Usher syndrome type 3

ORPHA: 231183

Overview

Usher syndrome characterized by progressive hearing loss typically beginning in late childhood, variable vestibular dysfunction and onset of retinitis pigmentosa by the second decade of life

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 Usher syndrome type 3, sourced from HPO and Orphanet clinical annotations.

Abnormal vestibular functionAbnormal cochlea morphologySensorineural hearing impairmentRod-cone dystrophyAbnormal electroretinogramVisual lossScotomaNyctalopiaVestibular hyporeflexiaIris hypopigmentationDepressionAnxiety

Classification & Codes

Orphanet Code

ORPHA:231183
Usher syndrome type 3
OrphanetORPHA:231183
Treatments0 drug(s)
Symptoms on record12 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO