SIMULATION VIDEO

Three Main Types of Usher Syndrome


Type 1Type 2Type 3
Hearing
Profound deafness in both ears from birth
Moderate to severe hearing loss at birthNormal at birth; progressive loss in childhood and early teens
VisionDecreased night vision before age 10
Decreased night vision begins in late childhood or teens
Varies in severity; night vision problems often begin in teens
Vestibular function (balance)
Balance problems from birth
NormalNormal to near-normal, chance of later problems


Usher syndrome is diagnosed through a series of hearing and eye tests to determine functioning of both senses. These tests, coupled with genetic testing will determine which type the person has and degree of hearing loss ad/or vision loss.

Although there is no cure for Usher Syndrome we are fortunate to have The Usher Syndrome Coalition in Massachusetts. The mission of The Coalition is to raise awareness and accelerate research for the most common cause of combined deafness and blindness.

The Coalition houses the Usher Syndrome Registry which directly connects clinicians and researchers with Usher Syndrome patients.
 
The Coalition counts a number of leading institutions and researchers among its members. Member institutions bring their expertise and commitment to the work of the Coalition, collaborating with partners and researchers from some of the finest international organizations in the world. In short, the Usher Syndrome Coalition strives to be the most comprehensive resource for the Usher syndrome community.

For more information on The Usher Coalition please visit them here.

sensory impairment information

Usher Syndrome

For more resources on Hearing Aids please visit “Helpful sites.”

Hearing loss is one of the most common birth defects. But, for those with a newly diagnosed child or someone struggling to communicate, the disability can be life-altering. There are a number of resources available to you to learn more about the different communication modes and listening devices, should you so choose. Below is an actual hearing loss simulation with and without an FM system in the educational setting:

Hearing Aids

Hearing aids are a first step to stimulating the auditory nerve with sound. Not everyone who uses a Hearing Aid will benefit from it. Why? Well, the speech banana dictates where speech is intelligible and where speech is unable to be accessed at.

As you can see from the above photo sounds in “the banana” is where speech is “Normal.” As you move further down the audiogram thee are the sounds that someone with the correlating hearing can “easily” hear as their “normal.”

Hearing aids essentially make speech louder and are therefore not appropriate for everyone. Someone with a mild-moderate loss may become mild whereas someone with a profound loss could hear in the normal range. There are so many varying factors and results are highly individualized.

What is the same, regardless of where a person’s hearing falls on an audiogram, is that hearing through a hearing aid is not perfect. Below is a simulation of a child hearing in the classroom setting. 

​Usher syndrome is the most common condition that affects both hearing and vision. The major symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. There are three main types of Usher Syndrome and all vary in severity, progression and age of onset.​

Hearing Loss