Living with Progressive deafness with stapes fixation
A form of hearing loss caused by a tiny middle ear bone that can no longer move freely.
Understanding Progressive deafness with stapes fixation
The stapes is one of the tiny bones in the middle ear. When it becomes fixed in place, sound can't pass well to the inner ear, so hearing is reduced. The hearing loss may be conductive or mixed. It can occur alone or alongside conditions like otosclerosis, Paget's disease, or osteogenesis imperfecta. Chronic ear infections can also lead to it. GARD lists onset in childhood.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- Hearing loss in both ears (conductive)
- A stuck stapes bone (stapes ankylosis)
- Hearing that worsens over time
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include ear, nose, and throat specialists and genetic specialists.
- 3Ask which tests can help
Your care team will decide which exams and tests can confirm the diagnosis.
Care & treatment
- Care team
- Ear, nose, and throat specialists (otolaryngologists); Genetic specialists (geneticists)
- Expert care
- Ask about multidisciplinary care centers for rare conditions
- Treatment
- GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
- Research
- Clinical trials and observational studies may be options. See the research section below.
General information, not medical advice. Your care team makes the decisions.
Everyday life
Living with a rare condition is easier with support.
- Connect with rare disease patient organizations for support
- Keep notes on symptoms, changes and questions for appointments
- Ask about patient registries that help research
- Look into financial aid and travel resources for care visits
Quick facts
The hearing loss comes from fixation of the stapes bone. GARD lists autosomal recessive inheritance, meaning a child inherits a changed copy of the gene from each parent, and notes it can also follow chronic ear infections.
Also known as: progressive hearing loss with stapes fixation; stapedo-vestibular ankylosis; thies reis syndrome; thies-reis syndrome
Patient organizations
Rare-disease groups that can help
- Global GenesRare Diseases · United States
- National Organization for Rare DisordersRare Diseases · United States
- Genetic AllianceRare Diseases · United States
- RARE FoundationRare Diseases · United States
You are more than your diagnosis
Perspectives from people navigating life with Progressive deafness with stapes fixation.
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