Hair Cell Regeneration
One of the promising medical technologies that could at
least partially restore hearing in some people with hearing loss is hair
cell regeneration. This process refers to the ability to cause the
cochlear hair cells to repair or replace themselves. Since the large
majority of hearing loss is caused by damage to these hair cells, this
technology offers great hope to many people with hearing loss.
December 2000 - Adult humans are unable to regenerate
damaged hair cells. But birds are able to do it quite readily. Do our
feathered friends offer hope that we may one day be able to regenerate
human hair cells? Find out what the University of Washington
Hair Cell Research has to say.
January 2001 - Progress in Hair Cell Regeneration
August 2001 - Hair Cell Regeneration Update from the House Ear Institute
July 2002 - From the 2002 SHHH Convention, here's a report on Dr.
Plubel's Plenary
Session on hair cell regeneration.
August 2002 - Hair Cells
"Self-Renew"
November 2002 - Deafness Discovery Showed Inner Hair
Cells Regenerated
June 2003 - Is this the holy grail of hair cell regeneration?
Scientists at the University of Michigan have achieved hair
cell regeneration in a mammal!
September 2003 - Hair cell regeneration and the related stem cell research
offer what is probably the best hope for hearing restoration in the next
few years. A reader who follows these developments just sent me four
stories, each of which discusses promising medical discoveries. Two of
the stories are late-breaking news and two are from earlier this summer.
Here are a couple of paragraphs from each story and a link to the full
article.
October 2003 - Here's more breaking news on hair cell regeneration. Sound
Pharmaceuticals has been issued a European patent for its hair cell
regeneration treatment.
September 2004 - Here's some more information on
hair cell regeneration in mammals.
October 2004 - Here's an update on hair cell
regeneration status and an interview with Dr. Edwin W. Rubel.
November 2004 - "The Scotsman" is reporting
on research at Sheffield University that may enable hearing restoration
in the foreseeable future.
January 2005 - Concerned that the hype regarding hair cell
regeneration may be inflating expectations, Dr. Neil
Bauman offers his analysis of where hair cell regeneration stands and
when we can expect it to be generally available.
January 2005 - Scientists have discovered a gene
that inhibits hair cell reproduction, and have learned that turning off
the gene causes hair cell proliferation.
February 2005 - UM Scientists Restore Guinea Pig's
Hearing
April 2005 - Work at Indiana University causes more
excitement about hair cell regeneration progress.
April 2005 - Scientists from the Medical Research
Council (MRC) and the University of Hong Kong have isolated the gene
responsible for sensory development in the inner ear.
June 2005 - Now that scientists have discovered
the gene that inhibits hair cell reproduction, the next hurdle is
developing a way to control that gene. Here's an article about a new
study that attempts to do just that!
September 2005 - The
2005 SHHH National Convention included a wonderful panel on the current
state of hair cell regeneration.
January 2006 - Most
hearing loss is due to damaged inner-ear hair cells, so scientists are
exploring ways to boost their growth. Success could help fight deafness.
Over the years, our ears take a beating. They're assaulted with
thunderous music, shrill sirens, blaring TV shows and the incessant
background thrum of modern life. Little wonder that by middle age,
millions of Americans suffer some degree of hearing loss, mostly due to
the cumulative destruction of the delicate sound-sensing cells inside
our ear from all this noise. Once these cells die, they're gone forever
and a hearing aid often lies somewhere down the road. But it may not
always be that way. Full
Story
April 2006 - Here's the latest report on the hair
cell regeneration work at Massachusetts General Hospital.
June 2006 - Scientists Show Mouse Ear Holds
Potential for Cell Regeneration to Restore Hearing
August 2006 - Stem Cells May Be Key
To Deafness Cure
June 2007 - The Miracle of Hair Cells and Prospects
for Regrowth
June 2007 - Genes In Human Inner Ear Cells Restored
September 2007 - The Hearing Regeneration
Initiative at UW
September 2007 - New Technique May be Boon for
Hair Cell Studies
September 2007 - Rotarians Raise Money for Haircell
Regeneration
November 2007 - Researchers Develop New Method of
Growing Hair Cells
August 2008 - OHSU Grows Hair Cells Involved in
Hearing
~~~~~~~~~~~~~~
January 2001
As most of our readers probably know, most hearing loss is caused by
deterioration of the hair cells in the cochlea. The hair cells move in
response to acoustic energy entering the ear, and stimulate the auditory
nerve with information regarding the characteristics of the incoming
sound. Drugs, heredity, or loud noises can damage or destroy the hair
cells, resulting in hearing loss.
