ANSD - a personal perspective

Please note: It is important to remember that people with Auditory Neuropathy Spectrum Disorder can experience a very wide range of listening abilities. Some find speech very clear when it is quiet but struggle a lot when it’s noisy. Some find it is difficult to understand speech even when it’s quiet, and some find they don’t even detect sounds in their environment very well. Jane falls into the area of the spectrum where it is very difficult to understand what people are saying even when it is quiet.

Do you experience fluctuations in your hearing? Do you have 'good days and bad days' that you can clearly identify? Are the differences extreme or subtle? 

I definitely had fluctuations in my hearing. I did have good days and bad days and I remember "towards the end" i.e. right before my implant, that I was very conscious and yet subconsciously aware of this. If I hadn't had a perfect night's sleep, if I hadn't gone to the gym regularly, if I hadn't had a cup of coffee or an energy drink, if I hadn't been eating properly and drunk enough water, then I knew I wouldn't hear properly. If I hadn't fulfilled these criteria (and probably other criteria that escape me at this point in time), then the differences were extreme. I basically couldn't make out anything versus I could hear reasonably well with some feedback. I definitely could NEVER hear anything "towards the end" without some visual form of reinforcement i.e. sign language, lipreading, cued speech, telling the person to slow down and speak up or whatever.

Could you describe how it sounds?

As to how it sounds...it really just sounds like mush, like someone's mumbling. You're really convinced that they're just not talking properly because for you this is "normal". I could sometimes, without visual input,  make out if someone was talking vs environmental sound, but not all the time. It usually took a while for it to reach my consciousness.

What would you have done differently?

I have to say I take an "I regret nothing" approach to life because I don't see the point in regretting anything. I tend to see it as: Suppose I hadn't done an honours degree in hearing sciences (though it means a year less that I could be studying), then I wouldn't have had the access I needed to the journal articles that told me precisely the same thing my audiologists were telling me - that an implant was definitely the way to go. During the time I was studying, there was a consensus conference on ANSD in Como, Italy in 2010. It was here that new guidelines for cochlear implantation for ANSD was recommended. Suppose I hadn't floundered with my degree (I spread it out over 8 years), then I wouldn't have delayed it long enough to get an implant and know how to successfully manipulate its outcomes, plus give me a good shot at doing my masters of clinical audiology degree. 

The only thing I truly do wish was different is my relationship with my brothers and sisters. We are currently estranged and I firmly believe that it boils down to my hearing in some shape or form, whereas they're convinced my hearing is an excuse. 

How much of an improvement has she had with the implant? How is her hearing different with the implant?

I can't put into words how much my life is different with my implant. I remember before my surgery that my left ear was my better ear. Post surgery (implanted right ear) sometimes if I'm not wearing my implant, I'll instinctively turn my head to the right and not the left so I can "hear" better!

The benchmark for a Cochlear Implant progress is eighteen months, so maybe come back in March 2013 and ask me then!

Do you have any recommendations for parents?

This is a really hard one! I'm not a parent myself which would definitely put a different spin on things. I really don't know how to answer this. I remember my mum yelling at me at one point because I couldn't hear her but she thought I just wasn't trying hard enough. Please remember this was before there was a widespread understanding of ANSD and by then we'd already gone to numerous audiologists and whatnot to try and work out what was "wrong" with me i.e., this was before there was even a definition of auditory neuropathy. 

Post diagnosis, there were the times we went to counselling and the minute her bum hit the chair, my mum would burst into tears and sob about how awful it was that I'm deaf. I ended up not getting any counselling and my mother getting all the attention. I guess I'm trying to say doing therapy together is not always the greatest of ideas. I appreciated the fact that recently my dad said to me that he felt my mum overdid it the emotional side of it. 

Have a variety of methods to manage communication and adapt your approach until you've hit on a solution. Be prepared that the right approach may change over time. I'd also recommend that if the child with ANSD wants to learn sign language, then let them. They may be expressing that they're struggling socially.

When did you start learning to sign?

I started learning in Year 6. I learnt it properly in 2000 when I did a Statement of Attainment in Signed English.

In the US, cued speech is commonly used to assist children with ANSD but this is not the case here. Why did you seek out a cued speech teacher? How has cued speech helped you?

Cued speech is something I've been keen to learn for years and years, ever since I had to do an assignment on it for university. The assignment I did noted that deaf people who used cued speech resulted with better academic and career results compared with their peers who signed who had better social results. It made sense to me to learn cued speech because I struggled with phonemic comprehension, something that cued speech is meant to give visual input for. 

I distinctly remember the first week of my Masters degree in audiology this year. The terminology was so out there and I really struggled to understand what was going on. Both the Auslan interpreters and I agreed that I had no idea what they were saying. Due to my implant, I was granted a deferral and did Certificate IV in Audiometric Assessment. I requested cued speech transliterators for a practical and noticed the difference straight away. I wasn't exhausted from having to translate everything from Auslan to English in my head.

You have been having singing lessons since your cochlear implant. Can you tell us about this?

It is my belief that most (not all) teenagers go through a period where "Music is my life" and I never really went through that phase (I definitely attempted to pretend to though and thought The Spice Girls were awesome when all my friends thought they weren't). Post implant, I have a voracious appetite for music and can't get enough of it. And they say that people with implants lose their music appreciation! Go figure.

My singing teacher Pat is the most wonderful, positive woman I have ever met. She's so thrilled to think that music might help me in some shape or form and is even happier to know I'm getting more benefit from music than possibly any of her other students. It might cost a lot, but I love going to someone who's so positive about the whole affair. I have almost no-one who is as enthused about this experience as Pat is. She's also gone out of her way to work out other, visual, ways to get the message across. 

I really started singing because it's a skill I always wanted to learn, though not sure if I have the dedication. I took part in a research study that teaches people how to sing by providing them with visual feedback - Sing and See program. That was how I met Pat - she was doing her doctoral thesis on the topic. 

Post singing, people have commented that I have more emotional inflection (intonation/prosody) in my voice. I feel I have more command over my voice and am probably more aware of music in a way that I wasn't before.

December 2011