Melanie McLaughlin, a 40-year-old insurance agent from Colorado Springs, did not feel well on New Year’s Day 2021. It started with an eye infection in her left eye. She thought it was conjunctivitis. The infection spread to her right eye and then to her ears and a couple weeks later, she had developed ulcers in both eyes and hearing loss in both ears. Her doctors were baffled. She did not have COVID-19.
McLaughlin was in and out of UCHealth Memorial North Hospital in January. She had multiple tests to try to figure out what had gone wrong with her body. Doctors prescribed steroids and believed her eye issues and hearing loss were related to an infection. While steroids helped with her eyes and her left ear, she could not understand what people were saying to her. She began seeing a slew of doctors for her eye and hearing problems as well as other ailments, including vertigo. Her health issues at one point became so severe, she was bedridden.
Without warning, her life had drastically changed.
“Going from being independent one day — being able to take care of yourself, shower, drive around, make your food, go to the restroom — and then the next day losing part of your hearing and then not being able to do any of that, as you can imagine, it was life-changing,” McLaughlin said.
Sudden and severe hearing loss: Cogan’s Syndrome
In February 2021, Dr. Samuel Gubbels, a UCHealth otolaryngologist, was called upon to help McLaughlin by Dr. Joe Hegarty, a colleague who practices neurotology, a subspecialty of otolaryngology that focuses on complex inner ear problems.
“[Hegarty] wanted another opinion on what was a complex, complicated situation that wasn’t making sense,” Gubbels said.
Hegarty’s working diagnosis was Cogan’s Syndrome, a rare autoimmune disease that affects the eyes and inner ears. Gubbels concurred, as did McLaughlin’s ophthalmologist Dr. Alan Palestine at the Sue Anschutz-Rodgers Eye Center on the Anschutz Medical Campus. What triggered the life-altering syndrome remained a mystery.
“We don’t really know why this disorder necessarily starts. There are different theories that an infection could trigger it, but we don’t know that if you get X virus, you’ll get Cogan’s. You get this inflammatory picture. A lot of your testing is negative, which is one of the complexities. You don’t typically have a smoking gun as far as what happened that started it,” he said.
Nevertheless, McLaughlin’s profound hearing loss had to be addressed as soon as possible. People with gradual hearing loss usually receive hearing aids because they amplify sound for people who have some natural hearing ability. But in McLaughlin’s case, since she had no speech recognition and her loss was severe, hearing aids would not be effective. Cochlear implants were the best option for her to hear again.
McLaughlin’s husband, who had to do most of the communicating for her at doctor’s appointments, was hesitant to tell her that cochlear implants were her best option.
“He thought it would upset me because it’s a big deal having cochlear implants. It’s a lot of work,” she explained. “But, I told him that I was willing to do whatever it takes to get my hearing back, to have a conversation with him and talk to my then 8-year-old.”
What are cochlear implants?
Cochlear implants consist of an electrical device inserted into the cochlea, a part of the inner ear. An external device sits on top of the skin near the ear that helps captures sound in the environment and sends signals to the internal device. Madeline Horan, a UCHealth audiologist who specializes in cochlear implant evaluations, first saw McLaughlin in May 2021.
“Typically, most cochlear implant patients I would see have hearing loss for a long time. Melanie’s case was different,” Horan said. “When she first came to us, she couldn’t hear anything. She was communicating via a transcription app on her phone.”
Working with Gubbels on the Anschutz Medical Campus, McLaughlin chose an Advanced Bionics cochlear implant. McLaughlin still needed to be tested for hearing aids, however. “With an initial cochlear implant consult, we test the unaided hearing, then put on hearing aids and complete some testing — that’s the FDA criteria. McLaughlin got zeros across the board,” Horan said.
Complications and urgency
While McLaughlin’s specialists agreed that cochlear implants were her best option to regain hearing and that something that needed to be done as soon as possible, the current steroid dose she was on made surgery unsafe. It needed to be slowly reduced.
