Living With Hearing Loss

Sarah Kirwan and Carl Deriso married in August 2020, after meeting through a multiple sclerosis self-help group.

A Complicated and Special Case

With another condition clouding the symptoms, a diagnosis of an inner ear problem is long in coming but sparks a mission to help others with multiple

disabilities. By Sarah Kirwan

In June 2010, I stepped off a plane in Los Angeles with a painfully full left ear, muffled hearing, throbbing eyes, vertigo, and a migraine. I also got off that plane with no idea I would straddle the line between a nondisabled and disabled identity for the rest of my life.

Over the next eight months, I sat in offices answering questions and completing tests, while medical specialists sat baffled. There was an otolaryngologist, neuroophthalmologist, neuro-psychometrist, rheumatologist, clinical psychologist, audiologist, and neurologist, among others.

And the tests were never-ending: from evoked potential tests, MRIs, and batteries of blood tests to vestibular-evoked myogenic potential and neuro-cognitive testing.

A decade later, the doctor visits and medical testing continue, but the frenzied pace has slowed and I can finally breathe again. I think back to those times when I was seeing two or three doctors each week, and I can remember one specialist smiling at me and saying, “Yours is a complicated and special case.” At that time, I didn’t realize how right he was.

Searching for a Diagnosis

I am in my early 40s and diagnosed with both multiple sclerosis and superior semicircular canal dehiscence, a very rare inner ear disease affecting 1 to 2 percent of the world’s population. While my MS diagnosis took about eight months, my SSCD went undiagnosed and misdiagnosed for almost nine years.

UCLA Health defines SSCD as “a tiny hole (dehiscence) that develops in one of the three canals inside the ear. Healthy individuals have two holes or ‘mobile windows’ in their dense otic capsule bone, but those with SSCD have developed a third hole.” The exact cause of SSCD is unknown. The condition is usually diagnosed in middle age, though it has been seen in small children.

The UCLA website adds, “The thinning or absence of bone located in the semicircular canal triggers vertigo, hearing loss, disequilibrium, and other vestibular and auditory symptoms. A common clinical symptom of SSCD, reported by patients, is the abnormal amplification of internal body sounds, such as heartbeats and eye movements.” Hearing internal bodily sounds is called autophony.

Far left: Kirwan before the successful surgical repair of her left ear’s superior semicircular canal dehiscence.

Kirwan is the volunteer director of the only women’s wheelchair basketball team in California, having played and coached for years.

MS and SSCD share symptoms, such as fatigue, acquired nystagmus (uncontrollable rapid eye movements), migraines, vertigo, and balance loss—and I was experiencing all of these. I was also experiencing other identifiable MS symptoms, such as numbness and tingling sensations, Lhermitte’s sign (which feels like an electric shock), and muscle spasms.

However, the one symptom I was experiencing that could only be connected back to SSCD was autophony. Hearing my organs moving and functioning was so disturbing and debilitating that this symptom combined with every other symptom I was experiencing reduced my life to one of survival and scarcity. It almost sent me into a psychiatric hospital. Or worse yet, over the side of a bridge, as I wrestled with suicidal thoughts and ideations I’d never experienced before.

Autophony-Induced Anxiety

The first time I heard my eyeballs moving, I thought, “There’s no way I could’ve just heard that.” I sat there for a bit, looking from side to side, to see if the noise would stop. It only got louder. I was shocked. The sound was nauseating—a mixture of grinding, whooshing, and fingernails on chalkboards. Then came my heartbeat in my left ear. The more animated and excited I’d become, the louder my heartbeat would get.

It finally got to a point where I could barely function or hold conversations with people, as my brain fog worsened. After that, it was the sound of the heels of my feet connecting with the ground or floor beneath me as I walked. Then, my body adjusted its own walking, so the balls of my feet would hit first.

I could also hear the mechanics of the muscles in my throat as I swallowed. It was getting more difficult by the day to hear anything over the noise of my internal organs. I started retreating from life and isolating myself. I was becoming less likely to be the life of the party, as I’d been.

For nine years, I lived like this. Each morning, I was greeted by the sound of my heartbeat in my left ear and the sounds of my overwhelming tinnitus that had developed somewhere along the way.

