Inner Ear Restoration

Restoring the inner ear is obviously important and welcome.  This reports that serious progress is now been made and the next decade could produce a successful working protocol to essentially eliminate deafness by simple regrowth.  I got this item from an investment newsletter named Energy and Capital.

We are now seeing a wide spread of stories that all are all focused on replacement therapy and that all is about growing back damaged parts of the body.  It is also clear that while the big targets are taking longer, that is not so true for smaller targets.

I am expecting a rapid and full migration out of artificial prosthetics into restored body parts.  This will include actual regrowth of whole limbs although that is likely to be the last achieved.  Yet nerve regeneration can change that prospect also..

A Miracle of Biblical Proportions

By Jason Stutman | Monday, May 12th, 2014

At two years of age, the average child has a vocabulary of about 100 words. By two and a half, they will know close to 300 — and will likely be talking nonstop.

This sudden acquisition of new words is what experts call the “naming explosion.” And despite the common complaint from parents during this time that they can never seem to get a moment of peace, it's something to remain incredibly appreciative of.

The fact is, if your child isn't able to spit out this many words by age three, chances are high there's an underlying medical issue at hand.

Take Alex Denworth, for example. At 20 months, Alex could speak only four words: "Mama," "Dada," "hello," and "up."

At first glance, this may have seemed like an issue of intelligence. But Alex wasn't mentally disabled by any means. In fact, he was fully engaged and competent with his toy trucks, building blocks, and just about every other typical toddler activity you could think of.

The medical issue Alex was suffering from actually had nothing to do with his mental wit, but was rather an issue of hearing: Alex suffered from a deformity in his inner ear known as Mondini dysplasia alongside a separate and progressive condition, Enlarged Vestibular Aqueduct.

In short, this meant Alex was profoundly deaf in his right ear and moderately deaf in the left. More alarmingly, it meant that a simple bump on the head or sudden change in pressure would cause even further hearing loss.

To the dismay of his parents, Alex couldn't ride on a plane, swim, or even step into an elevator without the risk of going completely deaf.

“A Miracle of Biblical Proportions”

Upon being diagnosed, Alex immediately became a candidate for a cochlear implant, a small but complex electronic device that provides direct stimulation to the auditory nerves of a patient's inner ear.

Unlike standard hearing aids, which only amplify sound, cochlear implants turn sound waves into electrical waves the brain can interpret.

Cochlear implants in their crudest form have been around since the 1960s, but it was only recently that they became precise enough for patients to identify individual words or even enjoy the sounds of music.

Today, these implants are advanced enough to completely change a patient's way of life. In the case of Alex Denworth, the procedure was described by his father as “a miracle of biblical proportions.”

And if those words are not enough, here's an image of a child hearing for the very first time:

If that's not absolutely precious, I'm not quite sure what is.

Still, cochlear implants are by no means perfect. While it's enough to make most patients completely elated, what's actually heard is largely distorted — regular speech ends up sounding something like the Dalek robots from Dr. Who.

If the above reference isn't familiar, neuroscientist Gary Housley puts the issue into clear, scientific terms: “Cochlear implants are very effective for picking up speech, but they struggle to reproduce pitch, spectral range, and dynamics.”

To tackle this hurdle, researchers are finding new and exciting ways to regrow the natural mechanisms of the inner ear. In particular, Housley and fellow researchers are currently investigating the use of cochlear implants for gene therapy.

The group has recently made a breakthrough by demonstrating how to successfully regenerate auditory nerves using a combination of electrode stimulation and gene vectors.

Without getting too technical, Housley and company were able to regenerate nerves and show a better response to sound in guinea pigs, an animal with both a similar cochlea size and structure to humans.

Ultimately, the study suggests the ability to use cochlear implants to naturally improve the hearing of human patients in the near future and quite possibly bring about a cure for deafness.

Perhaps even more exciting is the fact that the world's first human gene therapy trial will take place just two months from now. The trial, led by Kansas Medical Center's Hinrich Staecker, takes a more conventional approach than Housley's electrode stimulation method, using a harmless virus to deliver gene therapy.

In Staecker's own words: "The holy grail is to give people natural hearing back. That's what we hope to do — we are essentially repairing the ear rather than artificially imitating what it does."

The trials follow successful results from last year when Staecker's team was able to use the same virus to deliver a gene called Atoh1 to improve the hearing of mice by 20 decibels. That's about the difference between cupping your hands over your ears and then taking them off again.

Incredibly enough, deafness is just the tip of the iceberg when it comes to regenerative medicine. At this very moment, there are several small companies we're aware of working with similar methods to cure blindness and even regrow tissue damaged from heart attacks.

More on that in just a few weeks.