We have known for some time that some animals (including many birds)
can spontaneously regenerate damaged hair cells, but regeneration has
never been observed in mammals - until now. The August 26, 2000 Issue of
the British medical Journal Lancet reported successful regeneration of
hair cells in a postnatal rat cochlea by introducing a particular gene
(Math1) to the cochlea. Researcher Wei-Qiang Gao (Genentech, San
Francisco, CA,USA) points out, "It wasn't just a few hair cells--we
had several hundred, so it's robust production".
The next step in the investigation is to determine whether similar
techniques can regenerate hair cells in mature rats. Success in these
experiments would bode well for eventual hair cell regeneration in
humans.
Another possibility to replace damaged hair cells is transplantation.
Matthew Holley (University of Bristol, UK) and his colleagues have
developed an "ear in a test tube", in which they have
successfully grown mouse hair cells. Future advances may allow growth
and transplantation of human hair cells.
~~~~~~~~~~~~~~~~
August 2001
The House Ear Institute has been actively involved in the research on
hair cell regeneration. They are pursuing two complementary strategies
in hopes of understanding the regeneration process and how to induce it
in humans. Here's a link to an article describing their recent work.
http://www.hei.org/research/projects/cmb/haircellchall.htm
~~~~~~~~~~~~~~~~
October 2003
Editor: Here's more breaking news on hair cell regeneration. Sound
Pharmaceuticals has been issued a European patent for its hair cell
regeneration treatment. Note that this development is only part of the
required solution, and it doesn't mean that a treatment will be widely
available next week. But I think it is a big step towards viable hair
cell regeneration. Here's the press release.
~~~~~~~~~~~~~~~~~~~
Sound Pharmaceuticals, Inc. (SPI) announced that its patent
"Method for the treatment of diseases or disorders of the inner
ear" has issued in Europe, effective Oct. 1, 2003.
SPI has developed a novel strategy to stimulate auditory hair cell
regeneration using proprietary cell cycle inhibition technology.
Typically, auditory hair cells in mammals are not replaced when injured
or lost. This results in permanent and often progressive sensorineural
hearing a disease that affects over 30 million in the US. In non-mammals
like birds, hair cell regeneration occurs through the spontaneous
proliferation of the adjacent supporting cell. These newly proliferating
supporting cells can go on to become replacement hair cells. However in
mammals, auditory supporting cells do not proliferate or regenerate into
hair cells even in the presence of growth factors.
SPI identified that p27Kip1, a cyclin dependent kinase inhibitor,
prevents supporting cells from proliferating after embryogenesis.
Compounds developed by SPI to inhibit p27Kip1 have been shown to
stimulate supporting cell proliferation after drug or noise induced hair
cell loss. "We are the only group that has demonstrated the ability
to stimulate proliferative regeneration in the cochlea of mammals"
says Dr. Jonathan Kil, President & CEO. "It is anticipated that
this revolutionary technology will be critical in developing treatments
to restore hearing in humans."
Sound Pharmaceuticals, Inc. is a drug development company focused on
treating hearing loss. To date, Sound Pharmaceuticals' drug discovery
program has identified targets for the prevention of hearing loss and
for the improvement of hearing in individuals with hearing loss. For
more information please visit http://www.soundpharmaceuticals.com
~~~~~~~~~~~~~~~~~~~
October 2004
Editor: For people interested in hearing loss "cures", hair
cell regeneration is the current best bet. It appears that virtually all
animals except mammals regenerate hair cells on a routine basis. Dr.
Edwin W. Rubel and his colleagues are among the researchers on the
forefront of this exciting technology. "Hearing Review"
recently published an interview of Dr. Rubel along with a synopsis of
the research status. Here's one question and answer from the interview.
The complete article is available at:
http://www.hearingreview.com/Articles.ASP?articleid=H0410F01
~~~~~~~~~~~~~~~~~~~
Hearing Review: If hair cell regeneration is indeed possible, do you
think this science will ever progress to a point where there will be
full restoration of hair cells, or do you think that it's far more
likely we would see a partial restoration of hair cells in the inner
ear?