During this time, I was diagnosed with major depressive disorder and general anxiety disorder. However, the SSCD continued to go undiagnosed, as providers tied all of these symptoms back to my MS, and insisted I had additional undiagnosed mental health conditions—possibly bipolar depression or schizophrenia. Fearfully, I stopped talking about hearing my internal organs moving.

In the summer of 2018, I finally found the answers for myself, when I came across an article about autophony during one of my medical research binges. This led me to SSCD and a self-diagnosis, which I took to my neurologist.

I remember pulling out my notebook with printed SSCD articles and explaining to her that this is what I’ve been talking about for all these years. I begged her to conduct a CT scan of my temporal bone, so we would know if my symptoms contributed to SSCD or not and move forward from there.

She refused, and once again claimed I had undiagnosed mental health conditions, made sure I was seeing a therapist, and told me there was nothing else going on with my health.

When I continued advocating for myself, she said we’d be able to see the inner ear canal in my next MRI, which was wholly untrue, and she became defensive and argumentative. I let it go, fearing retaliation and knowing I would get nowhere. I was keenly aware of how much I needed her—finding a new doctor when you’re barely surviving is not an option.

Living with multiple invisible disabilities presents numerous challenges that are often misunderstood by the nondisabled community, as well as by some folks within the disability community. My goal is to help individuals, businesses, and communities better understand visible and invisible disabilities, so we can make a more accessible and inclusive world for everyone.

Finding Answers

For the next three months, I was too depressed to do much. And then a day came when I felt that familiar feeling of determination rise up within me again. I worked around my neurologist and went directly to UCLA’s department of neurosurgery, where I’d found two world-renowned surgeons researching and providing treatment for SSCD: Quinton S. Gopen, M.D., and Isaac Yang, M.D.

I knew they could help me but when I called, in June 2019, I was told I needed an SSCD diagnosis to see them. I burst into tears, unable to hold back nine years of fear, sadness, anxiety, frustration, and loss. Through sobs, I asked, “Where can I get a diagnosis? No one will believe me. Where can I go where they’ll listen to me? No one is listening. Will you please help me? Please.”

Hearing the desperation in my voice, the staff member I spoke with took my case to her superiors. One week later, I was sitting in their office. I will never forget when the physician assistant looked into my eyes and said, “We believe you, and we’re going to help you.” Once again, I burst into tears. I did not belong in a psychiatric facility. I did not have bipolar depression or schizophrenia. What I did have was validation and an official diagnosis—bilateral SSCD—meaning both ears are affected.

At the time, I was the only patient Dr. Gopen and Dr. Yang had seen who was diagnosed with both MS and SSCD, so they proceeded with caution to treat me. In October 2019, they performed a surgical repair of my left ear SSCD, which includes a skull-base, middle-fossa craniotomy for canal-repair resurfacing, plugging, or capping the bony dehiscence (hole).

When I woke up from the surgery, I could no longer hear any of my internal organs. The heartbeat in my left ear was gone, and I could finally hear out of it. For the first time in nine years, the external noises of the world were louder than the internal sounds of my body. It was incredible, but it would take some getting used to.

Sounds were extremely loud and I couldn’t handle even the smallest crowds of people, but my body and brain slowly started adjusting. And now, two years post-surgery, 70 percent of the symptoms I initially presented with have been alleviated. Eventually, I will need the same surgery done on my right ear, but I rest easy knowing I won’t have to wait for my symptoms to debilitate me further before I can do so.

Access and Allyship

Living with multiple invisible disabilities presents numerous challenges that are often misunderstood by the nondisabled community, as well as by some folks within the disability community. My goal is to help individuals, businesses, and communities better understand visible and invisible disabilities, so we can make a more accessible and inclusive world for everyone.

In 2020, I officially launched my woman- and disability-owned small business, Eye Level Communications LLC and, in 2021, the “Incluse This!” podcast. Using an intersectional approach, Eye Level provides strategic consulting and training services for disability access, inclusion, and allyship. The podcast is a safe and coalitional space specifically created for critical conversations that bring disability to the forefront of the greater diversity conversation.

While the tools are different, the mission of both is the same—equity for all people living with disabilities. I want to change disability policies and procedures. Most importantly, I want to change mindsets and perceptions, along with how we talk about disability in America and around the world.

Sarah Kirwan lives in California. For more, see eyelevel.works.

Share your story: Tell us your hearing loss journey at editor@hhf.org.