Choosing a Cochlear Implant with Usher Syndrome

Martha Steele

How a cochlear implant helps me cope with progressive vision loss

This is part two of a series.  View part one here.

Over the following months, I had a comprehensive evaluation to determine my eligibility for an implant This included interviews with a surgeon, a social worker, an audiologist, and an academic researcher.  The latter had the job of telling me everything that could go wrong, including the fact that my hearing might actually be worse after the surgery than before.  I exchanged several emails with this researcher and finally concluded that his caution was appropriate but that he also seemed optimistic that the chances of success were at least better than average.  At the same time, I began talking to CI recipients, attending seminars and exchanging emails with other cochlear implant recipients, including a few who also had vision issues.

I felt supremely confident in my surgeon and did not worry about the surgery itself.  I was nervous about how effective the CI would be, knowing that if it was not an improvement, I could not turn back, as the surgery may damage residual hearing.  Still, I thought the likely benefits outweighed the risks, and I decided to take the plunge.

I was activated in late April 2010.  The initial sounds were very similar to one another and very tinny.  I couldn’t understand a word, but oh my, was I hearing!  That first day, I was hearing consonants that I had never heard before.  And to me, it was those consonants that made so much of a difference in speech understanding.

It has now been over two years since I was activated, and I still wear a hearing aid in the other ear.  The CI has been a stunning success, exponentially improving my ability to understand in all situations, including noisy environments.  It is not perfect, as I still struggle in very noisy restaurants with people sitting across the table from me.  But so much has improved at a time that I most needed help, at a time of losing my ability to see faces and, more specifically, lips.  Only six months after I was activated, testing in a sound booth of single syllable words revealed that I correctly repeated 85 percent of the words and was nearly correct on the remaining 15 percent.  My audiologist was ecstatic, as was I.  I am no longer hesitant to attend large meetings, no longer hesitant to ride in the back seat of a car with people who no longer have to turn to face me when they speak, and no longer hesitant to address questions from an audience in a large room.  I can hear the difference between the “ch” of Chicago and cheat.  And best of all, I can hear and identify bird songs, something that has restored so much of my enjoyment of my passion, birdwatching.

The next big question for me is whether to go bilateral and get a second CI.  That is a leap that I have found challenging to take.  I find the CI and HA complementary: the CI picks up the high sounds, the HA the low sounds.  The HA enables me to enjoy music with natural, though amplified, hearing; I find music with the CI still very mechanical sounding.  Given that my understanding has been so good even with just one CI, the added benefit of a second CI for speech understanding alone is not very clear to me, especially in light of the likely loss of all hearing in either ear.

However, my continued decline in vision may once again be a factor in my decision on whether to go bilateral.  I am more and more reliant on auditory cues to help navigate the world.  Street crossings often involve hearing the buzz of a walk signal, the direction of traffic flow, and the speed of vehicles approaching the intersection.  I find myself turning my head so that the CI ear is positioned best to listen for the auditory cues.  I also often position myself in other situations such that I favor the CI, such as having someone sit to my CI side to better understand.  Do these conscious maneuvers mean I should be considering a second implant?  Would a second implant enhance my safety?  Clearly, I need to consider this decision more now than ever before.

For now, though, this CI never ceases to amaze me and I never take this gift for granted.  It was clear that I was struggling more and more as I lost central vision acuity, and the CI has made coping with the vision loss so much more manageable.  It has dramatically improved my hearing experience in all types of environments; it has allowed me to know that I can pursue my passion of birding for a lifetime despite my vision loss; it has given me more confidence to interact with people I don’t know without being worried about missing important conversational pieces; it has simply made a profound difference in my life.

There is a similar device recently commercialized in Europe for people with total loss of sight. The retinal implant operates similar to a cochlear implant in that electrode excite the optic nerve to enable the recipient to see something. For now, this implant gives little more than shapes and shadows where total darkness had been, so it is not appropriate for everyone with vision loss.  It reminds me of the early days of the cochlear implant, when the first recipients had to carry around a suitcase of wires.  Over time, of course, the technology improved and the CI provided benefits to more and more people with the improved technology.  I expect similar and rapid advances in retinal implant technology. Perhaps one day, that technology will help me sport a bionic ear and a bionic eye!  In the meantime, I continue to be stunned by the CI experience and may well opt to go bilateral, especially if having two CIs will significantly improve my safety navigating the world.  That is my next decision and my next challenge.