Usher Syndrome: Teaching Strategies for Deaf Blind Education

Usher Syndrome is a condition which affects a relatively small number of individuals within the population - the rate of incidence is around 4 per 100 000 live births. It is an autosomal recessive condition, and for those of who who are a little rusty on your genetics, that means the condition goes something like this:

If a baby is born to two parents who are both carriers for Usher Syndrome, the chance of them inheriting the condition is 1 in 4. There is also a 2 in 4 chance that they themselves will become a carrier for Usher Syndrome, and a 1 in 4 chance of them being neither a carrier nor having the condition.

The major characteristics and symptoms of Usher Syndrome are a combination of deafness and blindness, although the degree of both hearing and vision impairment will vary depending upon both the type of Usher Syndrome (there are three types) and the age of the person. In many people there is also great difficulty shown in balancing tasks such as sitting, standing and walking, and these milestones will often be delayed in a young child with Usher Syndrome.

Generally the condition is a progressive one, with hearing and vision being affected to a far greater degree in later childhood and into adulthood. For a person with Type 1 Usher Syndrome, they are generally profoundly deaf from birth and will have gradually deteriorating visual skills over time begining with loss of night vision.

One of the key educational goals for a child with Usher Syndrome is to establish a communication method which will be appropriate to use as they lose their visual skills over time. This may include the use of Makaton or key word signing, Auslan (Australian sign language) American Sign Language or other assistive communication methods. They may also benefit from early introduction of orientation and mobility skills development which will become useful as they begin to experience deteriorating vision over time. Teachers working with children with Usher Syndrome need to work along with therapists and medical specialists, as well as opthamologists or behavioural optometrists and family members/advocates as part of a multi-disciplinary team approach.

Regular IEP meetings will be required and the teachers will need to ensure they remain up to date with the vision and hearing and balance skills of the child over time. It will also be important that a thorough hand over of teaching notes, goals and IEP documentation is completed each year. In some cases it may be useful for a teacher to remain as the class teacher for a child with Usher Syndrome over a period of years in order to promote and maintain their communication and sense of security and consistency within the school environment.