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OK this is plain weird ...
Transcript of an interview.

Norman Swan: Hello and welcome to the programme.

Today on The Health Report: something I suspect you've never come across before ' people with almost supernatural powers of hearing, so sensitive that they can hear the scratch of insects, the blood in their bodies, or a defective bearing in a motor a few doors down the street.

Mattea Sarpa: Eyeballs sound like creaking doors, eyelids opening and closing have a scratchy sound, bones and joints creak. Even if I have pain, I have actually hurt a shoulder, the pain in my shoulder has the type of noise to it.

Michael Halmagyi: We think these people would be ideal earthquake detectors, just like frogs and crocodiles they can hear footfall a long, long way away because their whole body acts as a microphone.

There are some odd symptoms in medicine which if you haven't heard them before you think these can't be real, but these are real.

Aviva Ziegler: If you think that what you've just heard is science fiction you're mistaken, it isn't. This is a real story about a woman who suffers from a rare medical condition. But the path to discovering that it was a medical condition and not a sign of madness has been a long and frustrating one for Mattea Sarpa.

Mattea Sarpa: I first noticed something strange was, one night at a presentation and awards night, the microphone did a microphone squeal and the next thing I knew I was being helped off the floor.

I had actually fallen off a chair that I was sitting on and then soon after I felt headachy and annoyed for long periods of time and the next time something happened was my husband whistled loudly calling the dogs and that whistle was of a pitch that also made me pass out on the spot. And I realised that there are certain noises that are able to do that to me, so I started to see the local GP and he did say he felt that it was an inner ear infection and proceeded to give me antibiotics which didn't help at all.

Then I was referred to a specialist who said I had probably a type of fungal growth in my ear and gave me fluoride tablets which made me terribly sick but didn't help at all.

Aviva Ziegler: It was nearly two years of believing that she was actually going crazy before Mattea was finally referred to a neurologist who was able to help her.

At the Hearing and Balance Clinic at Sydneys Royal Prince Alfred Hospital, Professor Michael Halmagyi recognised her symptoms and told her that what she had was superior canal dehiscence, a rare condition where people become hyper sensitive to sound usually caused by a hole or crack in the protective casing that surrounds the inner ear.

Our ears are made up of three parts; the first is the outer ear canal that leads to the eardrum and behind that is the middle ear where tiny connecting bones transmit sound to the third part, the inner ear. And thats where Mattea has her problem.

The inner ear is encased in a very thick part of the skull bone. Its the thickest part of the skull bone and in fact one of the thickest bones in the body because its a very delicate vibrations sensor. But the inner ear has two vibration sensors, it has the hearing part which of course is frequencies between 250 and about 6000 cycles per second and then it also has a very low frequency vibration sensor part which goes from static, which is gravity, to about 20 cycles a second.
And this sensors not so much vibration of the air as vibration of the head. So that is the motion sensor part. So the sound sensor and the motion sensor are in principle very, very similar organs, and in fact they are in the same inner ear organ encased.

But when this casing develops a hole two major things happen and they both relate to the fact that when you remove the case you make this sensor hyper sensitive. One of the benefits of having it in such a thick casing is to reduce its sensitivity. Very crudely speaking its a bit like how tender does a nail bed feel when you remove the nail? Its not meant to be without a casing and if you even make a hole in the casing of the inner ear it becomes too sensitive both to sound and to motion. And not only that it tends to convert one into the other, so in particular sounds stimulate the motion sensors because normally thats dampened out. This is a person in whom loud sounds are really uncomfortably loud and those sounds will cause a sense of imbalance.

Mattea Sarpa: Just say there is a fluorescent light that is flickering, that is almost as bad as hearing a really loud noise because the hearing and the vision is connected and there have been times where a flickering light almost is enough to get me into a state of a fit. And flickering on and off does make a noise and at a high speed. I can hear all the workings of the inside of my body from the heart beat and pulse, the blood passing near my ear, my eyelids opening and closing, my actual eyeballs turning make a noise.

Michael Halmagyi: Not only external sounds appear too loud but these people become aware of their internal sounds and in particular what they find sometimes quite troubling is the persistent sound of their own hearts. The reason for that is double in other words the main artery and vein that supplies and drains blood from the brain goes about 2 millimetres away from the inner ear. And normally we cant hear that because of the bony casing but if that bony casing is damaged you suddenly become aware,its like living next to a train line.

Mattea Sarpa: Walking into an arcade shop where theres all those arcade games and noises going left right and centre and beeps and pings, I hear that type of noise in day to day life. Some days by the time I come home I feel that I am exhausted, my brain is just exhausted hearing and deciphering because my brain wants to understand all the noise it hears.

When there is more than one, two, three, twenty sounds I can actually hear all of them, it becomes like a cacophony of muddled noise because I can hear whats happening right next to me and I can hear whats happening 20 metres away just as loud.

