Introduction to the Implant Clinic

Tuesday was the day. The next step into the cloud, the next station along the line: our introduction to the Implant Clinic. I will confess that I have been to the Clinic before, but that was about 5-6yrs ago with Jarrah, and we didn't get past the 1st appointment as they felt he was functioning too well to be a candidate. This was quite different. I knew there was little chance Jonica would not be a candidate, although there was still a nagging doubt because of how well she functions relative to her audiograms. Still, I knew we were there with intent, not in the air of investigation.

A bus ride later, and we were there. Ah, Mater Audiology, how I have not missed thee. Like riding a bike really, once you go there enough times you never forget. The difference this time was being there with a 3yr-old instead of a newborn, so we stayed within the dept rather than going up various combinations of lifts to get to where we needed to go. But the same facilities, the same waiting area, the same collection of parents with tiny children waiting their turn...

After paperwork and a brief toilet stop (always count on the 3yr-old needing to go to the toilet as soon as it's her turn to be seen!), we met the audiologist and speech pathologist for our appt. Sadly they hadn't received Jonica's history so I gave a brief history of everything, with particular emphasis on what had happened recently. They did have her latest audiogram, so at least they had a baseline from which to judge what they saw in the appt.

Once they had a picture of where things were up to, we went to the sound booth to do some testing. At first Jonica was very unsure of the *enormous* headphones they wanted her to wear, but then when i suggested and they offered the usual inserts instead she decided that was ok. *Phew!* nothing worse than a child who won't participate in the testing you're there to do! Fortunately Jonica is a very reliable tester, her responses are very consistent and she stays on-task well, so we got another full set of air-conduction results.

Right ear: mostly stable, only some variation at 3kHz.

Left ear: all frequencies down again by 10-20dB

250Hz: 100dB

500Hz: 100dB

1kHz: can't remember, about 110dB?

2kHz: can't remember, about 115dB?

4kHz: no response 

What?!?

I must confess, that really caught me by surprise. I knew her left ear was bad, and I knew it was really in implant territory, but I hadn't expected it to deteriorate again, in only 4wks. This time, nothing was better than 100dB, and in fact she gave no response *at all* at 4kHz. Her high frequencies appear to have disappeared altogether. She's usually so stable between her big drops, I was just so surprised to see those results...

So, there's no question really. She's certainly a candidate. That doesn't mean we *have* to go ahead, but if she's struggling, why wouldn't we? Why let her struggle just because she's bright enough to get by? What if her right ear suddenly disappears, what then? And why shouldn't she have the best chance, if it's possible? I'm beginning to feel like we really don't have too many options here...

More appointments. Following up from this one, we'll be back at the clinic on Tues 10th of June for Speech assessments at 9am, followed by more audiology at 10:30. We're waiting to hear back from the surgeon about arrangements for a CT scan to be done under general anaesthetic. Then there's likely to be more ENT, followed by surgery. Then it really begins! 2-4wks after surgery there is switch-on, when they actually turn on the device, then an unbelievable schedule of appts until 6mths post-switch-on. Seriously, can't they put all this on ONE piece of paper? I've had to piece this together from several sources, and I'm still not convinced I've got everything. Maybe that's what I'll do, put it all on one piece of paper...

Next appts: Tues 3rd June Optometrist (all kids, just checking...)

                   Tues 10th June Implant Clinic Speech & Audiology

Back to ears...

Having decided that an implant was really the only viable option for Jonica's left ear, we had an initial appointment with the Implant Surgeon. Of course this involved getting the whole family into the city by 8:15am on a Monday morning, so that was an adventure in itself...

We asked questions, Surgeon described how an implant works. After viewing her audiograms he agreed that an implant was the best solution. Again, not urgent, but best not left too long. But nobody seems to be able to give us any idea about the process, what happens next, what even needs to happen. I'm sure there's some sort of standard-ish procedure, but nobody seems to be able to tell us what it is!

Around the time of this appointment, I received a letter from the Implant Clinic, telling us we had an appointment on Tues 27th May, at the hospital's audiology department. I just love it how they consult families about these appointments. One phone call, what days work for you, then they just make the appointments for you. Anyway, looks like this is starting to get real.

The following Friday, 23rd May, we had an Aus Hearing appt already booked. Back in February I had ordered Jonica an FM system cos I thought it might help her at Kindy. Basically, the teacher wears a microphone transmitter and Jonica has a receiver that plugs into her hearing aid. That way, the teacher's voice goes straight to her hearing aid and she hears instructions and directions more clearly, no matter where in the room she is. After all the appts in April, we decided to keep this one, and turn it into a full FM fitting rather than a hearing check.

