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Deafness-oligodontia syndrome

ORPHA:3230· ICD-10 H90.3

Definition

Deafness-oligodontia syndrome is characterised by sensorineural hearing loss and oligodontia/hypodontia. It has been described in two pairs of siblings and in one isolated case. Dizziness was reported in one of the pairs of siblings. Transmission appears to be autosomal recessive.

Prevalence
<1 / 1 000 000
Age of onset
Infancy, Neonatal