Paula Hijne (58) experiences daily the impact of a poorly functioning vestibular system. She now knows all about it. “Your vestibular organs are part of the sense ‘balance’. Many people don’t know that it is a sense, but it belongs in the list of sight, hearing, smell, taste and touch. Your vestibular organs are on both sides of your head, in your inner ear, right next to your hearing organ. Together they form the labyrinth in the inner ear. If there is something wrong with your vestibular system, it disrupts your balance.”
For a long time Paula was a primary school teacher, but when she got the balance disorder Ménière, she had to let that passion go. “I’ve suffered from hearing loss all my life. In the beginning it wasn’t that bad, but later I came home more and more tired after a day of work. A class full of high-pitched children’s voices is quite difficult if you can’t understand them well.”
‘You really fall over’
In 2000, Paula got the flu, which left her with tinnitus. “Tinnitus is also called ringing in the ears. It started as a soft noise for me, but it got louder over the years. Combined with the hearing loss I already had, hearing well became almost impossible. I bought hearing aids and hoped that my problems with that were over. The opposite turned out to be true.”
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Less than two weeks after Paula started wearing her hearing aids, she started experiencing severe bouts of vertigo. “This is a specific form of vertigo where you can’t stand up straight. You really fall over and have no control over it. Those attacks kept coming as soon as I put the hearing aids on.”
“I could no longer wear the hearing aids and eventually stopped wearing them, but the seizures continued. To this day I am still unstable on a daily basis: I can only walk and listen with concentrated attention. It is with a heavy heart that I say goodbye have to take my job in teaching.”
Complete chaos
Paula remembers her first Ménière attack well. “It was at school, during a team outing. I had noticed for a while that I was unstable and felt pressure on my ears all the time. I cycled home, went to bed and immediately fell asleep. The next morning I couldn’t anymore. I had lost control of my whole body. I was also nauseous, I was cold and I was sweating a lot. I couldn’t go to the toilet on my own and in my head it was complete chaos.”
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Because it was the first time, Paula didn’t know it was a vertigo attack. “It was very scary. I didn’t know what happened to me or if it would pass. It was almost impossible to lie down, it sometimes took 5-6 minutes before I could lie flat again after getting up. In the end the attack lasted 12 hours. That long It took until the chaos in my head was finally over. After that attack I went to the doctor and four months later I was diagnosed with Ménière’s disease, a condition that causes vertigo, ringing in the ears, hearing loss and damaged vestibular organs .”
‘Learn to live with it’
After the diagnosis, the doctors could do little for Paula. “My ENT doctor said: ‘Learn to live with it’. I thought that was very bad. I understand that there is no immediate solution for Ménière, but it would have helped me if I had some more information about the disease. How do you deal with having something like that?Give me at least a book, some sort of guideline.In recent years, I’ve found that this lack of help is because little is actually known about balance disorders—and balance at all. ” In the end, Paula wrote it herself guideline.
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Wanting to know more about it, Paula did a lot of research on balance herself. Two ENT doctors from Maastricht UMC+ have helped with this. “Balance is your most important sense to be able to move properly. Many people do not realize this. During my research I discovered that a clear, Dutch book has never been written about our vestibular organs and the equilibrium system. It is my mission to improve the sense I think that information about such an important sense should be included in the curriculum of both secondary schools and (para)medical training.”
Marja (67): ‘Happy when I was diagnosed with DFNA9’
Marja is almost deaf and has almost no balance.
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Marja de Children (67) has also been diagnosed with a balance disorder. She has DFNA9: a rare, hereditary condition in which both your hearing and balance organs slowly fail. “I am actually completely deaf, but thanks to a cochlear implant I can still hear reasonably well. This implant is a kind of hearing aid that converts sound into electronic signals. My vestibular system has also deteriorated sharply, so that I regularly lose my balance and no longer can walk straight.”
‘Like I’m drunk’
‘Walking down the street’, is what Marja calls it. “People sometimes think I’m drunk, but that’s not the case. I have a lot of imbalance due to my condition. For example, I can no longer get on a normal bicycle. For years I rowed, but now when I try to get into a rowing boat , then there is a good chance that I will fall into the water. I also find it difficult to climb stairs and crooked sidewalks. I really have to pay attention, otherwise I will lie on my nose.”
The first symptoms arose when Marja was 50 years old. “My hearing had deteriorated for a while, but at a certain point my balance also became less. It started with small things, such as getting on my bike. Or finding my way in the dark. Then I quickly became disoriented and I couldn’t As the years passed, the disorientation got worse. DFNA9 is a progressive condition, so slowly you become deaf and your vestibular system loses its function completely.”
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DFNA9 is a hereditary condition that has been in Marja’s family for generations. “My father had it and I come from a family of six children, three of whom have it. I only found out late that what I have is hereditary, because the condition was only discovered in 1998. Little is known about our vestibular system , so I was happy when I got a diagnosis and because of that I know what to expect. Because we know so little about it, there is often little that can be done about it and that is annoying. Most ENT doctors also don’t know what to do along.”
Better independent walking thanks to balance belt
Despite her complaints, Marja makes the best of it. “I have found a number of things for myself that help me to improve the quality of my life. For example, I did a fall prevention course and I do a lot of Tai Chi. Because of this I learn to stand stronger, even on one leg. I’m not going to be a star at it, but it helps me control my movements.”
Marja has also had a balance belt for six months. This gives a kind of electric shock that corrects your posture. “When I wear that belt, I walk more firmly and straighter. I really enjoy that. Normally I often walk on people’s arms for strength and balance, but the balance belt makes it easier for me to walk independently.”
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Normal cycling is no longer possible, but with her tricycle, Marja and her husband cover a lot of kilometers. “I followed a course to be able to cycle well on my tricycle bike. Cycling gives me a lot of freedom and that’s why it makes me happy. Isn’t that nice? Despite my limitations, I’m still a happy person. That’s because I’m still alive. Of course I sometimes feel frustrated and think: ‘Why can’t I do all those other things?’ But then I always look at how it can be done.”
Recognize what you can’t
Marja likes to transfer her life force and energy to others. “Don’t give up. Yes, it’s annoying and of course I shed tears, but life goes on. Acknowledge what you can’t do. Accept that and look for a solution or another way to be able to do something I noticed that especially with cycling. I still cycle, although at first I thought I would never be able to do that again. I’m proud of that.”
Although there are currently few concrete solutions for DFNA9, Marja knows that doctors are developing a ‘vestibular implant’. “This is an artificial vestibular system. It is expected that this will be available to people like me in five years. Perhaps I can then have surgery and have my hearing and balance corrected. It is my heart’s desire to undergo this treatment, so there I very much hope so.”
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