Usher syndrome

MeSH: D052245ORPHA: 886

Overview

syndrome characterized by a combination of hearing loss and visual impairment

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, sourced from HPO and Orphanet clinical annotations.

Sensorineural hearing impairmentVisual impairmentAbnormal electroretinogramProgressive visual lossBlindnessNyctalopiaVisual field defectAbnormal vestibular functionAbnormal retinal pigmentationVestibular areflexiaCataractMyopiaAtaxiaHigh hypermetropiaTinnitusAstigmatismNystagmusCarious teethAbnormal dental enamel morphologyMicrodontiaPsychosisDepressionHallucinationsAnxietyHypertrophic cardiomyopathyCerebral cortical atrophyDelayed gross motor developmentClumsinessMyopathyEMG abnormalityAplasia/Hypoplasia of the cerebellumPeripheral visual field lossFloppy infantHyperacusisAbnormal cardiovascular system physiologyAbnormality of dental colorDelayed ability to walk

Classification & Codes

MeSH Code

D052245

Orphanet Code

ORPHA:886
Usher syndrome
MeSHD052245
OrphanetORPHA:886
Treatments0 drug(s)
Symptoms on record37 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO