Nav: Home

Diminuendo -- New mouse model for understanding cause of progressive hearing loss

April 27, 2009

The respective microRNA seed region influences the production of sensory hair cells in the inner ear, both in the mouse and in humans. The findings have been published ahead of print in the current online issue of Nature Genetics. This study represents a major step forward in elucidating the common phenomenon of progressive hearing loss, opening up new avenues for treatment.

Scientists of Helmholtz Zentrum München, led by Professor Martin Hrabé de Angelis, director of the Institute of Experimental Genetics, have developed a new mouse model with a genetic mutant in which a single base of a specific microRNA seed region has been altered. Mice carrying this miR-96 mutation suffer progressive hearing loss as they get older. Moreover, if they carry two of these mutants, their sensory hair cells are impaired from birth on.

A number of genes associated with hearing loss were already known. "However, we were very surprised when with our new mouse model we discovered this new class of genes -microRNA - as genetic cause for this clinical picture," explained Dr. Helmut Fuchs, who conceived the idea of this mouse model and who is scientific -technical head of the German Mouse Clinic at Helmholtz Zentrum München.

The new mouse model is called diminuendo, named after the term in music theory meaning "becoming gradually softer". The mice were bred using the ENU method in which the male mice are administered N-ethyl-N-nitrosurea (ENU), thus influencing the DNA of their sperm. Successor generations develop dominant or recessive mutations. Using methods like these, Martin Hrabé de Angelis and his colleagues in the German Mouse Clinic can thus identify mutants that develop diseases similar to human diseases. They made the diminuendo mouse model available to colleagues of the Wellcome Trust Sanger Institute in Cambridge, UK, who - based on specific characterizations - ultimately found the association with the miR-96 mutation.

In Germany alone, around 13 million people have impaired hearing, according to estimates of the German Deaf Association (Deutscher Schwerhörigenbund). There are diverse causes for this, including deafness simply due to old age, hearing loss caused by infections and damage due to chronic noise. However, progressive hearing loss can also have genetic causes.

"We assume that our mouse model will be of far-reaching significance for the development of treatment strategies against genetically caused progressive hearing loss in humans," Dr. Fuchs explained. Colleagues from Spain confirm his assumption. They have already performed first examinations on patients diagnosed with progressive hearing loss. In them the microRNA cluster Mirn96 was mutated in the same seed region as in the mouse model. Now, with the aid of this mouse model, the international research consortium hopes to identify factors which are necessary for long-term survival of hair cells and thus to find new approaches for treatment of progressive hearing loss.
-end-
Original Publication: Lewis M. et al.: An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice. Nature Genetics online April 12, 2009
http://dx.doi.org/10.1038/ng.369

Helmholtz Zentrum München - German Research Center for Environmental Health

Related Hearing Loss Articles:

Researchers listen to zebrafish to understand human hearing loss
Can a fish with a malformed jaw tell us something about hearing loss in mice and humans?
Postmenopausal hormone therapy associated with higher risk of hearing loss
Use of postmenopausal hormone therapy was associated with higher risk of hearing loss, and the risk tended to increase with longer duration of use.
Few researchers consider hearing loss in healthcare communication: Study
Of the 67 papers reviewed, only 16 (23.9 percent) included any mention of hearing loss.
Few studies consider hearing loss when assessing communication with physicians
Doctors believe that communication with those under their care is important, but most studies of communication between physicians and older adults do not mention that hearing loss may affect this interaction.
Study shows hearing tests miss common form of hearing loss
Traditional clinical hearing tests often fail to diagnose patients with a common form of inner ear damage that might otherwise be detected by more challenging behavioral tests, according to the findings of a University at Buffalo-led study published in the journal Frontiers in Neuroscience.
Drug treatment could combat hearing loss
Researchers at MIT and Brigham and Women's Hospital have discovered a combination of drugs that induces supporting cells in the ear to differentiate into hair cells, offering a potential new way to treat hearing loss.
Second cause of hidden hearing loss identified
Some people can pass a hearing test but have trouble understanding speech in a noisy environment.
U study: Law aiding infants at risk for hearing loss
A Utah law has led to increased early identification of infants with hearing loss due to a congenital infection, according to a new study by University of Utah and Utah Department of Health researchers.
MED-EL convenes global hearing researchers for age-related hearing loss workshop
Leading scientists and hearing experts from around the world will gather for a scientific workshop sponsored by hearing implant leader MED-EL.
Iron deficiency anemia associated with hearing loss
In a study published online by JAMA Otolaryngology-Head & Neck Surgery, Kathleen M.

Related Hearing Loss Reading:

Best Science Podcasts 2019

We have hand picked the best science podcasts for 2019. Sit back and enjoy new science podcasts updated daily from your favorite science news services and scientists.
Now Playing: TED Radio Hour

Digital Manipulation
Technology has reshaped our lives in amazing ways. But at what cost? This hour, TED speakers reveal how what we see, read, believe — even how we vote — can be manipulated by the technology we use. Guests include journalist Carole Cadwalladr, consumer advocate Finn Myrstad, writer and marketing professor Scott Galloway, behavioral designer Nir Eyal, and computer graphics researcher Doug Roble.
Now Playing: Science for the People

#529 Do You Really Want to Find Out Who's Your Daddy?
At least some of you by now have probably spit into a tube and mailed it off to find out who your closest relatives are, where you might be from, and what terrible diseases might await you. But what exactly did you find out? And what did you give away? In this live panel at Awesome Con we bring in science writer Tina Saey to talk about all her DNA testing, and bioethicist Debra Mathews, to determine whether Tina should have done it at all. Related links: What FamilyTreeDNA sharing genetic data with police means for you Crime solvers embraced...