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progressive deafness with stapes fixation - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

progressive deafness with stapes fixation

MONDO:0011080

Also known as:Stapedo-vestibular ankylosisThies-Reis syndromedeafness, progressive, with stapes fixation

Overview

Stapes fixation (stapedovestibular ankylosis) is a hearing loss condition that appears as a consequence of annular ligament destruction followed by excessive connective tissue production during the healing process. This condition is mainly observed in otosclerosis, but is also found in chronic otitis media with tympanosclerosis, and other rare bone diseases such as Paget's disease and osteogenesis imperfecta (Lobstein disease).

GeneticsAutosomal recessive

ResearchLimited published research (5 articles)

Clinical Presentation

Features include: Stapes ankylosis and Bilateral conductive hearing impairment.

Organ System	Phenotype Count	Example Features
Ears	1	Bilateral conductive hearing impairment

Explore clinical features

Diagnosis

Biomarker and diagnostic research for progressive deafness with stapes fixation has been reported in the published literature.

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for progressive deafness with stapes fixation.

5 publications have been identified in PubMed for progressive deafness with stapes fixation. Research spans Review / Meta-Analysis (60%), Diagnostic / Biomarker (20%), and Case Report / Case Series (20%).

Recent Publications

De Vito A (2025). [PMID: 40534442](https://pubmed.ncbi.nlm.nih.gov/40534442/). *Acta Otorhinolaryngol Ital*. [Review / Meta-Analysis]
Forli F (2025). [PMID: 40534441](https://pubmed.ncbi.nlm.nih.gov/40534441/). *Acta Otorhinolaryngol Ital*. [Review / Meta-Analysis]
Sharaf K (2025). [PMID: 39589410](https://pubmed.ncbi.nlm.nih.gov/39589410/). *Eur Arch Otorhinolaryngol*. [Case Report / Case Series]
Bouatay R (2024). [PMID: 38530462](https://pubmed.ncbi.nlm.nih.gov/38530462/). *Eur Arch Otorhinolaryngol*. [Diagnostic / Biomarker]
Rajan D (2024). [PMID: 39649726](https://pubmed.ncbi.nlm.nih.gov/39649726/). *Laryngoscope Investig Otolaryngol*. [Review / Meta-Analysis]

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 2:55 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:601449

Orphanet

European rare disease database

Orphanet:3235

GARD

Genetic and Rare Diseases Info Center

GARD:5170

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
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progressive deafness with stapes fixation

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team