Overview
Cochleovestibular malformation is a rare condition where the inner ear does not develop normally before birth. The inner ear has two main parts: the cochlea, which helps you hear, and the vestibular system, which helps you balance. In this condition, one or both of these structures are formed incorrectly, which can affect hearing, balance, or both. The condition is also sometimes called inner ear dysplasia or inner ear developmental anomaly, and it falls under the broader group of inner ear malformations. People with cochleovestibular malformation most often experience hearing loss, which can range from mild to profound. Some people are born completely deaf in one or both ears. Balance problems are also common, and some children may be slower to sit up, stand, or walk because their balance system is not working properly. The severity of symptoms varies widely from person to person. Treatment focuses on managing hearing loss and supporting development. Hearing aids can help some people, while others may benefit from a cochlear implant, a small electronic device placed in the ear to restore a sense of sound. Early speech therapy and educational support are very important. There is no cure for the underlying structural problem, but with the right support, many people with this condition can lead full and meaningful lives.
Key symptoms:
Hearing loss in one or both ears, ranging from mild to complete deafnessBalance problems or difficulty with coordinationDelayed sitting, standing, or walking in infants and young childrenDizziness or a spinning sensation (vertigo)Ringing or buzzing sounds in the ears (tinnitus)Difficulty understanding speech, especially in noisy environmentsDelayed speech and language development in children
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Cochleovestibular malformation.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Cochleovestibular malformation at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Cochleovestibular malformation.
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 Cochleovestibular malformation.
Community
No community posts yet. Be the first to share your experience with Cochleovestibular malformation.
Start the conversation →Latest news about Cochleovestibular malformation
No recent news articles for Cochleovestibular malformation.
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.What specific type of inner ear malformation does my child have, and how does that affect treatment options?,Is my child a good candidate for a cochlear implant, and what results can we realistically expect?,Should we do genetic testing, and which genes should be tested?,Is there an increased risk of meningitis, and should we get additional vaccinations?,What therapies should we start right away to support speech and balance development?,Are other family members at risk, and should they be tested?,What educational supports should we put in place, and who can help us navigate that process?
Common questions about Cochleovestibular malformation
What is Cochleovestibular malformation?
Cochleovestibular malformation is a rare condition where the inner ear does not develop normally before birth. The inner ear has two main parts: the cochlea, which helps you hear, and the vestibular system, which helps you balance. In this condition, one or both of these structures are formed incorrectly, which can affect hearing, balance, or both. The condition is also sometimes called inner ear dysplasia or inner ear developmental anomaly, and it falls under the broader group of inner ear malformations. People with cochleovestibular malformation most often experience hearing loss, which can
At what age does Cochleovestibular malformation typically begin?
Typical onset of Cochleovestibular malformation is neonatal. Age of onset can vary across affected individuals.