As a child, I didn’t know that the way I heard the world around me wasn’t the way everyone else did.
Because of the work I did to fit in, even the school nurse couldn’t notice my condition during annual hearing and vision checkups.
But by the time I was a prospective student at age 16 — and fell in love for the first time — my condition, caused by tumors on my auditory nerves, was unavoidable: My hearing was going fast, and fast.
Medicine has come a long way in the past thirty years.
As recently as 1993, only about 5 percent of newborns were tested for hearing loss before leaving the hospital. Now it is almost universal. Parents must opt out of the test, and few do.
In the north of 97 percent of newborns have a hearing test within the first few days of life. The reason for this dramatic shift is the knowledge of what is possible through early detection.
Many cognitive pathways that permanently influence speech and overall development are formed between birth and age three. Early detection leads to early treatment. That’s why you see many more children with hearing aids today than 30 or 40 years ago.
Matt in hospital with his then girlfriend Norah after receiving his diagnosis
Matt Hay has written about coping with hearing loss by tapping into his memories using his favorite songs, from Beck to Simon and Garfunkel
At the other end of the age spectrum, only about 7 percent of people over 50 with hearing loss receive treatment, including hearing aids. That number rises to approximately 17 percent of people over 70 who need hearing assistance.
Although more seniors are getting hearing aids when they need them, the numbers are still low.
You would expect older people to accept the reality of hearing loss and take advantage of the technology available. But a majority who need help do not get it. This is partly due to cost, partly due to vanity, partly due to an aversion to technology or a hesitation to change.
Whatever the reason, almost 80 percent of people over 70 who need hearing aids do not get them.
As big a problem as that may be, there is still a segment of the population for which virtually no data is available: people who experience hearing loss sometime between their first day of kindergarten and their 50th birthday.
That’s a lot of people stranded on an island for years. There’s a lot going on in that part of life. It would be nice to know your options.
Those were some of the countless thoughts I had as I read everything I could about neurofibromatosis type 2, commonly called NF2, the diagnosis I received from a team of specialists not long after my first call from IU Medical Center when I 19.
Children are now universally tested for hearing loss from birth, but when Matt was born, only about 5 percent were tested
At the other end of the age spectrum, only about 7 percent of people over 50 with hearing loss receive treatment, including hearing aids.
For starters, I learned that my condition was genetic and relatively rare. I have not contracted a virus or developed any disease.
Sometimes NF2 is passed on from parents to children and sometimes it appears spontaneously. Either way, it’s caused by a defect in the gene that gives rise to something called schwannomin, a structural protein found on chromosome 22.
The result of this defect is benign tumors in the brain, spinal cord, and peripheral nerves, most of which are cranial nerve VIII, the auditory vestibular nerve.
That’s a complicated way of saying there was corrosive buildup on the wiring between my ears and my brain. It’s also a great opportunity to make a dark joke about “NF tumors getting on my nerves.”
The ears themselves, all the little bones and hair follicles that capture and filter sound, seemed to work fine. My eardrum vibrated as much as anyone else’s. The blockage occurred in the nerves that carried that sound to my brain.
Neurofibromatosis type 2 affected the nerves in Matt’s ears, causing a blockage in the transmission of sound to his brain
NF2 causes benign tumors in the brain, spinal cord and peripheral nerves
At first I thought this was good news. Now that we had isolated the problem, the doctors were able to remove the tumors and I was good to go; like connecting a broken wire, voilà, everything is fine.
I presented that hypothesis to my doctors, who kindly refrained from laughing out loud at me. Unfortunately, as I soon learned, the nerves in your brain are different from the wires in your car. You can’t replace them or cut away the bad parts and tie the two ends together with electrical tape.
Removing my tumors was literally brain surgery. And the nerves in that part of the body don’t respond well to scalpels. Surgeons had to cut out the tumors, but no one was sure what the resulting damage would be.
Then there was the source problem. Even if I got through this cutting process without any damage, I would still have the gene defect, which meant more tumors in the future. We may be on repeat for a while.
As depressing as that realization was, things continued to get worse. The specialists told me that even with this surgery and other surgeries I might need in the future, I would eventually become deaf: not “hard of hearing,” not “turn up the volume,” but stone deaf. Medical experts were able to slow the process through treatment, but one day I woke up and heard nothing at all.
Hearing that news was like being hit between the eyes with a hammer. My mother’s soft hum; my father’s stories, which were repeated over and over again; the bad jokes of my fraternity brothers; the throaty roar of a fast car; the ticking of a clock; the steady rumble of a train: everything would be gone.
And the music. Did this mean I would never hear Paul and Artie’s tight harmonies as they ran through the spice rack of ‘Scarborough Fair’? “Hello darkness, my old friend,” indeed.
When he learned he was going deaf, Matt thought about all the sounds he would miss, including the tight harmonies of Simon and Garfunkel.
“Did this mean I would never hear Paul and Artie’s tight harmonies as they ran through the spice rack at Scarborough Fair? ‘Hello darkness, my old friend’, indeed’
Beck’s Beautiful Way was on the car radio when Matt took his now wife Norah on their first date – it would be one of the songs most clearly etched in his memory
Matt and Norah – the couple is now married and has three teenage children
Sometimes fear precedes loss. When I heard that deafness was a done deal, I initially panicked. How do I hear a smoke detector? Can deaf people still drive even though they can’t hear horns, trucks or train whistles?
At that point, those worries were years away, but the brain tends to race when processing bad news.
I too have gone through all the stages of grief. Some people say there are five; others say seven. I didn’t count. At first I didn’t believe the doctors. They had to be wrong. I was otherwise healthy. According to the materials I could find on NF2, balance should have been an issue. I also read where you developed cataracts in your eyes, and there were skin lesions. I didn’t have any of that. The diagnosis had to be wrong.
The doctors nodded. They had heard all this before. Yes, sometimes there were other symptoms, some quite serious, but the most common was progressive hearing loss. My case, they assured me, was textbook NF2.
A personal soundtrack was my determined compensation for this diagnosis. As a typical Midwestern kid growing up in the 1980s and whose life events were tied to pop music, I planned to commit my favorite songs to memory.
I made a mental playlist of the bands I loved and created a way to tap into my most resonant memories. And the job I most clearly needed to cement? The one that me and my new girlfriend Nora – the love of my life – listened to in the car on our first date: Beautiful Way by Beck:
‘Searchlights on the skyline, I’m just looking for a friend Who’s gonna love my baby When she turns the corner. Egyptian bells ring, When it’s her birthday. Honey, I’m talking about you. There’s a hurricane blowing your way.’
Of the book The soundtrack of silence by Matt Hay. Copyright © 2024 by Matt Hay and reprinted by permission of St. Martin’s Publishing Group