National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Progressive deafness with stapes fixation

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

The following information may help to address your question:


What is progressive deafness with stapes fixation?

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 otosclerosis, 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: 1/30/2017

What are the signs and symptoms of progressive deafness with stapes fixation? 

Progressive deafness with stapes fixation is characterized by hearing loss in both ears that can be conductive, sensorineural, or a mix of both and abnormal development of the stapes, one of the three small bones in the middle ear. In this condition, the stapes becomes fixed in place by an abnormally placed piece of connective tissue. Hearing loss associated with this condition typically begins between ages 8 and 24.[1][2] Depending on the underlying cause of progressive deafness and stapes fixations, there may be other associated signs and symptoms. 
Last updated: 1/30/2017

What causes progressive deafness with stapes fixation?

The exact cause of progressive deafness with stapes fixation depends on whether it is associated with an underlying condition or infection. This condition may be associated with a number of conditions, including otosclerosis, 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).[1][2] Isolated cases may be inherited. Autosomal dominant, autosomal recessive, and X-linked cases have been reported.[1][4][6] In some cases, no underlying cause can be identified.  
Last updated: 1/30/2017

How might progressive deafness with stapes fixation be treated?

Treatment for progressive deafness with stapes fixation be treated 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 prosthesis most commonly achieves satisfactory results with minimal complications.[4][7]
Last updated: 1/30/2017

What is the long term outlook for individuals with progressive deafness 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: 1/30/2017

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

Please see our Disclaimer.

  1. Lurie IW. Deafness, Progressive, with Stapes Fixation. Online Mendelian Inheritance in Man (OMIM). 1996;
  2. Bozorg Grayeli A. Progressive deafness with stapes fixation. Orphanet. 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3235.
  3. Otosclerosis. National Institute on Deafness and Other Communication Disorders (NIDCD). September 2013; https://www.nidcd.nih.gov/health/otosclerosis.
  4. Albert S, Roger Gm Rouillon I, Chauvin P, Denoyelle F, Derbez R, Delattre J, Triglia JM, Garabedian EN. Congenital stapes ankylosis: study of 28 cases and surgical results.. Laryngoscope. 2006; 116(7):1153-1157. http://www.ncbi.nlm.nih.gov/pubmed/16826051.
  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.
  6. Zhang G, Sun L, Wang X, Wang H, Zhang B, Jin H, Wang H. [Clinical study of a single congenital stapes malformation with possible X-linked dominant inheritance]. Lin Chuang Er Bi Yan Hou Ke Za Zhi. August 2005; 19(16):745-747. http://www.ncbi.nlm.nih.gov/pubmed/16320716.
  7. Salahuddin I, Salahuddin A. Experience with stapedectomy in a developing country: a review of 200 cases. Ear Nose Throat. August 2002; 81(8):551-552. http://www.ncbi.nlm.nih.gov/pubmed/12199172.