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Синдром Ушера (Ашера)

ORPHA:886· ICD-10 H35.5· Usher syndrome

Визначення(English summary)

A rare ciliopathy characterized by congenital or childhood onset sensorineural hearing loss (HL) and retinitis pigmentosa (RP) that occurs in a second step with a night blindness and a progressive vision loss and, in some cases, vestibular dysfunction.

Поширеність
1-9 / 100 000
Успадкування
Autosomal recessive
Вік початку
Childhood, Infancy, Neonatal