The FM fitting was a resounding success. They're actually no longer FM's in the true sense of the word, they no longer use FM channels for transmission. They actually use 2.4Ghz wireless transmission, which solves a bunch of technical issues found with FM. In essence, each transmitter creates its own ad hoc wireless network and receivers can join that network and listen in to the stream of sound being sent from the microphone. This means there are no crosstalk issues as there were with FM systems (two systems on the same channel in close proximity would hear each other), and it's now a digital signal so it's even clearer in the hearing aid. The receiver automatically detects and joins the network when it becomes available, and the transmitter can still transmit an FM signal at the same time as the wireless signal. Brilliant. FM receivers find the channel automatically too, so when I pick Kaelen up from school while wearing the transmitter, his FM receiver automatically connects to my transmitter, no button pushing required. It's simply amazing. It's how technology should be - it just works!

Technical distractions aside...I used the transmitter with Jonica all day on Friday, and it cut the instance of "What?" down by about 75%. Which is amazing, because that was probably the most common word coming out of her mouth. If she couldn't see my face, she was really struggling to understand anything I said, even in good listening environments, and I was getting really tired of (a) repeating myself, (b) yelling, and/or (c) speaking like a tortoise so she could understand me. I used it in the car on the way home, and it was like a dream - I could talk in my normal voice and she understood me! Because she really only has one functioning ear at the moment, I'm using the transmitter all the time to give her the best access to our speech. Sure, she might become dependent on it, but I'd rather she understand me than wear herself out trying.

Next appt: Tues 27th May Implant Clinic Audiology & Speech appt

Teeth?!?

So this next appointment, Mon 12th May, may or may not have had something to do with hearing. Jonica had an appointment with a Paediatric Dental Specialist.

Back in January the kids visited our regular Dentist, and she picked up some hypoplastic enamel on Jonica's 2yr-old molars. We were given a referral to the Dental Specialist but it wasn't urgent so I didn't do much about it. The Dentist asked at the time is her hearing loss what part of a syndrome, but since we haven't found anything else I kind of shrugged my shoulders. Then, once her hearing dropped in April I began to wonder again, so I thought it would be good to look into this just in case it was connected to something.

Thank goodness we weren't late for our 10:30am appt, we were seen promptly at 11am :-\ A fairly standard appt, they were great with Jonica considering her age and having to look in her mouth, confirmed that she has hypoplastic enamel on all four 2yr-old molars and they need stainless steel caps. Under General Anaesthetic. Hmmm...

Having done some more reading since this appt, I don't think it's actually connected to the hearing loss. We don't have to have the caps done right away - although sooner would be better - so I'm going to see if there's any way they can do the teeth and the implant at the same time. Every GA comes with risk, so the fewer the better...

Next appt: Monday 19th May, initial consultation with Implant Surgeon

A New Journey

Oops, about 4 appointments have actually passed since I last wrote here...and it's going to get busier. I'll try to be more regular!

First, 28th April Australian Hearing follow-up.
This appointment was to confirm the results from the 11th April tests showing right ear recovery and left ear deterioration. Jonica was absolutely brilliant as they did a huge range of testing and she remained engaged the whole time, even as lunchtime approached. We did air-conduction on both sides at the usual 5 frequencies, we did unmasked bone conduction at 4 frequencies to get the response from whichever ear is better, then they did masked bone conduction on the left side at 3 frequencies to get an idea of what the left ear was doing. All up nearly an hour of testing, and she was super-reliable and compliant. What a champion!

The results were a mixed blessing. Middle ears were clear on both sides today, so the fluid on the right side has mostly gone, just a blocked eustachian tube still. Here they are:
Right Ear, air conduction
250Hz - 65dB
500Hz - 70dB
1kHz - 65dB
2kHz - 65dB
3kHz - 75dB
4kHz - 105dB
Slight improvements at 250Hz & 2kHz though still within the margin of error, significant improvement at 4kHz, all due to clearing of fluid from middle ears, as expected.

Left Ear, air conduction
250Hz - 80dB
500Hz - 90dB
1kHz - 95dB
2kHz - 105dB
4kHz - 120dB
Slight improvements at 250Hz & 1kHz though still within the margin of error, and further deterioration at 4kHz

Umasked Bone Conduction, response from the better ear
500Hz - 30dB (!!! Previously 55dB)
1kHz - 40dB (!! Previously 50dB)
2kHz - 55dB (Previously 70dB)
4kHz - 80dB (Previously 75dB)
These are very odd results, as they include significant improvements on previous results. Since we also isolated the left ear and none of its results were better, we can reasonably assume that this is what her right ear can do.

