A A A Accessibility A A A A

Having Usher Syndrome is a rollercoaster of emotions as your vision ebbs away.

Going blind is all consuming so much so we almost lose sight of the real importance of access to sound.

For me going blind took precedent!

I had retinitis pigmentosa and I had macular oedema.  My consultant was so concerned about the macular oedema I was given drops, I was given tablets and ultimately I had a small procedure on one of my eyes.  I was seen every 3 months for the first 4 years after my diagnosis.  I had appointment after appointment, low vision clinic, VI Liaison Officer, this test, that test the list went on and on and it was absolutely exhausting.

To be honest I didn’t really understand it all back then, I had been used to appointments, audiology and speech therapy. Looking back my life had very much been about working with one adult or another on a one to one or in small groups because of my deafness. 

However, as time progressed and I matured I began researching retinitis pigmentosa, macular oedema and Usher Syndrome - naturally I began to realise the severity of my condition and over the years I have felt a strange kind of grieving for my sight.  I have been sad, I have cried, I have been angry, I have felt emotions I never knew existed, the result of which I found myself feeling very different, very isolated and on occasion very scared of the future.

Whilst we were all dealing with my progressive sight loss the one thing that enables me to be me is my deafness, as strange as it sounds my deafness had become the one thing that could, would and does enable me to live a pretty positive life, but only with assistive technology.  There is some amazing assistive technology to enable deaf people.

I was born deaf, I never grieved for my loss of sound, I’d never had it to lose it!  I had hearing aids from 18 months old, they could enhance and enabled me to access sound, thanks to those old analogue hearing aids I learnt to speak.  It took a time but I was very well supported.  The digital hearing aids I got then enabled me to really get going and along with a radio aid I did really well and was able to be as inclusive as I chose.  There were difficulties but I had developed coping strategies because deafness and hearing aids were all I knew.

One of the first things I noticed just before my Usher Syndrome diagnosis was that I felt more deaf.  I was really struggling to hear, what I hadn’t realised straight away was that my sight loss had denied me the ability to lipread, see facial expression and gestures, things that help enable deaf people to hear, yes, us deafies hear with our eyes.  Lipreading is a huge part of understanding and enablement, up to 40% is heard with our eyes, I had lost that.

Looking back now losing the ability to hear with my eyes made my life so much more challenging.  I don’t think people realise how tiring being deaf is, always having to concentrate on listening, having to use your sight to access information, its tough, however deafblind is beyond exhausting and sometimes totally overwhelming. 

Having Usher Syndrome is truly challenging and painful for the people closest to us, often a feeling of helplessness because so far there is no cure, however there is some incredible assistive technology available.  In my opinion we need access to it and we must embrace it to live as positive a life as possible.

Being deaf and going blind is gut wrenching but what many tend to do is concentrate so much more on the blind part of the condition when in actual fact and in my opinion we are missing out on the real importance of our ears. 

Much more consideration should be given to our deafness and what hearing aids can do to enhance our lives.

For those following my story I have been fortunate to be wearing GN Hearing smart hearing aids for several years. 

My Resound LiNXQuattro smart hearing aids have enabled me to literally fill in the gaps made from the loss of visual clues like lipreading, facial expression and gestures and more.  These visual strategies are said to be 40% or more of how deaf people access sound! 

Today I hear so efficiently that I no longer miss the visual clues.  I can hear around doors, I can hear inside and outside, I have directional hearing, I hear tone and clarity.  I have depth in my hearing, I am very fortunate. 

Naturally my hearing aids have an app, Resound 3D, it enables me control over my hearing. 

I can use my iPhone or applewatch to make adjustments: I can adjust volume settings, mute my hearing aids.  Adjust speech focus, noise and wind noise levels. Adjust treble and base to suit.  There is a tinnitus manager.  There is a help locate lost or misplaced hearing aids.  There is a remote audiology assist.  The list goes on and on.

I can use my iPhone as a phone thanks to bluetooth connectivity and streaming, imagine life without access to a phone, I had that for 20 years!

