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Deafness-vitiligo-achalasia syndrome

ORPHA:3239· ICD-10 Q87.8

Definition

A rare otorhinolaryngologic disease characterized by congenital deafness with profound sensorineural hearing loss, short stature, vitiligo, muscle wasting, and achalasia. There have been no further descriptions in the literature since 1971.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal