Causes

There are many potential causes of deafblindness. Individuals can be born deafblind, others can be related to health conditions and in many cases the hearing and/or vision loss occurs later in life.

Deafblindness later in life

A person with acquired deafblindness may be born without a hearing or sight problem and then later can lose part or all of both senses.

Alternatively, someone may be born with either a hearing or vision problem, and then later loses part or all of the other sense.

Thigs that contribute to acquired deafblindness include:

Age related sensory loss

As we get older, this can develop gradually, the person themselves may not realise their vision and/or hearing is getting worse.

Signs that you may notice:

  • needing to turn up the volume on the television or radio
  • difficulty following a conversation
  • not hearing noises such as a knock at the door
  • asking others to speak loudly, slowly and more clearly
  • needing to hold books or newspapers very close, or sitting close to the television
  • difficulty moving around unfamiliar places

If someone already has either a hearing or vision problem, it’s important to look out for signs that suggest the other sense may be getting worse too.

As with all sight loss conditions, low vision aids such as magnifying lenses and task lighting can help to make the most of the remaining vision.

Macular degeneration

Age related macular degeneration, also known as AMD is an eye condition that causes loss to the central vision, usually of both eyes.

There are two types

  • Dry AMD is the most common this causing gradual sight loss over many years. There is no cure for dry AMD.
  • Wet AMD is more serious and without treatment, vision can deteriorate within days. Wet AMD can be treated to try and prevent further sight loss, the injection treatments often can lead to an improvement in the vision.

Cataracts

Cataracts cause vision to become cloudy or misty when changes to the lens in the eye cause it to become less transparent. Cataracts usually develop over many years and so are more common in older people.

They can occur in one or both eyes, and often can be treated with a simple surgery.

Cataracts alone do not cause deafblindness but as they are most common in older people, it is likely that someone with cataracts may have some age related hearing loss as well.

Glaucoma

Glaucoma can occur for a number of reasons. In most it is caused by a build-up of pressure in the eye when fluid is unable to drain properly. This increase in pressure then damages the nerve that connects the eye to the brain (optic nerve).

Glaucoma doesn’t usually cause any symptoms to begin with. It tends to develop slowly over many years and affects the edges of your vision (peripheral vision) first. For this reason, many people don’t realise they have glaucoma, and it’s often only picked up during a routine eye test.

Symptoms can include blurred vision, or seeing rainbow-coloured circles around bright lights. Both eyes are usually affected, although it may be worse in one eye.

Retinitis Pigmentosa (RP)

Retinitis Pigmentosa, known as RP is a cause of sight loss in people with Usher syndrome. It is a condition which causes progressive and gradual loss of vision.

RP causes a build-up of pigment on the retina at the back of the eye which has varying effects on a person’s vision, such as:

Loss of peripheral vision (or tunnel vision) where all outer vision is lost (eventually this occurs in all instances of RP)

Loss of up/downwards vision, but still able to see outwards.

Loss of outer vision but able to see up/down.

Patchy vision all over.

Ushers Syndrome

Everybody with Usher syndrome experiences the condition in a different way. The time of onset, as well as how your sight, hearing and balance are affected, varies from person to person. It also changes over time.

The main symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa (RP).

RP causes nightblindness as RP progresses the field of vision narrows – a condition sometimes known as ‘tunnel vision’ – until only the ability to see straight ahead remains.

If you have Usher syndrome, you may also have balance problems.

There are three types of Usher syndrome.

Usher type 1

People with Usher type 1 are usually born with profound hearing loss in both ears. Balance problems are also common. Babies may show delays in sitting and walking, and experience disorientation throughout their life, particularly as their sight changes.

Usher type 2

People who have Usher type 2 are usually born with a mild to severe hearing loss in both ears. This is typically within the higher frequency ranges. Type 2 is not associated with balance problems, which is a characteristic that distinguishes it from type 1.

Usher type 3

Usher type 3 is characterised by gradual sight and hearing loss, which occurs later in life after a person has learnt to speak. Some people may develop severe hearing loss while others may not. People with Usher type 3 typically have problems with their balance. It’s common for people to be misdiagnosed as having type 1 if they demonstrate balance problems and severe hearing loss.

Deafblindness from birth

Deafblindness from birth is known as congenital deafblindness.

It can be caused by:

Rubella syndrome

Rubella (or German Measles) is a mild virus. However, if a pregnant woman catches Rubella in the first 20 weeks of pregnancy, it can cause both hearing and sight impairments, as well as other developmental abnormalities to the unborn child.

A baby who has been affected by their mother contracting Rubella is said to have Congential rubella syndrome (or Rubella Etymology) which affects everyone in different ways but symptoms include:

  • Eye problems such as cataracts and sight deterioration
  • Sensorineural hearing impairments
  • Heart abnormalities
  • Brain damage

Rubella is now rare in the UK thanks to the introduction of the MMR vaccine.

CHARGE syndrome

CHARGE syndrome is a congenital condition (present from birth) that affects many areas of the body. Signs and symptoms vary among people with this condition; however, infants often have multiple life-threatening medical conditions.

The diagnosis of CHARGE syndrome is based on a combination of major and minor characteristics.

Cerebral palsy

A group of lifelong conditions that affect movement and co-ordination, caused by a problem with the brain that occurs before, during or soon after birth.

The symptoms of cerebral palsy aren’t usually obvious just after a baby is born. They normally become noticeable during the first two or three years of a child’s life. Symptoms can include, delays in reaching development milestones – for example, not sitting by eight months or not walking by 18 months, seeming too stiff or too floppy, weak arms or legs, fidgety, jerky or clumsy movements, speaking problems and vision problems. The severity of symptoms can vary significantly. Some people only have minor problems, while others may be severely disabled.

To learn more about types of sight and hearing loss, please click here