currently no cure for Usher syndrome.

·      Usher syndrome Type 1

This is the most common form of Usher syndrome. People with Type 1 experience profound congenital deafness. Retinitis pigmentosa usually becomes apparent within the first 10 years of life, but their level of vision loss varies over time.

There are balance problems. These don’t get worse with age but may seem worse as the person’s field of vision reduces. People with Type 1 tend to prefer signing as their mode of communication.

·      Usher syndrome Type 2

This is the second most common type of Usher syndrome. For people with Type 2, hearing loss ranges from mild to severe and is not usually progressive but is affected by the ageing process. Retinitis pigmentosa is apparent from late adolescence to late twenties. Type 2 people do not experience balance problems.

·      Usher syndrome Type 3

Type 3 is extremely rare and not much information is available. We know that people with Type 3 are born hearing and sighted. They usually begin to lose their hearing and vision in their thirties, and the loss is often rapid.

·      Retinitis pigmentosa

Retinitis pigmentosa is a genetic eye condition that causes degeneration of the retina – the light-sensitive tissue inside the eye where the first stages of ‘seeing’ take place. In cases of retinitis pigmentosa, the retina slowly degenerates and loses its ability to transmit pictures to the brain. In advanced stages, clumps of pigment appear in the retina. The first symptom of retinitis pigmentosa is often night blindness and an inability to see in poor lighting conditions. A narrowing of side vision, which is called tunnel vision, also occurs. In some cases retinitis pigmentosa leads to serious vision impairment, but most people retain some useful vision for many years.

Ageing

Older people are increasingly developing hearing and vision problems. Often the loss comes on very gradually – so gradually that older people themselves and those around them may not recognise or understand what is happening.

Combined hearing and sight loss can cause challenges with everyday activities like communication, getting around, and accessing information. It can make people feel isolated and excluded.

One of the main challenges for older people with deafblindness is being able to move around safely. Making simple changes to their environment, mobility training and guiding can all make a big difference.

Many older people who live in care homes or receive care in their own homes have hearing and vision loss. Providing good quality care means recognising their needs.

Signs of hearing loss in an elderly person

  • Complaining that other people mumble or speak too quickly
  • Asking people to repeat what they have said
  • Asking people to speak louder
  • Repeating words to verify what’s been said
  • Finding it difficult to keep up conversations in a noisy environment
  • Having difficulty understanding unfamiliar people or accents
  • Appearing confused
  • Getting tired in conversations because of the need to concentrate
  • Withdrawing from situations where conversation is expected
  • Needing the TV or radio volume louder than is comfortable for other people
  • Finding it hard to hear the telephone or doorbell
  • Using a hearing aid or loop system.

Signs of vision loss in an elderly person

  • Finding it hard to identify objects or familiar faces
  • Needing more light for reading and other activities
  • Finding it hard to cope with glare, such as bright sunlight
  • Sitting close to the TV
  • Holding a book close to their face
  • Giving up reading, watching television and other activities
  • Struggling to find small objects
  • Spilling food or knocking over cups
  • Stopping eating because they can’t see their food
  • Wearing mismatched colours or having stained clothing
  • Having difficulty moving around
  • Walking slowly or with less confidence
  • Bumping into things
  • Having difficulties when light levels change
  • Having difficulties with unfamiliar routes or places.
Skip to content

Sign up for our e-news

Contrast
Font
Increase
Decrease
Reset
Close