And the worst possible types of noises are grinding, vibrating type of mechanical noise. Voice noises is OK but mechanical noises seem to be getting into that pitch that is too loud for my ear, its almost grinding right into my brain.

Aviva Ziegler: How much more intense is it than the average would you say?

Michael Halmagyi: We have as much as 10 decibels better than a normal hearer. Now 10 decibels at that range would be nearly 20 times as good in terms of energy.

In some range, in some of the frequencies, very low frequencies she'll be 100 times as good as an ordinary hearer. We'd been aware of people like that for years and years. This phenomenon of balance activation by sound was well described a long time ago and carries actually in medical parlance the name of Tullio.

Aviva Ziegler: But its just not sound thats involved, vision is as well because vision is partly controlled by balance. Our ears make our eyes move involuntarily and irresistibly. For example Dr Halmagyi can diagnose balance problems by looking at abnormal eye movements. The direction of these beating, jerking, movements of the eye called nystagmus can actually tell a doctor which part of the inner ear has a problem.

Michael Halmagyi: But the real breakthrough came in 1998 when a doctor in the United States, Dr Lloyd Minor looked at the involuntary eye movements that are produced by sound in this so called Tullio effect and realised that they had to come from one particular canal of the inner ear. And eventually realised that if you do very thin and fine cat scans you will see that that canal is missing its roof. And so what he found is that the bone between the top of the inner ear and the brain was abnormally thin. And really completely defined this condition.

Aviva Zieglaer: When Dr Lloyd Minor the American specialist visited Australia some years ago Mattea went to see him and he became very intrigued by her condition.

Mattea Sarpa: He talked to me and videoed me actually passing out. They reproduced a noise in some headsets, many apologies, but he said that it was invaluable for his research. My hearing starts way before anybody else can normally hear , I already hear.

Aviva Ziegler: You could make a good spy.

Mattea Sarpa: Thats correct, Dr Halmagyi wanted to sell me to the CIA but my kids wouldnt be in it.

Of a night when its quiet I can hear things in my house from great distances. We live on acreage so I can hear dogs barking miles away. I can hear scrabbling noises throughout my house, if theres a mouse somewhere I can hear him because its so quiet and controlled I am able to hear the slightest noise. A door creaking, anything even the fridge running from a bedroom to kitchen, I can still hear it. If I was to walk with high heels on wooden floors yeah, thats really bad because I actually hear through my body as well. That vibration goes straight through me.

Michael Halmagyi: Its like, a bit like you know when the Indians used to put their ears on the rail track to hear the train coming. Well they dont need to put their ears on the rail track they can actually hear those ground vibrations.

Aviva Ziegler: How many people would present with this sort of problem?

Michael Halmagyi: We run a busy dizzy clinic and a hearing clinic, yeah we would see one a month.

Aviva Ziegler: Although Professor Halmagyi knew what Matteas problem was he still wasnt sure what had caused it. The fortuitous arrival of a new patient from Queensland who was complaining of similar symptoms to Mattea gave the medical team the clue theyd been looking for. The new woman had been hit on the head with a hockey stick.

Mattea Sarpa: And they found that she had had a blow to the head just behind the ear and this had caused a slight fracture in the skull and was making her have like amplified hearing.

So they came back to me and said have you had some sort of accident and its true, because I did have a blow to the head, to the side of the head in a car accident. And it was soon after that that it started. And because it had been years ago I was not physically ill at the time, the blow to my head was not of as great force or I didnt feel I was suffering from it. It had actually caused a by product.

So theres like a hairline fracture in a certain position amplifies the hearing because we are able to hear through the ear and through the skull at the same time. I had a special bone x-ray done of the head and there is the fracture just there, in the right spot.

Michael Halmagyi: We think what happens in some people theres a very thin layer of bone covering the ear and theyve had that most of their lives and then a wack on the ear or something just finally is the straw that breaks the ear.

We know that theres a big variety in the thickness of this bony roof and it may be that in some people it is already is very thin and over years it gets thinner and then it passes the critical point. We dont think its entirely something that develops theres got to be a certain predisposition to it.

Weve got over 40 patients now who come to us with this. Sometimes they just feel theres a blockage in the ear, sometimes they become aware that they can sense vibrations or they can hear vibrations. Often the presentation can be subtle. In Matteas case it was pretty obvious or obvious now.

Mattea Sarpa: With mechanical things I can actually tell when things are not running well, they make odd noises. And in the shopping centre I advise the butcher next door that his fridge was going to break down and they all laughed, and sure enough the next morning there was a mechanic pulling his fridge apart. So the mechanical noises are the ones that really stand out a lot.

Aviva Ziegler: Is there any sort of music that you can listen to?

