Ear tubes tomorrow morning

I got the long awaited call, it seems it was worth while pushing to be on the cancellation list...

Fi's surgery is tomorrow morning.

I have noticed since about mid- October that her hearing was down again (She passed hearing test in August). Hearing test confirmed in November that she had moderate bilateral hearing loss. 45 dB both sides. Saw ENT first week of Dec and it took until now after numerous phone calls to get the surgery. I don't think this is too bad. So, wish us luck. No more liquids past 5 AM...

She has been learning so many words lately, I am so excited to see how her speech will pick up even more post surgery.

Here we go again

I knew this would happen so it wasn't a shock. Dr Pauli couldn't see a light reflex at the end of October when we were there and I wasn't so sure sometimes if Fi could hear well. By mid November it was pretty clear that she couldn't hear well at all. She promptly failed her hearing test at the end of Nov, fluid on boht ears, hearing at 45 dB. We saw the ENT early Dec and we are just waiting for the surgery date in January. We will go for another set of regular tubes, I have done lots of reading on both T-tubes and titanium tubes and wouldn't have felt too comfortable doing either of them instead, thankfully the ENT agreed.
I want to copy here what Dr Pauli recommended to do as the guidance seems to have changed somewhat compared to what he told us last year: "Compared with children with achondroplasia, individuals with hypochondroplasia have a much more variable course of middle ear dysfunction, it seems. That means that there is no strong argument for using either collared button tubes vs. longer lasting T-tubes."

I may seem to be very matter of fact about this, and I suppose I am, over the last year and a half medical issues and having to do research about them has become part of my every day life. I try not to give in to the sadness that the poor chicken probably hasn't heard us well for the last 2 months and it might be another month before her surgery is scheduled, that is 3 months out of her speech development during this crucial time... At the same time, we couldn't have watched her more closely than with audiology visits every 3 months...

I think what really shocked me was that my eldest has fluid in her ears again, but more on that topic another day...

Audiology update

Fi passed her hearing test on Friday with flying colours. Both her ear tubes are out, so we put this down to the summer. I am delighted, it buys us at least another 3 months without having to go through another surgery. We need to go back for another hearing test in 3 months... YAY.
Tuesday we see the neurologist for the first time...

ENT update

Instead of moaning, I will just focus on the outcome of the ENT appointment on Wednesday. So, you might recall from this post that last month we saw the audiologist and one of the tubes was out, the other one was on the way out, but Fi's hearing was not impacted yet. I had no illusions I was quite certain that fluid would rapidly accumulate in her middle ear and sure enough the ENT consultant could already see it on last Wednesday. She can still hear well, but I think it might start to impact her hearing slightly already. The tube still hasn't fallen out of the other ear yet, so she is grand there.
Since July-Aug is the holiday season here, we have agreed that I will get her hearing tested late Aug and we made an appointment for Sep 5 to see her again. I must say I like this doctor, she remembered everything about us, or read her notes, she is kind and interested in Fi, asked about her motor skills for example, and she could do all this in 3 minutes. The fast that she didn't have us wait for an hour was an added bonus.
I think there is a very small tiny chance that the issue will resolve itself as we are heading into the summer and will be in warm climate for 3 weeks, but I am not banking on it. So I guess I need to start reading up on different types of ear tubes, Dr P. said to look into a more permanent solution, as if she has fluid now, it means that she will probably need tubes until she is about 8 or so. I guess in the US they would do T-tubes, but it's not something commonly used here... The ENT suggested titanium tubes before as something that may last for 1.5 - 2 years with slightly increased risk of perforation, but not as high of a risk as with T-tubes... Not sure, from what I have read about them, that I am convinced... Need to balance the risk of perforation with the risk of annual surgery and there is only so many tubes an eardrum can take apparently... I have 2 assignments for university over the next 2 weeks, but once I am done, this will be my next project, YAY it never ends.

Ear tubes, set 1

I cannot believe that I didn't post here about Fiona's ear tube surgery back in Nov. I better do it now, as there is more to come on the ENT front and it will only make sense that way. Here is the original post about her first hearing test, which she passed, but it already showed signs of middle ear dysfunction. Our audiologist, who I think is the BEST audiologist in the world, was very knowledgeable about skeletal dysplasias and their potential impact on hearing and she suggested a retest every 3 months. This is actually a lot more pro-active than what Dr. Pauli suggests, who says: "Behavioral audiometric and tympanometric assessment, first at 9-12 months of age and at least yearly throughout early childhood." Anyhow, I followed Sandy's advice and returned with Fi at the age of 5 months for another hearing test, where there was evidence of fluid in the pressure test bilaterally and she promptly failed her emissions on both sides too. Now this was a real shocker to me, as I have 2 other children and watch Fi like a hawk and I could have never said that she couldn't hear well. So I have armed myself with the results and Dr Pauli's hand-out and saw an ENT, who was first pushing for hearing aids - that's what they seem to do a lot of for kids with Down Syndrome in the UK, who similarly to our kids are very much at risk for middle ear infections. However after reading Dr Pauli's handout, she agreed to do the grommets, even though the youngest patient she has ever had was 10 months old. The surgery was completely uneventful. I have given them this handout about anesthesia for children with achondroplasia, as the same can apply for hypo and they were very receptive and reviewed it in detail with me. Her hearing was immediately back to normal following the surgery and she started babbling a lot more within a week or so. Sandy offered to do a quick check on her (free of charge!!!) every 3 months just to be sure that she can hear well.

First hearing test

This was our first real medical test of any relevance since Fiona was born.
Sure they did the X-rays for diagnostic purposes and we have been referred for a million things, none of which have of course happened yet...

I read this in Dr. Pauli's file about hypo:

"Problem: Ears and Hearing

Expectations: Many infants and young children with hypochondroplasia will develop recurrent or persistent middle ear dysfunction with conductive hearing loss (although this risk is considerably less than in children with achondroplasia). If not aggressively treated, this may contribute to delays in language and speech development. Middle ear dysfunction is often resistant to medical management.

Monitoring: Behavioral audiometric and tympanometric assessment, first at 9-12 months of age and at least yearly throughout early childhood. One should have a high level of suspicion that middle ear problems are present.

Intervention: Aggressive use of myringotomy and tube placement. If a child needs ventilation tubes, then they should be maintained until 6-8 years of age, since it appears that eustachian tube autonomy typically does not develop until then."

So I naively thought that I would just self-refer for a newborn hearing test just to have a baseline and then return every 6 months or so, I never expected any problems already...

The good news is that she did pass her hearing test, the bad news is that without me saying anything about her condition they could immediately see that there was a lot of negative pressure there and that her Eustachian tubes were quite small. We need to return for the next test in 3 months in the mean time I need to get into the system for a pediatric ENT specialist, so we wouldn't have to wait for months if she will need grommets...
So we shall see...