Disease Directory Usher Syndrome
Ophthalmological

Usher Syndrome

Also known as: Usher syndrome type 1/2/3, deaf-blindness, USH

Prevalence

4–17 per 100,000

Onset

Congenital (hearing loss); childhood to adolescence (vision loss)

Type

Autosomal recessive

Gene

MYO7A, USH2A, CLRN1 (multiple)

About Usher Syndrome

Usher Syndrome is the most common cause of combined deaf-blindness, characterised by sensorineural hearing loss present at birth or in early childhood and progressive retinitis pigmentosa that typically begins in adolescence. Three clinical types are distinguished by the degree and onset of hearing loss, the presence of vestibular dysfunction, and the age of visual symptom onset, with Type 1 being most severe. Mutations in at least 10 genes encoding proteins of the Usher protein complex, which maintains the structural integrity of photoreceptors and cochlear hair cells, are responsible.

Common Clinical Features

Congenital or early-onset sensorineural hearing loss Progressive night blindness beginning in adolescence Tunnel vision due to peripheral field constriction Vestibular dysfunction and balance problems (Type 1) Bone-spicule pigmentation on retinal examination Reduced ERG responses Photophobia Late central vision loss leading to legal blindness

Clinical Trial Eligibility Tips

What to know before applying to Usher Syndrome trials.

Specify your Usher type and causative gene in trial applications; USH2A-targeted therapies and MYO7A gene therapy trials have distinct eligibility criteria based on subtype.

Audiological records and vestibular assessment reports are often required alongside ophthalmological data; compile a complete multidisciplinary file before screening.

Cochlear implant history does not typically exclude patients from retinal-focused trials, but always disclose device implants during the screening interview.

Patient Resources

Patient Organization

Usher Syndrome Coalition

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Natural History Registry

Usher Syndrome Registry

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Orphanet

European reference resource for rare diseases (ORPHA:886)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Usher Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Usher Syndrome. Updated daily.

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