Waardenburg syndrome type 3

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Waardenburg syndrome type 3

ORPHA:896Clinical subtype

Also called Klein-Waardenburg syndrome · WS3 · Waardenburg syndrome type III · Waardenburg syndrome with limb anomalies

What it is

A very rare subtype of Waardenburg syndrome (WS) that is characterized by limb anomalies in association with congenital hearing loss, minor defects in structures arising from neural crest, resulting in pigmentation anomalies of eyes, hair, and skin.

Key facts

Age of onset
Neonatal
Inheritance
Autosomal dominant, Autosomal recessive
Classified as
Clinical subtype

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (at birth, Europe)Waardenburg syndrome

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

PAX3Disease-causing germline mutation(s)

ICD-10 codes

E70.3filed under a broader ICD-10 category — shared with 35 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 5523MONDO 0007862OMIM 148820UMLS C0079661

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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