Otosclerosis a hereditary cause of hearing loss

Otosclerosis is a condition of the middle ear which affects tiny bones called stapes in the ear. Hearing loss is usually gradual though in my case there was a sudden drop in hearing followed by a gradual decline. My example is that in April 1980 I attended a lecture room and sat in the back row and heard everything. After the holidays I returned and sitting in the same lecture room in October I had to sit in the front row. If I was to go in the same room now without hearing aids I would have absolutely no idea what was being said.

Soundwaves come in through your ear and arrive at the eardrums as vibrations which pass to the middle ear bones, the Malleus, Incus and Stapes. It is these bones that transmit the vibrations to the cochlea in the inner ear. It is the cochlea's role to convert these vibrations into sound which is then sent via nerves to the brain and therefore we hear.

The ear canals have fluids which flow around as we change our bodily position. As the fluid moves it is sensed by tiny hairs in the ear canals which send messages to the brain, down nerve endings which help us to keep our balance and posture.

It affects the stapes in the middle ear. In order to work effectively the tiny stapes bones need room to vibrate but with otosclerosis there is an extra bone growth which reduces the movement of the stapes. The bones keep on growing so that vibration continually reduces as does the amount of vibrations sent to the cochlea. It usually affects both ears so you don't have the luxury of one really good ear. In older age it can lead to a severe loss of hearing or total deafness in one or both of the ears.

Otosclerosis does seem to be hereditary. The experts say that one in three children of a sufferer will inherit. My mother and late Uncle had Otosclerosis, mum from the age of 16 but my uncle was not diagnosed until later life. I developed it from the age of 20 but could not persuade a doctor to diagnose me until I was 26! Women are believed to suffer more than men and pregnancy may make the condition worse. Luckily, I found that out after the birth of my three children!

Loss of hearing. Mine was quite dramatic. Over the University summer holidays I went from sitting in the back row of the auditorium to the front row- just to hear.

Tinnitus- now that is not much fun. I had it very badly at first but have learnt to mask it by focusing on external sources of sound. Tinnitus is noise in your head, but it seems to come from within and makes all sorts of whooshing and ringing noises. Sometimes I can hear my cats calling me but as I can see them sleeping I know it is not the case, its just the dreaded tinnitus.

Paracusis- This is the most relaxing and wonderful part of my condition. My hearing is much better where there is loud background noise. In fact I often cope better than others with good hearing.

Vertigo (dizziness and balance) problems, The high heeled shoes have had to go. Sometimes when walking downstairs it is scary. As an ardent historian I love to visit sites of interest with my son but some old properties are so scary, they seem to be so out of balance. My son knows when I need to grab his hand in case I fall.

Hearing Aids have improved over the years I have been wearing them and digital hearing aids have made sound so much sharper and clearer. Size wise they are now very small, packed with electronics. Technological changes happen all the time, My hearing aids are three years old and when they are replaced in two years time they will be smaller yet more powerful.The problem is the cost, my current pair were £1599 but the new ones may only be £1200, oh joy! However they were even more! Technology has advanced and now I have a pair of hearing aids that give even more clarity meaning that I can hear in the majority of situations- the only problem was the price, £1600!

Surgery- The stapes bone can be replaced by an artificial bone made of plastic or metal called a stapedectomy. It is often successful but it is a delicate operation with a small risk of failure. In my case the surgeon told me that I had cute little ears and he didn't fancy doing the operation- not surprisingly, I agreed! With surgery they operate on the worst ear leaving the "good" one of it goes wrong. In my case the "good" one is not "good" so until I can't manage the operation is out. Perhaps in my heart I am awaiting technological advances to secure 100% success.

If you need help with your hearing go to your doctor and press for a hearing test and follow up consultation with a hospital clinician.

If you are in the UK there is help and advice from:

The Royal National Institute for Deaf People www.rnid.org.uk

British Tinnitus Association www.tinnitus.org.uk

There are other organisations throughout the world and I recommend that you " google" "deaf associations" to see what is available in your country.