Otosclerosis - a disease of the middle ear

The ear is divided into three parts (see diagram above): the outer - external - ear, the middle ear and the inner ear.

The middle ear behind the eardrum is filled with air. Air comes from the back of the nose up a thin channel called the Eustachian tube. In the middle ear there are three tiny bones (ossicles) - the malleus, incus and stapes. These are the smallest bones in the human body.

The inner ear has two parts:

The cochlea, responsible for hearing

The vestibular system, responsible for balance

The cochlea is a fluid-filled chamber that looks a bit like a snail shell. The vestibular system is also filled with fluid and has five small sections that detect the movement of the head in different directions. The fluid in each of these sections moves as your head move and it is this fluid that sends signals to the brain via tiny hairs, which together with your vision and sensors in your joints, helps you maintain your balance and posture.

In a person with normal hearing sound waves enter the outer (external) ear and hit the eardrum, which causes the eardrum to vibrate. The sound vibrations pass from the eardrum to the middle ear bones. The bones then transmit the vibrations to the cochlea in the inner ear. The cochlea converts the vibrations to sound signals which are sent down the ear nerve to the brain which processes the sounds.

Otosclerosis is a condition that affects the stapes, one of the tiny bony ossicles in the middle ear. To have normal hearing, the ossicles need to be able to move freely in response to sound waves. Otosclerosis causes abnormal bone material to grow in and around the stapes.

The condition usually starts at the foot of the stapes, where it attaches to the cochlea. Bone is continually being broken down and remodelled but the remodelling process of the stapes becomes faulty. New bone is not made properly and gradually abnormal bone forms. In time, more and more of the stapes becomes covered by the abnormal bone growth. This abnormal bone reduces the movement of the stapes, which reduces the amount of sound that is transferred to the cochlea. Eventually the stapes becomes 'fixed' which causes severe hearing loss.

In most cases, only the stapes is affected. Sometimes the disease also affects the bony shell of the cochlea. Both ears are usually affected but sometimes only one.

The cause of otosclerosis is not known. Hereditary (genetic) factors are probably contributary and a gene linked to otosclerosis has been identified by a team at the University of Antwerp.

About 2 in 3 people with otosclerosis have other family members who also have this condition. It is also thought that fluoride may possibly help to prevent the condition. The number of cases in the UK went down after fluoride was routinely added to drinking water.

Also, a virus may play a part. People with otosclerosis may inherit a genetic tendency to develop the condition, but it may need a virus infection to 'trigger' the disease process.

Otosclerosis usually first develops between the ages of 15 and 35, but can develop in children. It is more common in women, who are affected twice as much as men. Pregnancy is not a cause but may make the condition worse so symptoms can often be first noticed during pregnancy.

Post mortem studies have shown that as many as 10% of people may have otosclerotic lesions in their middle ear, but apparently never had the symptoms, so they never sought a diagnosis.

People of African-American descent rarely have otosclerosis - it is a condition usually found in people of Caucasian or Oriental descent.

Hearing loss is the most frequent symptom of otosclerosis. The loss may appear very gradually. Many people with otosclerosis first notice that they cannot hear low-pitched sounds or that they can no longer hear a whisper.

In addition to hearing loss, some people with otosclerosis may experience dizziness, balance and co-ordination problems and tinnutus. Tinnitus is a sensation of ringing, roaring, buzzing, or hissing in the ears or head that accompanies many forms of hearing loss.

Other symptoms:

Paracusis is common. This means you tend to hear better when there is a lot of background noise. For example, when talking to someone in a pub or cafe that is full of other people.

Vertigo (dizziness and balance problems) develops in about 1 in 4 cases. This occurs when the balance mechanism in the cochlea (the semi-circular canals) is affected.

An examination by an Otolaryngologist (ear, nose, and throat physician) or otologist (ear physician) is needed to rule out other diseases or health problems that may cause these same symptoms.

Various tests may be conducted and these will include procedures to assess balance function as well as hearing. Do not be alarmed if you are referred for MRI and/or CT Scans - these will probably just be used to rule out other more serious conditions. You will also probably be tested by more than one audiologist throughout the diagnostic process

In the early stages of otosclerosis, or when the condition is mild, you might not need any treatment.

