Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome

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ORPHA:436174OMIM:616007E88.8
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Overview

Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome is an extremely rare multi-system genetic disorder that affects several parts of the body at the same time. As the name describes, people with this condition experience a combination of eye problems (cataracts, which are clouding of the lens in the eye), short stature due to a lack of growth hormone, nerve damage that affects sensation in the hands and feet (sensory neuropathy), hearing loss caused by inner ear or nerve problems (sensorineural hearing loss), and abnormal bone development (skeletal dysplasia). Because so many body systems are involved, the condition can significantly impact a person's daily life from an early age. Children may be noticeably shorter than their peers, have difficulty seeing or hearing, and may experience numbness or tingling in their limbs. The treatment approach is mainly supportive and focuses on managing each symptom individually. Growth hormone replacement therapy may help with short stature, surgery can address cataracts, hearing aids or cochlear implants may improve hearing, and physical therapy or pain management can help with neuropathy symptoms. Because this syndrome is so rare, there is limited published research, and management is typically coordinated by a team of specialists.

Also known as:

Key symptoms:

Clouding of the eye lens (cataracts)Short stature or slow growthHearing lossNumbness or tingling in hands and feetReduced ability to feel pain or temperatureAbnormal bone developmentJoint problems or stiffnessDelayed pubertyVision problemsDifficulty with balanceMuscle weakness in the limbsBone deformities

Clinical phenotype terms (24)— hover any for plain English
Prelingual sensorineural hearing impairmentHP:0000399Progressive sensorineural hearing impairmentHP:0000408AchalasiaHP:0002571Spondyloepiphyseal dysplasiaHP:0002655Fasting hypoglycemiaHP:0003162Spinal canal stenosisHP:0003416Thoracic kyphoscoliosisHP:0005659
Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome.

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No actively recruiting trials found for Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome at this time.

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No specialists are currently listed for Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia 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 Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome.

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Community

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Latest news about Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome

1 articles
Clinical trialUNITERAREApr 3, 2026
New Recruiting Trial: Phase 3 Study of LUM-201 in Children With Growth Hormone Deficiency
Lumos Pharma is testing a new drug called LUM-201 in children who don't produce enough growth hormone naturally. This Phase 3 trial is the final stage of testin
See all news about Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific tests do we need to confirm this diagnosis?,Should we pursue genetic testing, and what type is recommended?,Is growth hormone therapy appropriate, and when should it be started?,How often should my child's vision and hearing be checked?,What can we do to protect against injuries caused by sensory neuropathy?,Are there any clinical trials or research studies we could participate in?,What specialists should be part of our care team, and how often should we see them?

Common questions about Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome

What is Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome?

Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome is an extremely rare multi-system genetic disorder that affects several parts of the body at the same time. As the name describes, people with this condition experience a combination of eye problems (cataracts, which are clouding of the lens in the eye), short stature due to a lack of growth hormone, nerve damage that affects sensation in the hands and feet (sensory neuropathy), hearing loss caused by inner ear or nerve problems (sensorineural hearing loss), and abnormal bone developmen

How is Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome inherited?

Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome typically begin?

Typical onset of Cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome is childhood. Age of onset can vary across affected individuals.