Pioneering the Hybrid implant
While Leone's hearing loss is genetic, her kind of loss is different to that of the rest of her family. Hearing aids could not help her to hear. She struggled for years with poor hearing until she heard about the Cochlear Hybrid implant - a device which would help her hear high frequencies but maintain her natural low frequencies. This is her story.
Leone Sperling October 2009
Download a copy of Leone's Story Brochure
In 1989, at the age of 52, I started losing my hearing due to genetic nerve degeneration. My grandmother, mother, aunt and brother all had a hearing loss and had to wear hearing aids once they reached their 60s. However, my hearing loss is different from theirs. I have a 'ski-slope' loss which means I have very good, normal hearing over a wide range of low frequency sounds but then my ability to hear drops away dramatically to profound/severe deafness in the middle and high frequencies.
With a hearing loss like this, for many years I could actually function in the real world without hearing aids and without an implant. I could understand everything spoken in a quiet environment with one or two people, as long as I could also lip-read. However, I couldn't hear when people turned their heads away or if there was any background noise. But even with my good residual natural hearing I couldn't understand the TV and needed the additional help of captions. I couldn't understand the sound at the movies or the theatre and I couldn't hear people speaking in cars or in restaurants.
Hearing aids are of very little use for a 'ski-slope' hearing loss because they amplify the full range of sound frequencies. I can't tolerate amplification in the low frequencies but am so deaf in most of the middle and high sounds that restoring them is beyond the capability of a hearing aid. An audiologist once told me my kind of hearing loss is an audiologist's nightmare.
I managed without hearing aids for about seven years but in 1996 I experienced a sudden and dramatic loss in my right ear. I was fitted with an in-the-ear hearing aid in my better, left ear but was told my right ear was so bad, a hearing aid would be of no use. Then in 1999 I suffered a similar sudden loss of hearing in my left ear.
I was referred to Professor Gibson and sent to Cochlear for assessment. However I performed so well on tests that involved listening to sentences that I was sent away, being told I could hear better than anyone with an implant and the technology had not yet been invented to help people like me.
The reason I managed so well was because I was using every skill I had to help me to hear. Most of my adult life had been spent reading, studying, teaching and writing English so I have a great facility with the English language. This enabled me to become very proficient at guessing words from context. In addition, I'm also a good lip-reader and together, these skills enabled me to score well on hearing tests involving sentences.
Because I appeared to manage well, people didn't seem to understand how exhausting listening was and I felt isolated from members of my large family - four children, their partners and five grandchildren - because I couldn't hear anything that was going on at family gatherings.
Professor Gibson's audiologist fitted me with two behind-the-ear hearing aids but they really didn't help and I only wore them to be polite.
About nine years ago I joined Better Hearing and attended lip-reading classes. Although I was good at lip-reading to start with, my skill improved greatly over the four years I took classes. As well, at Better Hearing I learned a great deal about how to manage my hearing disability.
I kept hoping new technology could help me
Through the years I consulted other audiologists, always hoping for some help and although I met with sympathy and understanding, there was still nothing available which would help with my kind of hearing loss.
I first heard about the Cochlear Hybrid implant at a Better Hearing Conference in September 2006. At last there was a device that utilized, rather than destroyed, good residual low frequency hearing. I asked to be part of the research project to be undertaken. I was assessed as a suitable candidate and in June 2007, became the first person in Sydney to receive the Hybrid implant. Although I knew there was some chance of losing my hearing in the implanted ear, I was sure this would not happen. I felt the hand of Fate was working here. I had been waiting for something like the Hybrid to be invented and here I was in the right place at the right time to be the first person in the Sydney trial.
Professor Gibson and the Cochlear research audiologists, particularly Paula Incerti, treated me very specially and I found the 15 month trial interesting and rewarding, though certainly time-consuming.
Although warned not to have high expectations, I still anticipated hearing perfectly at 'switch-on' and was disappointed to hear this terrible noise - high-pitched, robot-like, artificial, Donald Duckish sounds. Gradually this improved and I was able to hear that the 'voice' of the implant had the tonal variation and sound patterns that mirrored the words I was hearing. But the voice of the implant was not clear and even now, over two years since I received the implant, that voice is still not clear.
For me the Hybrid works in this way. My good hearing in the low frequency sounds enters my ear unimpeded through the wide vent of the hearing aid component of the Hybrid device. This component picks up and amplifies a small range of the lower middle sounds while the implant itself is programmed to supply me with all the middle and high sounds that I have lost due to my deafness. The effect for me is that somehow the input of sound to the middle and high frequencies enhances what hearing I have left so I feel as if I am hearing better naturally.
The implant has been beneficial for me. I can now hear a lot better in social situations. I can participate in conversations at family gatherings although I still have difficulty hearing what my grandchildren are saying because of their high pitched voices.
I can hear people across the table in a restaurant and I can have a conversation in the car. I can hear quite a lot at the theatre if I sit in the first few rows and only need to glance at the captions occasionally and I can hear some of the voice-overs in TV news programs. I can hear in meetings and I have been studying Latin for the last seven years and can now often hear what fellow students are saying right across the classroom.
I still can't hear well enough to listen to the radio or watch TV effectively without captions and I can't understand enough to go to English language movies. I don't understand the announcements in airports (but then hearing people tell me neither can they!) and I only hear about one word in ten of tour guides' explanations and descriptions.
However, when I turn my implant off I feel terribly deaf and have become dependent on it. I always wear it when I'm going to have contact with other people. I now find a slight benefit when I use both the hearing aid component of the Hybrid device and wear my behind-the-ear hearing aid in my left ear.
Although there are still so many things I can't hear, I feel privileged to have been a part of this research and I am very grateful for the improvements the Hybrid implant has made to my ability to hear in social situations.