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Deafness, progressive with stapes fixation
Other Names for this Disease
- Progressive deafness with stapes fixation
- Stapedo-vestibular ankylosis
- Thies Reis syndrome
- Thies-Reis syndrome
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conductive or mixed hearing loss caused by fixation of the stapes. 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. 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. 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.Deafness, progressive with stapes fixation, also known as Thies Reis syndrome, is a form of
Last updated: 12/11/2009
- 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.
- 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.
- Otosclerosis. National Institute on Deafness and Other Communication Disorders (NIDCD). 2008; http://www.nidcd.nih.gov/health/hearing/otosclerosis.asp. Accessed 12/11/2009.
- 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.
- 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.
- Genetics Home Reference (GHR) contains information on Deafness, progressive with stapes fixation. This website is maintained by the National Library of Medicine.
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In Depth Information
- The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Deafness, progressive with stapes fixation. Click on the link to go to OMIM and review these resources.
- 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 Deafness, progressive with stapes fixation. Click on the link to view a sample search on this topic.