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Usher syndrome: Gene therapy restores hearing and balance

Researchers at Institut Pasteur successfully restored hearing and balance in a mouse model of USH1G syndrome using gene therapy, offering new hope for the treatment of hereditary deafness. The study demonstrates that inner ear defects caused by mutations in the USH1G gene can be reversed even after birth with durable efficacy.

SourceInstitut Pasteur·JournalProceedings of the National Academy of Sciences·DateSep 22, 2017

Hearing restoration and visual cues

A study found that visually stimulated cross-modal activation of auditory brain regions in deaf individuals improved speech comprehension after cochlear implantation. Auditory cortex activation developed in tandem with both auditory and visual speech following implantation, suggesting a positive effect on hearing restoration.

SourceProceedings of the National Academy of Sciences·JournalProceedings of the National Academy of Sciences·DateAug 14, 2017

When writing interferes with hearing

Researchers found that certain 'super-readers' with reorganized brain circuits perform better in tasks involving written words, while others with intact auditory-visual integration achieve more accurate results. This study highlights the crucial role of brain interactions in cochlear implant success.

SourceUniversité de Genève·JournalNature Communications·DateMar 28, 2017

The architecture of audition

A new study published in Scientific Reports found that the auditory cortex of profoundly deaf and hearing people has virtually identical neural architecture. The research raises questions about the role of experience in processing sensory information and could lead to potential new avenues for intervention in deafness.

SourceHarvard University·JournalScientific Reports·DateJul 17, 2016

Wbp2 is a novel gene implicated in deafness

Researchers discovered a direct link between Wbp2 and progressive high-frequency hearing loss in mouse models and human clinical cases. The study found that Wbp2 expression loss leads to reduced hormone receptor expression and hearing impairment.

SourceEMBO·JournalEMBO Molecular Medicine·DateFeb 8, 2016

Gene therapy restores hearing in deaf mice

Researchers at Boston Children's Hospital and Harvard Medical School used gene therapy to restore hearing in mice with genetic deafness. The treatment successfully restored the ability of sensory hair cells to respond to sound and partially restored actual hearing in deaf mice.

SourceBoston Children's Hospital·JournalScience Translational Medicine·DateJul 8, 2015

Osteoporosis diagnosis contributes to hearing loss risk

A study published in the Endocrine Society's Journal of Clinical Endocrinology & Metabolism found that people with osteoporosis are at a 1.76-fold higher risk of developing sudden sensorineural hearing loss. Researchers analyzed medical records of over 10,600 individuals with osteoporosis and found that they were more likely to be diag...

SourceThe Endocrine Society·JournalThe Journal of Clinical Endocrinology & Metabolism·DateApr 16, 2015

Can't sing? Do it more often

New research from Northwestern University suggests that singing accurately is a learned skill that can decline over time if not used. Regular practice can help improve singing skills and prevent the 'use it or lose it' effect, where adults may struggle to sing on key as they age.

SourceNorthwestern University·JournalMusic Perception An Interdisciplinary Journal·DateFeb 9, 2015

Hearing protein required to convert sound into brain signals

A specific protein, protocadherin-15 CD2, is crucial for hearing by forming tip-links that convert sound into electrical signals. The absence of this protein leads to profound deafness in mice and humans, providing a major breakthrough in understanding the auditory mechanotransduction machinery.

SourceEMBO·JournalEMBO Molecular Medicine·DateJun 17, 2014

Mice show innate ability to vocalize

Researchers found that mice do not need to learn to vocalize and can produce the same suite of ultrasonic sounds regardless of whether they are deaf or hearing. This discovery points the way to a genetic tool for studying speech and its disorders, shifting focus from learning to genetic mechanisms.

Scientists prevent development of deafness in animals engineered to have Usher syndrome

Researchers have developed a novel therapeutic approach to treat deafness and balance impairment by injecting mice with an antisense oligonucleotide that targets a specific gene mutation. The treatment, which was tested on mice engineered to model human Usher syndrome, successfully rescues hearing and cures balance problems.

Researchers develop Rx for deafness, impaired balance in mouse model of Usher syndrome

A new therapy called an antisense oligonucleotide (ASO) has been developed by researchers at LSU Health Sciences Center, which partially corrects gene and protein expression and rescues hearing and balance in a mouse model of Usher syndrome. This breakthrough suggests the potential for treating deafness and balance disorders.