Mattea Sarpa: Music in general is good unless its got the real clashy sounds in it because its certain pitches of music and voice and mechanical noises that when it gets to that level it wont matter what type of noise, whether its singing or mechanical, my eyes vibrate and I know its going to happen so I can try and avoid it.

I went to see Andrea Bocelli live, that made absolutely no impact on me but what did make an impact was when everyone clapped, oh 10,000 people clapping was not very comfortable.

Michael Halmagyi: Obviously the ear is frequency tuned and there is a particular frequency around 500 cycles a second that is the most potent at activating the balance system. We had one person who noticed that when he sang a certain note his vision would tilt. So if he looked at the horizon, if he made this sound, the horizon would move. Pressure or the sound would activate the balance system in one ear, the static balance system, so every time he made this sound his eyes would turn and therefore he had the illusion that the world had tilted.

Another patient who was a fire chief and the combination of ladders and sirens is not a good one for him. Going up a fire ladder and the sirens going he really thought hed fall off.

Mattea Sarpa: Recently I was in a shopping centre and I turned to my husband and I said to him the fire alarm is about to go off. And he said oh, dont be like that, he said I couldnt handle you being silly today. Sure enough within seconds the alarm went off and the whole centre was evacuated.

Aviva Ziegler: In the five years since Mattea was first diagnosed her sensitivity has worsened and shes found working in her family business increasingly difficult.

Mattea Sarpa: We owned a fruit market, a large fruit market, in a shopping centre so the amounts and varied types of noises from cash registers to scanners to trolleys, crowds of people and of course the worst of all was the fire alarm, I would get dragged out of the shopping centre by someone because Id just dropped.

By the time I came home I was not good, unable to function. I had constant headaches and I needed to be in a dark quiet place to recover from a day at the shop. And so that was not making my life very good and my family life was suffering terribly and in the end it was not worth me being in business. And so we decided to sell and now Im home and I do feel a lot better because I can control the surroundings that Im in.

Aviva Ziegler: Have you got any way of cutting yourself off from any of it?

Mattea Sarpa: I do wear ear plugs especially if I have a function where theres a large gathering, weddings and parties. I wear ear plugs, so everything is very muffled. I tend not to speak much, Im a bit of a wallflower, Im there but Im not there.

I went to see a show in the city, We Will Rock You. Yes, that was not good. Even with earplugs in the theatre, I had moments of near passing out.

Aviva Ziegler: Finding an earplug solution that gives the right balance between cutting out some but not all sounds has been challenging for the medical team. Their most recent experiment has been with a commercially available hearing impeder.

Michael Halmagyi: Theres a special type of hearing protector which is marketed for hunters and these hearing protectors will transmit sounds perfectly well up to about 80 decibels. Its fairly loud but above 80 or you can set it to higher, they would totally cut off and protect the ear. And we are trying that with Mattea because her problem is in a way similar that soft sounds are OK but once you get above 80 or certainly 90 db they become not only just unpleasantly loud, they can make her off balance.

Aviva Ziegler: Christopher Whitfeld is the audiologist whos been working with Mattea and regularly monitors her progress with the hearing aids.

Christopher Whitfeld: Hello, I havent seen you for a few weeks. Ill look up the last session with you.

Mattea Sarpa: You fine tuned the aid a bit.

Christopher Whitfeld: Have you been using it most days?

Mattea Sarpa: I have used it up until the end of December, I used it very frequently but we have made some big changes to our lifestyle and weve sold our business and Im at home now out on a property thats away from traffic and any type of noise and I very rarely use the earpiece except if I go out.

Christopher Whitfeld: What about in places like in the kitchen or somewhere where there could be loud sounds that you dont expect sometimes like plates and cups. Are those things loud enough to upset you?

Mattea Sarpa: They are loud enough to upset me but I think Im learning to live with those occasional sounds rather than the constant ones. It was the ones that were constantly around me that upset me more.

Christopher Whitfeld: The hearing aid will work as well as putting a plug in there.

Mattea Sarpa: It does.

Christopher Whitfeld: But it just keeps the ear open to all the softer sounds.

Mattea Sarpa: At home Id prefer to have some actual music on loud enough to cut out any other annoying sounds but not too loud that it upsets me.

Christopher Whitfeld: You havent got it with you today?

Mattea Sarpa: No.

Christopher Whitfeld: OK.

Mattea Sarpa: It is all still working well.

Christopher Whitfeld: Its really doing what an earplug would do but without blocking up your ear for softer sounds.

Mattea Sarpa: Now my own family since weve sort of discovered what it is seem to be not just sympathetic but they seem to have a better understanding that until recently I didnt even know what was wrong and it was difficult to try and reason why I was feeling like this.

Christopher Whitfeld: Well some of the symptoms that you get having your balance organ stimulated by loud sounds are similar to those that you could get from anxiety.