There is limited evidence that fluoride tablets may possibly slow the progression of the disease in some cases. This is thought to be mainly in the uncommon situation where the cochlea is affected. However, this treatment is not widely used.

Hearing aids can be very helpful and it makes sense to try them before considering surgery. However, otosclerosis will continue to progress and hearing aids will not stop you developing profound deafness in the long term.

If you do need surgery, then an operation called a stapedectomy can be carried out. However, if you suffer from tinnitus, surgery may not relieve this and if your inner ear is also affected, surgery may not improve your hearing either.

Alternatively you may of course decide to do nothing!

This operation aims to improve your hearing by replacing the stapes with an artificial plastic bone, called a piston. The piston helps to restore the movement of the ossicles, so transmitting sound into the inner ear.

A surgeon will remove most of the stapes bone, leaving just the portion called the footplate, which sits in contact with the link between the middle and inner ear - the oval window. A small hole is then drilled in the footplate and the piston is inserted so that it sits in contact with the oval window. At its other end, the piston is attached to the incus, the middle of the three ossicles.

About 85% of people find the operation a success and report a good improvement in hearing. However, there is a very small risk - about 1% - that fluid will leak from the inner ear and cause a complete loss of hearing in that ear. There is also a small risk of fluid loss in the years following surgery - for example, if you have a sudden blow to the head or sudden pressure change.

Occasionally the piston may move slightly and you will need a second operation to put it back into place. If you have this second operation there is a higher risk - about 5% - that you will lose your hearing completely in that ear.

Because of the small chance of serious complications, it may be a difficult decision to decide when to have an operation. Some people will persevere with hearing aids until the hearing becomes so bad that the hearing aids are not helping very much. Other people opt for surgery earlier so as not to need hearing aids.

When surgery is decided upon, the ear that is most badly affected is operated on first. This is the ear with most to gain if the operation is successful. This means that the hearing in the best ear is preserved in the small number of cases where the operation fails.

I found a lot of this information on Wikipedia:

Beethoven - the Composer

Howard Hughes - the pioneering American aviator, engineer, industrialist and film producer

Frankie Valli - lead singer of The Four Seasons, suffered from Otosclerosis in the 1970s but surgery restored most of his hearing by 1980

Richard Thomas - John Boy in "The Waltons"

Margaret Sullavan - the Actress

Queen Alexandra - wife of King Edward VII

Buzz Aldrin - Astronaut and the second man on the moon

Florence Henderson - America's favourite Mum in The Brady Bunch

And then of course there's ME!

Otosclerosis is even featured in a popular TV drama!

In an episode titled: "High and Low", avid viewers of CSI: Crime Scene Investigation learn that one of their favourite characters Gil Grissom, played by William L Peterson, starts to experience hearing problems. Subsequent investigations reveal that he has Otosclerosis.

It was no surprise to me when a hearing test in November 2007 showed that my hearing had deteriorated. I had already had a test three years previously, which showed I had slight hearing loss in my right ear but nothing to cause any alarm. However, if I had known then what I know now, then I would have asked for more detailed investigations sooner.

But, that November like many Mums where Christmas just "magically happens" for their families, I was too pre-occupied with the forthcoming festivities, in addition to being involved in a couple of projects that were very time consuming.

So, I left it and it was not until April 2008, that I realised that in addition to finding it more and more difficult to hear in my right ear, I was starting to experience some extremely worrying symptoms.

During this time I was experiencing a lot of stress, due to the actions of other people and I wonder if this was a contributing factor. I was finding it very hard to walk in a straight line and would accidentally bump in to people who were walking with me on my left hand side; I kept hitting the kerb when turning left out of our road when driving the car; I started feeling very dizzy if I stood up suddenly; I was having trouble with my speech and noticed a slight slurring of words sometimes; on a couple of occasions after just one glass of wine, which would not normally affect me, I thought the alcohol was going straight to my head; I found it hard to stand without worrying I was going to fall over; I had frequent headaches; my forehead would hurt if I tried to pick up anything off the floor; I kept dropping things; but worst of all I was utterly exhausted.

It became obvious that I had something wrong with me in addition to the hearing loss.

