‘Deaf’ is a broad concept. Sometimes a person is called ‘deaf’ when he or she does not correctly hear one word in a conversation. Someone wearing hearing aids who can correctly hear speech is also often called deaf or considers him/herself as being deaf. And someone who can hear almost nothing can also be called deaf.
‘Deaf’, written with a capital D, refers to people who do not experience their ‘being Deaf’ as a handicap but as a characteristic. They have strong relationships within the Deaf community and the Nederlandse Gebarentaal (NGT) (Dutch Sign Language) is their native language.
Actually, we can hardly ever speak of complete deafness in an audiological sense. Everyone can hear something and recognise speech in some way. We use the concept of hard of hearing, because we can indicate levels in this. For example, in order to be eligible to receiving cochlear implants for hearing or hearing aids.
Usher Syndrome knows a lot of variations in loss of hearing. The consequences of this can be far-reaching when at a later age the eyesight of a person deteriorates as well. Much depends on the opportunities and possibilities a person is given and the support that is received.
Some Ushers are born hard of hearing. Thanks to screening the hearing of a new-born baby and the possibility of a DNA test, deafness and the cause of this can nowadays be discovered at an early stage. In order to stimulate language development, children are given a cochlear implant for both ears at a very young age already. This enables them to grow up as being ‘hard of hearing’. With the support of sign language and/or lip reading and adequate guidance, the development of language and speech can be stimulated further.
A part of the people suffering from Usher Syndrome was born deaf and grew up without the innovation of cochlear implants in the Deaf community. They make use of sign language and sign language interpreters when communicating with people who can hear well. As their eyesight deteriorates, they make use of small signing space, tactic sign language, LORM and haptic signs.
Deafness and seriously bad hearing can be accompanied by balance problems.
Some people who were born hard of hearing or who became hard of hearing in their childhood have a progressive loss of hearing. Eventually, hearing aids will no longer be adequate. The language and speech have developed successfully and most people have been able to function reasonably well in the hearing world with the help of lip reading and hearing aids. As people suffering from Usher Syndrome also have a progressive visual impairment and are therefore highly dependent on their hearing, adults with serious deafblindness caused by Usher Syndrome may since the year 2016 be eligible for two cochlear implants, which makes it possible for them to localise sound and to understand speech (even without lip reading) again after a good hearing rehabilitation.
Progressive loss of hearing sometimes takes place so gradually, that it is not (timely) noticed. Only when hearing and understanding speech is improves by means of a cochlear implant, persons realise how much they have ‘missed in terms of sound’ and how much impact this had on their quality of life. A progressive loss of hearing also has far-reaching consequences for the lives of partners, children and colleagues.