Progress in deciphering the genetic architecture of human sensorineural hearing
impairment (SNHI) or loss, and multidisciplinary studies of mouse models, have led to the …

Usher syndrome (USH) is the most common cause of deaf–blindness in humans, with a
prevalence of about 1/10,000 (~ 400,000 people worldwide). Cochlear implants are …

Mutations in Atp2b2, an outer hair cell gene, cause dominant hearing loss in humans. Using
a mouse model Atp2b2 Obl/+, with a dominant hearing loss mutation (Oblivion), we show …

Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe,
efficient, and clinically relevant delivery modalities,. Here we demonstrate the safety and …

Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital
deafness. Adeno-associated virus (AAV)-based gene therapy is a promising therapeutic …

Because there are currently no biological treatments for hearing loss, we sought to advance
gene therapy approaches to treat genetic deafness. We focused on Usher syndrome, a …

Hearing impairment is the most frequent sensory deficit in humans of all age groups, from
children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital …

Hereditary hearing loss often results from mutation of genes expressed by cochlear hair
cells. Gene addition using AAV vectors has shown some efficacy in mouse models, but …

The use of viral vectors for inner ear gene therapy is receiving increased attention for
treatment of genetic hearing disorders. Most animal studies to date have injected viral …

Dizziness and hearing loss are among the most common disabilities. Many forms of
hereditary balance and hearing disorders are caused by abnormal development of …