Imagine being born profoundly deaf, missing the vital nerve needed to hear.
Without it, you had no options. Now, a new device and procedure can fix this problem.
As little Grayson found out, the outcome is life changing.
Three-year-old Grayson Clamp could hear his dad's voice for the first time.
Born without the nerve that carries sound to the brain, Grayson became one of the first children in the US to receive an auditory brain stem implant at the University of North Carolina at Chapel Hill.
Nicole Clamp, Grayson’s mother, says, "the day that they turned it on was, was amazing."
Since then, his mom says Grayson has blossomed, going from unable to hear to hearing distinguishing sounds.
He is even developing some speech.
Nicole says, "He just seems calmer; he's just more comfortable in his environment in general."
The ABI works by stimulating the brain stem directly, turning sound received by a processor into electrical impulses delivered to the brain.
Doctor Craig Buchman, Director of UNC Ear and Hearing Center at
University of North Carolina at Chapel Hill, says, "Where he was before he wouldn't hear a jet on the tarmac, and now he can hear, you know, the softest of whispers."
Since Grayson has the first implant, it is hard to predict how his hearing will develop.
However, Dr. Buchman says, “I'm hopeful. I'm super hopeful. And it seems like we are off in the right, going in the right direction."
With his dog Tully in tow, Grayson will be just fine.
Since Grayson was missing his auditory nerve, attempts at using a cochlear implant did not work.
That is why he enrolled in this trial.
His mom says he has been making great strides.
She plans to sign him up for a kindergarten readiness school in August.
This will allow him to be around kids his own age with no hearing difficulties.
TOPIC: A NEW WAY TO HEAR FOR GRAYSON: BRAIN STEM IMPLANT
REPORT: MB #3744
BACKGROUND: More than 12,000 babies are born with hearing loss each year in the United States. Current technology now allows the accurate assessments of hearing in children starting within a few hours of birth. In fact, all states have mandates that testing of hearing be done in the newborn prior to discharge from the hospital. Children with undetected hearing loss may not be able to develop normal speech and language or acquire the cognitive abilities, like thinking, knowing, and judging, needed for learning. The early detection of hearing loss allows treatment and rehabilitation of the hearing-impaired child at a very young age. (Source: http://www.medicinenet.com/detecting_hearing_loss_in_children/article.htm)
RISK FACTORS: About 50 percent of hearing loss cases are genetic. However, there are a number of risk factors for hearing loss in children, so there are numerous reasons why a child's hearing may need to be screened or tested. Common indications for a hearing evaluation include:
* Speech delay
* Poor school performance
* Infectious diseases that cause hearing loss (like Down syndrome)
* Frequent or recurrent ear infections
* Medical treatments that may have hearing loss as a side effect, like some chemotherapy agents
* Diagnosis of a learning disability or other disorder, like autism
NEW TECHNOLOGY: Cochlear implants (CI) are highly successful at restoring functional hearing, but Grayson was born without the nerve that carries sound to the brain. The auditory brainstem implant is similar in design and function to a CI, but the electrode is placed on the first auditory relay station in the brainstem, called the cochlear nucleus. The ABI stimulates neurons directly at the brainstem, bypassing the inner ear and hearing nerve entirely. Doctors start by setting frequencies in the implant for up to 20 electrodes. In the United States ABI's were previously only approved for adults with certain conditions. But now the FDA is testing it on children. (Source: http://www.asha.org/Publications/leader/2011/110315/Auditory-Brainstem-Implants.htm and http://www.mayoclinic.org/tests-procedures/auditory-brainstem-implant/basics/definition/prc-20022457)
FOR MORE INFORMATION, PLEASE CONTACT:
Craig A. Buchman, MD, FACS
Department of Otolaryngology-Head & Neck Surgery
University of North Carolina at Chapel Hill