Genetic 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!
January 2006 - Genetics and Hearing Loss: An
Overview
May 2006 -
Genetic cause of hearing loss in aging
August 2006 -
DNA Differences Between People With Hearing Loss and Those
Without
August 2006 - Gene linked to Hearing Loss in Aged
Identified
August 2006 - New Study Aims to Uncover Genetic
Factors for Hearing Loss
February 2007 - Scientists identify molecular cause for
one form of deafness
June 2007 - Boffin identifies gene causing otosclerosis
September 2007 -
Genetic Basis of Hearing Loss
November 2007 - Genes Influence Age-Related Hearing
Loss
March 2008 - Researchers Find a Genetic Cause for
Hearing Loss That's Inherited from Mother
June 2008 - Smoking and weight-gain linked to
hearing loss
August 2008 - UM Scientists Discover Another Mutation
that Causes Hearing Loss
August 2008 -
Two Genes Associated with Hearing Loss
November 2008 -
New mouse mutant contains clue to progressive
hearing loss
February 2009 -
Going Deaf and Blind in a City of Noise and Lights
March 2009 - Scientists find hearing loss gene
March 2009 - Proteins Linked To
Congenital Deafness Help Build, Maintain Inner Ear Stereocilia
April 2009 -
Sound science - Exploring the genetics of hearing loss
April 2009 - Researchers Find Lack of Key
Molecule Leads to Deafness
April 2009 - Holding out hope of a cure for deafness
April 2009 - 'Dimenuendo' Mouse Used To Understand Cause of
Progressive Hearing Loss
July 2009 - Deafness-Causing Mutation In a Gene
Associated with Cancer
September 2009 - Scripps Research Scientists
Identify Genetic Cause for Type of Deafness
November 2009 - Mouse study sheds light on
age-related hearing loss
January 2010 - Elusive protein points to mechanism
behind hearing loss
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January 2006
Knowing the
exact cause of a child's hearing loss can assist clinicians and parents
in making decisions regarding treatment and educational options. It may
surprise some parents to know that more than half of all children who
are born deaf or who become deaf very early in life have a genetic cause
for their hearing loss. In fact, recent
studies have revealed that approximately 50-60% of moderate to
profound, congenital, or early-onset hearing loss is genetic. The
remaining 40-50% of hearing loss is due to non-genetic effects, such as
maternal infection (CMV or rubella), prematurity, postnata infection
(meningitis, otitis media), ototoxic drugs, or acoustic/ cranial trauma.
Full
Story
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August
2006
Drugs to
stop old people going deaf may be a step nearer after scientists
identified the gene at fault. They found huge DNA differences between
people who lose their hearing and those who do not.
Now they
hope the KCNQ4 gene discovery paves the way for drugs to prevent deafness
and bring relief to Britain's 6.5million over-60s affected.
Dr Ralph
Holme of the Royal National Institute for the Deaf, which funded the
study, said: "We are optimistic that people will no longer face the
prospect of losing their hearing as they age."
Full Story
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August 2006
Loss of
hearing in the elderly has been blamed on defects in a specific gene
according to studies by Dutch researchers. It has been estimated that about
37% of Britons from ages 61 to 70 and 60% of those from ages 71 to 80 about
6.5m people are said to have age-related hearing loss. The Human Mutation
study that was conducted on 1,200 people revealed subtle changes in the
gene, KCNQ4 more frequent among those with age-related hearing problems.
According to the Royal National Institute for Deaf People, which funded the
work, the study offered "real hope for treatments."
Full Story
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June 2007
A new study has identified a gene responsible for
otosclerosis - the single most common cause of hearing loss among white
adults. Melissa Thys, from the Department of Medical Genetics, University of
Antwerp, Belgium, said that this finding may be a step towards new
treatments for otosclerosis, which affects approximately 1 in 250 people.
Otosclerosis is a multifactorial disease, caused by an interaction of
genetic and environmental factors. The outcome is a progressive hearing loss
as the growing bone in the middle ear interrupts the sound waves passing to
the inner ear. While the causative factors remain unknown, now one of the
genetic components has been identified.
