Navigating the sudden grief of severe hearing loss

It was one of those foggy, yet shimmering evenings that makes San Francisco the city it is. My wife and I met our oldest friends at a way-too-hip restaurant on lower Divisadero Street, near where we all used to live. It was a very San Francisco place, having been in a previous life a laundromat and Bank of Italy branch. We toasted to our friendship of three decades. Clinks all around. Friends since our now-grown children had been infants.

More people jammed into the restaurant. The buzz grew into a roar. We found ourselves shouting, but that was all right. We were part of a gathering of merrymakers. Iris was trying to convince Barry that the four of us ought to go to Portugal for a vacation. Val and I were talking about when our respective sons were toddlers, throwing from a crib a mountain of Beanie Babies while smiling like Cheshire cats.

At least, that’s what I think we were talking about. I was feeling good, but nowhere near tipsy.

And that’s when I realized: I was going deaf.

Nothing seemed to be coming out of Val’s mouth. She was moving her lips, definitely talking. But the speech bubble between us was empty. I could hear bits and pieces, but I couldn’t make out what they meant. The guts of each word had been stripped bare. Like a fish deboned, leaving a skeleton.

The moment is forever etched in my brain and it was terrifying.

I nodded and smiled, picking up scraps of what I was left trying to figure out. I asked Val to repeat herself, touching my index finger to my ear, angling my chin to the crowd six-deep at the bar. This wasn’t the time to fess up that something horribly wrong was happening. I felt like I had stepped into an Edvard Munch painting.

That night I didn’t say anything to my wife about the episode. I chalked it up to yet another high-decibel brasserie that made me feel like I was dining in a hangar at SFO.

We returned home to Iowa City, where I’m a professor at the University of Iowa. But when classes started in the fall, I noticed something was askew. I had to ask a few students to “speak up.” This wasn’t cause for concern — a lot of students mumble.

But the signs became impossible to ignore. I made an appointment for a hearing test at the university hospital, where I was put in a soundproof vault and outfitted with headphones and asked to repeat what was drilled into my ears. “Say the word ‘hello.’ Say the word ‘baseball.’”

The audiologist noted that I had missed several. When I met with an ENT physician following my vault experience, after examining my ears for wax and finding none, he made the suggestion that I might have a neuroma, or flap, which might be blocking sound coming into one or both of my ears.

“It’s unusual but an MRI will tell. Just to be sure,” they said.

The technician positioned me to slide through the metal arc of an MRI machine while the sound of jackhammers pounded. If I already wasn’t deaf, the racket surely pushed me along the way. In a week, the test came back “unremarkable.” I wasn’t sure whether this was a summary of my inner-ear workings or my brain acuity. There was no neuroma.

Over the next six months, in class and in meetings, my hearing got worse. I found myself strategically moving to position myself in front of whoever was talking, at least when I could. I took to focusing on the speaker’s mouth; sometimes I nodded at conversation stops even though I wasn’t sure what I was agreeing with. At restaurants, I tried to snag a seat with my back against the wall.

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Nine months later, I went back into the hearing vault. My hearing had gotten worse, as shown on an audiogram filled with X’s, O’s and squiggles connecting them. Another ENT physician suggested that I was suffering from general and assorted hearing loss — and yes, I really should consider hearing aids.

“Jeez,” I said more to myself than to the physician. It was my turn to mutter.

“Everyone’s got something in their ears these days,” he replied, trying to soften the blow.

In fact, as I was to learn, not having some degree of hearing loss is unusual, particularly for those who are middle-aged and older. Men in their fifties are three times as likely to have hearing loss than women of the same age, and the average age of a person buying hearing aids today is 60.

But why did I have to wear hearing aids in the first place? Was it something I did? Too many after-shower Q-Tips? Should I not have sat front row for the 1985 Born in the U.S.A. Tour?

And, if indeed my hearing was going, in the high-tech world of medicine, why wasn’t there some operation to improve it? You can get a replacement heart, ocular lens, liver, knee, hip, kidney, why not an ear — or the delicate bones inside it? It turns out that not only are there no such transplants, but little surgery is ever done on the ear.

Unlike eye surgery, which is common, operations aren’t often performed on the pliable bones and hair cell receptors of the middle ear, the source of natural hearing. There’s no equivalent of LASIK or cataract surgery when it comes to ears. Apart from a rare stapedectomy performed in response to a condition known as otosclerosis, if you’re hearing disabled, no operation can fix the condition.

Without other options, I figured with some embarrassment that I’d try hearing aids. I was gobsmacked at how expensive they were. The money, though, wasn’t the only thing that set me back. It was what wearing them meant. I had to face up to the fact that medically nothing was going to help me.

It’s with particular irony that I find myself with hearing aids. My head is large — I have a difficult time finding hats that fit — and my ears are correspondingly oversized, so large that a dreaded childhood taunt bullies used to hurl at me was “Ears!” The fleshy auriculae protruding on either side of my head are a caricature artist’s dream.

