Usher Syndrome News and Research

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Usher syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom. The major symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. The retina is a light-sensitive tissue at the back of the eye and is crucial for vision. As RP progresses, the field of vision narrows—a condition known as “tunnel vision”—until only central vision (the ability to see straight ahead) remains. Many people with Usher syndrome also have severe balance problems.

There are three clinical types of Usher syndrome: type 1, type 2, and type 3. In the United States, types 1 and 2 are the most common types. Together, they account for approximately 90 to 95 percent of all cases of children who have Usher syndrome.
CIB2 gene responsible for progression and severity of Usher syndrome

CIB2 gene responsible for progression and severity of Usher syndrome

CIB2 genetic mutation responsible for deafness associated with Usher syndrome type 1

CIB2 genetic mutation responsible for deafness associated with Usher syndrome type 1

Planarian flatworms now a model system for studying eye development and eye diseases

Planarian flatworms now a model system for studying eye development and eye diseases

Foundation Fighting Blindness grants US$125,000 to Oxford BioMedica for UshStat Phase I/IIa trial

Foundation Fighting Blindness grants US$125,000 to Oxford BioMedica for UshStat Phase I/IIa trial

Study details development of first mouse model for Usher syndrome III

Study details development of first mouse model for Usher syndrome III

Optogenetic Therapies for Vision workshop to be held on June 1

Optogenetic Therapies for Vision workshop to be held on June 1

Two scientists receive The Brain Prize 2012 for pioneering work on hearing and deafness

Two scientists receive The Brain Prize 2012 for pioneering work on hearing and deafness

Mayo Clinic, Oxford BioMedica collaborate to develop gene therapy for glaucoma

Mayo Clinic, Oxford BioMedica collaborate to develop gene therapy for glaucoma

FDA approves Oxford BioMedica's UshStat IND to treat Usher syndrome type 1B

FDA approves Oxford BioMedica's UshStat IND to treat Usher syndrome type 1B

AGTC receives FFB grant to evaluate gene therapy treatment for X-linked Retinoschisis

AGTC receives FFB grant to evaluate gene therapy treatment for X-linked Retinoschisis

New role for harmonin protein in Usher syndrome

New role for harmonin protein in Usher syndrome

Small molecule treatment holds promise for Usher syndrome

Small molecule treatment holds promise for Usher syndrome

Oxford BioMedica reports RetinoStat progress in Phase I study against wet AMD

Oxford BioMedica reports RetinoStat progress in Phase I study against wet AMD

Mary Ann Liebert's peer-reviewed journal highlights latest developments in ocular gene therapy

Mary Ann Liebert's peer-reviewed journal highlights latest developments in ocular gene therapy

OtoSCOPE method offers single-run genetic testing for hearing loss

OtoSCOPE method offers single-run genetic testing for hearing loss

Dr. Hastings receives grant for Spinal Muscular Atrophy research

Dr. Hastings receives grant for Spinal Muscular Atrophy research

Research on genetic mutations could lead to accurate diagnosis of USH, says scientist

Research on genetic mutations could lead to accurate diagnosis of USH, says scientist

Acucela to present data on ACU-4429 oral visual cycle modulator for dry AMD at Retina International meeting

Acucela to present data on ACU-4429 oral visual cycle modulator for dry AMD at Retina International meeting

NYU professor conducts genetic screening at academic campuses in Boston

NYU professor conducts genetic screening at academic campuses in Boston

Gene therapy may help correct molecular flaws that cause brain disease

Gene therapy may help correct molecular flaws that cause brain disease