Before smart hearing I would switch off my hearing aids whenever I could choosing silence to relax after a busy tiring day, today I relax listening to music, music I can hear, lyrics I can hear and I totally enjoy it, music is so therapeutic.

Without a doubt I’d love to be able to see again but its not happening anytime soon so I embrace as much enabling technology as I can to live a fulfilled life.

My smart hearing aids are the catalyst to my accessibility toolkit and should be made readily available to all hearing aid users living with Usher Syndrome.

 

Tuesday, 03 March 2020 06:51

Typical Sunday!

Catching up with family and repacking for a trip up to the north of Scotland to visit family. 
Anybody who has hearing aids knows it crucial to make sure you always , always, always carry a spare set of aids if possible, just in case!  As I was packing I came across my old NHS pair of hearing aids, at the time (2019) I was assured they were the best on offer!  They had served me ok in the past, they had to!  I decided to check them out and change over new batteries. I decided to put them in to remind myself of the sounds I used to hear.  The horrible sound of feedback, whistling and a rustle through the moulds. I started talking to Lyn my wife and realised just how different my voice sounded to myself.  It sounded like I had a cold and was very dull. I found it difficult to wear them and quickly changed back to the clarity of my LiNX Quattro, there really is no going back, there really is no comparrison.  I remember Molly saying there is no way she could go back to her old hearing aids, now I know exactly why.  It’s only been a few months with my new smart hearing aids but today was a sharp reminder as to just how good the GN Hearing smart hearing aid technology really is.  To deafblind people like myself having access to clarity of sound is essential.

Many thanks again to Molly of the Molly Watt trust and her dedicated family also to gn resound . Let’s get everybody on these. They do change lives. 

Tuesday, 03 March 2020 06:29

Finally Connecting with Sound

On February 3rd 2020 at the age of 31 I finally entered into the hearing world properly for the first time. I’ve had years of NHS hearing aids and the best they could offer still wasn’t enough as my eyes deteriorated I needed to tweak my hearing aids constantly in an attempt to hear as well as others, but issues with feedback, echoing and blurring of words still persisted no matter what I did. So the best in the NHS for someone with DeafBlindness still didn’t suffice as I strived for independence with my guide dog. 

So on this morning I felt nerves and skepticism as to what these Resound LiNX Quattro 9s from the renowned GN Hearing could really offer me. My daughter Lily (9) was excited for me to have them she was looking forward so seeing what technology they had In store. Jane & Molly Watt arrived at my home to take me to Correct Hearing in Carlton Nottingham to see audiologist Sarah Vokes.

On arrival I entered into the audiologist Sarah’s room and we began by getting to know each other, finding out what I could get from these hearing aids in my day to day life asking my hobbies and commitments so that later on Sarah could adjust the LiNX Quattro’s specifically to suit me. I needed a more up to date audiology test which I had there and then, this again was used to tailer the hearing aids to my ‘prescription’ to ensure I got the most out of them as they would adapt to my hearing loss. 

Then Sarah showed me the new hearing aids, my mind was blown! They were the tiniest ones I’d ever had and the tube that went into my ear wasn’t a tube but a thin wire with a processor at the tip where the dome went round and fitted snug in the ear canal close to the ear drum! I’d never had this and didn’t even realise that technology was possible, this was amazing to me. Then Sarah talked me through the settings as she synced the aids with my audiogram and tailored the settings to my lifestyle.  

Then came time to pop them in and switch them on! Immediately I heard conversations behind me and surround sound and I could accurately pinpoint where sounds where coming from, something invaluable to someone with tunnel vision.  I felt like a baby hearing familiar sounds but in a totally new way, we went outside and tested traffic, I could hear cars approaching from a distance accurately, again something invaluable for me with my guide dog to keep us safe when walking the streets.  

