Got hearing aids

Phew, we have the hearing aids and new moulds.

The bad news is that the new moulds are rubbish we will have to get new ones made and they wont be available in time. Oh well, at least we still have the old left mould still.

Australian hearing and signing playgroup

Another Australian Hearing appointment today confirmed that the grommets are no longer in effect and it looks like he might have more fluid in his right ear. We will have to decide whether we want them to be reinserted during his next operation.

He performed as expected on his hearing tests and new moulds were taken to try to resolve the increasing problems that we are having from feedback (squealing).

It was a long appointment and we arrived at Raphael's new signing playgroup with only 15 minutes left. We had a brash introduction to the teachers there and I was a little disappointed that there was a lot of talking happening there. I was hoping that it might be a speech-free playgroup to encourage Auslan development for the children but there seemed to be more English than Auslan. Maybe all of the administrative work necessitated using a language that most people were fluent with and maybe the quantity of signing will increase next time.

New hearing aids and weight check (10.36kg)

I weighed Raphael today at 10.36kg. This is a milestone for him becuase he has finally weighed more than the first weight check at the beginning of the trial (10.3kg). But before I hear you cheer too loudly, this only represents a 60g gain since 9/7/2007 (10 weeks).

Speaking of loudness, we also took Raphael to Australian hearing today to get new hearing aids for him that can supply higher volume for his left ear. Unfortunately, one of the new aids was missing the childproof battery lock and so he will have to wait for that one to be repaired. The good news is that it was the right aid that was missing the lock and so he is able to wear his old right hearing aid (with the same volume), and he can wear the new left hearing aid with the enhanced volume that his left ear needs.

Early learning and Australian Hearing

My wife forgot about attending Early learning this morning and they called her asking if she was coming. Scrambling everything together, she was there within fifteen minutes and found that the she was the only person scheduled to attend the session that day. The whole place had been setup just for Raphael. I think that she felt quite guilty about forgetting because of this.

I certainly don't mean this to sound like I am berating my wife for forgetting; but quite the opposite. I mention it becuase this is the first appointment that we have forgotten in the hundreds of appointments that we have had. Annie has been the solely responsible for scheduling appointments with practically no help from me at all and to have only forgotten one, amongst the countless numbers that we have had, I think is amazing.

Well done Annie! I cannot think of a high enough accolade for you, you are priceless.

In the afternoon we went to Australian Hearing to have more accurate hearing tests and new molds made. His left ear is only hearing above 90-100db which places that ear in the profound hearing loss zone. His right ear is much better, being a 45-55db loss (from memory).

Hearing aid adjustments

We visited Australian Hearing this afternoon for some adjustments to Raphael's hearing aids. The first task was to fit a new right ear mould. This latest mould fits far better than the previous attempts and so we were hoping that this will stop the dreaded squealing.

We then tested his hearing with another VROA test but this time he was tested with one hearing aid in at a time. This showed that his right hearing aid is boosting his hearing ability down to about 25db which is good enough for conversational hearing. His left ear however still behaved poorly even with his hearing aid.

The Audiologist further amplified the relevant frequencies of Raphael's left hearing aid but noticed that he mould is getting a bit loose. she took another mould of Raphael's left ear to get another mould made (that sentence is a bit confusing, I wonder what the real terminology is). This new mould will be posted to us when it is ready.

Sure enough, by the time we got Raphael home, both hearing aids were playing a chorus of squeals. Annie will be going to Aus hearing on Friday for the RIDBC teleconference and so she will ask them to do something about it then.

The factors are working against us:

• His right ear doesn't have enough cartilage in it to hold a hearing aid properly and so any movement, jogs the aid out slightly and starts a feedback squeal. The new mould is better but still not perfect.
  
• His left ear requires more amplification but because the aid is a little loose, this amplification has resulted in the left aid squealing.
• The combination of: cold weather, low set ears and not much neck to speak of means that his clothes are usually pressing the bottom of his ears when he is sitting and this of course dislodges the moulds slightly causing more squealing.

Hearing aid mould modifications

I took Raphael to Australian Hearing this morning for another attempt to resolve the squealing right hearing aid. The Audiologist cut excess material from the existing mould and took a new impression to make yet another mould.

New hearing aid moulds

Raphael finally received his new hearing aid moulds today and the Audiologist turned up the volume on the high frequency sounds of his hearing aid again in the hope that he new moulds would stop the really annoying feedback. Of course it sounded good at Australian Hearing but as soon as we got him home his right aid started to squeal relentlessly. On the bright side though, his left aid seems to be fine.

First attempts with CPAP

Yesterday after his Gymbaroo and Australian Hearing appointment (to get new moulds made) we rushed back to hospital to try his CPAP at 3:00pm. The intensivist tried a couple of different masks but Raphael was in a fighting mood and so this first attempt was abandoned.

At 9:00pm we tried again, we had a new headpiece (a nice soft one) and even though we had an initial struggle to get it on him he finally went to sleep with it on. All seemed good for the first hour but after that he would "desat" (blood oxygen level would drop) periodically with no apparent reason. This seemed to happen more when he was on his back or right hand side.

