FEATURES:
NS040828
 #58  Falling on deaf ears: Far from being grateful, some deaf
          people bitterly resent scientists' efforts to cure
          them. Pat Hagan finds out why

 Pat Hagan; Pat Hagan is a medical journalist in London.
(Additional reporting by Clare Wilson)

PHILIP Aiello has been deaf since 1943. He was just three months
old when his hearing was destroyed by meningitis. More than
half-a-century later, medicine had advanced to the point where a
tiny device called a cochlear implant could help. The implant is
no instant cure for deafness, but it can restore some degree of
hearing. For Aiello, by this time 55, the chance was too good to
miss.

In June 1998, he underwent surgery to have the device implanted.
The first thing he heard was the soft hum of the electrodes
warming up. The second was his wife Myrna saying hello.

But if the joy of hearing her voice for the first time is etched
on his memory, so too are the reactions he received. While he was
still learning to use the implant, Aiello went to Texas to attend
a deaf congress. Aiello, from Wheaton in Maryland, recalls what
happened when people started noticing the implant's distinctive
wire and hearing-aid type headpiece. 'They would finish the
conversation to run and tell their friends,' he says.

People began to keep their distance. Or they'd creep up behind him
and make noises -- if he didn't hear them, they'd tell their
friends that the implant didn't work. Aiello felt like he no
longer belonged in the deaf community.

His ostracism demonstrates just how polarised opinions are on the
treatment of deafness. As cochlear implants and other devices
become increasingly sophisticated , scientists are getting closer
to 'curing' deafness. But they are on a collision course with
large sections of the deaf community who believe they are not
sick and do not need curing. To some, it's a personal right to
remain deaf.

But the current conflicts are nothing to the furore set to erupt
when more effective and permanent treatments for deafness become
available. Scientists believe that within the next decade or so
they may be able to completely restore deaf people's hearing.
Various high-tech strategies are being investigated, including
gene therapy and stem cell therapy. But to some deaf people, such
approaches are akin to eugenics. 'We consider such research an
appalling waste of money,' says Paddy Ladd, a lecturer in deaf
studies at the University of Bristol, who has been deaf from
birth. 'Do they really think we are damaged and deficient people
who are simply a biological mistake?'

About 5 per cent of people in the west have significant hearing
problems, and just under 1 per cent are profoundly deaf. Causes
include genetics, infections in the womb or during childhood,
certain drugs, chronic exposure to loud noise, or simply the
ageing process.

Many people who have been profoundly deaf from an early age
immerse themselves in the deaf community, where sign language is
the primary communication tool. The UK signing community has long
fought for official recognition of British Sign Language (BSL) as
an indigenous language, arguing that about 70,000 people in the
UK use it as their first or preferred form of communication. Last
year they achieved this aim, when the government granted BSL
official recognition. But the deaf community argues this must be
translated into better provision of interpreters and video
phones, and funding for teaching of BSL.

Many in the signing community reject the popular notion that they
are 'disabled', arguing they are simply a minority with their own
rich culture and language. 'These people were born deaf and they
want to be deaf and the rest of us have to come to terms with
that,' says Pamela Morrisey, head of fund-raising for the British
Deaf Association.

These battles have far-reaching implications for the way that
doctors treat deafness, or whether they treat it at all. In
particular, they will determine whether it is ethically
defensible to restore, or refuse, hearing to a deaf child too
young to decide for themselves.

In many cases of deafness, the problem lies with the cochlea, the
snail-shaped cavity in the inner ear whose job is to convert
sound waves into nerve impulses (see Diagram). In the functioning
ear, sound waves striking the eardrum trigger movement of fluid
inside the cochlea, stimulating auditory hair cells on its lining
to produce electrical signals; these travel along the auditory
nerve to the brain. But the hair cells are particularly
vulnerable to damage, and cannot be regenerated.

A cochlear implant is an electrode that is embedded within the
cochlea, where it stimulates the auditory nerve directly,
bypassing the hair cells. The user wears a microphone on the back
of the ear, which picks up sound waves and transmits them to the
implant. To avoid passing a wire through the skin, they are
transmitted via radio waves.

Cochlear implants by no means restore hearing to normal. The
sounds produced have an artificial, tinny quality, and it can
take months of effort for people to get used to them, with
varying degrees of success. Children in particular need a lot of
support. Some people never really succeed with the device. What's
more, a cochlear implant can destroy any remaining natural
hearing a person might have, because the surgery kills off any
hair cells that are still functioning.