It was overwhelming, speech so clear I could hear my own voice and mis pronunciations even hear my own chewing for the first time, trust me that was a weird sensation for me as I’d never heard my own jaw before! These hearing aids were enabling me to hear for the first time IN my ear canal the natural cone shape of the ears were for the first time useful as I’d only ever had microphones behind my ear, these had a microphone IN my ear so yes at 31 years of age I was hearing like a normal person would for the first time!

After some fine tuning with Sarah ensuring I wasn’t too overwhelmed initially, she connected my Apple Phone and AppleWatch up to the Resound 3D app so I could change ‘channels’ and turn up or down the volume etc once I knew everything that was needed for now, we left with an appointment for a months time to check everything is going well.

When we left me Molly, Jane and our Guide Dogs Minnie & Bella went for a bite to eat to put my hearing aids to the test, I switched to ‘Restaurant’ mode and wow, yes I could hear the surrounding chatter but I could also tune in directly to Molly & Jane's voices, clearly, their speech sounded familiar but so different as I’d never heard such clarity in voices before. I could hear things were far away and what was close to me, for the first time since my sight declined I had depth perception thanks to these Line Quattro smart hearing aids. I was so happy!

Over the last few weeks since my switch on, I’ve had my follow up appointment which has turned me up to 100% of my hearing prescription, we did this in stages to allow my brain to adjust to the new sounds. The past few weeks have been interesting, I’ve been playing with the app, so I’ve set the GPS for when I take my daughter to school my hearing aids automatically switch to outdoor mode with speech clarity and noise reduction on low so this enables me to hear all around me knowing if children in the playground are close or far away and most importantly I can pinpoint my own daughters voice amongst the playground chaos.

I've done similar for when I attend tennis lessons or go on dog walks, the connectivity of these hearing aids is something I’ve never had but now I do I’m finding it so useful in daily life. Im not constantly walking around adjusting the volume like I used to, I’m now just turning speech clarity on or the treble up or just using a preset setting like ‘Outdoors’ or ‘Restaurant’.

Since becoming adjusted to the Linx Quattro’s I’ve heard bird song, drones, the Clock Ticking even my own dog makes noise when she yawns, I’ve never heard them before. I’ve had to remove the clock from my wall as I’d never realised how loud the ticking is! I've heard the low sounds of London Tubes under my feet while at an appointment at Moorfields eye hospital, I knew they were there before but I’d never actually heard them before.  

Hearing bird song, struck something emotional in me, my mother passed away 2 years ago, she used to love hearing, watching and feeding the garden birds something I struggled to indulge with but my daughter did wholeheartedly and it became a real grandmother & granddaughter connection for them both. So for me to now hear them sing as spring approaches is amazing, and it doesn’t have to be quiet for me to hear them either, I can hear them when the trees are rustling and children and playing near by, these hearing aids pick up that higher tone of bird song I couldn’t hear before. A precious gift to me.

I could go on, for example the way I hear music now is totally different. But for now I’ll leave it on that note until my next blog. In the mean time I’d just like to say a personal emotional heartfelt thank you to the Molly Watt Trust and GN Resound for enabling me to be Case Study 2 along with Sarah at Correct Hearing, you really have no idea what you have given me, words cannot describe the difference this has made to my life. What you have given me and my family is truly priceless, Thank you!

Wednesday, 19 February 2020 08:44

Claire's Fitting

Thursday, 28 February 2019 13:54

Equal access to sound for all

Today is ‘Rare Disease Day -2019’

So I decided on a blog about hearing aid technology that can and does enhance lives. 

This http://www.nciua.org.uk/latest-new/ was brought to my attention several weeks ago.  My first thought was FANTASTIC for those choosing cochlear implant but the more I thought about it and re-read it the more concerned I have become.

Of course this is really great news for those choosing cochlear implant, great that the criteria has changed making, I assume, application, assessment and presumably funding easier, however what about those of us using hearing aids, isn’t our access to the best available hearing aid technology as important?

I then read this: http://www.hearingreview.com/2019/01/better-hearing-better-living/ and felt compelled to have my say!