Outfitted with the top-of-the-line Phonak Audéo model, the devices were far from perfect. The hearing aids had to be calibrated numerous times. Yes, they improved my hearing, but in no way corrected it. The sound was tinny, distorted, mechanical and artificial-sounding. It felt like a miniature and flimsy speaker cone jammed into my ear. It itched, too. Where was all the rich high-fidelity we’ve come to expect from Sensurround electronics?

It’s now been three years since I first got outfitted. Do I like them? No. Do I need them? Yes.

Unlike glasses or contacts, they don’t correct hearing to the equivalent of 20/20 eyesight. I switched audiologists. As in all professions, not all audiologists are equally skilled. Adjusting hearing aids, I learned, is as much an art as it is a skill. Some audiologists will rush you. Some may talk (too loudly or softly) your ear off. Others might balk at the myriad adjustments you’ll ask them to tweak.

While no longer resembling a bulky transmitter, the basic hearing-aid concept is the same as when I was in high school 50 years ago. Essentially, they are combination microphones and amplifiers. They can be fully inserted in your ear or positioned atop it. They’ve gotten smaller over the years , and they’re now equipped with bluetooth, so talking on the phone, watching TV or listening to music through them is easier and more seamless.

They can also narrow or widen the field of sound of what the devices pick up, minimizing disrupting background noise. But they don’t materially alter the quality or substance of the sound that comes into your ears. They amplify sound directly into your ear canal. No matter how advanced they are, they don’t do much to make the sound clearer — just louder and slightly reformatted.

Because the FDA now allows the public to purchase hearing aids without a prescription, some devices have dropped in price. But for anyone with more than moderate hearing loss, getting an audiologist to perform a hearing test and then outfitting you with an appropriate device is the preferential way to go — if you can afford it.

I’ve seen a half dozen physicians who specialize in diseases of the ear, nose and throat, known technically as otolaryngologists and at least in my case, few of these physicians seemed all that interested in my hearing deficiency. Unless you have severe-to-profound hearing loss that makes you a candidate for a cochlear implant, these doctors seemed to have better things to do. Another aging guy whose wife tells him to turn down the TV.

The otolaryngologists I saw at three major university hospitals took a cursory look in my ears and then sent me on my way. The visits were over in ten minutes.

To improve hearing, it’s the device that’s essential. Most of the extras hearing-aid companies tout today include adjustments for comfort, clarity, speech and ambient sound, but few users will rely on any of these settings three months into wearing them, opting instead to default to the hearing aid’s preset settings. Additional presets (like while driving or for windy walks on the beach) can be made by an authorized dealer with access to the manufacturer’s programs custom set to your individual device. The wearer can make adjustments for noisy locations like restaurants, but in my case, that helped only minimally.

You’ll likely want to get an intuitive hearing aid that can be operated with minimal training via your smartphone, which carries the device’s controls. Each brand is different, so it makes sense to try out several models made by different manufacturers. Your provider should allow you to take units home and experiment.

There are some nifty innovations on the market that may change the shape of things to hear. One is a patented device pioneered by Silicon Valley neuroscientist David Eagleman called the Neosensory Clarify. This device seeks to help hearing-disabled users with a Fitbit-looking device strapped on the wrist. It uses ancillary senses to pick up the slack when the body’s primary anatomical organ — the ear — starts to falter.

The Clarify supposedly redeploys the brain to pick up sounds translated to vibrations on the wrist. In a sense, it seeks to do for the hearing-impaired what braille does for the blind: It creates an alternative pathway to cognition. After wearing these devices for a month, the Clarify purports to train the brain to translate the electrical impulses to register in your brain as sounds. The device asks a revolutionary question: Do we really need our ears to hear? Might we be able to get the same data to our brains through another peripheral? Instead of your ears, why not your skin? Some users say the device works, others suggest it’s snake oil.

My initial vanity — that the hearing aids would stick out like a flashing neon sign — proved to be mostly a non-issue. The devices I ended up with rest comfortably on the top of my ear. I don’t feel them and their weight is inconsequential. They’re small, kidney-shaped, and come in color-coordinated shades, which camouflages them to near-invisibility. I chose a subtle salt-and-pepper gray model. A clear, hollow tube connects the microphone housing to the miniature speakers inserted into my ears. To the casual observer, I’m going ear commando.

Audiologists advise wearing the hearing aids from the moment you wake till the second you hit your pillow. Even if you’re alone all day. Even if you don’t anticipate encountering another soul. The reason: Wearing the devices just occasionally tricks your brain into thinking you don’t need them, and that’ll confuse the prized three-pound master cranial organ a top your spinal cord. On-and-off wearing is not recommended.

But wearing the devices is a commitment to being dependent on them. The design of hearing aids muffles or blocks almost all of the natural unamplified sounds that stream into your ear canal.

