Usher, Cochlear Implants and You What is Usher? People with Usher syndrome are deaf or partially hearing with an eye condition called Retinitis Pigmentosa (RP). What is RP? The retina at the back of the eye is very sensitive to light. What you see is caught by the retina and sent along the optic nerve to the brain. With RP, the retina slowly becomes damaged and unable to send pictures to the brain. There is no treatment available yet which will stop the sight from changing. The symptoms of RP are: Difficulty seeing at night, no side or up and down vision, but you can see straight ahead. What does having a Cochlear Implant involve? - Assessment You would have a detailed hearing assessment involving a hearing aid trial, evaluation of lip-reading skills, speech perception and discussion of expectations. - Surgery You would stay in hospital for one or two days. The operation lasts between 2 and 3 hours. The surgeon makes a small cut behind the ear with no need to remove any of your hair. - Programming and Lifelong Care Initial programming takes place about four weeks after surgery. The external parts of the system are fitted, and the process of tuning the device to create an individual programme begins. Regular visits to the hospital are required to monitor the functioning of the implant system. Some Implant Centes offer support to younger users to listen and develop language skills. Then you would need to visit the Cochlear Implant Centre every year to make sure the implant system is working well. Claire’s story I decided to have a Cochlear Implant (C.I.) because hearing aids do not help me at all. A C.I. is also useful because of my Usher vision. My Cochlear Implant has made me aware of everyday sounds and has given me confidence in crossing roads. I can hear the traffic, so I can check for traffic before crossing the road. I can find my keys more quickly if I drop them because I can hear them hit the floor. Hearing the sound of my voice is incredible! David’s story David had his Cochlear Implant (C.I.) when he was 18 months old. He was diagnosed as having Usher Type 1 at 3 years 9 months old. Before his implant David was using some residual hearing through high powered hearing aids. His communication was based on sign, gesture and his own bubbly personality. Having a Cochlear Implant has enabled David to develop language through listening and sign and now has appropriate language for a 6 year old. David wears a hearing aid in one ear and his implant in the other ear. He attends his local mainstream school and he participates fully in all classroom and out of school activities with his deaf and hearing friends. Russ’s story Hello! I have Usher Type 3. I have always loved music. Surprisingly, my Cochlear Implant (C.I.) has helped me to hear music better. Now, I am able to hear many different musical instruments and to hear my voice while I sing. I have been able to continue as a music therapist. When listening to TV and radio, I can hear the spoken language in a clear and new way. I use the C.I. plus a hearing aid in my other ear which works well for me. My musical journey continues… Jane’s story Jane is 20 and was diagnosed as having Usher Type 1 just before she had her Cochlear Implant (C.I.) at 11 years old. She attended her local school for the deaf and successfully took her exams. After carefully considering her options, she is now enrolled at college taking a business studies course. Jane uses her Cochlear Implant to monitor environmental sounds. She has developed her lipreading and language skills enough to have relaxed communication with her close hearing friends and continues to sign with her deaf friends. Jane is very independent and enjoys travelling. What is Cochlear Implant? A Cochlear Implant is an electronic hearing aid, which provides sound to the hearing nerve. It can be used when your normal hearing aids do not provide enough sound to recognise speech. It can also tell you about the sounds happening around you. A Cochlear Implant consists of: - The internal parts - a receiver implanted by the surgeon in the mastoid bone behind the ear, and electrodes placed directly in the cochlea (inner ear). - The external parts - a microphone, processor and transmitter. The transmitter carries sound through the skin to the receiver. Types of Usher Type 1 People with Type 1 are profoundly deaf from birth. They cannot hear speech, even with hearing aids. Most people with Type 1 use British Sign Language (B.S.L.) or Sign Supported English (S.S.E.). They have poor balance. The RP is usually noticeable at around 10 years old. Type 2 People with Type 2 have partial to severe hearing loss from birth. They can hear some speech with hearing aids but they will need to lipread too. Some people with Type 2 sign as well as using hearing aids. They have normal balance and the RP is usually noticeable from teenage years upwards. Type 3 People with Type 3 have normal hearing or partial hearing from birth. Later on their hearing changes and RP may also be diagnosed at that time. Some people with Type 3 have good balance, others don't. This is a rare form of Usher in young people. Further information about Usher and Cochlear Implants can be obtained from Sense Information and Advice 101 Pentonville Road London N1 9LG Tel 0845 127 0066 Textphone 0845 127 0066 Fax 0845 127 0061 Email info@sense.org.uk www.sense.org.uk Nottingham Cochlear Implant Team Ropewalk House, 113 The Ropewalk Nottingham NG1 6HA Tel 0115 948 5549 Fax: 0115 948 5560 Email ncip@nuh.nhs.uk www.ci-4teenz.com The Usher and Cochlear Implant Partnership consists of Sense and the Nottingham Cochlear Implant Programme, with the support of Advanced Bionics. With grateful thanks also to Russell Gilmore.