Mattea Sarpa: I think that I was actually probably getting anxiety as a by product.

Christopher Whitfeld: Oh yeah, you would.

Mattea Sarpa: Of the way I was feeling.

Christopher Whitfeld: Once youve learned that those situations are going to make you feel nauseous then every time you know youre going to be exposed to loud sounds you dont want to go. Im happy Mattea, just keep going on the way you are and let us know if you need anything.

Aviva Ziegler: Apart from the hearing aids there is another solution on offer for Mattea. Its a new operation to repair her fracture. But so far only a few dozen have been performed in Australia and although mostly successful, only three of Dr Halmagys patients have so far taken the plunge. It was the American expert, Dr Lloyd Minor who realised that sound amplification could be stopped if the damaged casing was given a new surface.

Michael Halmagyi: Not only was the condition defined by Dr Minor they actually devised the operation to fix it which is basically resurfacing the casing, its like putting new tiles on your bathroom floor. The only thing is the bathroom floor is the floor of your skull on which your brain rests so weve got to open your skull, pull your brain aside and put a new surface on top of your ear.

Often people who have this have a number of tiles that have lifted so it is not just one hole in the canal its a person has a very thin floor, very thin floor. We call it the roof of the ear but the roof of the ear is the floor of the brain and youre coming in from the first storey and trying to repair what is in fact the floor but what the persons complaining of is a hole in the roof or the ceiling. But youll see there are a number of holes and so the trick is to identify exactly that hole and the scans with the stereo tactical localisations now are good enough to do that.

Aviva Ziegler: In terms of the prognosis for her what would you say that was?

Michael Halmagyi: I believe the thing could be fixed by an operation but you know quite rightly shes quite sensibly tossing up the material risk of the surgery versus the unpleasant inconvenience of the condition. But the surgery has a material risk, in any sort of surgery theres a certain death rate. This canal is the top canal of the inner ear so it is actually right under the brain. We dont actually open the brain, we dont even open the sack in which the brain is contained called the dura, we just pull it aside. But theres always a risk, its a small risk but an irreducible risk.

But you know, it is a new operation and therefore you know you learn as you go and quite rightly you know shes waiting to see whether there are better ways. We think there may be an indirect endoscopic way of doing it but the problem with this canal is that you need to go through the skull to get at it at the moment.

Mattea Sarpa: The fact that they would have to move the brain aside to reline the inside of the skull is very delicate, so I feel at this point its not life threatening to me to be like this, I just have to learn to live with it.

Aviva Ziegler: Not a good thing to have to learn to live with though.

Mattea Sarpa: We have a very noisy environment and people dont realise how noisy our environment is with all the mechanicals and all the equipment and televisions and fridges and blinking lights and things. But it does make life difficult in certain ways that other people wouldnt even dream of.

Aviva Ziegler: So if you want to relax, what do you do?

Mattea Sarpa: I put earplugs in so that sounds are reduced to a muffle. Again if I have background music on it tends to cut the sharpness off any other type of noise. The times of day that I can relax are very late at night or very early in the morning. Im up at 5.30 to have a cup of tea or a cup of coffee in absolute silence. Thats when I can relax.

Aviva Ziegler: And is it absolute silence?

Mattea Sarpa: I dont think it ever will be. Even if I wore a helmet to block off every noise Id still hear my heart beating or my eye balls turning.

Aviva Ziegler: Whats the worst case youve ever seen?

Michael Halmagyi: I think Mattea is the worst, not only that shes an extremely intelligent and erudite person who can tell you about it. And so we have a very clear idea second only to her, we know exactly what she must feel.

One of the joys of this job is when you have someone like that who teaches you a lot, I mean we learnt from her what to ask for. Shes been very helpful in the advance of this field.

Mattea Sarpa: The problem is there are very, very few people out there that are aware of this and thats made me feel bad because people dont understand and they think that Im silly or you know, slightly crazy. Whereas before it was just me thinking that I was abnormal. I would like to help other people realise what an ordinary life must be like for people who hear too much.

Aviva Ziegler: Well some people would say it;s a wonderful thing to hear but to a degree.

Mattea Sarpa: When you hear everything there are just some things you dont need to hear, dont want to hear.

Aviva Ziegler: Do you have any favourite sounds?

Mattea Sarpa: Dr Halmagyi put me in a soundproof room and that was the best sound Id heard in a long time. But he had to switch the light off cause the light was making a noise and that caused him to be very excited that I could hear the light. So we had the light switched off and I sat in quiet, in absolute silence and it was the best noise I had never heard.

Norman Swan: That amazing documentary was made by Aviva Ziegler and the sound engineer was Mark Don. Our thanks to Professor Michael Halmagyi for his assistance.
By netchicken: posted on 15-10-2005

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