Occasionally, depending on what interactions I have planned for the day, I won’t put them in — despite my provider’s cautionary advice. Going au natural means not hearing a lot of the annoying sounds we all endure: leaf blowers, airplanes, car alarms, neighbors and their relatives chowing down brats in the backyard. Without the devices, there’s a sublime and supreme sense of quietude.

When I fly, I don’t need to wear noise-canceling headsets or Airbuds; all I need to do is keep the hearing aids in and turned off.

The fact that hearing loss doesn’t happen suddenly might be considered the cruelest part of having to wear hearing aids. Every month, you stealthily realize you’re in the process of losing more and more. It's scary to realize that one of your senses, one you’ve cherished and taken for granted for decades, is taking a hike and never returning.

Hearing is the intimate sense that made it possible for you to comprehend a parent’s first words to you, your teenage crush whispering, “I love you”; your newborn’s nightingale cooing, not to mention the enjoyment of a performance of “Der Ring des Nibelungen” or marveling over Elis Regina dulcet singing of “The Girl from Ipanema.”

The scariest part is that hearing loss is irreversible. To have possessed something, to have nurtured and treasured it, and for it to be stolen from you never to return, that can be crushing.

Sometimes I get angry about the disparity of health, however much of a nonevent hearing disability is when compared to other maladies. Perhaps to comfort myself, I peel my eyes whenever I’m in any similarly-aged crowd to see if others are wearing hearing aids. I’ve gotten so adept that I try to figure out which brand and model they’re sporting.

I still hate putting them in most mornings. I have mixed results in a classroom. Zoom classes work better for me than in-person classes, during which I can utilize the bluetooth capability. When I’m out of a controlled environment, when I’m in an airport or at the supermarket or post office, I’ll sometimes preemptively say, “I’m hearing disabled,” even though I hate doing that and it usually does nothing.

Understanding someone wearing a COVID-19 mask is nearly impossible for me. Trying to decipher a pronounced foreign accent is equally difficult. Crowded restaurants are still difficult for me to navigate; large parties and functions are not generally welcomed. I don’t lipread, but I find myself looking to the speaker’s mouth for clues, and that seems to help. I have to be aware of how loud or soft my own voice is because my ears don’t register it as resolutely as they used to. Hearing aids carry their own eccentricities. I can hear the faint creaking of floorboards in my house yet when I’m in the middle of a robust three-way conversation, I feel like the odd man out.

It’s all exhausting. You don’t realize how complicated comprehending speech can be until your hearing abilities are compromised.

Where will it all end? My hearing acuity continues to get worse. There are four main stages of hearing loss: mild, moderate, severe and profound. I’ve gone from mild to moderate to moderately severe in three years. That's what a stack of audiograms indicate. The slope of decline is steeper than a ski jump at the Sochi Olympics.

Being the journalist I am, from the beginning of this strange and bewildering acoustic saga, I wanted to determine the etiology of my hearing decline. How bad was it going to get? How come such an unrelenting, unforgiving decline? Will I eventually go deaf? Should I start learning American Sign Language?

My hearing deficiency likely had been going downhill for years. But it was declining so incrementally that I hadn’t really noticed it until that evening at the sound-ricocheting restaurant. But I thought there had to be something more to it than just age-related hearing loss. It seemed to be slipping so fast and precipitously that its cause had to be more than the accumulation of nonspecific skirmishes with aging.

Why was a mystery — until my son called several months ago.

Michael and his wife, wanting to start a family, did what lots of young people do: They got preemptive, routine genetic counseling. Three weeks later, their tests came back. Everything normal. No cause for alarm. They can and ought to proceed, the counselor told them.

Michael called to share the news. As an afterthought, he added that on his genetic report card it was noted that he tested positive for an obscure genetic condition, so rare that it’s not even considered a disease, just a “syndrome,” called Alport. Some 150,000 Americans are estimated to have either the dominant or recessive gene that causes Alport syndrome, named after a South African physician, Arthur Cecil Alport, an expert on malaria, who wrote about the mutation in 1927 in The British Medical Journal.

“It’s something about irregular collagen in three parts of the body: your kidneys, inner ear, and eyes,” Michael said.

I signed up for my own genetic test, which confirmed that I, too, have Alport Syndrome (my variant is autosomal dominant Alport syndrome), one that has spared my eyes, but has manifested in my kidneys and ears. My kidneys are holding fairly strong. It’s the progressive hearing loss that seemingly is the byproduct of Alport syndrome, which I likely inherited from my father.

For now, my aural symptoms aren’t much different from those someone with age-related hearing loss might experience. Some Alport patients are deaf; others have moderate hearing impairment. It’s the type IV collagen in the basement membrane of my cochlea, or inner ear, that is abnormal and contributes to sensorineural hearing loss.

Until there’s something better to stick in my ear, it seems that I’ll need to make a separate peace with my not-so-trusty but none-the-less essential salt-and-pepper hearing aids. Will I eventually go deaf? No one can predict. But if I live another 20 years, I’ll likely have other assorted maladies so that my hearing loss will be just another medical issue that I’ll have to learn to contend with.