Deaf – blindness is a complex disability involving both vision and hearing loss. There are around 70 different causes of deaf-blindness. A distinction is made between an acquired and a congenital disability.
What is deafblindness?
In addition to the two major distinguishing criteria of acquired and congenital deaf- blindness, six specified categories are named:
- Born blind and deaf before learning to speak
- Born blind and deaf after learning the language
- born deafblind
- Born deaf and blind in childhood
- Born deaf and blind in old age
- Deaf and blind only much later, either at the same time or at different times.
In contrast to those who are blind or deaf, it is not possible for deaf-blind people to compensate for one sense with the other. Mobility, orientation and communication are extremely difficult as a result, so that those affected are dependent on lifelong support. An independent way of life, school education, choice of profession, partnership and raising children is extremely difficult for those affected, especially since the ability to communicate is severely restricted. For what are crush wounds, please visit gradinmath.com.
There are around 70 known causes of deaf-blindness, including rubella infection during pregnancy, extreme premature birth, after which the babies in the incubator may have too much or too little oxygen, toxoplasmosis, cytomegaly or drug abuse by the expectant mother. By far the most common trigger is Usher syndrome, which is diagnosed in every second patient.
This is an autosomal recessive inherited disease. Although both parents are carriers of the disability, they do not suffer from it themselves. There are several genetic defects that are located on different chromosomes and are responsible for deaf-blindness. There are twelve different types of Usher, which involve different genes and result in a greater or lesser degree of disability.
Common to all subtypes is deafness or very severe sensorineural hearing loss, which is present from birth. The reason is damage to the hair cells in the cochlea of the inner ear. The loss of vision, on the other hand, sets in later. It is caused by retinopathy pigmentosa, RP for short, in which the photoreceptors die off. RP begins around the age of ten with night blindness and gradually reduces the field of vision. Depending on the Usher type, RP can lead to blindness.
Symptoms, Ailments & Signs
While the hearing impairment can be diagnosed at a very early stage, the vision problem only occurs over the years. Deaf-blindness does not necessarily mean complete deafness and absolute blindness. Nevertheless, those affected perceive their environment differently because, unlike the blind or deaf, they cannot replace one sense with the other.
This double sensory disability only enables those affected to communicate via the sense of touch. Deaf-blindness is almost always accompanied by further damage. In many cases, there are learning disabilities, mental impairments and even neurological abnormalities. Often there is also a severe impairment of the overall development. However, this is highly dependent on when the disability occurred. Loss of balance is common in Usher patients, while others are prone to, although rare, epileptic seizures.
Diagnosis & course of disease
The diagnosis of deaf-blindness can now be made more quickly thanks to improved DNA analyses. This applies above all to the complex genetic defect in Usher syndrome, which is the main cause of the disability. In this way, hearing impairment can be diagnosed in newborns. Timely diagnosis plays a particularly important role in Usher syndrome.
If blindness is added to the hearing impairment at school age, this can lead to severe trauma for the children. The clinical features of Usher syndrome are threefold:
- Usher type 1 (USH1) shows the most severe course of the disease with congenital deafness and the onset of retinopathy of pigmentosa from the 10th year of life.
- Usher type 2 (USH2) has a constant, severe hearing loss, with RP beginning during puberty.
- In Usher type 3 (USH2), the hearing loss begins after language acquisition and progresses continuously, retinopathy of pigmentosa begins around the second half of life.
Deaf-blindness has a very negative effect on the everyday life of those affected and can lead to significant limitations in life. Especially in young people, deaf-blindness can delay development considerably, so that there can also be limitations in adulthood. Those affected suffer from severe visual and hearing problems.
As a result, it may not be possible to correctly assess and recognize dangers in everyday life, which means that accidents may occur more frequently. Most of those affected also suffer from balance disorders. The patients depend on the help of other people in their everyday life and cannot master many activities on their own. Epileptic seizures can also occur in deaf-blindness and, in the worst case, lead to the death of the person concerned.
