Leo is deaf, not completely, but quite deaf.  We hope that, in distilling here some of the tsunami of information that has flooded over us since his diagnosis, it may answer some of your questions and guide you in how you can support Leo in the future. They're not 'frequently asked questions' over on the left, but perhaps the questions we have been asking ourselves. And please don't hesitate to ask us anything about Leo. Danielle & David

How did we find out about Leo’s deafness?

In the last 7 years or so every child born in NSW has had their hearing tested before leaving the hospital as part of the SWISH program.  (I think other states have equivalent programs)  He failed the two tests he had while in his extended stay in the hospital but we were told that the SWISH test does throw up false-positives so we weren’t too stressed about the follow-up test at the audiologist.  With a postponement because of the second of Leo’s hernia operations, that test was done when Leo was about 10 weeks old and showed our optimism was misplaced and he did have a hearing loss.  Had we not had the SWISH test, it would have been at least many months before his hearing loss was spotted.

How the hell can they tell what a baby can hear?

They measure the activity in the parts of the brain that respond to signals from the ear?  As a result they can, and indeed prefer to do the tests on a sleeping baby.

How deaf is Leo?

A bit more technically than quite deaf, his hearing loss is severe in one ear (his left) and profound in the other.  What does that mean?  Without assistance, he couldn’t hear speech.  A profound hearing loss means he would hear a plane taking off and maybe a vacuum cleaner, but that’s about it.

How does the ear actually work?

I’m going to try and do this from memory without referring to anywhere else in the hope I can sum up the key things without straying into areas too technical.  I think I’ve got the basics right.  Here it is in a nutshell…

Sound waves travel down the ear canal where they vibrate slightly the eardrum, a skin-like membrane that seals the ear off.  To here, it is the outer ear.

Attached to the other side of the eardrum is the first of three tiny bones that are joined to each other.  I think the order is hammer, anvil and stirrup, but the key thing is that the bones amplify the vibrations of the eardrum.  This part of the ear is the middle ear and is joined to the nose, throat and eye by the eustachian tube.

At the end of this chain of bones is a piston like device that fits into one end of a snail shell looking inner-ear or cochlear.  The amped-up vibrations cause this piston to create amplified pressure waves that travel up the snail shell.  These waves are now strong enough for very fine hair-like structures along the spiral to be moved.  As the ‘shell’ gets narrower, the hairs are vibrated by waves associated with sounds of lower pitch.  The hairs are attached to nerves that send the signals to the brain.

It is an awesome piece of engineering and how this process works so instantaneously and with the precision for us to make out the most beautiful sounds is one of those miracles of (a) evolution (b) a creator [c] both [you choose!]

What isn’t working in Leo’s ears?

Leo has mixed hearing loss in both of his ears.  This term means that issues in both his middle and inner ears are contributing to it.  They can determine this by working out what his brain responds to for ‘normal’ sound travelling down the ear canal and also to vibrations sent to his inner ear via the skull from a vibrating tag they stick just behind the ear.  By working out the ‘volume’ that Leo starts responding to different frequencies to both these tests, and then doing some arithmetic they can work out what component of his hearing loss is due to middle and inner ear issues.

Some, but not all, of the middle ear hearing loss when he was tested was due to his middle ear being full of fluid rather than air as it should be.  However, this can’t explain all of it, but we can’t tell what the issue is yet.  Nor can we tell what is wrong with his inner ear, but it is apparently not damage to the nerve, which is good news.  Most likely the hairs inside the cochlear have not formed properly.

Will his hearing stay that way?

We are hopeful that with his middle ears clearing, his hearing will improve a bit.  Not miraculously, but a bit.  At the moment we are giving him a chance for it to clear naturally, but if that doesn’t he can have it drained by getting some grommets (tiny valve like devices) through his eardrum under a general anesthetic.

There are some conditions where the hearing continues to deteriorate, but we have no signs yet that indicate this might be the case with Leo.

