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Diseases

Genetic and Rare Diseases Information Center (GARD)

Progressive deafness with stapes fixation


Other Names for this Disease
  • Thies Reis syndrome
  • Thies-Reis syndrome
  • Stapedo-vestibular ankylosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Progressive deafness with stapes fixation, also known as Thies Reis syndrome, is a form of conductive or mixed hearing loss caused by fixation of the stapes.[1][2] The stapes is one of the tiny bones in the middle ear. It rests in the entrance to the inner ear, allowing sounds to pass to the inner ear. If it becomes fixated, sound waves cannot pass through to the inner ear, resulting in loss of hearing.[3] This condition may be associated with a number of conditions, including ostosclerosis, Paget's disease and osteogenesis imperfecta, or it may be found in isolation.[2][4][5] It may also result from chronic ear infections (otitis media with tympanosclerosis). The progression of hearing loss is generally slow, rarely profound, and usually resolves following treatment. Conductive hearing loss can be restored through surgery or hearing aids. Sensorineural hearing loss can be managed with hearing aids or cochlear implants.[2]
Last updated: 12/6/2015

References

  1. Deafness, Progressive, with Stapes Fixation. Online Mendelian Inheritance in Man (OMIM). 1996; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601449. Accessed 12/11/2009.
  2. Bozorg Grayeli A. Deafness, progressive, with stapes fixation. Orphanet. 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3235. Accessed 12/11/2009.
  3. Otosclerosis. National Institute on Deafness and Other Communication Disorders (NIDCD). 2008; http://www.nidcd.nih.gov/health/hearing/otosclerosis.asp. Accessed 12/11/2009.
  4. Albert S, Roger Gm Rouillon I, Chauvin P, Denoyelle F, Derbez R, Delattre J, Triglia JM, Garabedian EN. Laryngoscope. 2006; http://www.ncbi.nlm.nih.gov/pubmed/16826051. Accessed 12/11/2009.
  5. Hara A, Ase Y, Kusakari J, Kurosaki Y. Arch Otolaryngol Head Neck Surg. 1997; http://www.ncbi.nlm.nih.gov/pubmed/9339992. Accessed 12/11/2009.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Progressive deafness with stapes fixation. This website is maintained by the National Library of Medicine.

General Information

  • The !LINK! provides more information on this topic. You can reach them by calling toll-free at 800-241-1044 or by visiting their Web site.

In Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a 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 Progressive deafness with stapes fixation. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Thies Reis syndrome
  • Thies-Reis syndrome
  • Stapedo-vestibular ankylosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.