New understanding of hearing loss

A major breakthrough in the understanding of hearing and noise-induced hearing loss has been made by hearing scientists from three Pacific Rim universities.

Scientists from The University of Auckland, the University of New South Wales in Sydney, and the University of California in San Diego have collaborated for nearly 20 years on this research.

“This work represents a paradigm shift in understanding how our ears respond to noise exposure,” says Professor Peter Thorne from The University of Auckland, who is one of the co-authors of two papers published recently in the prestigious journal, the Proceedings of the National Academy of Sciences (PNAS) [1, 2].

“We demonstrate that what we traditionally regard as a temporary hearing loss from noise exposure is in fact the cochlea of the inner ear adapting to the noisy environment, turning itself down in order to be able to detect new signals that appear in the noise,” he says.

After the noise is turned off, hearing remains temporarily dull for some time while it readjusts to the lack of noise.

“Clinically, this is what we measure as a temporary hearing loss,” says Professor Thorne. “This has always been regarded as an indication of noise damage rather than, in our new view, a normal physiological process.”

The researchers show that this is due to a molecular signalling pathway in the cochlea, mediated by a chemical compound called ATP, released by the cochlear tissue with noise and activating specific ATP receptors in the cochlear cells.

“Interestingly, if the pathway is removed, such as by genetic manipulations, this adaptive mechanism doesn’t occur and the ear becomes very vulnerable to longer term noise exposure and the effects of age, eventually resulting in permanent hearing loss.”

“In other words the adaptive mechanism also protects the ear,” says Professor Thorne.

The second paper, done in collaboration with United States colleagues, reveals a new genetic cause of deafness in humans which involves exactly the same mechanism.

People (two families in China) who had a mutation in the ATP receptor showed a rapidly progressing hearing loss which was accelerated if they worked in noisy environments.

“This work is important because it shows that our ears naturally adapt to their environment, a bit like pupils of the eye which dilate or constrict with light, but over a longer time course,” Professor Thorne says.

This inherent adaptive process also provides protection to the ear from noise and age-related wear and tear. If people don’t have the genes that produce this protection, then they are more likely susceptible to developing hearing loss.

“This may go some way to explaining why some people are very vulnerable to noise or develop hearing loss with age and others don’t,” he says.

“Our research demonstrates that what we have always thought was temporary noise damage (i.e. the temporary hearing loss experienced in night clubs or a day’s work in factories), may not be this, but instead, is the ear regulating its sensitivity in background noise”.

“Although our research suggests that our hearing adapts in some noise environments, this has limits,” says Professor Thorne. “If we exceed the safe dose of noise, our ears can still be damaged permanently despite this apparent protective mechanism.”

“People need to protect their ears from constant noise exposure to prevent hearing loss and this is particularly important in the workplace and with personal music devices which can deliver high sound levels for long periods of time,” he says.

New implant replaces impaired middle ear

Functionally deaf patients can gain normal hearing with a new implant that replaces the middle ear. The unique invention from the Chalmers University of Technology has been approved for a clinical study. The first operation was performed on a patient in December 2012.

With the new hearing implant, developed at Chalmers in collaboration with Sahlgrenska University Hospital in Gothenburg, the patient has an operation to insert an implant slightly less than six centimetres long just behind the ear, under the skin and attached to the skull bone itself. The new technique uses the skull bone to transmit sound vibrations to the inner ear, so-called bone conduction.

“You hear 50 percent of your own voice through bone conduction, so you perceive this sound as quite natural”, says Professor Bo Håkansson, of the Department of Signals and Systems, Chalmers.

The new implant, BCI (Bone Conduction Implant), was developed by Bo Håkansson and his team of researchers. Unlike the type of bone-conduction device used today, the new hearing implant does not need to be anchored in the skull bone using a titanium screw through the skin. The patient has no need to fear losing the screw and there is no risk of skin infections arising around the fixing.

The first operation was performed on 5 December 2012 by Måns Eeg-Olofsson, Senior Physician at Sahlgrenska University Hospital, Gothenburg, and went entirely according to plan.

“Once the implant was in place, we tested its function and everything seems to be working as intended so far. Now, the wound needs to heal for six weeks before we can turn the hearing sound processor on”, says Måns Eeg-Olofsson, who has been in charge of the medical aspects of the project for the past two years.

The technique has been designed to treat mechanical hearing loss in individuals who have been affected by chronic inflammation of the outer or middle ear, or bone disease, or who have congenital malformations of the outer ear, auditory canal or middle ear. Such people often have major problems with their hearing. Normal hearing aids, which compensate for neurological problems in the inner ear, rarely work for them. On the other hand, bone-anchored devices often provide a dramatic improvement.

In addition, the new device may also help people with impaired inner ear. “Patients can probably have a neural impairment of down to 30-40 dB even in the cochlea. We are going to try to establish how much of an impairment can be tolerated through this clinical study”, says Bo Håkansson.

If the technique works, patients have even more to gain. Earlier tests indicate that the volume may be around 5 decibels higher and the quality of sound at high frequencies will be better with BCI than with previous bone-anchored techniques. Now it’s soon time to activate the first patient’s implant, and adapt it to the patient’s hearing and wishes. Then hearing tests and checks will be performed roughly every three months until a year after the operation.

