MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome

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ORPHA:397744OMIM:614369G60.0
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Overview

MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome is a rare genetic condition caused by changes (mutations) in the MYH14 gene. This gene provides instructions for making a protein called nonmuscle myosin heavy chain IIC, which plays an important role in how cells move and maintain their structure. When this gene is not working properly, it can affect several parts of the body at once. The syndrome is characterized by a combination of four main problems: peripheral neuropathy (damage to the nerves in the hands and feet causing numbness, tingling, or weakness), myopathy (muscle weakness), hoarseness of the voice, and hearing loss that typically worsens over time. The hearing loss is usually sensorineural, meaning it involves the inner ear or the nerve pathways to the brain. Symptoms often begin in adulthood, though the age of onset can vary between individuals and even within the same family. There is currently no cure for this condition. Treatment focuses on managing each symptom individually. Hearing aids or cochlear implants may help with hearing loss. Physical therapy and occupational therapy can help maintain muscle strength and function. Speech therapy may be recommended for voice problems. Regular monitoring by a team of specialists is important to track how the condition progresses and adjust treatments as needed.

Also known as:

Key symptoms:

Hearing loss that gets worse over timeHoarse or raspy voiceNumbness or tingling in hands and feetMuscle weakness, especially in the legsDifficulty walking or balance problemsReduced reflexesMuscle wasting in the lower legsFoot drop (difficulty lifting the front of the foot)FatigueDifficulty with fine motor tasks in the handsHigh-arched feet or foot deformitiesTrouble speaking clearly

Clinical phenotype terms (21)— hover any for plain English
Bilateral sensorineural hearing impairmentHP:0008619Progressive distal muscle weaknessHP:0009063Structural foot deformityHP:0010219Fatty replacement of skeletal muscleHP:0012548Mitochondrial swellingHP:0030774Vocal cord paralysisHP:0001605
Inheritance

Autosomal dominant

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

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome.

View clinical trials →

No actively recruiting trials found for MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome at this time.

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No specialists are currently listed for MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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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 quickly is this condition likely to progress in my case?,What type of hearing aids or devices would be most helpful for my hearing loss?,Should my family members be tested for the MYH14 gene change?,What physical therapy exercises are best for maintaining my muscle strength?,Are there any clinical trials or new treatments being studied for this condition?,How often should I have my hearing and nerve function checked?,What can be done to help with my voice hoarseness?

Common questions about MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome

What is MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome?

MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome is a rare genetic condition caused by changes (mutations) in the MYH14 gene. This gene provides instructions for making a protein called nonmuscle myosin heavy chain IIC, which plays an important role in how cells move and maintain their structure. When this gene is not working properly, it can affect several parts of the body at once. The syndrome is characterized by a combination of four main problems: peripheral neuropathy (damage to the nerves in the hands and feet causing numbness, tingling, or weakness), myopa

How is MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome inherited?

MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome typically begin?

Typical onset of MYH14-related peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome is adult. Age of onset can vary across affected individuals.