Apart from that due to fluid in the middle ear, hearing loss almost never improves naturally. Some middle ear issues (such as the three bones not working mechanically as they should) can sometimes be operated on to attempt to improve their efficiency. However, the impact if things go wrong can be catastrophic! If this is even an option, the recommendation is to wait until at least mid teens when Leo can be involved in the decision.

What caused his deafness?

There is no answer to this one yet, but deafness (like a lot of things) is more common in babies that are born premature. There is no histroy of deafness in our families.

Are we upset about Leo’s deafness?

Were we disappointed when we got the news?  Of course.  It wasn’t what we were hoping for, and armed with his initial test results and a generic hearing loss booklet we ran through all of the worst case scenarios.

Fortunately we sought and received some excellent advice that put his hearing loss in context and explained that there is likely to be some improvement and that he can have a mostly normal life.

The way we have come to see it, Leo is deaf, there is nothing we can do about it but to do the very best we can for him.  We also feel that there is so much to him already than his deafness and we don't want him to be defined by it.

Also, at the risk of soun4yt worse place to be born into: we have the medical support and technology available to us, the education and the support of wonderful family and friends all around us.

Also, as our time in the neonatal intensive care sadly showed us, there are many worse things for babies than being deaf.

What are you doing going overseas with Leo?

As we write this, we are in Italy for our 2½ month experience in Italy.  It has been our dream for a long time to take advantage of Danielle’s maternity leave for an extended stay.

We had the tickets booked and everything when we got the test results (Remember that optimism we mentioned after the SWISH tests!) and it felt like our trip was doomed.

However, as we saw more and more of the wonderful, professional people that have helped us and Leo so far (Ear Nose Throat Specialists, Audiologists, Hearing Support Staff, Audio Technicians) everyone encouraged us to go.  The ENT specialist said the summer weather could help clear Leo’s middle ear naturally.

Other people were great at moving appointments forward so we could get his hearing aids and some of the training done on how best to interact with him before we left.

The point made to us was that Leo is still, especially in adjusted terms, quite young and his brain isn’t yet processing sound the way it will be when it is more critical at, say, around 6 months of age.  Therefore, people told us that it wasn’t too critical that hearing aids are tweaked to achieve their maximum.  We also need his middle ears to clear before they can do that.

Speaking of middle ears, they didn’t give Leo any trouble on the flights.

Will Leo (and the people around him) have to learn sign language?

Even if his hearing doesn’t improve from its current levels, with hearing aids, therapy and/or implants he should still be capable of hearing and speaking to a degree that shouldn’t require him to use sign language.

Are cochlear implants an option?

Cochlear implants are an option if the hearing loss is due to the hairs not working inside the cochlear. (One of the possible reasons for some of Leo’s hearing loss)  In this process, the malfunctioning hairs are shaved off (removing any natural hearing) and an implant inserted into the cochlear.  The signals come from a microphone near the ear and are passed through the skin to equipment implanted in a gap created where a piece of the skull has been removed.  The result is a sound that is far removed from what most people experience, but with training they can make a big difference to those they are fitted to. 

The implants are used for severe to profound hearing losses and we are hopeful that with the clearing of his middle ear, the use of hearing aids and all the programs of Audio Verbal Therapy (AVT) ahead of us will mean he won’t need an implant.  Cochlear implants involve ongoing operations for life as batteries are changed and hardware is upgraded.

Why did you choose that colour for his hearing aids?

There is a surprisingly large range of colours available for hearing aids.  It is our view that you can’t hide a hearing aid, especially on a bald baby, no matter how closely the colour matches the skin.  People told us that they can get lost amongst clothes and pale blue features a lot in Leo’s current wardrobe, so we chose a Cobalt Blue, the same colour as the vertical band on the far left and right of this page.

It was at least better than the poo-brown he had for the first week (see right) while they waited for the best hearing aids for Leo to arrive.

How do the hearing aids work?

Simply, they have a microphone that picks up sound, amplifies it, then channels it into the ear canal.  Modern hearing aids do a lot more though.  They can amplify certain frequencies (or pitches) of sound selectively to match the hearing loss of the wearer.  They can also try to selectively amplify speech out of the clutter of background noise that is around.  There are even Bluetooth enabled hearing aids that allow the wearer to directly receive input from TVs, ipods, etc.

