Look closely at Grayson’s stunned response to hearing his father’s voice, and you might notice him cueing. While it was initially unclear to some what he was cueing precisely—which sparked some discussion on the Cued Speech group page on Facebook—Len Clamp, Grayson’s father, put the debate to rest: “He cued ‘TV.’ Then he cued a TV off,’” Len said.
“Up to that point, Holly, his main audiologist, was pinging his device electronically, then turning on the TV to get him acknowledging that he heard or felt the impulse. When I spoke to him, he turned to the TV and cued ‘TV’ because he expected the TV to be on [and] because he heard the sound.”
Len and his wife, Nicole Clamp, who work as a banking executive and nurse practitioner, respectively, had been fostering Grayson for a year before they adopted him in July 2011. In the same month as the adoption, Grayson received a cochlear implant, but it was ineffective. Determined to find another way for Grayson to have auditory access, Len and Nicole decided to try the ABI, which they learned about through their otolaryngologist at University of North Carolina at Chapel Hill (UNC) Hospital, Dr. Craig Buchman, who performed both surgeries.
The cochlear implant did not work due to the nature of Grayson’s hearing loss. Unlike most children who are candidates for a cochlear implant, Grayson has auditory europathy spectrum disorder (ANSD), which is characterized by either a nonfunctioning cochlea or auditory nerve. Grayson was born without cochlear nerves, which qualified him for an auditory brainstem implant. But prior to Grayson receiving a cochlear implant, it was not evident to Grayson’s doctors, Dr. Buchman and Dr. Matt Ewend, a neurosurgeon at UNC Hospital, whether or not Grayson had cochlear nerves.
(Photos courtesy of Len Clamp)
The aftermath of Grayson’s ABI was shaky. Following the eight-hour surgery, he underwent two follow-up surgeries to repair a major complication—a cranial spinal fluid leak. Afterward, Grayson laid flat in the pediatric ICU for fourteen days with a spinal tap in his back.
Optimistic for future ABI’s in other children, Len said that “now [doctors] have the perfect blueprint for all future surgeries.” But because of the complications that followed Grayson’s surgery and the relative newness of the procedure as applied to children, Dr. Ewend says that the ABI still requires further study. “I’m excited for the family, but nobody knows how the ABI will work out because there’s been so few done. That’s why it’s being done on a clinical study. It should be done on clinical trials.”
In addition to ANSD, Grayson was born with a host of other medical issues, including a condition called CHARGE, which caused in Grayson a severe heart defect, blindness in his left eye, and profound bilateral deafness. Grayson underwent open-heart surgery at a month old. “So far everything looks great on the cardiology front,” Len said.
Len and Nicole Clamp, who were high school sweethearts where they grew up in South Carolina, are enthusiastic about Grayson’s future. “God knew Grayson needed a unique couple to care for Grayson’s special needs,” Len said. “He had been preparing us for that job, probably since we first started dating in high school.” Grayson’s parents believe that his expressive and receptive language has improved considerably with the aid of his ABI and Cued Speech, and that he has started to “make sounds and cue at the same time,” according to Nicole. “He loves his ABI,” she said. “We never have to make him wear it, and he will ask us for it sometimes. Since the activation on May 21, we have seen a lot of progress. He has started to babble consistently and now sometimes will cue the sound he is making. He was not moving his mouth at all with his cues [before], and still does not a lot. But he has started to.”
Len and Nicole initially learned about Cued Speech from Sharon Addison of Charlotte, North Carolina, who, like Len, is employed at Bank of America and also has a deaf daughter. “They encouraged us to use and educated us about how Cued Speech helped their daughter. They also put us in touch with one of Grayson’s current transliterators, who taught us to cue at our house.”
Grayson currently receives auditory verbal therapy from UNC and instruction from specialists in deaf education from Charlotte Mecklenburg Schools. His favorite activities are going to the Discovery Place Kids Zone; playing with his parents’ iPad; and watching to his father cue books to him.
Grayson has developed a strong bond with his brother Ethan and even seems to appreciate the sounds of his brother’s voice, Nicole said. “The first sound Grayson ever identified by audition alone was the sound of Ethan’s cry. When he hears it, he will look for Ethan. Yesterday, he was cueing “Ethan” over and over and pointing to his ear to tell me he heard him,” she added. “Smiling and laughing the whole time.”
“Everyone involved is so happy about how well he is doing with [the ABI],” she added.