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Types of Genetic Hearing Loss

Read this page to learn about the different kinds of genetic hearing loss.

There are many different types of genetic deafness. Saying hearing loss is "genetic" means that there is a change in a gene.

  • Genes are in every cell of our bodies.
  • Genes store the instructions needed to make our bodies work.
  • When there is a change in a gene, it is called a mutation.
  • A mutation may mean that some part of the body doesnít work right.


About 30% of people with a genetic type of hearing loss have what is called syndromic hearing loss. The other 70% have a type called non-syndromic hearing loss.

Syndromic hearing loss
Syndromic means that the hearing loss is just one part of a condition that may have many other features. These other features can be:

  • things that make a child look different, like eyes that are different colors
  • things that you canít see, like kidney or heart problems

How to find out if your child has a syndromic hearing loss

If your child has a hearing loss, your doctor will want to see if he has certain features that suggest a syndrome:

  • He will look for things he can see.
  • He may measure things like the distance between your childís eyes.
  • He may order other medical tests.

What to do if your child has a syndrome

If you find out your child has a syndrome, you and your doctor will want to watch to see if she has other features that are part of that syndrome. Hearing loss is a feature in more than 400 known syndromes. The list below tells you about other features found in some of the most common syndromes that include hearing loss.

  • Your child may have only one feature of a syndrome or more than one. Everyone is different.
  • Even if several people in your family have the syndrome, they may all have different features.
  • Your doctor will watch for other possible medical problems.
  • Your doctor may be able to treat other medical problems.

Common Syndromes Associated with Hearing Loss
Name of syndrome Other features that may occur (besides hearing loss)
Alport Kidney problems
Branchio-oto-renal (BOR) Neck cysts and kidney problems
Jervell and Lange-Nielsen Heart problems
Pendred Thyroid enlargement or low thyroid function
Stickler Unusual facial features, cleft palate, eye problems (nearsightedness, cataracts, or retinal detachment,) arthritis, heart problems
Usher Progressive blindness
Waardenburg White patch of hair or light-colored skin patches; eyes of two different colors, or bright blue eyes, or widely spaced eyes

Non-syndromic genetic hearing loss
Non-syndromic means that the hearing loss is not associated with any other medical problems. If your child doesnít have a syndrome and you donít know of any other reason for his hearing loss, it may be a genetic non-syndromic hearing loss.

There are different types of non-syndromic hearing loss. Read about the different types on the next page, Inheritance of Hearing Loss.

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