Masked Bone Conduction, left ear only
Masked bone conduction is interesting. We've never had it done before, so i was interested in how it worked. They use the same test frequencies and the same bone conductor appliance, but they have to keep the other ear busy so they only get a response from the target ear. To do this, they put an air conduction insert into the other ear and play a beach-like sound (undulating white noise, perhaps?), then send the test frequency only to the bone conductor on the target ear. Amazing! Anyway, technical distraction...
500Hz - 80dB
1kHz - 75dB
2kHz - no response at 80dB
Bone conduction can only be tested up to 80dB, so at 2kHz there was no response at the maximum level. 4kHz was not tested, probably because since there was no response at 2kHz and her air conduction gets worse in the higher frequencies, they wouldn't expect any response at 4kHz.

For those of you who prefer a visual representation, here's the audiogram:

So, this confirms it. The only thing to do is to consider an implant for Jonica's left ear. She can still wear her hearing aid, and they adjusted it while we went and got pizza for lunch, but it's really not going to do enough for her. She's especially lacking in the high frequencies and mostly at 4kHz, which is where a number of speech sounds sit, so she's missing sounds like "sss" and "t". Her speech quality is still deteriorating, so it's becoming clear that an implant is the way to go.

Cranial surgery on my 3yr-old. Fantastic. How do I explain this to her? How do i tell her that they're going to put something in her head to help her hear without scaring the daylights out of her? She's aware enough to be frightened by new things, strange things, things she doesn't understand, people she's not familiar with, so I really need to discuss this with her, I can't just send her off to surgery without telling her something about what's going to happen. She knows her left ear is quiet, she knows it doesn't work like it used to. A few delicate conversations coming, methinks.

I think I'll do the other appointments as separate posts...

Next appt: Mon 12th May Paediatric Dental specialist for Jonica. I will explain...

Partial Recovery!

So much has happened in the last few days...where to start...

After the adventure that was Wednesday, we got up Thursday morning and got on with the day...Jonica was going to Kindy again, the boys and I were going to meet friends...when I noticed at breakfast, with hearing aids in, that Jonica seemed to be hearing and interacting with Kaelen across the breakfast table, about 3ft! I asked her if she could hear me, and she nodded. I asked her if she could hear Kaelen, and she nodded! She could hear something! To confirm *what* she was hearing I did a quick hack Ling sound test - put my hands in front of my face so she couldn't see my mouth - and she correctly identified 5 out of 7 sounds!

Recovery!

I can't describe the relief that I felt in that moment. Although I knew it could go again as quickly as it returned, I was just thankful that something had returned! I kept an eye on her through the morning and noticed that at about 5m there was still no response, so she clearly didn't have everything back, but she was no longer in her own little world, she was chatting again, and you could have a conversation with her at about 1m. I still gave her the medication as prescribed, but was pleased to be able to tell Kindy that she was doing better that day. 

We kept our Aus Hearing appt as scheduled on Friday, cos I knew she wasn't 100%, I knew she hadn't got everything back, so it was important that we knew exactly what she did have so we knew where to go from here. She was brilliant at the test and listened very well for a long time, they got full air conduction results for both ears...and what they found was somewhat unexpected.

Her right ear, the one with the fluid behind the eardrum, appears to have recovered to the same levels as before the drop on Monday, the only variation being what they would expect from fluid behind the eardrums. But her left ear, the one with no fluid, was 20-40db down across all frequencies. This means that her best frequency in that ear is 80db, which is about as loud as a shopping centre food court. All other frequencies were 100db or worse, akin to a domestic vacuum cleaner. No wonder she wasn't hearing anything on Tuesday & Wednesday if both ears were anything like that! Of course we don't know what her right ear really did on Tues & Wed, but she was so unresponsive that it would be hard to imagine it was much different to the left ear.

So the next step is to confirm these levels in a week or so, and if they seem to be permanent then we have some decisions to make. At those levels in her left ear, a hearing aid is pretty much useless. If those levels don't improve before the next test, we are looking at having to consider a cochlear implant for her left ear. A whole new learning curve for me!