Rubel: In my opinion, it's not a question of if we will regenerate,
restore, or protect hair cells, it's a question of when. Because we now
know that it's possible, it's only a matter of time until we can apply
this science to humans. My best prediction is 10-20 years. I certainly
hope to see it in my lifetime.
With respect to the degree of hair cell regeneration or restoration,
my gut feeling is that it will all depend on what type of hearing loss a
person has to begin with. One possibility for regeneration are people
who have complete loss of hair cells due to some genetic anomaly,
ototoxins, aminoglycosides, etc. In these cases, hearing care
professionals may someday have a choice between recommending a cochlear
implant versus an approach for growing enough hair cells where hearing
aids could be used more effectively and provide much more acoustic
information to that patient. As another example, you might see a patient
who has a 50% loss of their outer hair cells. In this case, maybe we
will be able to stimulate the regrowth and replacement of these
"cochlear amplifier" cells.
~~~~~~~~~~~~~~~~~~~
November 2004
Editor: "The Scotsman" is reporting on research at
Sheffield University that may enable hearing restoration in the
foreseeable future. Here are excerpts from the story. For the complete
article, please point your browser to http://news.scotsman.com/uk.cfm?id=1346202004
~~~~~~~~~~~~~~~~~~~
SCIENTISTS hope that stem-cell research could lead to a cure for
deafness in as little as ten years.
Researchers from Sheffield University are using embryonic stem cells
in efforts to grow new cells in the inner ear.
Although in its early stages, the team from Sheffield University
hopes it could lead to a cure for deafness in ten to 15 years.
[snip]
Dr Rivolta added that his team hoped to undertake the first tests on
animals in two years. "It could then be possible to do human trials
in three to four years, but that would depend on the animal
trials."
~~~~~~~~~~~~~~~~~
June 2007
Here's a great article that explains the structure
and function of inner and outer hair cells and also looks at some of the
research into regrowing these cells. If you're interested in this topic,
it's very much worth the read!
Full Story
~~~~~~~~~~~~~~~~~
June 2007
Dr. Jeffrey Holt, associate professor of
neuroscience and otolaryngology at UVa, and his research team, including
Dr. Bradley Kesser, an assistant professor of otolaryngology, targeted a
gene known as KCNQ4, which causes genetic hearing loss in humans when
mutated. They engineered a correct form of the gene and created a gene
therapy delivery system that successfully transferred the KCNQ4 gene into
human hair cells harvested from the inner ears of patients with hearing
loss. "Our results show that gene therapy reagents are effective in human
inner ear tissue. Taken together with the results from another group of
scientists who showed that similar gene therapy compounds can produce new
hair cells and restore hearing function in guinea pigs suggest that the
future of gene therapy in the human inner ear is sound," Holt said.
Full
Story
~~~~~~~~~~~~~~~~~
September 2007
Woodstick, is the incredible annual event
sponsored by the Rotarians for Hearing Regeneration: A Rotarian Action
Group during which we: Attempt to break the Guinness World record by
assembling the largest number of drummers with drum sets playing one song
simultaneously (Woodstick currently holds the existing record of 533
drummers!) Raise money for worthwhile causes. Our featured cause is
Hearing Regeneration. Our goal is to raise 15 million dollars over the
next ten years for the Virginia Merrill Bloedel Hearing Research Center at
the University of Washington. Also, we will be raising money for the
homeless for Network Services, and funding around $10,000 worth of musical
instruments and equipment to local schools for school music programs.
Full Story
~~~~~~~~~~~~~~~~~
November 2007
Researchers at the University of Virginia have
developed a new method of growing inner-ear hair cells that will aid
research to help people regain their hearing. Dr. Jeffrey T. Corwin, a
professor of neuroscience at the UVa Health System, and Dr. Zhengqing Hu,
a neuroscience research assistant, have been growing cells from inner ears
of chicken embryos. They hope to extend that knowledge to re-grow the
inner-ear hair cells of humans. Mammals grow inner-ear hair cells only
before they are born, unlike amphibians and birds, which can re-grow
damaged or lost cells. These unique structures are lost over time as
mammals age, or if they contract certain infections or undergo trauma. The
loss of inner-ear hair cells results in hearing loss and balance
impairment. Hu and Corwin's process is able to grow chicken inner-ear hair
cells in a laboratory setting.
Full Story