Update: 13 February 2009
I saw a Cognitive Behavioural Therapist yesterday. I believe he is a Psychologist and he told me that the stress I was under last year has undoubtedly caused the Otosclerosis. It is amazing how stress can cause a physical disease!

In April 2008 I went to my Doctor and she referred me to an Ear Nose & Throat (ENT) specialist.

Prior to seeing him, I had a very comprehensive hearing test at a specialist hearing clinic, which confirmed that I had significant hearing loss in my right ear - see Audiograms above, where it clearly shows the difference between the healthy left ear and the diseased right ear.

The audiogram was faxed to the ENT Specialist and I saw him two days later. I told him about my symptoms and he said I should have an MRI Scan on my head as a precaution. By this time I was starting to worry that I may have had a mini-stroke or that I was in the initial stages of Parkinson's.

The MRI scan showed no problems, so at least I could be assured that I did not have a brain tumour, so it was back to my Doctor to decide next steps. She had already mentioned that I would probably need to see a Neurologist, so when she said she would like to refer me, I was prepared. However, it was very scary all the same.

Of course I researched my symptoms on the Internet and there were various conditions that the symptoms matched, but not being a Doctor, I had no idea what the actual problem was. So all I could do was wait for the hospital to contact me to make the appointment.

We are only a short train ride from London, so at my Doctor's recommendation, I elected to go to The National Hospital for Neurology and Neurosurgery, which she said was the leading Neurological hospital in the UK. There I was seen by a Neuro-otologist and had a huge array of tests done. These included being spun around in a chair, filling my ears with water to see how dizzy I became (fortunately I was lying down!) and an Utenberger's Test, which involved me marching on the spot, with my eyes closed and my hands clasped out in front of me. Without knowing it I turned 60 degrees to the right and had quite a shock when I was asked to stop and open my eyes!

After around two hours of tests, I received the diagnosis: Otosclerosis in the right ear. However, the left ear seems unaffected at the moment.

I left the hospital with mixed feelings. Relief as it could have been so much worse, but also some concerns as it is likely that the hearing in my right ear will worsen. I also have to have Vestibular Rehabilitation Treatment (VRT) to retrain my brain to compensate for the balance and co-ordination problems.

I now realise that I have had the symptoms of Otosclerosis for quite a few years. I am not sure when they first started but I have noticed increasing clumsiness. I am often dropping things and frequently become absolutely exhausted.

In the past we have tended to attribute my tiredness to having had my last two babies relatively late in life. While a lot of my friends are becoming grandparents, I am still putting children through Primary/Elementary school.

I now have a digital hearing aid, which is amazing. At first I would hear every tiny noise and the hearing aid would crackle a lot, but thanks to 21st century technology my hearing aid has "intelligent noise reduction" - it is literally teaching itself to work out what I need to hear and what I don't. Apparently these digital aids are so powerful that it would take two PCs to match their capabilities!

Although I can now hear better, I feel that my life is to a certain extent "on hold" until I can start the physiotherapy - in the UK there is often a long waiting list and I just have to wait my turn - which I hope will help me back on the road to recovery. I am unable to work and neither can I play tennis. Gardening is also difficult because of bending down.

It is also difficult for the families of people who have Otosclerosis. The disease is "invisible", no one can see the problem. I always say it would be easier for my children to understand why Mummy finds it difficult to walk in a straight line without holding their hand or why I have no energy if I had a leg in plaster and had to use crutches.

I have good days and bad days but at least I now know that my condition is not life threatening.

Yes, it most probably is. My mother has severe hearing loss in both ears and may well have Otosclerosis, but at 80, I don't think she will want to be spun around in a chair or have her ears filled with water to find out!

My cousin has been wearing hearing aids since she was a teenager. My consultant has asked if I can find out more about her condition.

My eldest daughter has hearing loss in one ear. She is currently waiting for tests.

Lizzie, my youngest daughter has Auditory Processing Disorder - I have written a lens about her APD - and now we will start the process to find out whether she has Otosclerosis as well.

I keep nagging, oops I mean encouraging, my son to get tested. He will probably turn his mind to it once his cricket season is over! Then there is my other daughter, who we are keeping a close eye on as well.

