Family Discovers 8-Year-Old’s Single-Sided Deafness, and a Remedy for It

October 7, 2019

Debbie will never forget the alarm she felt when her eight-year-old son told her he could no longer hear from one ear.

The revelation came one evening while she was helping him with his homework.

“Kyle told me that a friend had yelled in his ear that day and said that ‘I do not want my ear blown out like my other one,’” she recalls. She pressed him for details, and when he told her that he regularly had difficulty hearing, “I thought, ‘oh my gosh, how can this be?’”

She knew Kyle, her youngest son, was sometimes antsy in the classroom. However, he had passed school screening hearing tests, and his grades and academic test scores were satisfactory. Debbie and Kyle’s dad Tom never suspected that Kyle had hearing loss.

Finding Experts and the Right Diagnosis

Debbie made an appointment with John M addalozzo, MD, an otolaryngologist at Ann & Robert H. Lurie Children’s Hospital of Chicago. Dr. Maddalozzo had previously treated Kyle for fluid in the middle ear, the most common cause of temporary hearing loss in young children.

Dr. Maddalozzo ordered a hearing test which revealed heartbreaking news: a permanent profound hearing loss in Kyle’s left ear.

“Even though we already knew he wasn’t hearing well, to see him in the sound booth not hearing these loud blasts was just horrible,” Debbie said.

To find the best option for Kyle, Dr. Maddalozzo referred the family to Nancy Young, MD, the Founder and Medical Director of the Lurie Children’s Cochlear Implant Program, one of the largest programs in the United States. At Dr. Young’s suggestion, a special MRI was done in order to understand Kyle’s ear anatomy.

Is a Cochlear Implant the Answer?

Cochlear implants are electronic devices that help many children with significant hearing loss. The implant stimulates auditory nerve fibers in the inner ear, providing information to the brain about spoken language and environmental sounds. Traditionally cochlear implantation has been used to treat significant hearing loss in both ears. It is not commonly done for “single-sided deafness” or asymmetric hearing loss, situations in which the opposite ear has normal hearing or a limited degree of loss well treated with a hearing aid.

“Hearing from one ear only is a huge disadvantage, but one that often goes unrecognized,” said Dr. Young. “Hearing from both ears helps us to understand speech when background noise is present and tells us the direction where sound is coming from.” Difficulty hearing in the presence of noise is challenging, especially for children, since they often spend time in noisy classrooms. The extra effort needed to hear when background noise is present can be challenging and tiring for children able to hear with only one ear. In addition, difficulty localizing where sound is originating can be a safety concern.

The Right Choice for Kyle

Dr. Young counselled Kyle and his family about his hearing loss. “She was really good about being honest and laying out all the options for treatment, including their pros and cons,” said Debbie. “She spent almost an hour with us at the first appointment; I thought that was pretty impressive.”

Dr. Young told the family that Kyle was fortunate that his MRI revealed a normal inner ear and nerve of hearing. Because of his history and anatomy, Kyle was a good candidate for the cochlear implant, the only treatment that could return hearing to his deaf ear. Kyle and his family were also informed that weekly therapy after implantation was necessary to help his ear and brain learn to listen effectively with his cochlear implant system.

‘I Want to Have Surgery’

The family went home to weigh the decision. During that time, Debbie joined an online support group for families facing single-sided hearing loss. One parent shared that choosing a cochlear implant for her child with single-sided hearing loss was “the best decision she ever made,” Debbie recalls. In addition, Kyle made it clear to his parents he was ready to undergo surgery.

“He told us, ‘if it’s the only way I can hear, I want to have surgery,’” Debbie said.

The decision was final. In March 2018, he underwent the outpatient surgery with Dr. Young. Several weeks later his device was turned on by his implant audiologist, Beth Tournis, Au.D. Soon after, he began aural habilitation, or listening therapy, with Crystal Zgela, M.S., a speech language pathologist at Lurie Children’s who is also a Certified Listening and Spoken Language Specialist.

Progress and a Bright Future

Now 10, Kyle has flourished since receiving his cochlear implant. This summer he finished his listening therapy, and he continues to see his implant audiologist at Lurie Children’s several times a year.

His reading scores have notably improved since he began using his implant, Debbie said, and he is better able to focus in his fourth-grade classroom.

For other families considering cochlear implantation, Debbie said to keep in mind that it takes some work and commitment to therapy following surgery, but that it’s worth it in the end.

“Just stick with it,” she said. “It has made such a difference for Kyle.”

Often the biggest challenge for families of children with single-sided deafness or asymmetric hearing loss is obtaining approval from their insurance carrier for cochlear implantation, said Dr. Young. She hopes that this will change in light of recent U.S. Food and Drug Administration approval of the device Kyle received, the MED-EL Synchrony Cochlear Implant, for children and adults with these types of hearing problems. FDA approval for these conditions is a first and should enable more children and families struggling with hearing loss to obtain effective treatment, Dr. Young said.

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