Renal caliceal diverticuli-deafness syndrome

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Overview

Renal caliceal diverticuli-deafness syndrome is an extremely rare genetic condition that combines kidney abnormalities with hearing loss. The kidney problem involves caliceal diverticula, which are small pouch-like outgrowths from the calyces — the cup-shaped structures inside the kidney that collect urine before it flows into the ureter. These pouches can trap urine and sometimes lead to kidney stones, urinary tract infections, or other complications. The hearing loss in this syndrome is sensorineural, meaning it results from problems in the inner ear or the nerve pathways that carry sound signals to the brain. This type of hearing loss can range from mild to severe and may affect one or both ears. Because this syndrome is so rare, very few cases have been described in the medical literature. The exact genetic cause has not been fully identified, and treatment is mainly focused on managing symptoms. Hearing aids or cochlear implants may help with hearing loss, while kidney complications such as stones or infections are treated as they arise. Monitoring kidney function over time is important. Patients benefit from a team of specialists including kidney doctors and hearing specialists who work together to provide coordinated care. Research into this condition is limited, and much remains to be learned about its long-term outlook and underlying genetic mechanisms.

Also known as:

Key symptoms:

Hearing loss present from childhoodSmall pouches in the kidney collecting system (caliceal diverticula)Kidney stonesRecurrent urinary tract infectionsFlank or back pain related to kidney problemsBlood in the urineDifficulty hearing conversations, especially in noisy settings

Clinical phenotype terms (6)— hover any for plain English
HydroureterHP:0000072Abnormality of the upper urinary tractHP:0010935
Inheritance

Autosomal dominant

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Renal caliceal diverticuli-deafness syndrome.

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No actively recruiting trials found for Renal caliceal diverticuli-deafness syndrome at this time.

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No specialists are currently listed for Renal caliceal diverticuli-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 Renal caliceal diverticuli-deafness syndrome.

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Community

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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

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is the hearing loss, and what type of hearing device would be most helpful?,How often should kidney imaging and hearing tests be repeated?,What steps can we take to prevent kidney stones?,Are there any signs or symptoms we should watch for that would need urgent medical attention?,Should other family members be tested for this condition?,Is genetic testing available that might help identify the cause in our family?,Are there any clinical trials or research studies we could participate in?

Common questions about Renal caliceal diverticuli-deafness syndrome

What is Renal caliceal diverticuli-deafness syndrome?

Renal caliceal diverticuli-deafness syndrome is an extremely rare genetic condition that combines kidney abnormalities with hearing loss. The kidney problem involves caliceal diverticula, which are small pouch-like outgrowths from the calyces — the cup-shaped structures inside the kidney that collect urine before it flows into the ureter. These pouches can trap urine and sometimes lead to kidney stones, urinary tract infections, or other complications. The hearing loss in this syndrome is sensorineural, meaning it results from problems in the inner ear or the nerve pathways that carry sound si

How is Renal caliceal diverticuli-deafness syndrome inherited?

Renal caliceal diverticuli-deafness syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Renal caliceal diverticuli-deafness syndrome typically begin?

Typical onset of Renal caliceal diverticuli-deafness syndrome is childhood. Age of onset can vary across affected individuals.