Usher Syndrome

Usher’s is a genetic condition which causes hearing loss from birth and progressive loss of sight due to Retinitis Pigmentosa.  There are two types of Usher’s.

Usher’s Type 1

• Profound deafness from birth
• RP usually evident within the first 10 years of life
• People with Usher’s Type 1 usually use singing to communicate

Usher’s Type 2

• Hearing loss ranging from mild to severe
• RP evident from late adolescence to late twenties
• People with Usher’s Type 2 usually use hearing aids and lip read

Although Types 1 and 2 are now well recognized, there is some evidence to suggest that a Type 3 Usher syndrome exists.  People with this type appear to have normal hearing and sight at birth and in childhood.  RP is usually diagnosed between the ages of 20 to 30 years, followed by a degeneration of hearing in adult life.

How do you get Usher’s?

Usher’s is passed on genetically and is a recessive disorder, which means that a person must have two Usher’s genes, one from each parent to get the condition.  A person who has one gene will not develop Usher’s and is called a carrier.  If both parents are carriers, each of their children has a one-in-four chance of having Usher’s.