Hearing loss helps make her who she is

Addison Curry has two sets of ears. One she was born with, the other she got from a doctor. “I’ve had them my whole life.”

The second pair of what she calls “ears” are actually hearing aids. Addison, who goes by Addie, was born with mild-to-moderate hearing loss, and the Drayton Hall Elementary School fourth grader is comfortable talking about it with anybody who asks.

“It’s kind of fun,” she says. “It makes you special, and nobody’s perfect. Everybody has a problem. It feels kind of cool.”

As MUSC Health marks Better Hearing and Speech Month this May, Addie’s parents and doctors are encouraging other families to be aware of what they call a major public health issue. Audiologist Meredith Holcomb says treating hearing loss and speech problems is essential.

“Early diagnosis of hearing loss in children critical to spoken language development, communication, social interaction and cognition. For older adults, untreated hearing loss can negatively affect their overall quality of life and those with even mild hearing loss are twice as likely to develop dementia.”

Treating hearing loss is serious business at MUSC Health, which has the highest rated Ear, Nose and Throat Department in the Southeast and is ranked among the best in the country by U.S. News & World Report. Its doctors perform about 140 cochlear implant surgeries per year, fit about 400 hearing aids and do multiple other ear surgeries as well.

Addie has been an MUSC Health patient her entire life. Her mother, Ryan Howard, says when Addie was a newborn, she failed her first hearing test. “Addison’s dad and I were crushed. We looked at Meredith, our audiologist, and told her she must have not done the test correctly.”

Like about 90 percent of children with hearing loss, Addie was born to hearing parents. They needed time to adjust to the news, her mother says. “I didn’t fully understand how they were evaluating hearing on a newborn. After research and time, we accepted the diagnosis and began to move forward.  By 3 months, Addie was in hearing aids, and we started speech therapy at 6 months.”

Holcomb, Addie’s audiologist, says her hearing loss was caused by a recessive genetic disorder. “Addison’s hearing loss etiology was identified as connexin 26, which is the most common cause of congenital hearing loss. Connexin 26 mutations are non-syndromic, which means they produce isolated hearing loss.”

The most common causes of hearing loss in adults are aging and exposure to loud noise.

Holcomb highlights three treatments.

Digital hearing aids

“Digital hearing aids are recommended for patients with varying degrees of hearing loss,” Holcomb says. They turn sound waves into digital signals and have computer chips that analyze sounds and can be programmed to match the person’s type of hearing loss.

Cochlear implants

“Cochlear implants are recommended for patients with moderate-to-profound bilateral sensorineural hearing loss and who have difficulty with speech and understanding speech with hearing aids,” Holcomb says.

MUSC Health has the largest cochlear implant program in the state. The implants work by bypassing damaged structures in the ear and stimulating the auditory nerve.

Osseo-integrated devices

“Osseo-integrated devices are FDA-approved for patients with conductive hearing loss or single-sided-deafness,” Holcomb says. The bone-anchored devices work with the help of a tiny metal vibrator that goes behind the ear.

But devices are only part of the story for children such as Addie who were born with hearing loss. Holcomb describes Addie’s path to where she is now.

“Addison was fit with hearing aids soon after her diagnosis, and she began intensive weekly auditory verbal speech therapy to develop spoken language. Addison is now in mainstream elementary school and uses her hearing aids and a personal digital system to allow her to hear and understand her teacher. With the help of her determined family and the ENT, pediatric audiology and speech language pathology services at MUSC, Addison is a successful spoken language communicator.”

Addie’s mother adds some detail, showing the nitty gritty of what’s involved in raising a child with hearing loss. First, her family’s health insurance doesn’t cover Addie’s hearing aids. “We have to replace her hearing aids every three to five years. Each aid is around $2,500 to $3,000. She essentially has our first car on her ears.”

There is a proposal in the state legislature to require insurance coverage of children’s hearing aids, but it’s unclear if or when it might become more than that.

Ryan Howard says there’s also technology to consider when it comes to school. “Addie’s teachers wear an FM system to enhance their voices and it links to her hearing aids.  A lot of the school work is done on computers and with technology, so that has been our biggest barrier — incorporating ways for Addie to partake and fully hear these things.”

But they’re figuring it out, she says, and she wouldn’t have Addie any other way. “Addison was and is perfect, we wouldn’t trade anything about her, not even the hearing loss. It makes her who she is, and we love her indefinitely.”