However, the longer she waited to have surgery, the higher her risk of developing a secondary condition called labyrinthitis ossificans, a condition in which the inner ear starts to fill with fibrous tissue and then bone, Gubbels said.
“We were all nervous that if we wait too long to get her weaned down on steroids, we might end up where she develops this [labyrinthitis ossificans] and wouldn’t be able to put the cochlear implants in,” Gubbels said. “We would lose our opportunity.”
Gubbels worked with McLaughlin’s rheumatologist Dr. Jason Kolfenbach to gradually substitute her steroid treatment with a biologic, non-steroid medication that acts as a steroid and suppresses inflammation.
With the reduction of steroids, McLaughlin’s eyes flared-up and other complications manifested. In May, McLaughlin ended up back in the hospital and she received an increase in steroids to manage her symptoms. Then, back to tapering her dose, again.
“I honestly felt like I would never be in a range where my surgeon would consider it safe enough for surgery. So, it was a long journey. I think we scheduled surgery three times before it actually happened,” McLaughlin said.
In September 2021, despite some fibrous tissue present, the surgery was a success.
Cogan’s Syndrome and cochlear implants
After surgery, McLaughlin rested and healed for a couple of weeks before the implants were turned on, or activated, which is standard procedure. On the first day of activation, she heard sounds like beeps and boops and, then, speech. Although the speech sounded mechanical, which is normal as your brain adjusts to electric impulses instead of natural soundwaves, McLaughlin was overjoyed to hear sound again.
“I was fortunate because I did have some speech recognition after they turned both my implants on, which was really cool. And of course, it’s a very emotional experience to hear again after not hearing for quite a few months,” she said.
The first voice she heard was from her audiologist. Then she heard her husband and then her son. She said that, although it wasn’t perfect, it was “amazing” to be able to communicate with people again.
Since the activation in September 2021, McLaughlin has been practicing listening exercises with help from her husband and guidance from Horan. Practicing includes watching and listening to TV, music, audiobooks or having conversations. These exercises are essential for her brain to get used to translating digital signals into speech or sound.
In the beginning, she visited her audiologist every month for the first three months to get the electrical current adjusted. After that, she checks in after six months, then after nine months, and then annually. Horan says that it takes a full year after surgery to reap the full benefits. But, at her first follow-up appointment, she scored 100% on sentence recognition while in a quiet environment. Prior to her surgery, she scored 0%.
“I’m blown away by how well she’s been hearing with the implants. It’s so exciting to think back to when I first saw her and how she could only hear through a transcription app, and now she comes in and hears just fine on her own. It’s very exciting,” Horan said.
Gubbels said that one of the predictors of success with cochlear implants is how long a person has been deaf. So, while her sudden hearing loss was dramatic, it also contributed to her surgery success. If people wait too long after they’ve lost their hearing, the nerves in the ear may degenerate. People who have healthy nerves have better success with implants.
“So, she’s a pretty star performer, and a large majority of the reason why is that it wasn’t all that long between when she’d lost her hearing and when we got the implants in her,” Gubbels said.
While her hearing is vastly improved, her left ear is better than her right and, she said, her hearing is not back to “normal.”
“Your brain is amazing at adapting to the new way of hearing and some things sound exactly as I remember them,” McLaughlin said. “However, there will be some frequencies I will not be able to hear again. There are thousands of hair cells in your ears, and I am working off 16 electrodes. I don’t think that we realize how complex hearing is until it is gone.”
Also, she’s still on steroids and trying to wean herself off of them. She visits her rheumatologist and eye doctor every three to four months and considers herself lucky.
“Hearing my son’s voice again, that’s just incredible. And being able to carry on a conversation with my husband and just talk about the mundane things of life again has been great. Even though I’m still disabled in other areas, having my hearing back has improved my quality of life tenfold.”