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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Arts syndrome


Other Names for this Disease

  • ARTS
  • Lethal ataxia with deafness and optic atrophy
  • Lethal ataxia-deafness-optic atrophy
  • X-linked fatal ataxia with deafness and loss of vision
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Symptoms

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What are the symptoms of Arts syndrome?

Boys with Arts syndrome have sensorineural hearing loss, which is a complete or almost complete loss of hearing caused by abnormalities in the inner ear. Other features include weak muscle tone (hypotonia), impaired muscle coordination (ataxia), developmental delay, and intellectual disability. In early childhood, affected boys develop vision loss caused by degeneration of the nerves that carry information from the eyes to the brain (optic atrophy). They also experience loss of sensation and weakness in the limbs (peripheral neuropathy).[1]  

Boys with Arts syndrome also have problems with their immune system that lead to recurrent infections, especially involving the respiratory system. Because of these infections and their complications, affected boys often do not survive past early childhood.[1] 

Females can also be affected by Arts syndrome, but they typically have much milder symptoms. In some cases, hearing loss that begins in adulthood may be the only symptom.[1] 
Last updated: 4/8/2014

References
  1. Arts syndrome. Genetics Home Reference (GHR). August 2009; http://ghr.nlm.nih.gov/condition/arts-syndrome. Accessed 4/8/2014.


Other Names for this Disease
  • ARTS
  • Lethal ataxia with deafness and optic atrophy
  • Lethal ataxia-deafness-optic atrophy
  • X-linked fatal ataxia with deafness and loss of vision
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.