So today we have:

– Appointment at a local school to see whether, in due course, they might be able to cope with Alice and her needs and bring the best out in her.

– Appointment with Dr. Wendy at St. George’s for testing and to begin referral process for CI assessment in addition to a check up with another Audiologist on Alice’s Hearing Aids.

– Appointment and assessment at Great Ormond Street Hospital with Tony Sirimanna

– Cued Speech Lesson

not bad for a Thursday in November.

The school appointment goes well, although I am having to get used to people looking terrified and confused when you explain Alice’s problem to them. We have elected to look at private schools for Alice, so they are not obliged to take her on. I think this one we will keep as a plan B as we have already seen a school that we think might fit the bill (this all obviously depends on how well her treatment goes). We are a speaking family who grew up in the mainstream – we will strive as hard as possible (money and time no object) to allow Alice this opportunity too.

So straight from the school to St. George’s. Usual parking problems etc. We get to the clinic to discover that they have just changed booking system and that they aren’t sure who should be there and who shouldn’t. Either way, they are running hopelessly late. Fortunately, Dr. Wendy and her team know we are off to GoSH straight afterwards so we manage, somehow, to get seen on time.

More behavioral tests, but this time aided. Alice starts off well, but by the end is not responding to anything at any frequency. I am half convinced she has learnt these tests now and is predicting the outcomes (I think these are her 5th set of Visual Reinforcement Audiometry tests). We tell them her aids don’t seem to be providing any benfit at this stage – no, none at all. I suspect they are not surprised by this. They check them anyway and agree that they are happy to refer us on to the CI programme. However, we are given the choice, either to do this at St. George’s or, pending the outcome of our meeting at GoSH, at GoSH – we are to get back to Dr. Wendy with our decision.

Straight into the car, sandwich en-route, to Great Ormond Street (in Central London if you don’t know it). For once, the Gods shine on us and we get parked right out front.

Having spent years hearing about what a wonderful hospital GoSH is, we are surprised to see that it does rather resemble a Victorian Sanitorium. The quality of care, however, is undoubtedly very high.

Alice has a further set of VRA tests and this time doesn’t respond at all, even up to 105db at all frequencies. We then meet Tony Sirimanna.

Tony clearly knows AN/AD and, for once, has a clear opinion of how to deal with it (which is a revelation in this world of ambiguity). I have to say, he probably raised more questions than he answered. Tony’s picture of AN/AD is thus: Those children with the condition sit somewhere on a normal distribution curve. Those at one end develop language normally but have trouble differentiating in background noise. Generally they do well either aided or unaided. Those at the other end of the spectrum, who behave as if profoundly deaf are easier to treat and generally do well with an implant. It is those in the middle of the bell who are difficult to treat. Some of Alice ‘s audiograms suggest she is in this middle group (moderate to severe hearing loss) and some suggest a profound hearing loss. As I remarked to Tony, I can sit behind her with a snare drum and bang it as loud as I can with no response. All this said, exactly where Alice sits in the distribution is still difficult to read.

Equally, Tony suggested that those children whose AN/AD (or ANSD – Auditory Neuropathy Spectrum Disorder as they are now calling it) is cause by the Otoferlin mutation, seem to respond well to CI (although these children normally display a profound hearing loss and thus fit at the end of the distribution curve).

Tony suggests that if it were him, he’d go to Sydney for the EEABR (assuming money isn’t a problem) as Alice is clearly an ambiguous case (how can you be an ambiguous case of an ambiguous condition? how much more confusing can this get?) or if not, try Prom Stim at Nottingham. Equally, there is a lab in France who will test for the Otoferlin mutation for us if we ask nicely enough (I’m beginning to think we are really behind the curve here in the UK)

If any of these suggest she would be a good CI candidate, then he would push for bilateral implants. This is a new one to us, but I am happy to listen.

So we leave GoSH a little shell-shocked. Just to top it off, I have managed to get a parking ticket as the bay out front was for residents only – I knew my luck would run out sooner or later.

So home we go, children fed and to bed and on to cued speech lesson no.3. For anyone who can cue, you will understand how unfunny Catharine’s test of cueing Supercalifragilisticexpialidocious was after the day we’ve just had.

11pm we both collapse in a heap.

To the Lister Hospital in London (without Alice this time) to talk to David Selvadurai about what a CI would entail. Both of us have spent many hours trawling the internet to find out what we can about CIs in addition to email, telephone and face-to-face correspendence with a number of experts (Chuck Berlin, Neil Donnelly to name but two). I know what they are, how they work, who makes them (even down to the turnover of their businesses and their long term financial viability), what the benefits and weaknesses are of each model. We have watched videos of the surgeries, the switch ons and have a reasonable understanding of the equation of effort put in versus outcome.

All this said, will it work for Alice?

David, as ever, is supportive and very informed, particularly with regard the nuances of AN/AD. He takes us through the assessment procedure and what this will mean (speech and language assessment, assesment by a teacher of the deaf, scans – which she has had – and various other behavioral tests). He highlights the issue of funding but is reasonably comfortable that our PCT should be OK. That said, because Alice has displayed some behavioural responses at the 55db level, this will need further explanation when applying for funding (the PCTs have rarely dealt with anything so complex as AN/AD).

David feels Alice should be a good candidate based on her scans (good quality, intact nerve etc.), but suggests EEABR (Electrically Evoked Brainstem Responses) could be a useful predictor of CI outcome – the issue – these can only be done at present in Sydney or possibly in Manchester. There is another EABR test – Promontory Stimulation (prom stim) which can be done in this country (at Nottingham), but the nature of the electrode used means the tests can be unreliable. The EEABR in Sydney is done with an electrode shaped like a golf club which allows direct contact with the Cochlea. The Sydney CI centre make these on site. Manchester and GoSH hospitals are awaiting approval (more bureaucracy) to use these in the UK. He tells us he will speak to Prof. Gibson at the Sydney CI Centre (his former mentor on the Graham Fraser fellowship) to seek his advice.

However, we can kick start the whole process once Dr. Wendy formally refers us to him – we are seeing her on Thursday, along with Tony Sirimanna at GoSH (recognised as one of the only ‘experts’ on AN/AD in the UK).

off we go!