If her left ear recovers fully before the next test, I would be somewhat hesitant to go for an implant. Yes, her hearing fluctuates somewhat, and consistent hearing is important for speech & language development. But if this is all it is, 5 bad days every 2 years which then recover, I'd have to think very carefully about the decision to implant. Implants are fantastic for those who need them, but they do destroy all remaining natural hearing in the ear. That's fine if there is really none to work with anyway, but in Jonica's case, if this was how it kept going with just a few bad days every couple of years, I'd be very hesitant to blow away her residual hearing too soon. 

Not much to do about it now...just wait and see. I'm in Tasmania with Kaelen this week anyway, visiting my sister and her children, so plenty else to do and think about!

Next appt: Mon 28th April 11am - Aus Hearing follow-up for Jonica

Where does it go?

My posts seem to be fewer and further between these days, and for me that's a good thing. That means that things are stable, kids are moving along, doing what they're supposed to be doing, and life is generally good. I guess that means too that the creation of a post signals bad news...

There have been some adventures. Jarrah has had ongoing ear infections again, all in the left ear, so he spent most of 2013 with only one hearing aid in. He still had a grommet in that ear, so once it was removed things settled down quite a lot. I think there's still a hole in the eardrum (better check!), but that is apparently closing over so we seem to have avoided the need for further surgery there.

Kaelen is stable as ever. His language is progressing nicely, his intelligibility is improving, he's loving Prep and has recently taken to the piano. He's very self-motivated (when he wants to be!) so has made excellent progress in a short space of time for a little guy, and was sure to point out to me the other day a note on our piano that has gone out of tune with recent wet weather. His (amplified) ears work just fine!

Jonica has been doing incredibly well. Her hearing had settled after the last drop 2 years ago, and although she has very little hearing in the high frequencies (4kHz = 90-110db), she's been doing amazing things with what she has. Both her speech pathologist and her teacher of the deaf have been quite surprised at what she can do/hear/say, given the nature and shape of her loss. She's started using possessive pronouns which involve an 's' sound on the end, she's been putting lots of nice 't' sounds on the ends of words and we've been working on 'f' and 's' at the start of words - much more difficult and perhaps a little advanced for a hearing-impaired child of her age, but better to make her aware of it now rather than wait til there's a problem.

Well, now we have a problem. On Monday evening she said to me suddenly, "Mummy, my ears are quiet!" The kids normally say this if their batteries are low (or dead!), so i checked her hearing aids - nope, they seemed to be working fine, no blockages, batteries were fairly new. She hasn't always been reliable with this, so I didn't think too much of it. However, the next morning as we were having breakfast, I noticed that she was having real trouble understanding me, with words and sounds that she hasn't struggled with before. Then as we were getting ready for Kindy etc., I called to her a couple of times in ways that would normally have been fine and there was no response. Hmmm... I mentioned to Kindy that I thought she wasn't hearing too well, and to please use lots of gesture and tap her shoulder to get her attention etc. They were grateful for the warning, and agreed at pickup time that there was definitely something not right.

It wasn't until I really put some effort into observing her and our interactions that I discovered how bad things really were. In the morning I had called to her from about 4 feet away and got no response at all. In the evening Jarrah sat at the table less than 3 feet from her and started talking to her - no response. He moved closer, less than 2 feet away, and spoke loudly. Some response, but no real understanding of what he had said. After her bath but before I put her hearing aids back in, I called her from about 1 foot away, no response. I immediately put her hearing aids in and tried again, this time with some response - which means she looked up at me! We brushed her hair and I spoke point blank into her hearing aid; she understood some of what I said. The cat came into the room, meowing for his dinner, and he's a pretty noisy cat. She couldn't hear him. This morning we got out her little violin, she had a turn then I had a turn. She got quite upset when she couldn't hear me playing from about 1.5 feet away.

Oh dear.

I had a look in her ears to find quite a bit of wax. Knowing that the GP wouldn't do anything about it, I decided it was best to try to see our ENT as soon as possible to (a) get the wax cleared out, and (b) rule out any other medical issues which might have caused such a loss. Our ENT is a champion. I left a message on their answering machine at 8am, and at 9am they were calling me with a squeezed-in appointment. I had to almost jump down the phone at the GP's receptionist to get them to fax a referral through TODAY rather than jump through their hoops, but the ENT's receptionist asked no questions, just immediately offered a time. Today! Wonderful. Exactly what the Dr ordered...

ENT managed to get all the wax out without even a flinch from MissJ! Found some fluid behind one eardrum but nothing else obvious to cause such a change. Gave us some antibiotics for the fluid and a short course of steroids as an anti-inflammatory...and recommended an urgent hearing test. I had notified Aus Hearing of our situation before seeing the ENT and they had already mentioned an urgent review so that was my *next* phone call after the ENT appointment! Friday 8:15am. Excellent.

