Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.


Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Deafness, progressive with stapes fixation

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

Your Question

Please provide information about deafness, progressive with stapes fixation (Thies Reis syndrome), including symptoms, causes, treatment and prognosis.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is deafness, progressive with stapes fixation?

Deafness, progressive 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: 12/11/2009

What are the symptoms of deafness, progressive with stapes fixation?

Deafness, progressive with stapes fixation is characterized by bilateral hearing loss - either conductive or mixed - and stapes fixation.[1][2] Hearing loss typically begins between ages 8 and 24.[1]
Last updated: 12/11/2009

What causes deafness, progressive with stapes fixation?

The exact cause of deafness, progressive with stapes fixation depends on whether it is associated with an underlying condition or infection. Isolated cases may be inherited.  Autosomal dominant, autosomal recessive, and X-linked cases have been reported.[1][4][5][6] In some cases, no underlying cause can be identified.  
Last updated: 12/11/2009

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.[4][7]

Last updated: 12/11/2009

What is the prognosis for individuals with deafness, progressive with stapes fixation?

Surgical treatment of isolated deafness with stapes fixation allows good functional results regardless of age or type of surgery.[4] If other symptoms or complications are present, prognosis may be vary.
Last updated: 12/11/2009