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Genetic and Rare Diseases Information Center (GARD)

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Alström syndrome

Other Names for this Disease
  • ALMS
  • ALSS
  • Alstrom syndrome
  • Alstrom's syndrome
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Your Question

What percentage of patients with Alström syndrome become deaf? What is the average age of deafness?

Our Answer

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What percentage of patients with Alström syndrome become deaf?

Progressive sensorineural hearing loss is found in 88% of individuals with Alström syndrome.[1]  About 10% of affected indivduals progress to profound deafness and must rely on sign language for communication.[2]
Last updated: 9/27/2011

What is the average age of deafness in individuals with Alström syndrome?

In up to 70% of individuals with Alström syndrome, hearing loss becomes evident between ages one and ten years. The average age of onset is 9 years old but can range from 2 years to 25 years old.[1]  Hearing loss may progress to be more severe by the end of the first to second decade.[1]
Last updated: 9/27/2011