Update: 06 October 2008
My eldest daughter has a significant hearing loss in her right ear. She has been told she has scarring on her right ear drum, which means it may be a different cause than Otosclerosis. She has now been referred to a Consultant and will undergo tests similar to those I had, including an MRI scan.

Update: 21 July 2009
Yesterday I consented to have Genetic Testing done. My blood will be examined for Genes that are known to be realted to my ear problems.

The results will take about 8 weeks but I will update when I have them.

Why have therapy?

The aim of VRT is to retrain the brain to recognize and process signals from the vestibular system in coordination with information from vision and proprioception. This often involves desensitizing the balance system to movements that provoke symptoms.

If the brain cannot rely on the information it receives from the inner ear, your ability to maintain posture and balance can become overly dependent on vision or on the information received from the muscles and joints. This is called proprioception.

This can lead to developing new patterns of movement to compensate for the change and to avoid head movements that are likely to create symptoms of dizziness and nausea. For example, you might develop an exaggerated sway of the hips as a method of balancing, you might turn your entire body rather than just the head when turning to look at something, or you might always look down at the floor to avoid what appears as a confusing swirl of activity.

Unfortunately, this can result in headache, neckache, muscle stiffness and general fatigue. You can also find the simplest of tasks difficult. For example I am unable to use a broom to sweep the floor. The last time I tried it I almost fell over. Also when out walking I need to have someone hold my hand to help me stay in a straight line. As for pushing a trolley in the supermarket, I have had to give up on that as I cannot help crashing into people.

Trolley rage is not a pretty sight!

What happens during VRT?

A qualified therapist will perform a thorough evaluation by observing posture, balance, movement, and compensatory strategies. An individual treatment plan will then be deivised that will include exercises to be performed both in the therapy department and at home, which combine specific head and body movements with eye exercises.

Treatment may also include increasing activities and exercise in order to strengthen muscles and increase tolerance for certain stimuli. Some of the exercise and activities may at first cause an increase in symptoms, as the body and brain attempt to sort out the new pattern of movements. But with time and consistent work, coordination and balance will be regained, headaches and fatigue should diminish and the symptoms of dizziness and nausea will disappear.

To be honest I do not know!

My VRT starts in October 2008, but I have been told it could be quite a long time before I can lead a normal life again.

I will see the Consultant again at the end of 2008, as I will be having six monthly checkups. If my left ear remains healthy I will probably make do with the hearing aid in my right ear. However, if it starts to deteriorate then I will have to consider having my right ear operated on.

I am one of those people who tries not to worry about something unless it happens - most of the time I manage it!

Update: 16 October 2008
I have had my first physiotherapy session. I was warned that I would need accompanying to and from the hospital as the exercises could trigger my symptoms and make them worse. However, by the end of the session I had a slight headache but did not feel too bad.

I was given some very simple exercises to do and I had to note how they made me feel and whether they aggravated my symptoms:

0 = No symptoms
1 = Mild symptoms
2 = Moderate symptoms
3 = Severe symptoms

The exercises were numbered and I worked my way through the list. They increased in difficulty, starting with just moving my eyes to look up and down to bending forwards and backwards with my eyes closed and then on to walking with my eyes closed, with another person alongside, in case I became unsteady or fell.

Once I had identified the exercises that I found replicated my symptoms, I was told I would need to start working my way through them. The first exercise has to be done three times a day for one minute and I cannot move onto the next exercise until the first one no longer aggravates my symptoms.

I will have a follow up appointment in six weeks.

Although I had a headache by the end of the session, I feel a lot more positive about how long it will take to recover. If the exercises work then hopefully I should be getting back to normal within just a few weeks.

26 October 2008 - One step forward, two steps back.....

I am getting on quite well with the exercises and am noticing that I am less exhausted and able to do more around the house, without tiring so quickly. However, the latest exercise: sitting in a chair and bending forward to pick something up off the floor has aggravated the symptoms. I know I have to persevere, but it is not particularly nice doing something you know is going to make you giddy for awhile.

02 November 2008
I seem to be starting to recover. I have days now where I do not feel dizzy. Although any sort of stress still tires me out. I am hoping that I will feel confident enough to start driving again within the next 2-3 weeks!