Full Story
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September 2007
Editor: The folks at the Virginia Merrill Bloedel Hearing Research Center
at the University of Washington are looking at the genetic basis of hearing
loss. This article first appeared in their online newsletter at http://depts.washington.edu/hearing/pdf/bloedelsound.pdf
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As of 2006, researchers have identified 145 different genetic loci that
cause hearing loss in humans. Of these deafness-causing locations in the
genome, the specific mutated gene has been identified for 80 of them. The
complex structure of the auditory system and its demand for fast and precise
signaling to the brain is the likely reason why there are such a large
number of different genes, mutations in which cause hearing loss. The fact
that auditory system malfunctions are not lethal to either humans or mice
makes genetic analysis of hearing loss a particularly useful way to probe
the biological basis of hearing loss and deafness. While convenient for
understanding how hearing happens, the human cost of hearing loss is very
large, having both enormous social impact as well as significant economic
consequences. Detailed studies on each gene that contributes to hearing loss
provides information on the basic biology of hearing and provides clues as
to how hearing loss might be ameliorated in affected individuals.
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Going Deaf and
Blind in a City of Noise and Lights
February 2009
When Rebecca Alexander walks into the party at a
Hell's Kitchen condo, it's like someone turned on a 10,000-watt lightbulb.
The room migrates toward her, absorbing her aura, a raw, happy energy. She
had sold the party to me as "a regular event where young blind people meet
somewhere that's well lit." It looks like any other party: loud music,
appetizers, a dozen twenty- and thirtysomethings mingling in a room devoid
of furniture to trip over. Most everyone in the room has retinitis
pigmentosa, the progressive death of photoreceptor cells, which can lead to
blindness. Alexander, a pretty 29-year-old brunette who is also losing her
hearing, grins hello. The Kanye West song that's playing sounds to her like
an auditory blur, and she views everything through two small holes called
"doughnut vision," which is like if you squeeze your hands into little
circles in front of your eyes. A typical person can see 180 degrees without
turning their head; Alexander can see less than 15 degrees. Her pupils dart
back and forth, to figure out who's there, scanning to compensate for her
brain's unfortunate habit of wrongly filling in blank spots, putting empty
space where there's a person. Her condition is known as Usher syndrome type
III, a recessive genetic mutation causing her retinal cells and ear-hair
cells to die, an ailment shared by fewer than a thousand Americans. A
typical sufferer is blind and completely deaf by his forties.
Full Story
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April 2009
Progressive hearing loss is a common and
debilitating problem in humans, yet relatively little is known about the
hundreds of genes thought to be involved. Chrissie Giles spoke to Professor
Karen Steel, whose work with mice is shedding light on the complex process
of hearing. Headbanger, oblivion, catweasel: not Scandinavian death metal
bands, or the latest celebrity fragrances, but mutant mouse strains that
share defects in the sensory hair cells found in their ears. These defects
frequently affect the balance and/or hearing of the mice, and research into
them can bring new insights into human hearing. Now, we can add to the list
of mutant strains 'diminuendo'. This mouse carries a mutation that causes
hearing problems, and is the latest mutant to have been identified by
Professor Karen Steel at the Wellcome Trust Sanger Institute. Professor
Steel has been investigating the genetics of deafness for some 30 years. Her
ultimate aim is to help develop therapies to prevent or minimise the effects
of progressive hearing loss, a debilitating and distressing condition that
affects around 60 per cent of people over 70.
Full Story
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April 2009
An Israeli hearing loss expert has discovered one of
the root causes of deafness, opening up the potential for a whole new range
of treatments, and even possibly a cure for both genetic and age-related
deafness. In a landmark study, Prof. Karen Avraham of Tel Aviv University
has revealed that microRNAs - tiny molecules that regulate cell functions
found in the 'hair' cells of the ear - help us to hear. Deafness continues
to be a significant problem in medicine today. For all the progress made in
the last years, still one in 1,000 US babies are born deaf. The numbers
increase as the population grows older, with more than 50 percent of
pensioners in America experiencing some form of hearing loss, making it the
most common disability in humans. Despite this, doctors still don't know
what causes hearing loss in most people, but they do know where the process
starts to break down.
Full Story
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May 2009
Scientists of Helmholtz Zentrum München, the German
Research Center for Environmental Health, 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 reportedly impaired from
birth on. "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.
Full Story
