Hope to treat deafness at birth

Mutations in the connexin 26 protein are the most common cause of hearing loss at birth, accounting for more than 25% of hereditary hearing loss worldwide. This protein plays an essential role in the functioning of hair cells in the inner ear, which are responsible for transmitting sound signals to the brain. Through their studies they are the Jones researchers Hopkins university A breakthrough in the treatment of congenital deafness.

deafness congenital: The most important mechanisms for the development of the auditory system

John team Hopkins university Mice with congenital deafness associated with the absence of connexin 26 in the body were collected and followed. Scientists focused on animals Less than two weeks, when the ear canal is still closed, but also during the period immediately after opening, when the first sounds can be detected.

The results showed that many aspects of auditory development were not affected by the mutation. in First, the researchers observed that the cells generated spontaneous activity that triggered the normal activation of hair cells — the sensory cells of the inner ear — as well as neurons in the brain’s sound processing centers. This early sound-independent activity is essential for training the auditory system to respond correctly to sounds of different frequencies.the authors explain in their files communication.

The second item is highlighted: After opening the ear canal, different tones were correctly assigned by different subregions of the midbrain and auditory cortex. The problem was that the spontaneous activity and everything that followed stopped as soon as the ear canal was opened and the auditory system began to rely on sound for its activity. Thus, the sensitivity of neurons to sounds increased dramatically, similar to the hyperacusis that often accompanies hearing loss.Scientists say.

Results that may improve response to cochlear implants

According to the researchers, the two components detected in the auditory functions of mice with the connexin 26 mutation indicate that auditory activity Early spontaneity It could play a key role in developing new therapeutic interventions for congenital deafness.

Maintain this activity Early spontaneityInduced training in the face of deafness leading to mutants can prime the auditory system to respond well to cochlear implants, thus improving the effectiveness of early therapeutic interventions. It also suggests that early intervention may be important to avoid compensatory increases in sensitivity in the face of deafness leading to mutations that may predispose the auditory system to respond well to cochlear prostheses, thus improving the effectiveness of early therapeutic interventions.”

“This may allow the brain’s sound processing centers to mature so that they can correctly interpret the responses produced by cochlear implants in patients with this type of congenital deafness.”Dwight confirms Bergels, One of the authors of the article published in the journal Plus biologyJune 27, 2023.