In first grade, routine vision and hearing tests revealed I had hearing loss.
Further testing confirmed my right ear had no hearing, and my left had some loss. Doctors initially diagnosed me with Pendred Syndrome, a genetic disorder causing early hearing loss and balance issues. Fortunately, my insurance covered two Phonak hearing aids, sparing my family thousands of dollars.
Pendred Syndrome often causes fluctuating hearing loss. I wore hearing aids for years, using a red and blue pair—my favorite colors at the time. I later received an FM System in class, which helped me hear my teachers over background noise. An FM or frequency-modulated system is a microphone-like system that helps a student with hearing loss listen and hear better in loud places like a cafeteria or classroom. It’s a transmitter device worn by the teacher and connects wirelessly to the student’s hearing aid. However, my hearing worsened by sixth grade, especially in my left ear. My audiologist recommended a cochlear implant for my right ear, which insurance again covered, which I’m fortunate for. Cochlear Implants are significantly more expensive than hearing aids.
It was also around this time that my hearing diagnosis changed. It was determined that it was not Pendred Syndrome that I suffered from; it was EVA. EVA, or Enlarged Vestibular Aqueduct, which is often attributed to or confused with Pendred Syndrome, is an abnormally enlarged vestibular aqueduct. Vestibular aqueducts are narrow, bony canals that travel from our inner ears to our skulls. We all have vestibular aqueducts but as the National Institute on Deafness states, “Recent studies indicate that a vestibular aqueduct is abnormally enlarged if it is bigger than one millimeter, roughly the size of the head of a pen. This is called an enlarged vestibular aqueduct, or EVA; the condition is also known as a dilated vestibular aqueduct or a large vestibular aqueduct. If a vestibular aqueduct is enlarged, the endolymphatic duct and sac usually grow large too. The functions of the endolymphatic duct and sac are not completely understood. Scientists believe that the endolymphatic duct and sac help to ensure that the fluid in the inner ear contains the correct amounts of certain chemicals called ions. Ions are needed to help start the nerve signals that send sound and balance information to the brain.” (U.S. Department of Health and Human Services. (n.d.). Enlarged vestibular aqueducts and childhood hearing loss. National Institute of Deafness and Other Communication Disorders).
The surgery involved placing an electronic device under my skin and connecting a wire to my cochlea. After recovery, the implant was activated, allowing me to hear from my right side for the first time I could remember. Sounds were unfamiliar at first, but with practice, I adapted and got better. Speech especially sounded strange. Note that a cochlear implant doesn’t restore natural hearing, it just helps the brain to understand sound in a new way. Over time, the person learns to recognize speech and sound. It takes time and practice. In fact, it often takes years. Even today, speech doesn’t sound complete, but both devices are constantly getting updated technology. Perhaps one day it would pick up speech more than ever.
Adjusting to hearing loss has been challenging, from asking people to repeat themselves to using a microphone in class. It was rough adjusting to a hard-of-hearing life, one where I had to get comfortable asking people to repeat themselves, as I don’t like speaking much. I’ve had to get comfortable handing the microphone out to both professors and students and ask for it back. That too was a difficult journey that I still haven’t fully overcome. Though I still struggle with these, my devices have helped me navigate a world not designed for the hard of hearing or the deaf. But despite many fluctuations, both improvements and decreases, the devices I am fortunate enough to use have maintained my hearing and reminded me to keep pushing.