Genetic Hearing Loss Causes
We all know that genetics is one of the major causes of hearing loss, and
can be a contributing factor even when it's not considered the main cause.
We do know that there are scores of genes that can cause genetic hearing
loss. The good news is that these genes are being identified and genetic
solutions to hearing loss are being developed.
April 2013 - Protein Mutation Causes Hearing Loss
March 2013 - Tiny Genetic Patch Stops Deafness in Mice
February 2013 - Gene Linked to Nonsyndromic Deafness and
Usher Syndrome
February 2013 - House Research Institute Recruiting
Patients for NF2 Clinical Trial
February 2013 - Gene Tied to Multiple Types of Hearing
Loss
December 2012 - New Test Identifies Cause of Childhood
Deafness
November 2012 - Migraines may increase the risk of
hearing loss
October 2012 - Researchers Discover Gene That Causes
Deafness
August 2012 - Genetic Testing Can Explain Hearing Loss and
Guide Therapy
August 2012 - Study of zebra fish may speak to Fraser
syndrome hearing loss
May 2012 - Genetic loci identified for high-frequency
hearing loss
March 2012 - Drug halts organ damage in inflammatory
genetic disorder
February 2012 - Variant of Usher Syndrome Gene
Preserves Vision and Balance
February 2012 - How Mitochondrial DNA Defects Cause
Inherited Deafness
January 2012 - Harmonin study adds new piece to Usher
syndrome puzzle
January 2012 - New clues to human deafness found in
mice
August 2011 - Researchers Link Usher Syndrome Hearing
Loss to Harmonin Protein
July 2011 - Hearing Loss Proteins Discovered, Linked to
Usher Syndrome
June 2011 - Mutated Muscle Protein Causes Deafness
February 2011 - Function Of Molecule Underlying
Deafness Discovered
February 2011 - Nearly 70 Genes Identified As Hearing
Loss Causes
January 2011 - High tech genetic sequencing enables
screening for hereditary hearing loss
January 2011 - UK Researchers Find Gene Mutation That
Causes Deafness and Could Lead to Therapies
July 2010 - Gene Mutation That Causes Rare Form of Deafness
Identified
July 2010 - New gene makes the difference in Usher
syndrome
April 2010 - Structure of Inner-Ear Protein is Key to
Hearing and Inherited Deafness
January 2010 - Elusive protein points to mechanism
behind hearing loss
November 2009 - Mouse study sheds light on
age-related hearing loss
September 2009 - Scripps Research Scientists
Identify Genetic Cause for Type of Deafness
July 2009 - Deafness-Causing Mutation In a Gene
Associated with Cancer
April 2009 - Researchers Find Lack of Key
Molecule Leads to Deafness
March 2009 - Scientists find hearing loss gene
March 2009 - Proteins Linked To
Congenital Deafness Help Build, Maintain Inner Ear Stereocilia
November 2008 -
New mouse mutant contains clue to progressive
hearing loss
August 2008 - UM Scientists Discover Another Mutation
that Causes Hearing Loss
August 2008 -
Two Genes Associated with Hearing Loss
June 2008 - Smoking and weight-gain linked to
hearing loss
March 2008 - Researchers Find a Genetic Cause for
Hearing Loss That's Inherited from Mother
November 2007 - Genes Influence Age-Related Hearing
Loss
February 2007 - Scientists identify molecular cause for
one form of deafness
August 2006 - New Study Aims to Uncover Genetic
Factors for Hearing Loss
July 2005 - It's well established that genetic
mutations cause a large portion of hearing loss cases; now scientists
have found that other mutations can exacerbate the effects of the
original mutations!
More on this and related
topics
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March 2013
Researchers used a small genetic patch to partially
restore hearing and balance in deaf mice with Usher syndrome, according to a
new report published in Nature Medicine. The animal study, which is still in
its early stages, could eventually develop into new treatments for Usher
syndrome, a congenital hearing disorder which usually goes hand-in-hand with
blindness as well. When the scientists injected the profoundly deaf mice
with the genetic patch, they developed into partially hearing mice with no
balance problems.
Full Story
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February 2013
The list of genes shown to be associated with
hearing loss continues to grow, and that's promising news for patients, as
each newly identified mutation expands diagnostic opportunities and
highlights a potential pathway for future treatment. One of the latest
discoveries came from an international research team led by the University
of Cincinnati and Cincinnati Children's Hospital Medical Center. Along with
colleagues from the National Institute on Deafness and Other Communication
Disorders, Baylor College of Medicine, the University of Kentucky, and other
institutions, the team identified genetic mutations responsible for
nonsyndromic deafness and Usher syndrome type 1J. Nonsyndromic deafness
accounts for about 80% of genetic hearing loss. Children with Usher syndrome
type 1 typically are born profoundly deaf, have severe balance problems, and
develop vision problems in early childhood.
Full Story
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August 2012
Audiologists have been predicting for years that a
single blood sample would be used to quickly detect a multitude of
underlying genetic causes for hearing loss. A test at the University of Iowa
in Iowa City does just that, screening genome sequences associated with
syndromes ranging from Pendred to Usher and for genes linked to hearing
deficits. What does this advance mean to those who treat the hearing
impaired? A lot, said Jeff Simmons, who coordinates the Cochlear Implant
Center at Boys Town National Research Hospital in Omaha, NE. "I think it has
multiple uses," he said, aside from being able to pinpoint a cause. "From a
clinician's point of view, [genetic testing] can help tell us where we want
to go in terms of remediation."
Full Story
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January 2012
Scientists have long known that harmonin aids in
sensing sound in the inner ear, but a new finding suggests that the protein
is also involved in transmitting sound information from the inner ear to the
brain, adding a layer of understanding of harmonin's role in Usher syndrome.
"Usher syndrome is the most common inherited form of combined deafness and
blindness," said Amy Lee, PhD, an associate professor of molecular
physiology and biophysics and otolaryngology at the University of Iowa and
the lead author of the study in Nature Neuroscience (2011;14[9]:1109).
Experts estimate that four of every 100,000 babies born in the United States
are affected by Usher syndrome. Three to six percent of them are deaf, and
an additional three to six percent are hearing impaired, according to the
National Institute on Deafness and Other Communication Disorders (NIDCD).
Full Story