Deafblindness Causes, effects and management

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Deafblindness is when an individual has a combined sight and hearing impairment that causes difficulties with communication, access to information and mobility.

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These difficulties are caused by the way that sight loss and hearing loss affect each other even if taken separately, each sensory impairment is mild.

This includes people with progressive sight and hearing loss. Deafblindness has a significant impact on a person’s life, even if they are not totally blind or deaf.

Deafblindness is often referred to as dual sensory loss.

Deafblindness, most commonly affects older adults, although it can affect people of all ages, including children.

What causes deafblindness?

There are many potential causes of deafblindness. The condition can either be present at birth (congenital) or develop later in life (acquired).

Four basic groups of people who experience deafblindness have been identified as those who:

*Are hearing and sight-impaired from birth or early childhood.
*Are blind from birth or early childhood and then later start to lose their hearing.
*Are deaf from birth or early childhood and then later start to lose their vision
*Develop a hearing and sight loss later in life.
Congenital deafblindness is often caused by genetic conditions, such as Down’s syndrome and CHARGE (a rare condition that can affect various parts of the body including the ears, eyes, heart, nasal passages and genitals).

Other possible causes of congenital deafblindness include:

*Excessive drug or alcohol use during pregnancy.
*Viral infection during pregnancy, such as rubella
*A traumatic birth
*Premature birth

Acquired deafblindness can be the result of age, illness or injury. Most people with acquired deafblindness have been able to see or hear for most of their lives.

Some people are born without the ability to see or hear and then start to lose their other senses later in life. For example, someone born with a hearing impairment may develop a condition that affects their sight, such as glaucoma, when they are older.

Although someone with acquired deafblindness can often have a fairly good level of sight or hearing, the combined loss will still make day-to-day living difficult.

Testing hearing and vision
It is very important for the sight and hearing of someone who is deafblind to be accurately assessed. This ensures they receive the appropriate level of care and support.

Living with deafblindness

Deafblindness can be a challenging disability to live with. Each person will have a different level of hearing and sight loss, which means they will have their own individual care needs.

In cases of congenital deafblindness, treatment may not be possible, so care aims to:
*Preserve any remaining sight or hearing the person has.
*Teach the person alternative or informal methods of communication, such as hand-on-hand signing or objects of reference.
*Help the person to develop as much independence as possible – for example, by training them to use a long cane, a guide dog or a sighted guide.
*In cases of acquired deafblindness, it may be possible to identify and treat the cause of the hearing and sight loss. Social care support and rehabilitation training may also help.
For example, cataracts (cloudy patches that develop in the lens of the eye) can sometimes be treated with cataract surgery, and mild or moderate hearing loss can often be improved with a hearing aid.

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Symptoms of deafblindness

Hearing and sight loss levels vary between individuals who are deafblind.

Hearing loss

In deafblindness, hearing loss can occur from birth or may develop later after an infection or injury. In other cases, a person’s hearing may gradually deteriorate over time.

Someone with impaired hearing may find that speech and other noises sound muffled and indistinct and they may not be able to follow and understand conversations, particularly when there is background noise.

A person with a hearing problem may also need to turn up the volume on the television or radio and ask others to speak loudly, slowly and more clearly.

Sight loss

A person who is deafblind may have developed a condition that gradually causes their vision to deteriorate. For example, they may have an eye condition such as:
Cataracts – cloudy patches that form on the eye’s lens
Glaucoma – Pressure changes inside the eye that damage the optic nerve (the nerve that transmits images from the eye to the brain)
Retinopathy – A number of eye disorders that damage the blood vessels of the retina (the light-sensitive tissue at the back of the eye) and can lead to vision loss
Common symptoms of conditions that cause progressive sight loss include:
*Eye pain
*Blurred vision
*Halos around light sources
*Reduced night vision
*Difficulty seeing in bright sunlight or well-lit rooms

When to seek medical help

In cases of congenital deafblindness, hearing and sight loss are usually detected soon after birth during early routine checks.
You should visit your doctor as soon as possible if you notice any deterioration in your hearing or eyesight. They may refer you to a specialist for hearing tests or vision tests.

If they are diagnosed and treated early, eye conditions such as glaucoma and cataracts can be successfully treated before they cause significant damage to your sight.

You should also visit your doctor if you have repeated ear infections because they could increase your risk of hearing loss.

“It is important that the deterioration of one or both senses is identified at an early stage so that appropriate action can be taken. This is particularly important in the case of children”

Causes of deafblindness
There are more than 70 different causes of deafblindness. The condition can either be present at birth (congenital) or develop later in life (acquired).

Congenital deafblindness
In the past, rubella (German measles) was the leading cause of congenital deafblindness. Rubella is a highly infectious virus that can seriously damage a pregnant woman’s unborn baby. In particular, the baby’s eyes, ears and heart can be damaged.

However, since routine rubella vaccinations were introduced in 1988, and due to the success of the MMR vaccine, the number of babies affected by rubella has decreased significantly.
Problems associated with premature birth are now a more common cause of deafblindness. Smoking and excessive alcohol consumption are both major risk factors for premature birth, so you should avoid both if you are pregnant.

Acquired deafblindness
Acquired deafblindness is when a person is born without a hearing or sight impairment, or a single sensory impairment, and loses both senses or part or all of their other sense later. This can be due to age, illness or injury.

Sometimes, a child born deaf can gradually begin to lose their sight. This can be caused by a condition called Usher syndrome, where the retina (the light-sensitive layer of cells that line the back of the eye) gradually deteriorates.

As a person gets older, their hearing will often start to deteriorate as the cells inside their ear become damaged and are unable to repair themselves.
A similar process can also happen with a person’s sight. Over time, it can gradually deteriorate as the cells at the centre of the retina start to break down. This process is often referred to as age-related macular degeneration.

There are a number of other eye conditions that can cause sight loss if they are not identified and treated at an early stage. Some of the more common ones include:
Cataracts – Where cloudy patches develop in the lens of the eye, making a person’s vision misty or blurred; cataracts can usually be removed with cataract surgery
Glaucoma – where the tiny drainage tubes in the eye become blocked, causing pressure to build up inside the eye that can damage the optic nerve (the nerve that transmits images from your eye to your brain)

Retinopathy – A number of eye disorders that damage the tiny blood vessels of the retina; diabetic retinopathy is a common complication of diabetes where the cells of the retina are damaged by high blood sugar levels.

Diagnosing deafblindness

It is important that a deafblind person’s level of hearing and sight are accurately and regularly assessed. How well a person can hear and see will affect the level of care and support they need.

Deafblindness can have a significant impact on a person’s ability to live independently, including:

*The ability to make choices and take control of your life.
*Health and safety
*Managing the daily routines of personal and domestic life.
*Involvement in education, work, family, social and community activities.

Specialist assessment

A specialist assessment should be arranged as soon as deafblindness has been identified by your local authority.

The assessment should be carried out by a specially trained professional who is able to identify the deafblind person’s needs. During the assessment, the following criteria will be taken into consideration:
*Communication
*One-to-one human contact
*Social interaction
*Emotional wellbeing
*Support with mobility
*Assistive technology
*Rehabilitation

The assessment will also take into account current needs and those that will develop in the future. For example, it will be easier for a person to learn alternative forms of communication before their condition deteriorates.

It is important that the deterioration of one or both senses is identified at an early stage so that appropriate action can be taken. This is particularly important in the case of children.

A deafblind person should have access to services suitable to their level of hearing and sight and their individual needs. Mainstream services aimed mainly at blind people or deaf people may not be appropriate for someone with a dual sensory impairment.

*source@ livehealthily.com