Congenital hereditary facial paralysis-variable hearing loss syndrome

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ORPHA:306530OMIM:604185Q87.0
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Overview

Congenital hereditary facial paralysis with variable hearing loss syndrome is a very rare genetic condition that is present from birth. In this syndrome, the nerves that control the muscles of the face (called the facial nerves) do not develop or function properly, leading to paralysis or weakness of the facial muscles on one or both sides of the face. This means that affected individuals may have difficulty with facial expressions, closing their eyes, smiling, or moving their lips. In addition to facial paralysis, many people with this condition also experience some degree of hearing loss, which can range from mild to severe and may affect one or both ears. This condition is sometimes grouped under the broader category of Moebius syndrome-like disorders or hereditary congenital facial paresis syndromes. The facial paralysis is typically noticed at birth or very early in infancy when a baby does not move their face normally, especially when crying. The hearing loss may be identified later through newborn hearing screening or during early childhood. There is currently no cure for this condition. Treatment focuses on managing symptoms and improving quality of life. This may include speech therapy, physical therapy for facial muscles, hearing aids or cochlear implants for hearing loss, and sometimes surgical procedures to help with eye closure or improve facial movement. Early intervention with hearing support and developmental therapies can make a significant difference in a child's communication and social development.

Also known as:

Key symptoms:

Inability to move facial muscles on one or both sides of the faceDifficulty closing the eyes completelyInability to smile or make facial expressionsHearing loss that can range from mild to severeDifficulty with feeding or sucking in infancyDrooling due to poor lip controlSpeech difficultiesDry eyes due to incomplete eye closureFlat or mask-like facial appearanceDifficulty with chewing

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Congenital hereditary facial paralysis-variable hearing loss syndrome.

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No actively recruiting trials found for Congenital hereditary facial paralysis-variable hearing loss syndrome at this time.

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No specialists are currently listed for Congenital hereditary facial paralysis-variable hearing loss syndrome.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Congenital hereditary facial paralysis-variable hearing loss syndrome.

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Questions for your doctor

Bring these to your next appointment

  • Q1.Is the facial paralysis affecting one side or both sides of my child's face?,What type and degree of hearing loss does my child have, and what are the best options for hearing support?,Should we pursue genetic testing, and what would the results mean for our family?,What therapies should we start right away to support my child's development?,Are there surgical options to improve facial movement, and when would those be considered?,How often should my child's hearing and eye health be monitored?,What resources or support groups are available for families with this condition?

Common questions about Congenital hereditary facial paralysis-variable hearing loss syndrome

What is Congenital hereditary facial paralysis-variable hearing loss syndrome?

Congenital hereditary facial paralysis with variable hearing loss syndrome is a very rare genetic condition that is present from birth. In this syndrome, the nerves that control the muscles of the face (called the facial nerves) do not develop or function properly, leading to paralysis or weakness of the facial muscles on one or both sides of the face. This means that affected individuals may have difficulty with facial expressions, closing their eyes, smiling, or moving their lips. In addition to facial paralysis, many people with this condition also experience some degree of hearing loss, wh

How is Congenital hereditary facial paralysis-variable hearing loss syndrome inherited?

Congenital hereditary facial paralysis-variable hearing loss syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Congenital hereditary facial paralysis-variable hearing loss syndrome typically begin?

Typical onset of Congenital hereditary facial paralysis-variable hearing loss syndrome is neonatal. Age of onset can vary across affected individuals.