So, where does all this leave us?

* Daddy is currently overseas, arrives home midnight Friday
* At 1pm Friday, Kaelen & I fly to Tasmania to spend a week with my sister and her children, so Jonica & Jarrah will be looked after by my parents for some amount of that time
* Hearing test Friday will tell us more exactly where things are at, and how far her current hearing aids will stretch
* In the back of my mind is the possibility of Jonica needing cochlear implants.

Last time her hearing dropped, 2 years ago, it did appear to recover somewhat after about a week. There is no guarantee that this will or will not happen this time. All fluctuating hearing loss is unpredictable, both in time and magnitude, so there is every chance that she will never recover from this, just as much as there is every chance that she will. But we don't know if, or when. Her speech was already affected less than 24hours later, I can see some of her auditory memory is not quite secure enough yet for her to have full use of all the language she normally uses, and she's not chatting like she normally does when she plays. This afternoon we were at a friend's house after seeing the ENT, and she was playing by herself in another room, completely silent. Normally we can hear her very easily and can tell exactly what she's doing, but not today.

Where does it go?

When a deterioration like this happens, where does the hearing go? I know that's a stupid question, but there has to be some half-reasonable answer. Something must change, or cause a change, which impacts her hearing. What is going on?

Stay tuned for the next instalment...and huge thanks to my friend mc for offering unprompted to look after the boys while Jonica and I went to the ENT today. So much easier with 1 child than with three, as she well knows, so THANKYOU!


Next appt: Friday 11th April 8:15am Aus Hearing

A new type of marathon...

I've learned something over the years. Always allow about an hour more for Aus Hearing appointments than the diary says you'll need. Sometimes I still forget, but these appointments reminded me of this truth.

Mon 29th Oct was a follow-up for Kaelen (Mr4) after having slightly blocked ears back in August. Afternoon appointments are a bit of a nuisance now because I have the 3pm school pick-up to work around, so a 3pm appointment really means have lunch at 11:30, get Mr4 & Miss2 into bed by 12, get them up at about 2 or shortly after so we can arrive at school by 2:30, sign Mr6 out and still get to our appt close to 3pm. Who knew a simple hearing test appointment required so much planning?!? Anyway, his results were great - he's never fluctuated since the day he was born - so that was the easy bit.

It's a few months ago now so some of the details are a bit fuzzy, but I think we did impressions for new moulds for Jarrah while we were there, and I think I had a question about Jarrah's FM not working properly or something...whatever it was, it necessitated Jarrah's hearing aids having some attention also, which meant extra time and waiting around. Not too bad, although a little longer than I'd expected, and I had left the roast in the oven at home cos I had expected to be home by about 4:30-5pm. One thing led to another, there were some technical difficulties, and finally i think at about 4:45-5pm(!!) Jarrah's hearing aids were brought out finished; I went to put them in and discovered that they simply didn't work.

At all.

Not very useful for a schoolboy! Fortunately i had checked them immediately in the waiting room, so I called the audiologist back again, who was most apologetic, and we proceeded to wait. Again.

Eventually the audiologist returned, saying she couldn't make them work either, she'd have to get the technician to have a look at them and determine whether it was the software on her computer or the hearing aids themselves (they had given her some trouble as she had been trying to adjust them, hence the delay in the first place!) and we'd have to take some loan hearing aids as it was after 5 & the technician had gone home. Of course that meant that the loan hearing aids then had to be setup with Jarrah's prescription, and so we waited. Again.

Finally, they were ready! By now it was about 5:30-5:40pm, the kids were tired, hungry & dirty (bath time is 5pm in our house) and my roast was quickly becoming a BBQ...so we stepped out into the peak-hour traffic and I think we made it home by about 6:10pm. That was a marathon 4hrs from leaving home to arriving back again, just for a 1hr Aus Hearing appt at a centre that's 15mins from our house!

I did rescue my roast, it was not quite as charred as I thought it might have been, and we all slept well that night...

The following Monday, 5th Nov, was Jonica's Aus Hearing follow-up. Nowhere near as eventful (Praise The Lord!) and Jarrah was at school, so as I recall everything pretty much went to plan. Her results, while no better, were at least no worse than they had been in May, so it seems she hadn't had any fluctuations in the last few months. *Phew!*

Next time: January checkups, another marathon!