Despite these drawbacks, many people find the implants hugely
beneficial. The device significantly improves lip-reading in most
people and about 10 per cent of users end up being able to
communicate through sound alone. To date, an estimated 65,000
people worldwide have had a cochlear implant.

Debate rages among deaf people about the pros and cons of the
device. Many members of the signing community argue they have no
need to undergo invasive surgery that yields less-than-wonderful
results. 'Deaf people see themselves as not in need of 'cures','
says Ladd, who is author of Understanding Deaf Culture.

Most contentious, however, is what happens with children. Nearly
90 per cent of deaf children have hearing parents, who often see
implants as a logical choice. But numerous deaf associations,
including ones in Britain, the US and Australia, condemn cochlear
implants for children. Implants, says Ladd, 'cast [deaf children]
adrift in a world in which they are an inferior version of a
hearing person, rather than as a first-class deaf citizen'.

Equally, deaf parents who reject implants for their deaf children
also draw flak. One deaf couple recently came under heavy fire
when they took this a step further and deliberately conceived a
deaf child through IVF .

The decision on whether to give a child a cochlear implant cannot
be put off for long. The first six years of life are crucial for
language acquisition. Many centres advocate carrying out the
surgery on children of two, or even younger. Recent research
seems to support this approach. For example, a study published in
May in the journal Otology & Neurotology followed 10 congenitally
deaf infants fitted with implants between the ages of 6 and 18
months. Within one to four months of the surgery, the previously
silent babies started babbling -- the first stage of speech
development. The younger they were when the device was implanted,
the sooner this occurred.

The debate has been muddied by concerns that the implants raise
the risk of the potentially fatal brain infection meningitis,
perhaps due to the surgery or simply the presence of a foreign
object in the inner ear. Worldwide there have been 91 known cases
of meningitis in people with implants, including 17 deaths. New
implant recipients are now vaccinated, but passions on this issue
run high. At a Manchester conference on cochlear implants two
years ago, members of a group called the Deaf Liberation Front
staged a protest with banners saying 'Better deaf than dead'.

If controversy surrounds cochlear implants, what would happen if a
complete cure for deafness became available? Many groups of
scientists around the world are investigating other strategies to
restore hearing, including gene therapy and stem cell therapy.
The research is still at an early stage, but few doubt that
eventually a cure will emerge.

In the late 1980s, researchers found that unlike mammals, birds
can regenerate auditory hair cells. The new hair cells are
derived from cells in the cochlea called supporting cells, which
have a maintenance role.

In the developing embryo, both supporting cells and hair cells are
descendants of the same precursor cells. The ones that are
destined to become hair cells start expressing a gene called
Math1, whose protein product turns on a suite of other genes
characteristic of hair cells.

Researchers at the University of Michigan have used gene therapy
to turn supporting cells into hair cells in mammals (The Journal
of Neuroscience, vol 23, p 4395). They introduced the Math1 gene
into guinea pigs' supporting cells. Within two months they looked
like hair cells. 'They started changing their identity,' says
Yehoash Raphael, who led the research. The team is now seeing if
the new hair cells can transmit signals to the auditory nerve.

But gene therapy is not the only possible approach. Last year,
researchers at Harvard Medical School found that small numbers of
stem cells exist in the inner ear of adult mice. Unfortunately
these were in the balance organs of the inner ear, not the
cochlea. But the balance organs contain hair cells that are very
similar to those in the cochlea.

The team showed that when prodded with two biological signalling
molecules, the stem cells gave rise to what appeared to be new
hair cells, both in the test tube and when put into the
developing cochleae of chicken embryos. The researchers' next
goals are to repeat the feat in mice embryos, then adult animals,
and assess if the new cells function properly. 'That's the
ultimate test,' says cell biologist Stefan Heller, who leads the
team.

The finding has galvanised researchers in the field, for three
reasons. First, it is possible that inner ear stem cells could be
taken from people who donate organs after death. Second, studying
inner ear stem cells in the lab should make it much easier to
work out how to push embryonic stem cells along the road to
becoming hair cells. Third, it suggests that stem cells could
also be present in the cochlea itself. Perhaps with the right
chemical cues, they could be persuaded to form new hair cells
without the need for any invasive surgery. 'That's the hope of
the whole field,' says Heller. 'To find a drug that brings back
hearing.'

