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Tietz syndrome

Other Names for this Disease
  • Albinism-deafness of Tietz
  • Hypopigmentation/deafness of Tietz
  • Tietz albinism-deafness syndrome
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Tietz syndrome is a genetic condition characterized by profound hearing loss from birth, fair skin, and light-colored hair.[1] This condition is caused by mutations in the MITF gene. It is inherited in an autosomal dominant fashion with complete penetrance.[2]  
Last updated: 1/28/2010


  1. MITF. Genetics Home Reference (GHR). 2006; Accessed 1/28/2010.
  2. Smith SD, Kelley PM, Kenyon JB, Hoover D. J Med Genet. 2000; Accessed 1/28/2010.
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In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Tietz syndrome. Click on the link to view a sample search on this topic.