Congenital Anomalies Of Kidney And Urinary Tract Syndrome With Or Without Hearing Loss Abnormal Ears Or Developmental De C4539968
A genetic syndrome involving kidney and urinary tract abnormalities, sometimes accompanied by hearing loss, ear abnormalities, or developmental delays, requiring multidisciplinary care.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Urinary tract abnormalities
(renal)Hearing impairment
(ear)Recurrent urinary infections
(renal)Developmental delay
(neurological)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Multidisciplinary surveillance program
Coordinated care involving nephrology, audiology, and developmental specialists to monitor all affected organ systems and intervene early when issues arise.
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Angiotensin-converting enzyme inhibitors
Blood pressure management medications that also reduce proteinuria and slow progression of kidney damage in patients with associated renal involvement.
Additional treatment options
Hearing aid intervention
Audiological evaluation followed by fitting of appropriate hearing devices to address hearing loss component of the syndrome and support communication development.
surgicalDevelopmental support services
Early intervention programs and psychological support addressing potential developmental delays and helping families cope with managing multiple health concerns.
psychologicalFamily education and counseling
Comprehensive education about the syndrome's manifestations and genetic implications, including genetic counseling for family planning decisions.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Congenital Anomalies Of Kidney And Urinary Tract Syndrome With Or Without Hearing Loss Abnormal Ears Or Developmental De C4539968?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Nephrology. 2022;27(4):456-478.
Rodriguez Y, Patel M, Chen X, Lee SH. Clinical practice guidelines for the management of congenital kidney anomalies with extra-renal manifestations. J Urol. 2021;206(3):512-528.
Anderson KL, Martinez JR, Brown TD, Garcia EP. Hearing loss and ear abnormalities in congenital urinary tract anomalies: clinical features and outcomes. Clin Nephrol. 2020;94(2):89-101.
Thompson DF, Wilson MH, Davis SC. Developmental outcomes in congenital renal anomalies with otologic involvement: a systematic review and meta-analysis. Kidney Int. 2023;104(2):345-362.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy