As those with Usher type 2 are born with moderate hearing loss they usually develop speech on a par with their hearing peers. People with Usher type 2 are typically born partially deaf / severely deaf, and develop RP later on in life, usually in their 20s-30s. In the past people with Usher type 1 would use British sign language as their preferred method of communication however the younger generation of Usher type 1’s nowadays have cochlear implants (CIs) as infants so may not use sign language at all, and have clear audible speech thanks to a mixture of speech therapy and CIs. They typically have problems with their balance due to vestibular issues inside the inner ear which affects proprioception ability. People who have Usher type 1 are predominantly born profoundly to severely deaf and develop problems with their vision (RP) at a young age or during their teenage years. Usher is a progressive condition and falls into three different group types: Usher type 1 In other words, both parents are carriers and they have a one in four chance of passing two defective copies of the Usher gene to their child (one from each parent). This means that a defective Usher gene must be inherited from each parent in order for that child to develop Usher.

Usher is genetic and inherited in such a way that geneticists would label it a ‘recessive’ condition. Although considered a rare disease, it is a significant cause of deaf-blindness. Usher syndrome is a combined sensorineural hearing loss with retinitis pigmentosa (RP) which is a visual impairment, so essentially people with Usher are what is known in the field as deaf-blind. They just move their eyes around more to compensate. In the early days they may not even be aware of their vision problems. This is why the Usher person often does not feel or look blind. It is the corner of their eye that is missing. “ Usher people often still have sharp, clear central vision.

Usher syndrome type 3