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hearing loss

LabTV: Curious About Genetics of Deafness

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Joseph FosterWhat do Miami, music, and genetic research have in common? They are all central to the life of Joseph Foster, the young researcher who’s in the spotlight for our next installment of LabTV.

Foster, a research associate in Mustafa Tekin’s lab at the University of Miami’s Hussman Institute for Human Genomics, is involved in the hunt for the remaining genes responsible for congenital forms of deafness.This area of research is a good fit for Foster. Not only does he have a keen interest in genetic diseases (a close family member was born with cystic fibrosis), he’s a musician with a deep appreciation of the gift of hearing—loving to play the saxophone in his free time.


Snapshots of Life: The Biological Basis of Hearing

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sensory hair cells in a chicken's ear

Credit: Peter Barr-Gillespie and Kateri Spinelli, Oregon Health & Science University, Portland

Did you know that chickens have ears? Well, here’s the evidence—you’re looking at a micrograph of sensory hair cells that make up the inner ear of Gallus gallus domesticus, otherwise known as the domestic chicken. Protruding from each hair cell is a tall bundle of stiff appendages, called stereocilia, that capture vibrations and enable the chicken to hear everything from grain being poured into a feeder to the footsteps of a wily fox. The flatter area is occupied by supporting cells, which have recently been shown to have the capacity to regenerate damaged or destroyed hair cells.

Peter Barr-Gillespie and Kateri Spinelli of Oregon Health & Science University, Portland used a scanning electron microscope to capture this image—one of the winners of the Federation of American Societies for Experimental Biology’s 2014 BioArt competition—while studying how these cells convert sound waves into brain waves. It is generally known that sound waves cause the stereocilia on each hair cell to oscillate in concert. These vibrating stereocilia trigger electrical changes in the hair cells, which then send signals to the brain. Barr-Gillespie’s group focuses on the actual molecules that build the stereocilia and translate the vibrations into brain signals.


Vision Loss Boosts Auditory Perception

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Image of green specks with blobs of blue centered around a large red blob with tentacles

Caption: A neuron (red) in the auditory cortex of a mouse brain receives input from axons projecting from the thalamus (green). Also shown are the nuclei (blue) of other cells.
Credit: Emily Petrus, Johns Hopkins University, Baltimore

Many people with vision loss—including such gifted musicians as the late Doc Watson (my favorite guitar picker), Stevie Wonder, Andrea Bocelli, and the Blind Boys of Alabama—are thought to have supersensitive hearing. They are often much better at discriminating pitch, locating the origin of sounds, and hearing softer tones than people who can see. Now, a new animal study suggests that even a relatively brief period of simulated blindness may have the power to enhance hearing among those with normal vision.

In the study, NIH-funded researchers at the University of Maryland in College Park, and Johns Hopkins University in Baltimore, found that when they kept adult mice in complete darkness for one week, the animals’ ability to hear significantly improved [1]. What’s more, when they examined the animals’ brains, the researchers detected changes in the connections among neurons in the part of the brain where sound is processed, the auditory cortex.


Meet Alex—Before and After NIH Clinical Trial

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Photo of an infant with mottled skin adjacent to a photo of young man with clear skin being examined by a female doctor.

Caption: Alex, then and now, with Dr. Goldbach-Mansky
Credit: Kate Barton and Susan Bettendorf (NIH)

Alex Barton recently turned 17. That’s incredible because Alex was born with a rare, often fatal genetic disease and wasn’t expected to reach his teenage years.

When Alex was born, he looked like he’d been dipped in boiling water: his skin was bright red and blistered. He spent most of his time sleeping. When awake, he screamed in agony from headaches, joint pain, and rashes. After a torturous 14 months, a rheumatologist told his mother that Alex suffered from Neonatal-Onset Multisystem Inflammatory Disease (NOMID). The doctor showed her a brief and scary paragraph in a medical text. Kate Barton, Alex’s mother, admitted that it “knocked her over like a freight train.”