Living with Progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome
A rare genetic condition with hearing loss that worsens with age, a heart rhythm change, and mild heart muscle thickening.
Understanding Progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome
Hearing loss starts late and gets worse over time. It comes from the inner ear or hearing nerve (sensorineural). The QT interval is a part of the heartbeat pattern seen on an ECG, and in this condition it is longer than usual. GARD says it can start at any time in life.
Signs & symptoms
GARD doesn't list specific symptoms for this condition yet. The summary describes these features:
- Hearing loss that worsens over time
- A long QT interval on a heart tracing (ECG)
- Mild thickening of the heart muscle (cardiac hypertrophy)
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, genetic specialists and heart and blood vessel specialists.
- 3Consider genetic testing
Changes in MYO6 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.
- 4Connect with expert centers
Hearing Loss Association of America and Children's Cardiomyopathy Foundation may keep lists of experts for this condition.
Care & treatment
- Care team
- Ear, nose, and throat specialists (otolaryngologists); Genetic specialists (geneticists); Heart and blood vessel specialists (cardiologists)
- Expert care
- Expert lists: Hearing Loss Association of America, Children's Cardiomyopathy Foundation
- 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 Hearing Loss Association of America and other patient organizations
- 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
This condition is caused by changes in the MYO6 gene. It is autosomal dominant, meaning one changed copy of the gene from a parent is enough.
Also known as: deafness with hypertrophic cardiomyopathy, autosomal dominant, type 22; progressive neurosensory deafness-hypertrophic cardiomyopathy syndrome; progressive neurosensory hearing loss-hypertrophic cardiomyopathy syndrome; progressive sensorineural deafness and hypertrophic cardiomyopathy syndrome; progressive sensorineural deafness-hypertrophic cardiomyopathy syndrome; progressive sensorineural hearing loss and hypertrophic cardiomyopathy syndrome
Patient organizations
- Hearing Loss Association of AmericaProgressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome · United States
- Children's Cardiomyopathy FoundationCardiomyopathy · United States
You are more than your diagnosis
Perspectives from people navigating life with Progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome.
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