CLEVELAND — Maggie Gleason, 14, who was born deaf, heard sound for the first time in her life when hearing specialists at University Hospitals (UH) Case Medical Center turned on an innovative electronic device called an auditory brainstem implant (ABI). The implantable device provides a sense of sound to a person who is profoundly deaf.
Maggie was born without cochleas, the small snail-shaped bones in her inner ears which house the auditory (hearing) nerve. As a result of her condition, she had no auditory nerve. Maggie had to wait for a technology like ABI to advance to a stage where it could help her. ABI bypasses the ear and stimulates the brain stem.
According to one of her surgeons, Maroun Semaan, MD, of UH Case Medical Center, Maggie may be the first teen with this device for absent cochleas.
“For someone who has never heard, the perception and awareness of sound is extremely helpful,” said Dr. Semaan, Director, Otology, Neurotology, and Balance Disorders at UH Case Medical Center and Associate Professor, Otolaryngology, Case Western Reserve University School of Medicine.
ABI is a prosthetic hearing device that stimulates neurons directly at the human brainstem, bypassing the inner ear and hearing nerve entirely, which in Maggie’s case, did not even exist. The device consists of a tiny radio receiver implanted underneath the skin and tiny platinum electrodes implanted into the brain stem.
Maggie’s surgery was in September and the system was turned on and tested Dec. 28. Her family watched as the medical team activated the device and recorded the first moments of her being able to hear. Her dad’s voice, because it was low, came in the best for her.
“I always felt I would have a lot to say to her when the moment came,” said her father Frank Gleason, “but I was left speechless.”
Sounds, amplified and clarified by a special coil and miniature computer that slips onto the ear like a conventional hearing aid, are collected by the receiver, converted to electrical pulses and transmitted to the electrodes. From there the signals travel through the skin by radio frequency and connect to the brain stem at the same place the auditory nerve would normally connect. Although the patients can perceive sound and pitch, results may vary by patient and full hearing is not restored.
Maggie is a ninth grader at Lorain High School where she is in a class for the hearing impaired.
Science is her favorite class. She also likes to cook and she dances hip hop by feeling the beat of the music. Chocolate chip pancakes and sausage are her favorite things to cook.
Maggie has overcome several medical adversities in her life. According to her mother she flat-lined twice when she was born; was born without cochleae, had weak kidneys, a weak heart, and collapsed lungs; has had two cleft palate surgical repairs, and was on a gastric feeding tube for nine years.
“She didn’t crawl on her own, didn’t pick up her head on her own,” said Joanna. “It’s been a long road to this point.”