UNDERSTANDING DEAFNESS

RELATED CONDITIONS

Tinnitus

Daily life can be incredibly noisy and following exposure to loud noises for long periods people can be left experiencing a ringing or buzzing in the ears; altbough for most people, these noises are only temporary for over four million people in the UK these noises can be permanent. These noises are often severe, can cause depression, anxiety and frustration and are known as tinnitus. Individual cases and personal experiences of tinnitus vary dramatically with some people experiencing only very quiet 'whisper-like' sounds and air tunnels, to people who experience sounds that roar. For some people the noise is constant, and for others it only occurs from time to time.

Whilst only a small number of people suffer with tinnitus, it is thought that nearly all people experience some form of head noises. In the early 1950s a series of experiments were carried out into tinnitus involving 80 people with normal hearing. After being placed in a soundproofed room for five minutes, 93% reported hearing tinnitus sounds, although none had previously complained of tinnitus.

So what is it which makes these sounds so prominent for tinnitus sufferers? Although scientists disagree over the exact nature of tinnitus, the problem is believed to involve processes in both the ear and the brain. Some experts believe that tinnitus can sometimes be attributed to the brain focusing on the background electrical activity which is a natural part of the normal ear function. It is also known that symptoms can be exacerbated by environmental factors such as stress, depression and tiredness. Given that tinnitus itself can lead to anxiety, sleep deprivation and stress, those experiencing tinnitus can find themselves trapped in a vicious circle. There are a wide variety of different causes of tinnitus, including exposure to loud noise, use of some prescription drugs and middle ear infection. However, for many people, tinnitus, like hearing loss, is a natural part of the ageing process - believed by some experts to be linked to the deterioration of the tiny 'hair' cells in the inner ear.

There are many effective treatments of tinnitus which may lessen the effects or entirely eradicate the condition. For more information visit your GP.(21)

USHERS SYNDROME

Usher Syndrome (Usher) is a genetic disorder which combines a moderate to profound hearing loss and Retinitis Pigmentosa commonly (RP), which causes a progressive loss of sight.

Usher Syndrome is a genetic condition and therefore is inherited. It is caused by a recessive gene which means that most people with Usher Syndrome are the only ones in their families to have the condition . In fact, many families do not even have a history of hearing loss or RP.

Unfortunately, there is currently no genetic test to check if parents are likely to have a baby with the condition. Ten different genes are involved in the development of Usher's syndrome.

Hopefully within the next 5 years genetic counselling will be available for families affected by Ushers Syndrome. (20)

Types of Usher

There are three main types of Usher Syndrome identified at present.

Type 1 Type 2 Type 3
  • Born with a profound hearing loss.
  • Balance problems.
  • Develop night-blindness in their teens or earlier, due to RP.
  • Born with a moderate to severe hearing loss.
  • Develop night-blindness in their 20's, due to RP.
  • No balance problems in childhood.
  • Progressive hearing loss that gradually worsens.
  • Develop visual impairment around the age of 20-30 years due to RP.
  • May have balance problems later in life.

People, who have Type 1 Usher will often be sign language users and may attend a school for deaf children.

People who have Type 2 Usher will normally be able to speak, may or may not use sign language and may attend a mainstream school or school for the deaf children.

Type 3 Usher is extremely rare. A small number of people who have type 3 Usher have speech and do not normally sign. They would normally have attended a mainstream school.(20)

 

 

 

 

 

 

Many people assume that when a person is deaf, they are unable to hear any sound at all. In reality this is far from true. Whilst deaf people may struggle to distinguish individual sounds or noises, ie spoken language, the vast majority of people suffering from hearing loss still get an overall sensation of sound.

What causes deafness?????

Many people lose their hearing with age. Whilst others may be born with significant hearing loss. It is not entirely understood why some babies are born with hearing loss and the diagnosis can often be a shock to the parents. Fewer than 10% of deaf babies are born to deaf parents and those that are born to hearing parents may be born into a family with no history of deafness.For more information on this see children and deafness.A baby may also be born deaf if the mother contracts rubella whilst pregnant or if the baby has to be delivered prematurely. Other common causes of deafness are childhood illness such as meningitis, measles and mumps. Deafness can also result from trauma, ie a blow to the head or from prolonged exposure to loud sounds. These people are said to be 'deafened'.

Different types of deafness

There are several different 'types' of deafness. Whilst some people may find that their hearing loss prevents them from distinguishing all types of sound, others may find that they are only deaf to certain frequencies of sound. These people may be unable to hear sounds of very low or very high frequency. (14)

In addition to this, there are three commonly recognised forms of deafness (excluding traumatic injury):

Conductive deafness (outer and inner ear) is is the most common form of deafness and may be temporary of permanent. It usual occurs through the build up of wax in the ear canal or fluid in the middle ear. Such blockages means that sound is unable to pass though the outer ear and middle ear to the cochlea and auditory nerve. Sensori-neural deafness (inner ear) is also known as nerve deafness. The actual cause of this type of deafness is unknown but it is presumed that it can sometimes occur through meningitis, measles, mumps and rubella, lack of oxygen at birth, and in some cases be genetic. This particular deafness occurs in the inner ear where the sound is not being processed properly by the cochlea or the auditory nerve.Mixed deafness a s the name states is a mixed deafness of conductive and sensori-neural deafness.In addition to the types of deafness, there are degrees of severity within each classification. The recognised classifications are (14):

  • Mild deafness – struggle in noisy situations to follow speech. The person can hear quieter sounds roughly between 25 and 40 decibels.
  • Moderate deafness – struggle to hear or follow speech without the aid of a hearing aid. The person can hear quieter sounds oughly between 40 and 70 decibels
  • Severe deafness – relies on lip reading and sign language even with a hearing aid. The person can hear quieter sounds roughly between 70 and 95 decibels.
  • Profound deafness – this degree of deafness relies heavily on lip reading and sign language. The person can hear quieter sounds from 95 decibels. (15)