It seems clear to us that despite the idiosyncrasies of ANSD and, in particular, Alice’s seemingly unique place on this spectrum, the management of children with the condition is pretty straightforward. SLT aside, you can either do nothing, aid or have a CI fitted. This in mind, up to a point it is largely academic where on the spectrum Alice sits. She has no speech or language and communicates only through basic expression and gesture. Thus, a CI is the only sensible option for her if we want her to communicate through speech.
All this said, this doesn’t stop us from wanting to learn as much about her condition as is possible and, in some respects, take the altruistic view and do what we can to help other parents in the same boat as us. It was one of the reasons behind starting this blog.
And so, through another convoluted relationship, Alice and I set off to Manchester last Thursday to meet Catherine McMahon, the Head of Audiology from the Macquarie University in Sydney. Not only does Catherine know many of the individuals we have encountered along our ANSD journey (in particular those at the SCIC) but, more importantly, she has a research specialism in ANSD.
Uncle Gavin and Aunty Lizzie played host for the night before Alice and I set off to Manchester University the next morning. We met Catherine and Kai Uus, a teaching Paediatrician at the University who is also leading a PhD in ANSD. Catherine gave a fantastic and unbiased opinion of both the condition and Alice’s prognosis. We also went along to the Whithington Hospital for some testing so Catherine could witness Alice’s enigmatic test results first-hand. Like many before her, from the paperwork she had seen, Catherine concluded that Alice was in the moderate-severe range, but after seeing her do another VRA (her sixth), she concluded that she was, in fact, pretty bad.
In addition, Alice had a CAEP (Cortical Auditory Evoked Potential) test. This is normally used in infants who are too young to gain a behavioural response in VRA testing. More electrodes on the head, but this time awake. CAEP, supposedly, offers the ability to measure higher cortical processing of stimuli with complex acoustic features that are essential for the development of speech and language. Bless little Alice who just carried on regardless. Interestingly, the CAEP response was not in line with her behavioural response – it suggested there were signal peaks transmitted to her brain and thus she should be displaying better behavioural responses than she was in practice. Alice conspires to confuse again.
I hope Catherine got something out of the day too. I have to admit that by lunchtime I was starting to flag and the five hour journey home served only to finish me (and Alice, who again was an angel) off.
Regardless, Catherine is confident that we are following the right path and that Alice, with lots of hard work, should do well with a CI.