One of the youngest babies in the world to get a cochlear implant hears for the first time. Health24's Adiel Ismail witnessed Neave Barrett's discovery of the world of sound.
Neave Barrett's deep blue eyes darted around the room – and then came to a slow stop.
For the first time in her short life, the four-month-old who was born profoundly deaf could hear her parents say "I love you". It was a magical moment.
Tears of joy
Now a rich world of sound and auditory exploration awaits baby Neave who received the gift of hearing on Friday following a cochlear implant operation in January. Neave became the youngest baby in South Africa, and one of the youngest in the world to get the life-changing cochlear operation.
It was performed at Vergelegen Mediclinic in Somerset West by a surgery team from the Tygerberg Hospital Stellenbosch University Cochlear Implant Unit and the Carel du Toit Centre.
One month later, on 12 February, the implant to her right ear was switched on and Neave experienced sound for the first time.
Wearing a white babygrow with an elephant and turquoise shorts with white polkodots, Neave was cooing and gurgling when the cochlear implant was activated. For the first time her eyes blinked at the sound of clapping hands.
As Neave laughed and squealed, tears of joy washed over the faces of parents Mark and Mia from Hout Bay in Cape Town.
"It was amazing. It was just so heart-warming to see Neave hear and responding to sound. It was wonderful," said Mia who smiled brightly while tears streamed from her eyes.
The moment was profound for Mark too. Watching Neave hear, a tear rolled down his right eye. He wiped it off before reaching out to touch his daughter's tiny toes.
"It was very emotional and overwhelming. It was such a poignant, special moment and words fail me," said Mark.
Mia and Mark were devastated when a newborn hearing screening found that their daughter couldn't hear.
This test, which is not compulsory and parents can refuse to have it done, is offered at private health facilities, but it is not universal at public hospitals.
Recommended screening technologies include oto-acoustic emissions (OAE), which assess cochlear (inner ear) functioning, and auditory brainstem responses (ABR), which record neural activity in response to sounds.
The tests are accurate and take one to three minutes to perform; and has the same sensation as simply putting a finger in the infant’s ear.
The urgency of having the cochlear implant surgery for Neave was because of an unexplained build-up of inflammation in her ears, which could have resulted to bony growth, called ossification.
However, during the operation it was found that there was no ossification and therefore only one ear was implanted. Neave is expected to undergo the second surgery in May for her left ear.
A transducer of sound
Dr Derrick Wagenfeld, a ear, nose and throat specialist, who championed cochlear implant surgery in South Africa, told Health24 that a cochlear implant is used to bypass the inner ear as a transducer of sound.
He explained that the operation on Neave involved implanting a series of electrodes into the cochlea near small nerve endings.
"These are stimulated from an externally worn device which transmits the coded signal across an intact skin to a receiver stimulator, which is directly connected to the electrodes."
This is stimulated according to a code by an audiologist. Following surgery, the audiological management includes the fitting of the external speech processor, as well as the activation and MAPping of the internal cochlear implant electrode.
"MAPping is the word we use when we refer to the process of programming the processor and electrode array to the appropriate levels that the individual needs in order to hear," said Surida Booysen, an audiologist with the Carel du Toit Centre.
"We use special coding strategies that convert the acoustic sound into electrical energy."
Booysen indicated that it is deeply rewarding to be part of a process that enables someone to communicate.
"We have the humbling opportunity to walk the journey towards sound with our patients and their loved ones and see how their lives change, one sound at a time.
Ensuring optimal benefit
"The audiologist typically serves as the child's case manager during the pre-assessment period of the cochlear implant and will continue to manage the child for the rest of their lives," she said.
Following initial activation of the implant, Booysen will continue to see Neave and her parents to do regular MAPping sessions, habilitation sessions and hearing tests to ensure that the technology continues to function well, and ensure optimal benefit from the cochlear implants.
Although a cochlear implant bypasses the ineffective hearing organ and can give the brain direct access to sound, it cannot interpret the sound.
"It is still a very rudimentary device in comparison to the pea-sized cochleas people with typical hearing have. Cochlear implants are nowhere close to replicating the function of our typically functioning cochleas," said Booysen.
"Electrical impulses are meaningless until the auditory brain can give meaning to sound."
Cochlear implantation provides the potential for children who are implanted early enough to acquire spoken language.
Booysen said that significant research has been done about how the auditory brain functions, and has indicated that there is a sensitive period during which the brain can develop spoken language naturally in the first 3.5 years of life.
"In other words, if an infant is born with hearing loss and the auditory brain is not stimulated during this period, then the auditory centres of the brain may never again be used for what it was created for and the brain starts to reorganise itself."
She said cortical reorganisation occurs when the visual cortex attempt to take over the auditory part of the brain. "The sooner we can stimulate the auditory brain, the higher the likelihood of typical brain development," said Booysen.
"Currently Neave's brain is wired for sound and auditory language learning so the sooner we can give her brain access to sound, the easier it will be for her to attempt typical speech and language development with the right kind of intervention and support."
Sending and receiving clear signals
As a speech therapist, Babara Kellett has been carefully observing the bond and interaction between baby Neave and mom Mia.
She said a tuned-in parent and an interactive baby ensures that each party is sending, receiving and responding to clear communication signals.
"My role is to skillfully observe the interactions to ensure optimal attachment and the ability for both parties to send and receive clear signals so that the wonderful dance of communication can lay the foundation for later speech and language development."
Kellett reiterated that the areas of the brain responsible for listening, auditory memory and ultimately oral language need to be stimulated.
"If this access is early, easy and encompasses the full range of the speech spectrum, the ability to develop clear speech increases. This ease of access further allows typical language to develop through natural interaction and overhearing.
"If we can help achieve this, language can develop typically without being taught in a remedial way. This is really the goal of early intervention, great technology and appropriate support," she said.
Neave, spelt as Niamh in Irish, means radiant. "We like the sound of it," said Mia. "It is a beautiful, soft and Gaelic sound."
Now a new chapter of sound is ready to be explored.