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 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.

The four procedures

Grommets, ABR, CT scan and eye pressure test were the four tests to be done on my boy today.

My wife and I arrived shortly after 7:00am to the hospital. Raphael had already been given some preventative ventolin and the anaesthetis arrived shortly afterwards to give him some a drying medicine (to help with his secretions during the operation) and some other stuff. Actually with the number of syringe fulls of drugs that the anaesthetist injected into his line, I was half expecting Raphael to bulge a bit.

His fluid line was still in and the nurse came and asked us to put him in his baby hospital gown so we stripped his clothes of but of course his singlet could not be removed from the line Annie and I had a good chuckle about all of the possible ways that we could potentially get his singlet of him but eventually resigned ourselves to the fact that he would go into surgery with his grotty singlet dangling from the IV tube

Annie and I prayed that we would see his cheeky smile again.

Finally we brought him to the day surgery area where he was going to start his procedures with the insertion of grommets. We even ran into his normal Audiologist who had postponed her travel just so she could perform the ABR test on him.

After a short wait, I was asked to put on a surgical gown and hat in order to bring Raphael into the theatre. After several embarrassing attempts to correctly put on the surgical gown the nurse said that'll do. I can only assume that she would have a chuckle at my expense later, she did a good job at holding a straight face while I was fighting with it. In hindsight it really wasn't that hard to do. I can only assume that my mind was somewhere else completely, preventing me from completing even this simple task.

After wheeling him into the theatre and putting him on the operating table the surgeon advised me that the ophthalmologist would arrive in fifteen minutes to perform the eye pressure test. After that they ushered me out of the theatre and back to the waiting room.

We knew the procedures would probably take over 2 hours (if everything went well). After about 3 hours we were told that Raphael was awake and we went to see him in recovery. He was in a little pain and his eyes and lips were swollen from fluid (not an allergic reaction). He was in much better shape than last time he had a GA. We are grateful for the special preparation that the anaesthetist organised for him.

That afternoon he was hooked up to an oxygen saturation monitoring machine and while he was having an afternoon sleep he had two instances when he could not breath properly and had to be turned onto his side to get him breathing again properly. He had been given some codeine to help with his pain and since his episodes I heard a rumour that opioids like codeine can cause resperation difficulties in babies that are already at risk. This made me question why he was given codeine. I was told that the issue was that he had already had paracetamol, and aspirin was a bigger risk after surgery. There was also mitigation for giving him codeine in that he was given the minimum dose and also that there was a nurse constantly in the room monitoring the oxygen saturations of the kids in the room. It all turned out ok and to put this in context he has previously demonstrated that he can desaturate under normal circumstances when he is sleeping on his back so it may have had nothing to do with the opioid anyway.

Later that evening he even gave a smile although he wasn't quite back to his usual self yet.

ABR results

The results of the recent ABR test were not as good as what we were hoping.

The test demonstrate a hearing loss up to 50db on his right ear which is "consistent with a moderate, predominately conductive loss". The test also showed a hearing loss up to 40 or 50db in his left ear "consistent with a mild to moderate predominately sensorineural hearing loss."

It was recommended that Raphael be fitted with hearing aids in the short term, "until more accurate results can be obtained."

If his right ear's conductive hearing loss is due to otitis media (fluid in the middle ear), then it is possible that a simple surgery to insert grommets will resolve this hearing loss. Sensorineural loss is much more difficult to repair and I doubt that there is much that can be done to improve the hearing of his left ear.

A successful ABR

The audiologist performed another ABR on Raphael today and this one was much more successful than the last one.

We hooked him up with wires and headphones and even taped the bone conduction device to his forehead so that we wouldn't have to touch him once he fell asleep.

The bone conduction test is used to send sound signals straight through his skull which bypasses the middle ear altogether. This test is a good way to determine how good inner ear and nerves are working. This test showed that his inner ear can detect sounds as low as 30db. A fully developed hearing system can generally hear down to about 20db so this is pretty good especially becuase infants hearing generally continues to improve.

Unfortunately he struggled a bit before falling asleep and knocked the headphones off before could test his total hearing ability. He woke up when we put his headphones back on him but fell asleep quickly afterwards and then he slept for ages. This was enough time for the audiologist to get an excellent sample of information including different pitched sounds. She said that this test showed a small improvement since the last test, but at this age a child's hearing ability fluctuates from day to day.

The audiologist hasn't analysed the results properly yet and said it will be about a week before the results can be posted to all of the parties involved with his hearing.

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.

Another Auditory Brainstem Response (ABR) test

Before the ENT doctor commits to putting grommets in Raphael's ears, they wanted to have another ABR test done to see if his hearing had improved since the last one. So we went to another two hour appointment today with the audiologist.

Unfortunately he only slept for 30 minutes which was only enough time to test his right ear. This showed that he could only hear volumes over 60db.

Because there was not enough time to test his left ear, I will have to take him to another two hour appointment on Friday to have the test repeated. Hopefully there will also be time to perform a bone conduction hearing test as well. Because this requires touching him while he is sleeping there is a good chance that he'll wake up during this one so I don't know if that will be possible at all.

Audiologist appointment

Raphael had another Hearing test today called a "Hearing assessment using Auditory Brainstem Responses". Basically he was hooked up to a bunch of wires attached to his head and had a big set of headphones put on him and we then let him fall asleep in his pram. Then sounds were played through the headphones and the brain sensors looked for activity that coincided with the sounds. The findings of this test were that he had 40 decibel (db) hearing loss in one ear and 50db loss in the other. This effectively means that Raphael can probably hear us if we are sitting close and speaking in a reasonably loud voice.

Audiologist

Had an Audiologist appointment where some ear echo type tests were performed. These determined that there was some hearing loss and further test would be needed to establish how much.