Unsurprisingly, members of the signing community are less than
impressed. 'Scientists are patronising the deaf by assuming they
need 'curing',' says Lori Fuller, a deaf advocate for the
campaigning group Deaf Empowerment, based in Wisconsin in the US.
'It's hearing people who have a problem with hearing loss, not
deaf people.'

Ladd slates the scientists involved in this research. 'They do not
care about us, only about their own salaries or careers,' he
says. 'None of these people has any sustained contact with deaf
communities.'

Not all deaf people feel the same way of course. The research is
welcomed by Richard Roehm, a leading deaf activist based in
Irvine, California, although he acknowledges that 'the majority
of the adult deaf would perceive this as some sort of genocide'.

Few would argue that adults have the right to decide for
themselves if they want medical treatment, but as with cochlear
implants, the fiercest battles are likely to be fought over
children. When the day eventually comes, as it surely will, that
a safe cure for deafness becomes available, many hearing parents
of deaf children will want to use it. To the deaf community, this
is a challenge to their very right to exist, and must be resisted.

It's a viewpoint that most hearing people find hard to understand.
Deaf people 'have to accept that there are now real treatments',
says Heller. But to those who are sceptical of the deaf
community's viewpoint, Ladd suggests the following thought
experiment: 'Had [gene therapy] emerged in the 1950s, there would
have been immense social pressure to use this technology to
remove homosexuality,' he says. 'Fifty years later, we can
imagine the magnitude of the crime that would have been committed
in society's name.'
Designing deafness
Pat Hagan
Two years ago a deaf couple in the US hit the headlines when they
deliberately conceived a deaf child. Sharon Duchesneau and Candy
McCullough, a lesbian couple in Bethesda, Maryland, had the child
through IVF. For the father they turned to a deaf male friend
with five generations of deafness in his family.

The plan worked. Duchesneau gave birth to a boy, Gauvin, who was
profoundly deaf in his left ear and had only limited hearing in
his right. The couple rejected the offer of a hearing aid.

It was a bizarre twist to the concept of designer babies. Usually,
the selection process is about screening out disabilities -- not
creating them. Yet is the outcome so different to what goes on
naturally when disabled people marry someone who has the same
condition as them?

The incidence of the commonest form of genetic deafness has
roughly doubled in the US in the past 200 years. A paper
published in The American Journal of Human Genetics in June
suggests that one of the main causes was the development of sign
language in the 16th and 17th centuries (vol 74, p 1081). That
and the establishment of schools for the deaf made it more likely
that deaf people would marry each other and so have deaf children.



____________________________________________________________
Copyright 2004 Reed Business Information US, a division of Reed Elsevier
Inc.
 
INTERVIEW:
NS040828
 #60  Blessed voices: Molly Brown was left profoundly deaf after
          her auditory nerves were removed during treatment for a
          genetic illness. But late last year she became one of
          the first people to be given a radical new type of
          implant that attempts to recreate hearing by
          stimulating the brainstem directly. Of the five who
          received the implant, she has had the most success. She
          still finds the telephone difficult, so when she told
          Duncan Graham-Rowe about her strange new world they
          used instant messaging

 Duncan Graham-Rowe

Molly Brown has a genetic disease that causes tumours to grow on
the auditory nerves. She had one cochlea removed when a tumour
was taken out in her 20s, and later had a cochlear implant in the
other to stimulate the nerve electrically. When that cochlea also
had to be removed she became an ideal candidate for the new type
of implant, developed by the House Ear Institute in Los Angeles.
She is 44 and lives in Washington state, near Seattle

When did you first notice problems with your hearing?
In 1982, when I was 22, I noticed I was having some difficulty
talking on the phone. The sound seemed to be getting softer and
more garbled.

What did you do?
I went to an ear doctor, who diagnosed 'sinus difficulties'. But
it was getting worse, so after another year I switched doctors.
My new doctor straightaway suspected a brain tumour. He said,
'You are too young to lose that much hearing.'

What was the diagnosis?
I had neurofibromatosis type II (NF2), although it wasn't
diagnosed for certain until last October. It is a disease in
which chromosome 22 basically tells your body to develop
non-malignant growths or tumours on the hearing nerves, spine and
sometimes elsewhere in your body. It is present at conception.
You know, I almost feel better knowing that I have had this from
day one and that I was not doing something 'wrong'.

