Hypospadias-hypertelorism-coloboma and deafness syndrome

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Overview

Hypospadias-hypertelorism-coloboma and deafness syndrome is an extremely rare genetic condition that affects multiple parts of the body at the same time. The name describes its main features: hypospadias (where the opening of the urethra is on the underside of the penis rather than at the tip), hypertelorism (eyes that are spaced wider apart than usual), coloboma (a gap or notch in part of the eye structure, which can affect vision), and sensorineural deafness (hearing loss caused by problems in the inner ear or hearing nerve). Because this syndrome involves several organ systems, children born with it may face challenges related to their urinary tract, vision, hearing, and sometimes other developmental areas. This condition is present from birth, and the features are usually noticed in the newborn period or early infancy. Some affected individuals may also have additional findings such as facial differences, developmental delays, or other birth defects. Because the syndrome is so rare, there is limited information about the full range of symptoms and outcomes. There is currently no cure for this syndrome. Treatment focuses on managing each symptom individually. Surgical correction may be needed for hypospadias and possibly for eye abnormalities. Hearing aids or cochlear implants may help with hearing loss. A team of specialists working together is important to provide the best care for affected children and their families.

Also known as:

Key symptoms:

Opening of the urethra on the underside of the penis (hypospadias)Eyes spaced wider apart than normal (hypertelorism)Gap or notch in the eye structure (coloboma)Hearing loss or deafnessVision problemsUnusual facial featuresPossible developmental delaysPossible genital abnormalities beyond hypospadiasPossible kidney or urinary tract differences

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hypospadias-hypertelorism-coloboma and deafness syndrome.

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No actively recruiting trials found for Hypospadias-hypertelorism-coloboma and deafness syndrome at this time.

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No specialists are currently listed for Hypospadias-hypertelorism-coloboma and deafness 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 Hypospadias-hypertelorism-coloboma and deafness syndrome.

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Community

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Latest news about Hypospadias-hypertelorism-coloboma and deafness 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.What is the severity of my child's hearing loss, and what are the best options for hearing support?,How much does the coloboma affect my child's vision, and will it change over time?,When is the best time for hypospadias surgery, and what should we expect during recovery?,Should we pursue genetic testing, and what might the results tell us?,Are there any kidney or other organ problems we should screen for?,What early intervention services should we start right away?,Are there other families or support groups for this condition?

Common questions about Hypospadias-hypertelorism-coloboma and deafness syndrome

What is Hypospadias-hypertelorism-coloboma and deafness syndrome?

Hypospadias-hypertelorism-coloboma and deafness syndrome is an extremely rare genetic condition that affects multiple parts of the body at the same time. The name describes its main features: hypospadias (where the opening of the urethra is on the underside of the penis rather than at the tip), hypertelorism (eyes that are spaced wider apart than usual), coloboma (a gap or notch in part of the eye structure, which can affect vision), and sensorineural deafness (hearing loss caused by problems in the inner ear or hearing nerve). Because this syndrome involves several organ systems, children bor

At what age does Hypospadias-hypertelorism-coloboma and deafness syndrome typically begin?

Typical onset of Hypospadias-hypertelorism-coloboma and deafness syndrome is neonatal. Age of onset can vary across affected individuals.