What does it sound like for Leo with the hearing aids?

We really will never know as only Leo truly knows what makes it to his brain. However, if you're around and we are putting in/taking out Leo's hearing aids you are welcome to put one to your hear and you can get an impression of what the sound is like going into Leo's ear.

To us it is very clear, although a little 'tinny'. You also quickly get a picture for all the other incidental background noises that get picked up and amplified as well.

We have a hope that once we have a clearer picture of the profile of Leo's hearing that there will be some magic box or software or something that will be able to demonstrate to us something like how Leo hears the world. If there is such a thing and we do it, we will let you know.

Did having the hearing aids make a difference?

We believe so.  We can see that Leo responds significantly more to our voices than he does without them.  However, he is still small and babies this age can be a bit unpredictable as to what they respond to anyway.

What helps Leo to hear better?

The advice we have received is probably all common sense: reduce the background noise as much as possible, talk in a normal voice about 40cm in front of him, play games and sing songs just like you might do with a normal child.  There is no need to raise the voice as it gets distorted and can overload the hearing aids.

How do infants cope with hearing aids?

Good question.  So far, Leo has been great with them.  He can whinge a bit as his ear gets tugged and the mold gets shoved in and the aid hooked over his ear, but once it is in, he seems to respond positively to the improvement in sound. 

As of mid-October 2011 he is just beginning to show the coordination that will soon lead to him being able to pull them out himself.  If it comes to that, there is a cap that he can wear that leaves him resembling a WWI pilot but limits the ability to fool around with them.

Has it being expensive?

The happy truth is that we have hardly spent a cent.  Well, we paid to see a couple of ear, nose & throat specialists after getting the test results back, but we are very fortunate that everything to do with the hearing aids hasn’t cost us one cent and won’t until Leo is 26.  Also, earlier in year the federal government announced a $12 000 for therapy for a range of disabilities including deafness that will help Leo get exactly the help he needs.  Another article reports that for every dollar invested in such therapy returns two dollars to the community.

Will Leo speak with a funny voice?

The advice we have received suggests that with the modern therapy available Leo will be able to speak just like a normal child.

What support is available to help Leo speak properly?

We have started with the Shepherd Centre, a charity that has a philosophy that with early intervention, plenty of focused therapy from the parents and the best technical assistance, the vast majority of deaf kids should have an almost completely normal life.  Over 90% of children that go through the Shepherd Centre attend a normal school for Kindergarten.

We are mindful that this is one of a range of approaches that people may have to deafness and that there is a vibrant and unique culture in the deaf community amongst whom deafness is not seen as a disability, but rather part of the diversity of humankind.

As an example of these views, there was this recent article in the Sydney Morning Herald.

Will Leo go to a normal school?

That’s our hope.  He will certainly have hearing aids and he may us a FM transmitter that involves the teacher wearing a microphone that transmits the voice directly to the hearing aids of the wearer.  In fact, David has worn such an FM transmitter for a student in one of his classes.

How is Max coping?

Very well, we believe.  He is curious and interested in Leo’s deafness and is excellent at following instructions as to what assists Leo the best.  In fact, he corrects us if we over complicate the actions to some of the songs that we were shown by The Shepherd Centre.

What can I do to help?

Leo wearing his 'magic ears'

If you have made it this far in this webpage, that’s a good sign!!

Seriously, we firstly ask that you try to treat Leo exactly as you would any other kid of his age.  You might like to use some of the tips for helping him to hear with his hearing aids and if you’re interested, we would be happy to share with you some of the therapy exercises we have been taught by The Shepherd Centre.  Don’t worry, they are designed to be as fun and engaging (for Leo at least) as possible.

We welcome it if your children are curious about Leo's hearing aids. They are naturally curious and we would enjoy explaining how they work.

We are also mindful that Leo's experience with his hearing aids stays as positive as it can. (Maybe this is horribly transparent, but we refer to his hearing aids as magic ears.) We respectfully ask that you join us in being as positive about his hearing loss and hearing aids as you can.