So you had the tumour removed?
Yes, and they had to remove the cochlea with all the auditory
nerves as well, so I was left deaf in that ear. After that the
hearing in my good ear fluctuated wildly. There was a small
growth on that cochlea too. That growth had to be removed, which
damaged that cochlea and left me completely deaf. When my newborn
son was just three weeks old, I awoke to zero hearing. So I
decided to have an implant in the damaged cochlea.

How long were you profoundly deaf before you had the cochlear
implant?

About three years. My doctors felt I was not emotionally or
mentally ready to receive a cochlear implant. I was absolutely
devastated at my deafness. It is hard to put into words. I was so
lonely, so terribly sad. There was no guarantee a cochlear
implant would work, so my doctors and my family wanted me to
'accept' my deafness so I could move on. And I did.

When you eventually had the cochlear implant put in, how well did
it work?

It worked very well, especially at the beginning. I spoke on the
phone frequently, and did extremely well where I could combine it
with lip-reading. I loved it.

But it didn't last. What happened?
Last summer, I began having awful facial pain on the right side. I
thought it was a tooth. I give my dentist a lot of credit for
figuring that it might be a brain tumour. The pain is known as
trigeminal neuralgia -- the worst pain known to medical practice.
It felt like I was being electrically shocked.

What happened next?
My local doctor in Seattle had to do an MRI scan to get a better
look at the tumour, and that meant removing the cochlear implant.
You cannot have that much metal in your body with an MRI scan
because it interferes with the imaging. My doctor promised me he
would put it back if the tumour was not on the hearing nerve.
Unfortunately it was, so that cochlea had to be removed too.

What did it feel like to know that you might be left profoundly
deaf again?

Ah...I asked my children to tell me they loved me, because I might
never have heard their voices again. I wore the cochlear implant
right into the operating room where they did the MRI scan. My
audiologist had tears in her eyes when she said she had to take
it out. I wondered if hers would be the last voice I would hear
in my life. I was devastated.

But after about six months, you did hear again...
Yes. They gave me a 'penetrating electrode auditory brainstem
implant', which is a surgically implanted hearing device that
attempts to replace the actual hearing nerve by electrically
stimulating auditory nerves in the brainstem directly. It is
still only in trials; there was no guarantee it would work. But
for me, it has worked splendidly, especially when you consider I
have zero nerves to my brain for hearing. I can control it myself
via a signal processor that translates sounds into electrical
stimulations. I can control the volume.

What was it like when they turned the device on? What did you hear?

It was one of those moments you never forget. Steve Otto, my
audiologist at the House Ear Institute in Los Angeles, set me up
with his computer to test if it worked. I tried not to watch
their faces too much. I tried to concentrate on whether I could
hear any sound. I waited. I knew he was turning it up. And then
there it was. So soft. It was a series of beeps. I just said,
'There.' I was perfectly calm, but inside I could have jumped in
Steve's lap and shouted, 'Yahoo!'

After the test, whose voice did you hear first and what did they
say?

It was Steve's. He punched a button, and there was his
voice...beautiful! He said, 'Listen to my voice. How does it
sound? Testing one, two, three, four, baseball, cowboy, hot dog.'
I can remember. If I lived to be 1000 years old I would never
forget it.

How does it feel to be able to hear again after being profoundly
deaf?

About how you would think! I am thrilled, pleased and happy beyond
words. You must remember that I was facing a life with absolutely
no sound. To have this is beyond miraculous, and I am ever so
thankful. I am sitting here typing and enjoying the click-clack
of the keys.

How does it compare with when you had a cochlear implant?
I am always surprised at the similarities. It is not exactly the
same. But I compare them very favourably, which I was not
expecting. I was expecting to hear mostly beeps. I would say that
for speech I may have done a bit better in the very beginning
with the cochlear implant. But not near the end.

What can you hear?
It seems like I hear everything. I hear amazingly soft sounds,
such as the bubbles in a glass of soda, or pepper hitting a
dinner plate. My family also tells me that my speech is better. I
can hear my own voice again. But it is speech comprehension that
I am really after. The bubbles in a glass of pop are just icing
on the cake.

Are you having to relearn how to understand speech?
Yes, I am. I frequently try to understand people without looking,
but it is so much easier to combine with lip-reading. I am lazy.
As for the phone...

