Genetics of Hearing Loss

Genetics play a major role in hearing loss for people of all ages. The genetics of hearing loss can be a complicated matter, made even more complex by the fact that inherited and acquired hearing loss can co-occur. Inherited hearing loss is the result of genetic mutations, while acquired hearing loss is caused by environmental factors.

Hearing Loss Caused by Genetic Syndromes

cells in a petri dish

Not all cases of hereditary hearing loss are caused by any specific syndrome, but many are. Doctors estimate that there over 300 genetic syndromes that are associated hearing loss. These include:

  • Alport Syndrome
  • Branchio-Oto-Renal Syndrome
  • Goldenhar’s Syndrome
  • Jervell and Lange-Nielsen Syndrome
  • Mohn-Tranebjaerg Syndrome
  • Norrie Disease
  • Pendred Syndrome
  • Stickler Syndrome
  • Treacher Collins Syndrome
  • Waardenburg Syndrome
  • Usher Syndrome

Hearing loss that is associated with another syndrome is called syndromic hearing loss.

Other Causes of Genetic Hearing Loss

Most cases of hearing loss are not associated with a syndrome; these cases are called non-syndromic. Non-syndromic hearing loss is typically caused by recessive genes, meaning both parents are carriers of the gene. Some cases are caused by dominant genes, which means only one parent is a carrier.

About 70 percent of cases of genetic hearing loss are non-syndromic, and 80 percent of these are caused by recessive genes.

Identifying Genetic Hearing Loss

Identifying genetic causes of hearing loss can be tricky and sometimes requires the team of an otolaryngologist (ENT physician), audiologist, geneticist and a genetics counselor.

The otolaryngologist may take a thorough history and physical exam, then make a referral to an audiologist for a full hearing test.

The patient may also have lab work done to test for prenatal infections that can cause hearing loss in infants. Lab work can help confirm if any syndromes are related to the hearing loss.

A geneticist and genetic counselor can help narrow down which syndromes are likely and eliminate ones that are not. Specific genes can be tested for further confirmation.

If you or someone you love is suspected of having genetic hearing loss, there are options to find out the cause as well as a variety of treatments available. To learn more, call Sharp Hearing today.