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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.

Treatment

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How might deafness, progressive with stapes fixation be treated?

Treatment for deafness, progressive with stapes fixation typically involves surgery.[1][2] The conductive component of the hearing loss can be restored by surgery or hearing aids. The associated sensorineural component is managed by hearing aids or cochlear implants, depending on its severity.[2] Stapedotomy (a procedure where a laser is used to make a hole in the stapes) or partial stapedectomy (removal of the stapes) with stapes replacement using a prostheses most commonly achieves satisfactory results with minimal complications.[3][4]


Last updated: 12/11/2009

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. 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.
  4. Salahuddin I, Salahuddin A. Ear Nose Throat. 2002; http://www.ncbi.nlm.nih.gov/pubmed/12199172. Accessed 12/11/2009.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

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Clinical Trials & Research for this Disease

  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".
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.