Overview
Sinoatrial node dysfunction and deafness is an extremely rare genetic condition that affects both the heart and hearing. The sinoatrial (SA) node is the heart's natural pacemaker — a small cluster of cells that sends electrical signals telling the heart when to beat. In this condition, the SA node does not work properly, which can cause the heart to beat too slowly (bradycardia), have irregular rhythms, or pause between beats. At the same time, affected individuals experience sensorineural hearing loss, meaning the inner ear or the nerve pathways from the inner ear to the brain are damaged, leading to partial or complete deafness. Symptoms related to the heart may include fainting, dizziness, fatigue, and exercise intolerance. The hearing loss can range from moderate to severe and may be present from birth or develop in early childhood. Because the condition involves both the heart and the ears, patients typically need care from multiple specialists. Treatment focuses on managing symptoms. For the heart rhythm problems, a pacemaker may be needed to keep the heart beating at a normal rate. Hearing aids or cochlear implants can help with hearing loss. There is currently no cure that addresses the underlying genetic cause of this condition. Early diagnosis is important so that appropriate heart monitoring and hearing support can be started as soon as possible.
Also known as:
Key symptoms:
Slow heart rate (bradycardia)Irregular heartbeatFainting or near-fainting episodesDizziness or lightheadednessHearing loss or deafnessFatigue and low energyDifficulty exercising or keeping up with physical activityHeart pauses or skipped beatsShortness of breathPoor tolerance of physical exertion
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Sinoatrial node dysfunction and deafness.
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Specialists
View all specialists →No specialists are currently listed for Sinoatrial node dysfunction and deafness.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Sinoatrial node dysfunction and deafness.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is my child's (or my) sinoatrial node dysfunction, and will a pacemaker be needed?,What type and degree of hearing loss is present, and what are the best options for hearing support?,How often should heart rhythm monitoring and hearing tests be done?,Are there any physical activities or sports that should be avoided?,Should other family members be tested for this genetic condition?,What are the signs that the condition is getting worse and when should I seek emergency care?,Are there any clinical trials or new treatments being studied for this condition?
Common questions about Sinoatrial node dysfunction and deafness
What is Sinoatrial node dysfunction and deafness?
Sinoatrial node dysfunction and deafness is an extremely rare genetic condition that affects both the heart and hearing. The sinoatrial (SA) node is the heart's natural pacemaker — a small cluster of cells that sends electrical signals telling the heart when to beat. In this condition, the SA node does not work properly, which can cause the heart to beat too slowly (bradycardia), have irregular rhythms, or pause between beats. At the same time, affected individuals experience sensorineural hearing loss, meaning the inner ear or the nerve pathways from the inner ear to the brain are damaged, le
How is Sinoatrial node dysfunction and deafness inherited?
Sinoatrial node dysfunction and deafness follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Sinoatrial node dysfunction and deafness typically begin?
Typical onset of Sinoatrial node dysfunction and deafness is childhood. Age of onset can vary across affected individuals.