7th November 2008 – 11th November 2008

After a whole day of talking about Alice (not a difficult thing to do given how wonderful she is) we sleep (very well I might add) on what to do next.

Do we go down the GoSH route or stick with David Sevadurai at St. George’s? GoSH have performed some 300 CI surgeries and are the centre of excellence in the South of England and the St. George’s CI programme only 20. Heart or Head?

Fortunately, both our hearts and our heads tell us that David is the right man for the job.

We email David and Wendy at St. George’s to let them know of our decision and also to seek their advice on the EEABR/EABR and Otoferlin tests.

David replies on Friday evening (like I said before – does this man never rest) whilst I am sitting in my usual haunt of the Red Rose Indian Restaurant in Surbiton awaiting a takeaway.

I’m not sure Roki, who runs the restaurant, is used to an in-his-thirties man sitting at his bar crying. David is delighted we have chosen to go with him, but one thing he said will stick with me for a long time ” I will always strive to achieve the best outcome for Alice, and you”. Not a difficult thing to say, but to us both, it meant the world.

Several emails later and the process seems to be underway. We are just awaiting the details of the first appointment on what will, no doubt, be a lengthy assessment process. David is busy speaking to experts around the world to seek opinion on whether EEABR / EABR is worthwhile given the good results from the MRI. At this stage, he feels not.

Just to top it off, we receive news from Tony Sirimanna that he feels Alice’s inconsistent VRA results could be because of an “abnormality of the neurotransmitter that normally flows from the hair cells across the synaptic cleft to the nerve endings. It is just supposition that perhaps while there is enough transmitter she hears better, but once depleted she doesn’t hear as well for a while”

It’s all Greek to me but sounds plausible – David agrees, but reminds us this is only a theory and will have no impact on her CI outcome as the CI bypasses this junction anyway. Dr. Wendy also agrees that it is worthwhile pursuing the Otoferlin tests.

We are getting somewhere. We are both happy.

So here’s what I make of it.

Medicine, to me, is usually something akin to witchcraft (but then so is flying and anything else I don’t really understand). I do have a degree in Biology, so my understanding of some of the terms and principles is better than the average man on the street, but not by much.

Hearing, in particular, is a process that seems to baffle even the experts. Sound waves are converted into motion by the various small membranes, bones and hairs in the different sections of the ear and it is this motion that can be turned into a nervous impulse and sent to the brain. The brain then learns this as sound which brings us the delights that are the birds singing, the waves crashing and the Foo Fighters or the horrors of a grade 1 violin player and Take That.

As with any part of the body, if one part of a pathway breaks, the whole thing collapses. It doesn’t really matter where on the pathway the problem is. It’s like the signals at Piccadilly Circus tube station breaking down. All the other stations and lines on the underground may be working, but the trains can’t travel past Piccadilly Circus. In my slightly addled brain, this is how I have come to learn about deafness. If something in the ear or associated nervous system is broken or absent, the journey of sound breaks down and we don’t hear.

Auditory Neuropathy (AN) or Auditory Neuropathy/Auditory Dyysynchrony (AN/AD) or Auditory Neuropathy Spectrum Disorder (ANSD) or whatever the hell it is called is something of an enigma in hearing loss terms. Strictly speaking, the majority of children with this type of hearing loss are not deaf at all – they just can’t hear (do you see what I did there? Now you understand why I’m confused)

ANSD is more a description of a group of symptoms than it is a name for a particular condition in its own right. It seems to refer to the case where the ear is picking up sound, but for some reason, this sound is not transmitted as it should be to the brain. This could be for any number of reasons: A problem with the auditory nerves or associated synaptic junctions or, possibly, some problem with the synchronisation of the hair movements in the inner ear.

As a result, children hear some or all of their sound as distortion, making it difficult or impossible to discriminate speech. The best explanation of what the experts think it might be like is a ‘badly tuned radio’. You can hear some speech in there, but the white noise means it is very difficult to make it out properly. Thus, some children can get by without any intervention, but will suffer in a noisy background and others, like Alice, seem to hear no speech at all and, for at least a part of the time, act as if (or may as well be) profoundly deaf.

Little Alice’s nerves all seem to be working ok so the problem is somewhere else. Save for chopping her up and taking a look, there is no way of telling. The best guess is that she has an inner hair cell dyssynchrony meaning the signal to her brain is scrambled (hence the absent ABR). As we have now learnt, an absent ABR does not mean there is no signal going to the brain; it only detects a strong and coordinated signal. Alice could be hearing all sound as Pink Floyd tunes for all we know.

So there you have it. This is why Alice’s ears don’t work – witchcraft and guesswork. We are both mathematicians at heart. We understand cold, hard facts. Guessing games, theories and best estimates don’t sit very comfortably.

I just wish somebody could tell us what was really wrong.