“At that point, we will end the process with a final X-ray examination and final hearing tests. If we get good early indications we will continue operating other patients during this spring already”, says Måns Eeg-Olofsson.

The researchers anticipate being able to present the first clinical results in early 2013. But when will the bone-conduction implant be ready for regular patients?

“According to our plans, it could happen within a year or two. For the new technique to quickly achieve widespread use, major investments are needed right now, at the development stage”, says Bo Håkansson.

AHRF Researcher Describes Cochlear Amplification Using Novel Optical Technique

It has long been known that the inner ear actively amplifies sounds it receives, and that this amplification can be attributed to forces generated by outer hair cells in the cochlea. How the ear actually accomplishes this, however, has remained somewhat of a mystery. Now, Jonathan A. N. Fisher, PhD, and colleagues at The Rockefeller University, in New York, describe how the cochlea actively self-amplifies sound it receives to help increase the range of sounds that can be heard.

Fisher and colleagues used a new optical technique that inactivates prestin, a motor protein involved in the movement of the outer hair cells. The outer hair cells are part of the hair cell bundles (which also include the inner hair cells)- the true sensory cells of the inner ear. The main body of the hair cells sits in the basilar membrane- the tissue that lines the interior of the bony cochlea. The “hair” part of these cells, called the stereocilia, sticks up into the fluid-filled space of the cochlea, where they are pushed by the fluid as sound waves travel through it.

The sound waves traveling down the cochlea produce actual waves that can be observed along the basilar membrane as visualized in the animation (from the Howard Hughes Medical Institute). The cochlea picks up different sound frequencies along its length, with higher frequency sounds picked up at center of the “snail” and the lower frequency sounds being picked up at the part of the cochlea closest to the eardrum.

The outer hair cells have been known to provide amplification of sound waves picked up by the inner hair cells by actively changing their shape to increase the amplitudes of the sound waves. These outer hair cells can do this because the membrane protein can contract and cause the stereocillia to be deflected by the overlying tectorial membrane.

Fisher and colleagues developed a light-sensitive drug that when illuminated by an ultraviolet laser can inactivate prestin in select locations within the cochlea. Using this novel technique, the researchers were able to affect prestin at very specific locations along the basilar membrane.

The researchers found that by inactivating prestin at very specific locations, the sound-evoked waves that carry mechanical signals to sensory hair cells were re-shaped and of smaller amplitude- indicating that without prestin, amplification is dampened  compared to what the researchers saw when prestin was allowed to function normally.

Scripps Research Institute Scientists Identify Molecules in the Ear that Convert Sound into Brain Signals

For scientists who study the genetics of hearing and deafness, finding the exact genetic machinery in the inner ear that responds to sound waves and converts them into electrical impulses, the language of the brain, has been something of a holy grail.

Now this quest has come to fruition. Scientists at The Scripps Research Institute (TSRI) in La Jolla, CA, have identified a critical component of this ear-to-brain conversion—a protein called TMHS. This protein is a component of the so-called mechanotransduction channels in the ear, which convert the signals from mechanical sound waves into electrical impulses transmitted to the nervous system.

“Scientists have been trying for decades to identify the proteins that form mechanotransduction channels,” said Ulrich Mueller, PhD, a professor in the Department of Cell Biology and director of the Dorris Neuroscience Center at TSRI who led the new study, described in the December 7, 2012 issue of the journal Cell.

Not only have the scientists finally found a key protein in this process, but the work also suggests a promising new approach toward gene therapy. In the laboratory, the scientists were able to place functional TMHS into the sensory cells for sound perception of newborn deaf mice, restoring their function. “In some forms of human deafness, there may be a way to stick these genes back in and fix the cells after birth,” said Mueller.

TMHS appears to be the direct link between the spring-like mechanism in the inner ear that responds to sound and the machinery that shoots electrical signals to the brain. When the protein is missing in mice, these signals are not sent to their brains and they cannot perceive sound.

Specific genetic forms of this protein have previously been found in people with common inherited forms of deafness, and this discovery would seem to be the first explanation for how these genetic variations account for hearing loss.

The Unbalanced Sloth

Most creatures need a good sense of balance — especially tree-dwellers that swing among high branches. In mammals, the ability largely comes from three loop-shaped structures in the inner ear called semicircular canals; in most species, the size, shape, and arrangement of those loops (inset) is extremely consistent from one individual to another. But in three-toed sloths (such as Bradypus variegatus, the brown-throated three-toed sloth, pictured), many proportions of the semicircular canals are surprisingly variable from one sloth to another.

The overall variability is at least twice that seen in other species of mammals the team analyzed, researchers report online today in the Proceedings of the Royal Society B. That high degree of variation stems from the sloths’ languid lifestyle, the researchers suggest.

Sloths, which move extremely slowly when they move at all, don’t require the sense of balance that a swift, agile creature such as a primate needs. The finding supports one of Charles Darwin’s notions about evolution: If an organ isn’t crucial, variations in its structure or performance aren’t lost over time, keeping the potpourri in the population.