Regression and Recovery - Part 2

Updated: Mar 26, 2023

When Kathleen left for Montana, I threw myself into research. Support groups for SCDS, otosclerosis, tonic tensor tympani, middle ear myoclonus, and more. The latest operations to dampen hearing, but not destroy it. Endless medical journals analyzing the outcomes of various treatments and procedures. The latest in hearing aids, smart earplugs, and the Inflation Reduction Act’s provision to allow consumer electronics companies to bypass insurance and market assistive hearing devices direct to users. I read anything and everything, and spent a solid two weeks doing nothing but research and sleep (a good portion of which was spent camping in our friends Laura and Glenn’s driveway - love you guys and can’t thank you enough for helping us out when I was stuck).

At the end of it all, I had appointments with four field-leading doctors, slide decks and timelines outlining my symptoms, and a long list of questions for each of them. Due to a lack of standardization in diagnostic testing, I would need to travel to each them individually, and none of them were located near me in the PNW. This effectively threw the trip into reverse - instead of trucking northeast into the Canadian Rockies, I was headed back south - to San Francisco, LA, and eventually east to Boston. After these visits, I had three major takeaways:

Doctors are often heavily biased toward their diagnostic comfort zones, regardless of what the test data says. This is probably a ‘no duh’ takeaway, but I had naively hoped that the best of the best doctors would be immune to this sort of thing. I was wrong. Doctors 1 and 2 (numbered sequentially) both told me that I had classic symptoms of SCDS on both sides, that I was indeed ‘bilateral’, and that no CT scans were necessary because they were so sure. Never mind the negative VEMP tests, the only thing they could do was surgery plugging both holes (and both balance canals, with its massive risk of long term disability). Before I got halfway down my list of questions, they were asking when I could be available for my first craniotomy. Hard pass.
Basic statistics needs to be included in our high school mathematics curriculum. I was absolutely amazed by the blank stares I got when I asked for things like confidence intervals behind patient outcomes figures. Yes Doctor #3, you may have an 80% success rate with your surgical procedure, but you probably shouldn’t be telling your patients that when you’ve only done it 4 times, and the success rate in a well controlled 150 person study was less than 40%. Also, this same doctor proceeded to tell me that I had otosclerosis, bilateral SCDS, and middle ear myoclonus, AND that they were completely unrelated. I just happened to have three extraordinarily rare middle and inner ear conditions by sheer chance. So I threw some numbers at him - otosclerosis is 1 in 12, bilateral SCDS is 1 in 240, middle ear myoclonus is 1 in 1666. So that would make me a 1 in 4.8 million patient. He seemed unfazed by this. I asked him how many patients like me he’d seen in his career. There had been three. I told him he should invest in the lottery.
The average American is totally screwed if they have a slightly obscure medical condition. I am the ideal patient. I have great insurance (still Apple’s health plan because I’m privileged enough to be able to afford COBRA), I have a background in the subject matter behind my medical issue (I am a headphone designer after all), I do in fact have a grasp of basic statistics, and I have the time and money to be able visit expert doctors around the country. Despite all of this, I received horrible medical advice from three consecutive physicians - advice that could have needlessly destroyed my quality of life. What chance does Joe the plumber have?

You may be wondering what happened to Doctor #4. Luckily, he was one of those human beings that you feel fortunate to have come across in life.

When Kathleen came back from Montana, I was still in pretty rough shape. The meditative white noise of a solo drive to California had been a godsend, but I was terrified of being around people again, and the cacophony that comes with it. Our plan was to fly east together - first to a wedding in Nova Scotia (for our good friends Matt and Maddie, no way in hell I was missing that), and then to Boston to visit Doctor #4 as Mass Eye and Ear. I was prepared with hand-modified white noise generators, a regimen of supplements, massage techniques, new balance exercises, and emergency earplugs/headphones. The flight out was surprisingly uneventful, and I got through the wedding with my game face on. I may not have made it out on the dance floor, but the happiness radiating from the newlyweds was contagious, and it was hard to be anything but joyous around them. Kathleen and I flew into Boston in higher spirits having made it through that milestone.

The first order of business in Boston was testing - CT, VEMP, audiogram, acoustic reflex, and more. I had seen Doctor #4 once before and knew that he was very data driven, always a good sign. I arrived at the appointment an hour early - his first of the day - and he saw me right away. We went through my daunting slide deck and list of questions, and his answers were extraordinarily detailed and thoughtful. We talked coping strategies, and he expressed admiration for the hearing health features that the AirPods team had released the week prior, and made a few requests of his own. Finally, he asked a seemingly bizarre question that changed everything. “Have you lost weight?” I had, with credit to the biking and hiking of months prior. “Hmmm, do you take any allergy medication?” I did, with credit to the annoying grass allergy I had developed since moving to California. Just then, the results of my CT scan popped up on the computer screen. The radiologist’s report read “Dehiscence of the right side superior semi-circular canal, consistent with last year’s scan. Left side normal.”

Left. Side. Normal.

I almost lost my shit in the doctors office. I thought I was done - no more biking, no walks with future kid, no more life as I knew it. But now there was hope.

“I think I know what’s been making everything worse”. He called in a gaggle of residents. “The impedance of your right ear is already extremely low due to your dehiscence. In addition to that, I think the impedance of both of your ears has been reduced even further due to Patelous Eustachian Tube. You’re getting hit twice with the same punch.”

The residents scribbled notes. Patelous Eustachian Tube, he explained, occurs when the tube that connects your middle ear to your throat (the one that pops when you swallow) gets stuck open. There are several potential causes, but among them are weight loss and use of allergy medication.

“You must be absolutely miserable.” I concurred. He prescribed a nasal drop meant to inflame the tube and get it to seal again. “It’s always tricky to get the tube to seal once it’s been opened, but this is very treatable.” I concurred again, having built a career around solving similar acoustic seal problems in AirPods. “Put the allergy meds on pause, and try these drops for a month. And maybe have a cheeseburger.”

I practically skipped back to the hotel. When I got the medication, I felt something I wasn’t sure I’d ever feel again - a change for the better. Not perfect, not by a long shot. But a reduction in the nonstop banging and clattering of everyday external noise. My own voice returning to a somewhat reasonable volume. The feeling like I’d stepped off a boat and onto solid ground. Not back to normal, but back to where I was at the beginning of the trip, before the downward spiral began. It was time to get back in the saddle.

On the way back to California, camped near Mt. Hood