I have always advocated for those who wear hearing aids.  Having worn them since I was 18 months old they have been my access to the world, to sounds that enabled me to access speech and oralism, also very importantly to access the sounds of danger when combined with visual clues.

My belief is being able to hear opens the door to a whole world of inclusion and independence, that said it isn’t easy.  Learning speech takes years and lots of patience and commitment and it is exhausting. 

All people born deaf have to learn to listen and understand sounds, not just profoundly and severely deaf but also those with mild and moderate hearing loss who do have similar challenges accessing sound. It is fair to say all deaf people have to work really hard just being themselves! 

I have met profoundly deaf people doing well and mildly deaf people struggling.  Level of deafness does not always demonstrate need, just some cope better than others.  I have in my lifetime been bullied because I am “not deaf enough” the fact is I am deaf enough to need hearing aids, I hear only loud noises without my hearing aids and lipreading these days is out - deafblind is hugely challenging.

Since losing most of my sight I am almost totally reliant on hearing technology to access the world.

I am a hearing aid user, I do not require or want cochlear implants at this stage in my life I cope very well with state of the art smart hearing aid technology, technology that has enabled me to access so much more than I had ever imagined. 

I work and have many contacts around the UK working for the NHS and the funding of hearing aids seems to be so complicated I have been unable to get the answers I’m looking for. 

I am no expert on these things.  I have tried to find out who funds what, it is all so incredibly complicated, NHS Trusts, NHS England, the list goes on, then there is the post code lottery which dictates who gets what hearing aids.  Sadly many NHS Trusts have little or no experience of deafblindness and attempt to assume our needs!   In short we miss out.  It is a fact that the deafblind should be provided with two pairs of hearing aids and two pairs of glasses if appropriate, again this is a postcode lottery, but demonstrates the needs of those with dual sensory impairment do differ considerably.  Surely in recognising this why do we not go further and offer the very best in hearing aids as smart hearing aids can and do to a degree compensate for visual impairment.

I rely on my hearing aids more than ever before, they are my link to people, to speech and to education and I don’t just mean school, I mean learning to be a part of society, away from silence and it’s isolation and in my opinion that is where so many problems can begin.  The hearing aids currently provided by the NHS can only offer so much, having lost visual clues means they do not meet the needs of the deafblind. 

Inappropriate hearing aids can lead to a lack of confidence, a vulnerability and often isolation and depression as blindness sets in.

At 20 years old I was very fortunate to discover GN Hearing and to benefit from “Smart Hearing aids”.   In 2014 I was fitted with Resound LiNX2, then LiNX3D and now LiNXQuattro and it is really here that I want to begin:

Smart hearing aids have revolutionised access to sound, they are a real alternate to cochlear implant, they enable choice for those with all levels of deafness and whilst it could be said they are more expensive than the standard NHS hearing aids they are a fraction of the cost of cochlear implant and when considering what they provide for the deafblind they are priceless.  I cannot put into words how enriched my access to sound has become.  I now hear things I had never heard in my life, as a result I can do so much more.  Not only that but my confidence is at an all time high, I can access the world around me. 

Smart hearing aids have enabled me to use a telephone, they give me directional sound.  They literally help compensate for my vision loss.  Directional sound and enriched hearing means I can engage with people, I hear danger and I can live a pretty full life.

I’d really like to know why funding quality hearing aids is such a big issue, the ROI (return on Investment) like cochlear implants is clear, they provide inclusivity not just to sound but to our world.

Living with dual sensory impairment, deafblind is more than challenging and from my view point very frustrating as I know exactly what smart hearing aid technology is available and yet most are denied this because of cost. 

In a paragraph at 24 years old I have experienced the evolution of hearing aids, from analogue to digital and now to smart hearing aids resulting in me being able to “Hear more, Do more and Be more” doesn’t everybody deserve that?

Usher Syndrome is the most common cause of congenital deaf blindness, that said according to recent research we are between 1 in 10,000 and 1 in 20,000 so in the scheme of things rare. 

We met a lovely lady from Northern Ireland at a Molly Watt Trust ‘In it Together’ event in Edinburgh in October 2015, she has been a hearing aid user since she was 4 years old and was managing very well.

At that particular event we were very fortunate to have a presentation from GN ReSound’s Graham Roberts who was able to share with us the many impressive hearing aids and products available from the supplier.  

That presentation together with Molly’s in depth and ongoing testimony of her GN ReSound LiNX² and how they have enhanced and enabled her life led to an influx of enquiries about how and why these enabling smart aids are not available to those living with Usher Syndrome (deafblindness), particularly as they offer far more than most hearing aids provided by the NHS.

It is fair to say everybody at that event, particularly those using hearing aids where very interested in the enablement smart hearing aids can provide.

Molly coped with standard Phonak hearing aids provided by the NHS until she was 20 years old, however, from 14 years old when she became registered blind, deafblind she struggled and the reason for this in the main was because of her blindness.

For 6 years Molly insisted her hearing had deteriorated even though test after test showed her hearing, thankfully remained stable.  

In actual fact what had happened was her ability to lipread, to use facial expression and body language had gone along with her sight, her ability to access sound and communicate had dwindled, demonstrating just how much the deaf rely on their eyes to hear.  In other words she could no longer ‘fill in the gaps’ that her eyes had enabled.  

It is a fact that the deaf hear with their eyes.

GN ReSound LiNX² provide a hearing experience those who have experienced really benefit from.  They provide an incredible clarity of hearing, they enable directional sound meaning that whilst Molly is blind she can now turn towards a sound, something she could not do with her NHS provided Phonak hearing aids, she can also identify not just where sounds are coming from but most importantly she knows the sounds of danger - just imagine how important that is to safety for the deafblind.

These hearing aids have incredible bluetooth connectivity to iPhone and applewatch, also android devices giving the ability to many deafblind to access aurally things they could only dream of previously.  Using a telephone as a telephone for the first time ever is most certainly a huge positive.

Not only can they be adjusted be independently adjusted but enable a very personal hearing experience and in so doing bring great confidence and independence.

Our friend from North Ireland was one of many desperate to have access to GN ReSound LiNX² and indeed has asked her NHS audiology department, their response was somewhat bizarre - an appointment with the Cochlear Implant Clinic!

Cochlear implant is amazing technology for the deaf, however, not only is it not for everybody it is also incredibly intrusive, expensive and most importantly not what the patient wants why not consider more enabling hearing aids which happen to be a fraction of the cost. 

Figures suggest the cost of 1 cochlear implant would be close to the cost of 10 pairs of best quality smart hearing aids!

There are lots of people who have been hearing aid wearers a long time and like Molly have gotten on very well with them but now need more as Usher Syndrome/ blindness looms close.

GN ReSound have a variety of hearing aids including the ENZO2 for those with a profound hearing loss and whilst they are the creme de la creme of smart hearing aid technology their cost in comparison to cochlear implant is very reasonable.

Cochlear implant involves surgery, it is considered that deafblind people should have two implants to enable directional sound for safety and rightly so,  directional hearing is a must for the deafblind, safety is imperative for all, whatever hearing aids worn. 

Most get one implant at a time so often two surgeries after each substantial aftercare and rehab at huge cost.  

I’m not saying this shouldn't be the case, of course it should where appropriate, however not every deafblind person wants such invasive surgery preferring an alternate option.

Patients should always have a say in their care as it tends to be them who are the real professionals and who will have done the research based on their condition.

I know there is a long and in depth assessment for cochlear implant and so there should be but surely every patient’s individual requirements should be considered?

My friend in Northern Ireland was told there is a 3 year waiting list is this acceptable?  Why not consider best hearing aid options?

We continuously hear that our NHS remains financially fragile, that it needs more funding but very rarely do we hear about solutions, about genuine savings that do not include reducing staff - surely this scenario has to be seriously considered

I would like to say our friend in Northern Ireland’s scenario is unique but sadly it is not!

 

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