Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome

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ORPHA:3038OMIM:182875Q87.0
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8Treatment centers

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Overview

Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome (also known as Simosa syndrome or Simosa-Penchaszadeh-Bustos syndrome) is an extremely rare genetic condition that affects several parts of the body. It was first described in a small number of patients and is characterized by a combination of distinctive features present from birth or early childhood. The main features include delayed speech development, asymmetry of the face (where one side of the face looks slightly different from the other), strabismus (crossed eyes or eyes that do not align properly), and unusual creases or folds on the ear lobes. Some affected individuals may also have mild intellectual challenges and other minor physical differences. Because this syndrome is so rare, there is limited information about its full range of symptoms and long-term outcomes. There is currently no specific cure for this condition. Treatment focuses on managing individual symptoms, such as speech therapy for language delays, corrective measures for eye alignment problems, and supportive therapies to help with development. A team of specialists typically works together to address the various needs of affected individuals. Early intervention, particularly for speech and developmental delays, is considered important for achieving the best possible outcomes.

Also known as:

Key symptoms:

Delayed speech and language developmentFacial asymmetry (one side of the face looks different from the other)Crossed eyes or misaligned eyes (strabismus)Creases or folds on the ear lobesShort stature or growth delaysMild intellectual disability or learning difficultiesLow-set or unusually shaped earsBroad or flat nasal bridgeMild developmental delaysPossible hearing difficulties

Clinical phenotype terms (14)— hover any for plain English
Unilateral narrow palpebral fissureHP:0007946Anterior creases of earlobeHP:0009908
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome.

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No actively recruiting trials found for Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome at this time.

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No specialists are currently listed for Delayed speech-facial asymmetry-strabismus-ear lobe creases 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 Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific therapies do you recommend for my child's speech delay, and how often should they occur?,Does my child's strabismus need surgical correction, or can it be managed with glasses or patching?,Should we pursue genetic testing, and what might the results tell us?,What developmental milestones should I be watching for, and when should I be concerned?,Are there any hearing tests we should do to make sure hearing loss is not contributing to the speech delay?,What educational supports or early intervention programs are available for my child?,Is there a risk that future children could also be affected, and should we see a genetic counselor?

Common questions about Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome

What is Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome?

Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome (also known as Simosa syndrome or Simosa-Penchaszadeh-Bustos syndrome) is an extremely rare genetic condition that affects several parts of the body. It was first described in a small number of patients and is characterized by a combination of distinctive features present from birth or early childhood. The main features include delayed speech development, asymmetry of the face (where one side of the face looks slightly different from the other), strabismus (crossed eyes or eyes that do not align properly), and unusual creases

How is Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome inherited?

Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome typically begin?

Typical onset of Delayed speech-facial asymmetry-strabismus-ear lobe creases syndrome is neonatal. Age of onset can vary across affected individuals.