Usher syndrome type 2

ORPHA: 231178

Overview

Usher syndrome characterized by mild to severe congenital hearing impairment, normal vestibular function and later development of retinitis pigmentosa

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 2, sourced from HPO and Orphanet clinical annotations.

Abnormality of the inner earSensorineural hearing impairmentRod-cone dystrophyAbnormal electroretinogramVisual lossScotomaIris hypopigmentationCataractMyopiaNyctalopiaConstriction of peripheral visual fieldSleep disturbanceReduced visual acuityPeripheral visual field lossFatigueColor vision defectDepressionAnxietyGait imbalanceReduced contrast sensitivityAbnormal vestibular function

Classification & Codes

Orphanet Code

ORPHA:231178
Usher syndrome type 2
OrphanetORPHA:231178
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO