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Alström syndrome

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

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What percentage of patients with Alström syndrome with complete hearing loss?

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: 6/14/2016

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: 6/14/2016

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  1. Marshall J, Paisey RB, Carey C, Macdermott S. Alström syndrome. GeneReviews. May 31, 2012; http://www.ncbi.nlm.nih.gov/books/NBK1267/.
  2. Marshall J, et al. Alström Syndrome. European Journal of Human Genetics. 2001; 15:1193-1202. http://www.nature.com/ejhg/journal/v15/n12/full/5201933a.html.