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.
Researchers use modified AAV as ‘Trojan Horse’ for delivering genes to restore congenital deafness

Researchers use modified AAV as ‘Trojan Horse’ for delivering genes to restore congenital deafness

Inner ear gene therapy holds promise for treating patients with hearing loss and dizziness

Inner ear gene therapy holds promise for treating patients with hearing loss and dizziness

Researcher paints pitiless picture of life for people with acquired deaf-blindness

Researcher paints pitiless picture of life for people with acquired deaf-blindness

New glasses may help expand sight of person with limited peripheral vision

New glasses may help expand sight of person with limited peripheral vision

First small molecule targeted therapy holds promise for Usher syndrome

First small molecule targeted therapy holds promise for Usher syndrome

RPB grants $500,000 to accelerate treatments for Retinitis Pigmentosa

RPB grants $500,000 to accelerate treatments for Retinitis Pigmentosa

Study provides better understanding of intestinal pathologies associated with inherited, infectious diseases

Study provides better understanding of intestinal pathologies associated with inherited, infectious diseases

Researchers investigate how gene regulation affects evolution and development

Researchers investigate how gene regulation affects evolution and development

E-Rare funding recommended for European young investigators network for Usher syndrome

E-Rare funding recommended for European young investigators network for Usher syndrome

Scientists evaluate two different strategies to treat Usher syndrome

Scientists evaluate two different strategies to treat 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