During his sleep he would occasionally move and the mask would slip a little. This resulted in air escaping and blowing across his face. This made him panic and struggle making the leak worse. This meant that I didn't get much sleep last night.

Hearing aid adjustment

This morning we took Raphael to have his hearing aids adjusted to try to stop the awful squealing from the feedback.

They reduced the high pitch gain and this seems to have improved things a lot. It still squeals if he leans up against something but at least it isn't squealing all the time now.

Hearing aids

After the morning's gymbaroo, Raphael went to Australian Hearing to have hearing aids fitted. As usual (after gymbaroo) he was exhausted but he performed well for the practical hearing tests and these actually showed an improvement in hearing low frequency sounds.

They customised some digital hearing aids and fitted the ear moulds that had arrived since our last visit. Raphael was well and truly sick of being held by then and so we put him down on the floor where he was happier. While the audiologist explained the use and care of the hearing aids to us Raphael explored the room and the corridor outside the room.

The mould doesn't fit very well in his wonky ear becuase the cartilage isn't in the right places to hold the mould in properly. Apparently this can be a common problem for kids with CHARGE syndrome. Because of this poor fit he seems to get a bit of feedback on the device. The audiologist fitted some kind of feedback protection but now that we have got it home and have put the aids on him I am not convinced that the feedback protections does anything. In the end I just resorted to hold him down and jamming the hearing aid in as hard as I though safe to do. This used much more force than any mother would endorse, it was lucky that Annie wasn't home at the time.

Four appointments today

The four appointments today were: Gymbaroo, Australian Hearing, Let's Sign and a meeting with Raphael's future primary school principal.

I have talked about Gymbaroo before so I won't elaborate further here.

At today's Australian Hearing appointment, Raphael had another functional hearing test. He was seated on his mother's lap and sounds were played through speakers to his left or right and if he responded by turning towards the sound then he was rewarded with either a puppet in a window or a toy elephant drumming on the other side. The results of the test were the same as previous tests with regards to what volume and pitch he could hear. Ear moulds were then taken of his ears canals for the purposes of fitting hearing aids. He wasn't very keen on having his ears filled up with putty; but I have become quite adept at holding his head still.

Today was the last day of the 4 week Let's Sign course. I am a little disappointed that it is finished but I am very grateful to the teacher who went to the trouble of preparing extra signs just for me and giving me one-on-one help with the signs.

After the Let's Sign course we met up with the state coordinator of ELT (Early Learning Tasmania) and the principal of our local primary school to discuss Raphael and his older siblings in the schools. They are extremely supportive and have seem to have fabulous resources to help educate disabled kids and their families. I felt tears of gratitude well in my eyes when they told me what they thought they would be able to do for Raphael and his older siblings.

Australian Hearing audiologist appointment

Raphael had an appointment today at Australian Hearing with their paediatric audiologist. This is an Australian federal government organization responsible for assessing hearing and supplying hearing aids for children (and other specific groups).

They performed a formal behavioural hearing test on him and found that:

• he responds to sounds above 50-60db
• he is more responsive to high pitched sounds
• he is unresponsive to low pitched sounds (even at 80db)

They commented that this is consistent for babies with middle ear fluid (otitis media). An air pressure test also had results that were consistent with middle ear fluid but she could not make out much about his ear drums because of his small canals.

They said that this test shows that it is unlikely that he has a severe hearing loss and if we sit close to him and talk clearly with no background noise then "he will be getting a lot of what we are saying".

From here they would like us to continue with what we were already doing with regards to trying to improve his hearing (eg more tests at the hospital and possibly fitting grommets). After all of this is done then he needs to have his hearing assessed again to see whether it has improved. If his hearing cannot be improved to hear 30db then hearing aids would be appropriate to improve his hearing at this important early developmental stage.

RIDBC and Australian Hearing

Today we received a package from RIDBC (Royal Institute for Deaf and Blind Children) containing:

• A few toys to borrow (these are great, we had to forcibly extract these cool new toys from our older kids so we could play with them - erm I mean so that Raphael could play with them);
  
• Some suggestions on methods that we can use at home to test Raphael's vision and hearing; and
  
• Some specific games designed to solicit responses from visual and auditory stimulation.

Annie will hopefully go to a video conference with Raphael's RIDBC teacher who is in New South Wales. The teacher said that her speciality is for blind children, it is a shame that in our case Raphael is more deaf than blind, but we are very happy to get the multi-sensory loss early intervention services.

I say "hopefully go" because the venue that hosts the video conferencing facilities in Hobart is owned by Australian Hearing and they will only allow their own clients to use this facility. So guess what we did... that's right, we applied to become clients of Australian Hearing. This bureaucracy is actually good because we were not aware of Australian Hearing before. Australian Hearing are the Australian federal government group responsible for supplying hearing aids for children who are assessed as needing them. To qualify for this free service we need to apply and we are required to have a doctor's referral. So tomorrow morning we have a an appointment with our GP to get one of these. Hopefully all of this will be sorted out by Friday so we can use the video conferencing facility.