Relatives and parents are often affected and suffer from psychological problems and limitations. A causal treatment of deaf-blindness is not possible. The symptoms can be reduced with the help of visual aids and hearing aids. However, the symptoms can also worsen over time, so that the person affected becomes completely blind or loses their sense of hearing completely.
When should you go to the doctor?
If parents or relatives notice behavioral problems in their offspring or if they notice irregularities in natural reactions, a doctor should be informed of the observations. Peculiarities in the position of the eyes or a lack of reaction to strong noise are signs of a health problem. Discoloration of the eye, a blank stare, or lack of reflexes when a breeze hits the eye is uncommon and needs to be seen by a doctor. Vocal abnormalities, the absence of a startle reflex or irregularities in movement must be examined and treated.
Normally, deaf-blindness is diagnosed immediately after birth. The present obstetric team examines the newborn and already detects the first irregularities. In further tests and examinations a few days or weeks after the birth, the health impairments are also automatically determined by the pediatrician. In most cases, therefore, the relatives do not have to take action independently with this disease.
Nevertheless, you should consult a doctor if the first examinations at the beginning of life did not result in any diagnosis and the baby still shows behavioral problems and unnatural developments. If children react neither to visual contact nor to a noise, this is unusual. If the offspring only reacts to physical contact, this should be discussed with a doctor.
Treatment & Therapy
Since deaf-blindness is not a disease but a disability, the person affected needs special aids. These in turn are tailored to the degree of hearing and vision of the disabled person. Large screens or magnifying glasses provide support where there is little vision. A cochlear implant helps hearing-impaired children as long as the auditory nerve is still functional.
A touch language is required for communication, such as the Lormen or Tadoma. With Lormen, the communicating partner puts certain signs in the palm of the hand of the person concerned, which the deaf-blind person can arrange into letters and recognize as words and sentences. Tadoma is also a touch language. Here, the deaf-blind person feels the face of the speaker, registers the lip movements with his thumb and with his other fingers he feels over the neck and cheek.
Deaf-blind people depend on need-based support throughout their lives. Sign language and communication courses for parents and relatives are of fundamental importance in order to be able to communicate better with their children.
As a rule, this sensory impairment cannot be prevented, especially when it is a matter of genetic Usher syndrome. Vaccination can prevent rubella infection during pregnancy. Expectant mothers should be screened for toxoplasmosis and cytomegalovirus as a precaution.
According to scientific definition, deaf-blindness is not classified as a disease but as a disability. Medical aftercare aims primarily to prevent complications and to grant a high degree of independence through offers of help. Even in children, deaf-blindness entails enormous restrictions in the way of life.
They usually cannot attend a regular school and have special support needs due to their limitations. As a rule, however, this cannot compensate for the disability, which is why there are major restrictions in the job. Learning sign language is an important element of aftercare. This preserves the ability to communicate.
Furthermore, those affected depend on aids such as hearing aids, screens and reading magnifiers throughout their lives. It is not uncommon for mental problems to arise as a result of deaf-blindness. There is constant isolation in perception. A therapist can address difficulties and negative constellations and, if necessary, remedy them. A strong and stable environment as well as contact with other deaf-blind people often promise the best relief from mental suffering.
You can do that yourself
Deaf-blindness means a lifelong need for help. It’s not a disease, it’s a disability. A cure is impossible. The everyday life of those affected is severely restricted. They are unable to hear or see, or have limited ability to do so.
In order to cope with everyday life, they regularly have to use aids. Special magnifying glasses or screens and ear implants sometimes promise that the performance of the sensory organs will increase. Communication usually takes place via a touch language. This extreme handicap often leads to isolation, which is why clubs and associations work to integrate the sick. Apart from medical care, relatives in particular try to improve the situation of the deaf-blind. This often happens with the involvement of therapists and doctors.
Self-help is primarily aimed at independently coping with everyday life. Deaf-blind people are particularly good at routine processes. Structural measures can, for example, eliminate sources of danger in an apartment. A stable family environment can significantly increase the quality of life. However, elementary everyday processes such as preparing food are only possible with external help. They cannot explore new local conditions on their own.