Joanne Milne, who has Usher syndrome, heard sounds for the first time this week after receiving a cochlear implant. But what is Usher syndrome – and can it be treated?
Joanne Milne, who has Usher, was recently interviewed about her experience of receiving a cochlear implant to enable her to hear, writes Yemi Tadesse of the deaf-blind charity Sense.
Usher syndrome is a rare genetic condition that affects hearing, vision and, in some cases, balance.
The hearing loss is due to a problem within the inner ear, while the vision loss is caused by an eye condition known as retinitis pigmentosa (RP).
RP leads to a gradual and progressive reduction in vision, most commonly known as tunnel vision.
The impact of Usher syndrome varies from person to person. Some individuals may be born profoundly deaf while others begin to lose their hearing at a later stage in their life. Some individuals begin to lose their vision as early as 12 years of age, while for others RP does not emerge until later in life.
Usher syndrome’s impact on the communication and mobility skills of individuals varies. Individuals with Usher syndrome may be British Sign Language users (BSL) and deaf-blind manual users.
Braille may also be used by this group of individuals. Other prefer to use speech, either with the help of hearing aids and/or by lip reading.
As demonstrated by Jo Milne, some individuals with Usher syndrome may opt to have cochlear implants to help with their hearing loss.
There are various forms of mobility support that individuals with Usher syndrome can use. Some people use the red and white cane; others use a guide dog.
Alternatively, individuals may prefer to receive support from another individual, who is known as a communicator guide.
Both the guide dog and the communicator guide act as the eyes and ears of the deaf-blind person.
The onset of Usher syndrome varies from person to person. Before being diagnosed, individuals may have had a very independent life.
As well as losing your hearing, being told you also face vision loss can be a terrifying experience. This is why having the right emotional support is crucial.
In some cases, meeting other individuals with Usher syndrome is all the support an individual needs.
Sense supports and campaigns for deaf-blind people, including those with Usher, and we have launched the Sense Usher peer mentor scheme, which is run by Jo Milne herself.
With the right support, individuals with usher can lead independent and successful lives.
Yemi Tadesse is research officer at deaf-blind charity Sense. For more information on Usher, visit http://www.sense.org.uk/content/usher-syndrome