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Norrie disease

ORPHA:649· ICD-10 H35.5

Definition

A rare developmental defect during embryogenesis characterized by abnormal retinal development with congenital blindness. Common associated manifestations include sensorineural hearing loss and developmental delay, intellectual disability and/or behavioral disorders.

Prevalence
<1 / 1 000 000
Inheritance
X-linked recessive
Age of onset
Antenatal, Neonatal