The different types of Usher syndrome

Usher syndrome is broadly divided into three types.

About Usher type 1

Sensorineural hearing loss 

Usher type 1 is characterised, in most cases, by a profound hearing loss in both ears present at birth. Compared to other Usher types, the hearing loss occurs much earlier and is more profound.

Balance

Usher type 1 is characterised, in most cases, by balance problems from birth. This means that babies may demonstrate delays in sitting and walking. Walking tends to occur after 18 months of life. Significant disorientation may be experienced throughout life, particularly when vision has deteriorated.

Retinitis Pigmentosa (RP)

Usher type 1 is associated, in most cases, with the development of nightblindness within the first decade of life. This then progresses to tunnel vision followed by further reduced  vision and issues with discriminating between colours. This series of events for all cases of RP related that is caused by Usher syndrome. The development of other eye problems may reduce vision further, such as cataracts.

About Usher type 2

Sensorineural hearing loss 

Usher type 2 is characterised, in most cases, by hearing loss in both ears, usually, within the higher frequency ranges. Although hearing loss is present at birth, it commonly ranges from mild to severe, differing from the hearing loss seen in Usher type 1. 

Balance

Usher type 2, in most cases, is not associated with balance problems. This is usually a characteristic that is used to distinguish between individuals with type 1 and type 2 Usher.

Retinitis Pigmentosa (RP)

Usher type 2 is characterised, in most cases, with the development of RP within the second decade of life. RP follows the same series of steps described above.

About Usher type 3

Sensorineural hearing loss 

Usher type 3 is characterised, in most cases, by progressive hearing loss, which occurs relatively later in life once the individual has developed speech. This form of Usher varies from individual to individual. Some individuals may develop profound hearing loss whilst others do not. 

Balance

Usher type 3 is clinically characterised, in most cases, by impairments of the balance system. Individuals can be misdiagnosed as having type 1 if they demonstrate balance problems along with profound hearing loss. 

Retinitis Pigmentosa (RP)

Usher type 3 is characterised, in most cases, with the development of RP later in life.

 

Atypical cases

It is important to acknowledge that not all individuals with Usher syndrome fit into the three clinical classifications of Usher syndrome i.e. type 1, type 2 and type 3. Instead, the symptoms they exhibit may vary greatly and be difficult to diagnose as a specific Usher type.  Cases like this are often called atypical cases. 

 

More from Sense

Vision loss and Usher syndrome

Hearing loss and Usher syndrome

Balance and Usher syndrome


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Created: February 2016

Review due: February 2018

 

  

 

First published: Wednesday 2 December 2015
Updated: Monday 16 January 2017