13 November 2008
One step forward, two steps back again! Huge relapse, no chance of starting driving again yet. Hit by stress (people close to me with problems) and it has knocked me back. Just starting to feel better again over the last couple of days.

19 November 2008
The Physio is hard at the moment. I have to drop something on the floor, bend down (from standing) and pick it up. Just one minute, 3 times a day. Every time I do it, it leaves me with a headache. Feeling weary.

01 December 2008
For the first time I am really low. Saw physiotherapist today and she admitted that the VRT is not necessarily to going to help me towards a full recovery. I may have to have the Stapdectomy.

I see the Consultant in two weeks.

16 December 2008
I went into London yesterday to see my Consultant. Even having my Hubby with me did not make it easy. Boy was it tough going through the Underground. Hoards of people rushing and pushing past you does tend to make you feel a tad disorientated. Fortunately, it was only one stop on the Tube from the mainline station St Pancras then a short walk.

My hearing was tested. The left ear is still OK and no deterioration in the right ear - all very good news. Had one neurological test that involved sitting in a chair, three electrodes being attached to my head, lights out and being spun slowly around. This was to check that there was not anything more sinister going on in the brain. All clear in that department as well.

The journey home was worse, it was the rush hour, so had to stand on the train for 20 minutes - not easy and I can confirm that the age of chivalry is well and truly dead!

In the New Year I will see a Cognitive Behavioural Therapist who specialises in dealing with the anxiety and gradual loss of confidence that I have - I am told that this is normal with this condition. He will give me strategies to cope with the down times and this should have a positive effect on the physiotherapy.

We have also been offered genetic testing, to see if I have any genetic "markers" that relate to hearing problems, as my eldest daughter is also having tests for hearing loss and of course Lizzie has APD.

But on the whole feeling a bit better about things - I was like a bear with a sore head the night before the appointment. Very, very growly!

30 December 2008
Well I survived Christmas! I cooked Christmas Lunch for 9 and on Boxing Day there was 14 of us. The last visitors (my eldest daughter and her dog) left this morning and yes I am tired. But I am pleased that my energy levels were good and so was my patience and humour!!

Every time I think I am making improvements there is a setback, but fingers crossed...

2008 was a difficult year for many reasons but I look forward to the New Year knowing that no one could ask for a more loving, supportive and tolerant husband and family.

02 February 2009
Having a head cold and Otosclerosis do not mix! I have not had a cold for years and this one laid me very low. It's hard enough trying to think straight at times but with a cold it is ten times as hard. However, I am over it now and due to see a Cognitive Behavioural Therapist next week. Another trip to London.

I am a lot better than I was but over the last few days have found it difficult to bend over without my head hurting. This may be a result of the cold. I am still tired a lot of the time, which gets me down at times.

13 February 2009
I am very tired and if I am honest I feel a bit low. I went to the Neurological Hospital yesterday and the travelling in and out of London does make me tired for a day or two after. But I don't think it was just the travelling.

I saw a Cognitive Behavioural Therapist, who is going to work with me to help overcome the anxiety and loss of confidence I suffer when I am out in crowded places. The discussion was very interesting, if not rather depressing. It may take me a year to completely get my confidence back.

I am not going to go into too much detail now, as I am so tired and will save it for another time. But I do have to get a book and start working through it. My next appointment is in six weeks and by then the Therapist, a great Scottish guy, wants me to be going into a supermarket on my own!

28 March 2009
Well I did it! In the last couple of weeks I have actually been in to two different supermarkets on my own and done a bit of shopping. Have not done a full weekly shop yet but will try to do it soon - one problem though, I have always hated doing the weekly shop!

There's a full update on my blog

21 July 2009
I have continued to see my Cognitive Behavioural Therapist (CBT) once a month and in June managed the trip into London on my own.

I have underlying depression but have more good days than bad. However, I will also be visiting my local mental health care team as my CBT thinks there are some underlying problems that are hindering the work he is doing with my confidence and anxiety.

Yesterday I saw my Consultant about the physical problems that Otosclerosis causes me - my symptoms have improved and my hearing has not worsened. It's still one step forward and two steps back but I am heading in the right direction.

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