It should be such a simple question, but actually, for our ANSD babies, it’s the hardest to answer.
In standard hearing loss, you do an ABR or test them in a hearing booth. For our babies this just doesn’t cut it. Alice tested anywhere between a moderate and a profound loss within the same session and within minutes. Her hearing was consistent in that it was completely inconsistent. Even when we went for Ear No2, she was still able to completely confound us and her audiologists. Interestingly, when we ask what she hears, its anything between nothing and her new favourite, “Ghosties……..” So none the wiser. But none of that hearing was useful. She couldn’t make enough sense of it to make out speech.
But ANSD kiddies are all different; some hear nothing useful, some have windows of useful hearing and some hear well with amplification or technology that improves noise to sound ratios. Herein lies madness and it’s frustrating for parents, who want a straight answer to what should be a simple question and for professionals as they can’t give the straightforward answer.
We don’t think Ollie hears much; in the next month or so we’re going to find out where he tests in terms of VRA but last week was more exciting and much more useful.
Francesca from Manchester University has devised a test to try and capture the useful hearing in an ANSD baby. An amazing area of research and one that we are delighted to support. We have discovered that Oliver can hear, he can distinguish the different parts of speech (on a simple level consonants and vowels) and the signal can get through. The issue is that it took very loud sounds to do it – for Oliver, it was 105db, but it was still possible. For other ANSD children, the answers will be very different. It was fantastic and amazing and it will be an extremely useful piece of the puzzle for audiologists.
Alice's Ears 
I’m so glad I found your site. Thank u for sharing your journey. My son was diagnosed with ansd at 6months and we chose to wait until 18 months to begin hearing aid trials while continuing using speech therapy. There has been some speech development but speech perception is still difficult to determine. In the last paragraph of your post u noted a test done at manchester to detect useful hearing. Do you have a way to contact Francesca to see how this test was done? We are in Florida and would find such a test very helpful before making a final decision as to ci. Thank you 🙂
Danielle,
Thank you so much for finding us! I think the test I was referring to is called Electrocochleography or ECochG. They essentially try to stimulate the auditory nerve directly, thus bypassing the structures of the ear (and the possible site of ANSD lesion). it ‘can’ (and I stress. ‘Can’) be a good predictor of CI outcome.
I think it is quite readily available in some centres in the states.
Why not come along to our Facebook ANSD group – http://www.facebook.vom/groups/ANSDParents
Chris
Ignore my reply – I just re-read the post and it’s not ECochG I was referring to. It is a behavioural test.
I will get Alice’s mummy to post more about it.
Chris
Alice’s mummy here – essentially the test is focused on the detection of speech at varying levels. So they played a tape of speech sounds and then monitored responses. They used specific words or sound combinations to see if differences occurred at set levels. Francesca is Francesca Sterling at University of Manchester. It might be worth seeing if they can contact her directly.