cataract-ataxia-deafness syndrome

MeSH: C538283ORPHA: 1368

Overview

Cataract-ataxia-deafness syndrome is characterised by mild intellectual deficit, congenital cataract, progressive sensorineural deafness and ataxia. It has been described in two sisters. The inheritance is likely to be autosomal recessive.

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with cataract-ataxia-deafness syndrome, sourced from HPO and Orphanet clinical annotations.

Sensorineural hearing impairmentVisual impairmentDevelopmental cataractNystagmusDecreased nerve conduction velocityAtaxiaMild intellectual disabilityHypertoniaAreflexiaTremorShort statureAdult onset sensorineural hearing impairmentPeripheral neuropathy

Classification & Codes

MeSH Code

C538283

Orphanet Code

ORPHA:1368
cataract-ataxia-deafness syndrome
MeSHC538283
OrphanetORPHA:1368
Treatments0 drug(s)
Symptoms on record13 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO