I have Norrie disease which is a very rare X-linked genetic condition which causes mainly boys to be born blind or severely sight impaired. The majority of sufferers also experience progressive hearing loss. Other secondary symptoms can also include autism and cognitive impairment.
I first noticed something was wrong when I started getting severe bouts of tinitis in my left ear back in about 2001. It would hang around for four to six weeks, then disappear as though nothing had happened. I had no idea about steroid injections back then and I still have no idea what to tell a doctor about it if I were to try it. I just know that steroids apparently can have a positive effect.
I kept battling with these bouts of tinitis until 2005, when I had a severe bout that just wouldn’t go away. I was training to have a guide dog at the time and the episode hit me right at the time we started training. My hearing was fine the day before, then it just came on all of a sudden.
It was early in 2006 that I received my first hearing aids. The sound I remember most hearing that I hadn’t heard in a long time was my wife at the time, crunching on her cornflakes! It was quite a novelty back then!
My hearing continued to deteriorate, and I started to learn the caveats that go with hearing aids, such as the maintenance of tubing, wax blockages and all the things that can stop a hearing aid working to its full capacity. I had several blockages of wax in my ear itself and I played around with several different ways of dealing with it. In the end I found that just regular check-ups at the doctor was the best way to avoid complete blockages, no matter what cleaners you use. I even tried just rinsing my ears in the shower every night, but the doctor reported when he cleaned my ears out that mold had grown inside my ear! So that didn’t work either.
Then came the hassle of learning how to use the phone effectively as my hearing deteriorated to a point where I could no longer use the phone with my bare ear. I started using telecoils, but they just weren’t as effective for me at first as they should have been. I have always participated in a lot of teleconferences both for my employment, and for voluntary activities, and this work became extremely difficult. Then one day, I just decided I would try the phone in my left ear, and surprisingly, I got a much better result despite my left ear supposedly being the worse side. Each of my ears sound different, but my left hearing aid and telecoil gives me a much fuller sound on the phone than my right for some reason.
Then I discovered the wonders of Skype, good quality USB microphones and good quality headphones. Skype has made a huge difference to my life! So too has the IPhone and its very good quality sound through headphones, particularly when using Skype or Facetime. These tools have made an immeasurable difference to my level of stress when communicating with people in teleconference situations. It’s much easier to follow fast moving conversations. Doing this with one ear on a phone is very hard work.
I would like to focus for a while on hearing loss in the context of being a musician, and also being totally blind. I might diverge into other areas as well as I write this, but hopefully, that will add to the information you are looking for.
I worked as a producer and session musician in recording studios between 1998 and 2004. I loved the work, and in the few times I’ve had studio work since, I’ve found that I can still cut it as a producer and player in the genre that I’m familiar with.
To be honest, the loss of being asked to play and record by other musicians has been the biggest source of grief to me in relation to my hearing loss. During the time I worked, I dealt with the tinitis that I referred to earlier, all the time trying to hide that I was having any difficulty. Amazingly, I received a Facebook comment from my good friend Michael Fix recently that when we were mixing an album, I was sitting on the lounge in his studio at the back of the room, which he said was the worst spot for acoustics in the room. I was telling him what I wanted raised or lowered in the musical mix, and he said I got everything spot on! I don’t know whether that was during one of my bouts of tinitis, but without attempting to boast, it would have been at the height of my musical abilities that I did so.
Another comment from someone I worked with in the late 90’s stated that I was able to pick a note on a bass guitar track that was a semitone out from where it should have been, so it was disastrously out of place in terms of a commercially viable recording. Nobody else picked it.
That gives you an idea of the sensitivity in hearing that I once had. My vocals when I sang were almost always perfect. Takes on vocal tracks I did needed minimal adjusting or dropping in. I was master of all I did vocally, and I could tell an artist exactly what needed fixing in their performance and how to do it.
Live work though, was a different story. From when I was a teenager, I needed to be amplified really well on stage so that I could clearly hear what I was doing without any doubt. I dreaded playing on sound systems that had bad onstage monitoring, because I was always doubtful of what I was singing. This tells me in hindsight that my hearing was deteriorating from much earlier than I realised it. Other people didn’t care how good the foldback speakers were on stage, or indeed whether there were foldbacks there at all! They just performed effortlessly and worked with whatever they had without complaint. I envied them!
It was in 2008 that I eventually had to give up singing professionally. It happened suddenly. A month or two earlier, when my wife and her family came to see me sing, I was spot on. But in the intervening time, my hearing had deteriorated to a point where I could no longer tell that I was on pitch or not. I was wearing hearing aids by this stage, and in the few months before, I had experienced episodes where everything seemed to blur, and I could no longer tell what key the song I was about to sing was in. It was embarrassing, because I didn’t know what I was doing at all for a while there. But I kept trying to cope with it until eventually, one of the staff at the club I was playing at asked my wife why I was not singing in pitch. I knew then that it was time to quit. It was impossible to hide my difficulties anymore.
This was a big loss in income for me, but also, a big loss in terms of my identity as a professional musician. I have worked on and off over the years to try and get back what I had. Sometimes I think I’ve got it! Then I start to pick up faults, or hear from someone else that I’m off key, and I realise that it won’t ever be the way it was, when I just didn’t have to think about what I was singing. There will always be that margin of error, big enough that I can’t get away with it.
Playing can be flurry and has its share of doubt as well at times. Not the playing itself, but the worry that I’m hearing the chord changes in a song correctly if I’m trying to learn it to play behind someone else. I was involved heavily with a church for five years, and being a part of their music team gave me the opportunity of developing the skills to hear new music properly. I need to hear things close up through headphones, and I need new material in advance so I can make sure I’m hearing it right. This opportunity was there in the church. Unfortunately, I lost my position on that team in the church due to doctrinal reasoning let’s say, following the splitting of my marriage.
In later years, I actively pursued the music scene and tried to get known in Melbourne where I lived for three years, but I never got included in any projects. I was honest about who I am and the challenges I experience, and said that if I have material in advance, I could learn it and be professional. But it didn’t work. Sometimes, if there was a piano in one of the pubs they were playing in, the local musicians would get me up to play some old standard stuff, but they never asked me to be involved in upcoming gigs or in recording new material. I don’t know why. This is what causes me the most grief about my hearing loss. They obviously doubt my ability to be professional, and that is what breaks my heart. I am a musician by nature. I cannot change it. I’ve tried, but it doesn’t work. I always come back to it.
I have ideas about how to be involved in the music industry from a different perspective. I just haven’t had the time or energy to pursue them due to my paid work. But I will get to it, and I am determined that once again, I will become a valued member of the country music industry in Australia as I once was.
My return to Brisbane has led to my involvement in a band which specialises in original material, headed up by a friend of mine who is also blind. Along with Michael Fix whom I mentioned earlier, David Truong has unreservedly given me the opportunity to be part of a developing band. I really appreciate being able to keep my hand in and my skills up! Today I recorded some acoustic guitar parts on his upcoming single in the studio, and it was just as rewarding and natural as it always was for me.
Finally, a word on the importance of research into hearing loss. As a person who is also blind, employment opportunities are very hard to come by. I have been fortunate however, but so many others who are deafblind have not had the opportunities I’ve had. If we are able to restore hearing loss in people who have progressively lost their hearing, it would mean that they are given back something which one can only recognise as a gift from above which we took for granted until it was lost. I have no concept of what it is like to see. To me, sight is like science fiction. But hearing and its loss is very real in my experience. To be able to walk through a shopping mall and be able to instantly recognise a song playing through some distant speakers again would be amazing! I envy people who say as we’re walking along, “oh I haven’t heard that song in years! I love it”! I usually ask at present, “what song is it, I can’t hear it”! Simple pleasures would be restored if we were able to put our time, energy and money into research that would make an astronomical difference, even more so for others than for me. I would be able to sing again and to resume my career doing something that as I said, is a part of me. Others however, would be more employable than they have had the chance to be. It is sad to have to reflect on that reality, but it is certainly true that employers look first at a person’s level of function and efficiency. I certainly don’t endorse such a narrow focus, but it sadly is something which we have to contend with, even though as people who are deafblind, we have not only invaluable skills to offer, but also insights which cannot come from a life without disability.
Family Stories are the personal views and experiences of the authors and should not be read as action or advice from the Norrie Disease Foundation.