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. a publication of hearing health foundation
winter 2022
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