Overview
Sensorineural hearing loss-early graying-essential tremor syndrome is a very rare inherited condition that affects three main parts of the body: hearing, hair color, and movement control. People with this syndrome are born with or develop hearing loss caused by damage to the inner ear or the nerve that carries sound signals to the brain — this is called sensorineural hearing loss. They also experience early graying of the hair, often starting in their teens or twenties, much earlier than most people would expect. In addition, they develop essential tremor, which means their hands, arms, or other parts of the body shake in an uncontrolled way, especially during movement or when trying to hold a position. This condition is also sometimes referred to by its three main features together. It is thought to be inherited in an autosomal dominant pattern, meaning one changed copy of the responsible gene is enough to cause the syndrome. The exact gene responsible has not been fully confirmed in all reported cases, which makes diagnosis challenging. There is currently no cure for this syndrome. Treatment focuses on managing each symptom separately. Hearing aids or cochlear implants can help with hearing loss. Medications such as propranolol or primidone, which are commonly used for essential tremor, may help reduce shaking. Hair graying does not require medical treatment but can be addressed cosmetically. Regular follow-up with specialists is important to monitor how symptoms change over time.
Key symptoms:
Hearing loss caused by inner ear or nerve damage (sensorineural hearing loss)Early graying of hair, often starting in the teens or twentiesUncontrolled shaking or trembling of the hands or arms (essential tremor)Tremor that gets worse during movement or when holding objectsDifficulty hearing in noisy environmentsPossible balance difficulties related to inner ear involvementProgressive worsening of tremor over time
Clinical phenotype terms (5)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Sensorineural hearing loss-early graying-essential tremor syndrome.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Sensorineural hearing loss-early graying-essential tremor syndrome at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Sensorineural hearing loss-early graying-essential tremor syndrome.
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 Sensorineural hearing loss-early graying-essential tremor syndrome.
Community
No community posts yet. Be the first to share your experience with Sensorineural hearing loss-early graying-essential tremor syndrome.
Start the conversation →Latest news about Sensorineural hearing loss-early graying-essential tremor syndrome
No recent news articles for Sensorineural hearing loss-early graying-essential tremor syndrome.
Follow this condition to be notified when news becomes available.
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.Which gene or genes might be causing this syndrome in my family, and should I have genetic testing?,How quickly might my hearing loss or tremor get worse over time?,What hearing devices or treatments are best for my level of hearing loss?,Are there medications that can help my tremor, and what are the side effects?,Should other family members be tested or evaluated for this condition?,Are there any clinical trials or research studies I could join?,What specialists should I see regularly, and how often?
Common questions about Sensorineural hearing loss-early graying-essential tremor syndrome
What is Sensorineural hearing loss-early graying-essential tremor syndrome?
Sensorineural hearing loss-early graying-essential tremor syndrome is a very rare inherited condition that affects three main parts of the body: hearing, hair color, and movement control. People with this syndrome are born with or develop hearing loss caused by damage to the inner ear or the nerve that carries sound signals to the brain — this is called sensorineural hearing loss. They also experience early graying of the hair, often starting in their teens or twenties, much earlier than most people would expect. In addition, they develop essential tremor, which means their hands, arms, or oth
How is Sensorineural hearing loss-early graying-essential tremor syndrome inherited?
Sensorineural hearing loss-early graying-essential tremor syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.