Schwann cell disruption may lead to hidden hearing loss, researchers find
The Hearing Review Aug 02, 2017
Patients who complain they canÂt hear their friends at a noisy restaurant, but pass a hearing test in their doctorÂs office, may be describing hidden hearing loss.
Now, less than six years since its initial description, scientists have made great strides in understanding what hidden hearing loss is and what causes it.
In research published in the journal Nature Communications, University of Michigan researchers report a new unexpected cause for this auditory neuropathy, a step toward the eventual work to identify treatments, the school said in an announcement on its website.
ÂIf people can have hidden hearing loss for different reasons, having the ability to make the right diagnosis of the pathogenesis will be critical, said author Gabriel Corfas, PhD, director of the Kresge Hearing Research Institute at Michigan MedicineÂs Department of Otolaryngology  Head and Neck Surgery.
Corfas published the research with co–author Guoqiang Wan, now with Nanjing University in China. Using mice, they discovered that disruption in the Schwann cells that make myelin – which insulates the neuronal axons in the ear – leads to hidden hearing loss. This means hidden hearing loss could reportedly be behind auditory deficits seen in acute demyelinating disorders such as Guillain–Barré syndrome, which can be caused by Zika virus.
Corfas and Wan used genetic tools to induce loss of myelin in the auditory nerve of mice, modeling Guillain–Barré. Although the myelin regenerated in a few weeks, the mice developed a permanent hidden hearing loss. Even after the myelin regenerated, damage to a nerve structure called the heminode remained.
When the ear is exposed to loud noises over time, synapses connecting hair cells with the neurons in the inner ear are lost. This loss of synapses has previously been shown as a mechanism leading to hidden hearing loss.
In an audiologistÂs quiet testing room, only a few synapses are needed to pick up sounds, according to the University of Michigan. But in a noisy environment, the ear must activate specific synapses. If they arenÂt all there, itÂs difficult for people to make sense of the noise or words around them. That is hidden hearing loss, Corfas said.
ÂExposure to noise is increasing in our society, and children are exposing themselves to high levels of noise very early in life, Corfas said. ÂItÂs clear that being exposed to high levels of sound might contribute to increases in hidden hearing loss.Â
The newly identified cause – deficiency in Schwann cells – could occur in individuals who have already had noise exposure–driven hidden hearing loss as well.
ÂBoth forms of hidden hearing loss, noise exposure and loss of myelin, can occur in the same individual for an additive effect, Corfas said.
Previously, Corfas group succeeded in regenerating synapses in mice with hidden hearing loss, providing a path to explore for potential treatment.
While continuing this work, Corfas started to investigate other cells in the ear, which led to uncovering the new mechanism.
There are no current treatments for hidden hearing loss. But as understanding of the condition improves, the goal is for the research to lead to the development of drugs to treat it.
ÂOur findings should influence the way hidden hearing loss is diagnosed and drive the future of clinical trials searching for a treatment, Corfas said. ÂThe first step is to know whether a personÂs hidden hearing loss is due to synapse loss or myelin/heminode damage.Â
Go to Original
Now, less than six years since its initial description, scientists have made great strides in understanding what hidden hearing loss is and what causes it.
In research published in the journal Nature Communications, University of Michigan researchers report a new unexpected cause for this auditory neuropathy, a step toward the eventual work to identify treatments, the school said in an announcement on its website.
ÂIf people can have hidden hearing loss for different reasons, having the ability to make the right diagnosis of the pathogenesis will be critical, said author Gabriel Corfas, PhD, director of the Kresge Hearing Research Institute at Michigan MedicineÂs Department of Otolaryngology  Head and Neck Surgery.
Corfas published the research with co–author Guoqiang Wan, now with Nanjing University in China. Using mice, they discovered that disruption in the Schwann cells that make myelin – which insulates the neuronal axons in the ear – leads to hidden hearing loss. This means hidden hearing loss could reportedly be behind auditory deficits seen in acute demyelinating disorders such as Guillain–Barré syndrome, which can be caused by Zika virus.
Corfas and Wan used genetic tools to induce loss of myelin in the auditory nerve of mice, modeling Guillain–Barré. Although the myelin regenerated in a few weeks, the mice developed a permanent hidden hearing loss. Even after the myelin regenerated, damage to a nerve structure called the heminode remained.
When the ear is exposed to loud noises over time, synapses connecting hair cells with the neurons in the inner ear are lost. This loss of synapses has previously been shown as a mechanism leading to hidden hearing loss.
In an audiologistÂs quiet testing room, only a few synapses are needed to pick up sounds, according to the University of Michigan. But in a noisy environment, the ear must activate specific synapses. If they arenÂt all there, itÂs difficult for people to make sense of the noise or words around them. That is hidden hearing loss, Corfas said.
ÂExposure to noise is increasing in our society, and children are exposing themselves to high levels of noise very early in life, Corfas said. ÂItÂs clear that being exposed to high levels of sound might contribute to increases in hidden hearing loss.Â
The newly identified cause – deficiency in Schwann cells – could occur in individuals who have already had noise exposure–driven hidden hearing loss as well.
ÂBoth forms of hidden hearing loss, noise exposure and loss of myelin, can occur in the same individual for an additive effect, Corfas said.
Previously, Corfas group succeeded in regenerating synapses in mice with hidden hearing loss, providing a path to explore for potential treatment.
While continuing this work, Corfas started to investigate other cells in the ear, which led to uncovering the new mechanism.
There are no current treatments for hidden hearing loss. But as understanding of the condition improves, the goal is for the research to lead to the development of drugs to treat it.
ÂOur findings should influence the way hidden hearing loss is diagnosed and drive the future of clinical trials searching for a treatment, Corfas said. ÂThe first step is to know whether a personÂs hidden hearing loss is due to synapse loss or myelin/heminode damage.Â
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