Researchers At The John Hopkins University Discover The Proteins That Would Most Likely Restore Irreversible Deafness

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Source: https://www.hopkinsmedicine.org/

Johns Hopkins Medicine researchers have most likely successfully discovered a way to restore hearing with people that have irreversible deafness.

Researchers said that by using genetic tools found in mice, they identified proteins called hair cells that control the sound detection of cells found in the mammalian inner ear. This report on proteins can be found in eLife.

Associate professor of neuroscience at the John Hopkins University School of Medicine, Dr. Angelika Doetzlhofer said:

“Scientists in our field have long been looking for the molecular signals that trigger the formation of the hair cells that sense and transmit sound. These hair cells are a major player in hearing loss, and knowing more about how they develop will help us figure out ways to replace hair cells that are damaged.”

Mammals hear through sound vibrations that travel through the cochlea; which is a hollow, snail shell-looking structure. Inside the cochlea, you will find two types of sound-detecting cells – the inner and outer hair cells – which send all sound information it picks up directly to the brain.

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These hair cells, or damaged auditory nerves are the main causes of 90% of genetic hearing loss. When deafness is encountered through an exposure to massive noises or viral infections, it is because of the damage it causes to hair cells. Human hair cells do not regenerate, unlike those of birds or other mammals. Once hair cells are damaged, loss of hearing will be permanent.

Scientists have already gotten down that the first step to having a hair cell birth starts in the outer part of the cochlea where precursor cells transforms into hair cells and stops once it reaches the inner part of the structure. Knowing the starting point of hair cell development, Doetzlhofer and her team researched on molecular cues along the cochlear spiral.

Among the proteins that were studied, a pattern that stood out was found in Activin A and follistatin that was worth investigating further.

“In nature, we knew that Activin A and follistatin work in opposite ways to regulate cells. And so, it seems, based on our findings like in the ear, the two proteins perform a balancing act on precursor cells to control the orderly formation of hair cells along the cochlear spiral.” Doetzlhofer divulged.

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Researchers individually studied these two proteins to figure out exactly how they can coordinate hair cell births by a number of different experiments.

“The action of Activin A and follistatin is so precisely timed during development that any disturbance can negatively affect the organization of the cochlea. It’s like building a house—if the foundation is not laid correctly, anything built upon it is affected,” Doetzlhofer added.

She also notes that this fundamental research can reach to great potential solutions in treating deafness by the damaged hair cells: “We are interested in how hair cells evolved because it’s an interesting biological question,” she says. “But we also want to use that knowledge to improve or develop new treatment strategies for hearing loss.

 

 

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