That's the acid test in artificial hearing. How well can you
understand people on the phone?

I call it my love-hate relationship. I so want to do it. I hear it
ring and think, 'Just pick it up!' I talked to my daughter
recently, and she said something which I felt had nothing to do
with what I was asking. I get all confused, then it goes out the
window -- mostly because we start laughing too much -- but I'll
never get it unless I try. I did call Steve in Los Angeles one
morning, which was a big deal for me. I heard him laugh at
something I said. These things are a real accomplishment for me.

Do you think your hearing has been enhanced in any way compared
with your natural hearing before all this began?

Oh, I do. I know I hear things that others don't. I hear metal
detectors in buildings buzzing, and when I ask others if they
hear it they always say no.

How different do things sound now compared with before your
hearing problems?

My memory is very clear on sound. Things such as car horns or
water running are very similar. Music is very different, but my
daughter plays the piano for me to get a feel of each note and
how they sound now. But when people speak it sounds like they are
speaking in an extremely growly voice with their hand muffling
their mouth. I can always tell a man's voice from a woman's
without looking. I am learning the environmental sounds, to
differentiate between, say, someone speaking to me and the food
mixer running.

Do things sound artificial?
Oh, it's very artificial. Very synthesised. But the longer I use
it and get used to it, the more natural it sounds. The brain is
pretty adaptable.

You recently went back to the House Ear Institute for a 'retuning'
to help improve your speech comprehension. Can you explain what
they did?

The device has different types of electrodes. Some sit on the
surface of the brainstem, others penetrate it and try to target a
greater range of nerves and frequencies. When I go for
reprogramming, Steve changes the way I hear. So I need to
practise using it all over again. The penetrating electrode
program may have been too loud previously, it was sounding as if
I were standing under high-voltage power lines. Steve turned it
down a bit. I am still asking people around me, 'what was that
sound?'

Do you think you will ever become so accustomed to your new
hearing that you forget how things used to sound?

I will never forget. When I watch a singer on TV singing a song I
know, I remember. It tugs at your heart. But my joy at hearing
anything at all overrides that.

Given the dangers of tampering with the brainstem, weren't you
scared of the risks? Only one person had had the implant before
you and there were no results at the time...

I had no fear, zero. It felt like my millionth brain surgery. I
felt a lot better about trying it than if I had always been
wondering if I should have. It was my impatient nature. I felt
almost driven to try. I'm no hero here. That title belongs to the
researchers and audiologists who slave over these inventions
every day.

What were your expectations?
They said there were no guarantees that I would hear any sound at
all. There are many repercussions from having a brain tumour
removed. Besides the deafness, my face looks as if I have had a
stroke. My eye would not close, so I had a metal spring implanted
in it to open and shut it. In short, it is a difficult deal. I
had zero expectations. Of course you hope, but I was trying to
prepare myself for a lifetime of no sound.

What motivated you to volunteer?
My children. They have a 50 per cent chance of inheriting NF2 from
me. I so wanted to show them that you should not be afraid to try
things, that you don't need to be fearful. Plus, I want as much
research into NF2 as possible.

Do you know whether any of your children have NF2 or will develop
it?

Thanks be to God, they look clear of it. They recently had MRI
scans, which were clear. I would give my life for them, of
course. I would give back this miraculous hearing if they would
be free from NF2. But it looks very good for them. They are 25,
22 and 18. We have talked about the 'what-ifs'. If any of them
did develop NF2 I would counsel them and totally leave it up to
them. The only test available thus far is the MRI. Again, I want
more research so they will be able to check newborns and catch
these tumours when they are small.

But with NF2 the tumours can reoccur...
Once the hearing nerve is removed, most people do not develop more
tumours in the hearing section of the brain. I do have one teeny
tumour on my spine. I am lucky, as many NF2 sufferers are really
bothered by spinal tumours. In severe cases, they have to use
wheelchairs.

Do you turn your device off at night?
I do take it off at night. And once I am out, I am gone! I do
occasionally sleep with it. I can hear traffic from about half a
mile away and it drives me nuts. But I can turn it off. Sometimes
I get all tense at sounds I am hearing, or when people are
annoying me. But then I remember, ah, I can turn it off!



____________________________________________________________
Copyright 2004 Reed Business Information US, a division of Reed Elsevier
Inc.
 


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