*This article has been updated
Don't cry for me darling Neavie,
The truth is your daddy loves you,
So close your eyes now,
And go to sleep.
This personalised lullaby is soothing, but three-and-a-half-month-old Neave Barrett can't hear a word.
She was born profoundly deaf.
Her parents, Mark and Mia from Houtbay in Cape Town, are hopeful that a cochlear implant surgery on Monday will finally allow their daughter to hear their voices, along with the rich sounds of the world.
A medical emergency
The urgency of having this operation at such a young age is because of a build-up of inflammation in her ears, leading to bony growth - or ossification. This makes waiting for implants at a later stage impossible.
Fortunately, an early screening test, which is not compulsory and parents can refuse to have it done, picked up that there was a problem before Neave was discharged from hospital days after she was born.
Newborn hearing screening is offered at private health facilities, but it is not universal at public hospitals although a few pilot projects are being rolled out.
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.
Neave was just three weeks old when she was put through nearly half a dozen tests to assess her hearing ability.
"We were told, after initial tests, that she was at least suffering from moderate hearing loss. I was hopeful that that was all that we were dealing with, but we later found out it was profound hearing loss", Mia said.
Recalling how she felt on the day she found out Neave was deaf, Mia's pale visage instantly turned into a blush of pink. She cast her eyes over to Neave, who was in the arms of Mark, and in a gentle tone said: "I was sad that she was not being able to hear our voices, not able to hear us tell her that we love her. I love reading and I felt heart-broken at the thought of not being able to read to her, to tell her stories and talk to her. Mark is amazing as a dad. He always sings to his kids and makes up songs and makes them personal. I felt sad that she wouldn't hear that."
Mark proceeded to say that while his wife reads a couple of bedtime stories to their first born Lyra, he rocks Neave to sleep while bouncing on a gym ball or dancing to some music.
A sense of sound...
"I'll often sing her a made-up lullaby as she goes to sleep. I like to think that Neave can hear some of the bass sounds from my singing – or least feels the vibrations from my chest as she is in my arms."
Mia explained that one of the hardest things for her is when Neave is in the rear-facing car seat. “She can't see us and she gets upset and she can't hear if we call out to her.” Her voice then shifted into a staccato tone as she uttered: “That breaks my heart.”
She continued in a soft voice: “You never want your child to be deaf. You never want your child to have obstacles. You want them to have the best opportunities in life. You want life to be as easy as possible for them. This is not going to make life easy for her."
For Mark, one of the toughest moments was when Neave underwent one of the hearing screenings. He explained that a microphone device was inserted into the ear canals where pulses of sound are transmitted.
"They turn the volume of the pulses of sound up and they do this with different frequencies and it just got louder and louder and louder and not seeing her respond just filled me with dread. I could hear it quite loudly and she couldn't hear that."
Mia indicated that she and her husband felt mixed about Neave having surgery at such a young age.
"It is obviously very daunting and very scary, but it is a choice that we made and we think it is the best choice for her. We are also very grateful that is can happen and that we found a surgeon who is willing to do it because not everyone would be prepared to do it so young. We don't have the luxury to wait."
"At 3½ months of age Neave will be one of the youngest patients done anywhere in the world, and she certainly will be the youngest baby in South Africa and Africa to be getting one," said Dr Derrick Wagenfeld, who is an ear, nose and throat specialist.
He pioneered cochlear implant surgery in South Africa and will be doing the operation on Neave at Vergelegen Mediclinic in Somerset West.
"Our overseas colleagues, with whom I’ve had contact over Neave’s case, have all been mightily impressed with how we have been able to diagnose this condition so early, something which even in the most developed countries is often not achieved. It really does speak volumes for the dedication of the medical staff in this country, under conditions of infrastructure which certainly leave a lot to be desired."
Dr Wagenfeld explains to Health24 why the operation for Neave was something of a medical emergency
"In Neave’s case, with the inflammation which has been detected on the routine scanning we do while evaluating these patients, there is obvious inflammation within the cochlea and this can have dire consequences as it can lead to fibrous tissue forming in the inner ear with subsequent healing by bone formation or ossification.
"This can make cochlear implantation impossible later on and will thus render her profoundly hearing impaired for the rest of her life. The reason why we do this so early in her case is to try to get the electrodes in before this bone has a chance to develop".
According to Dr Wagenfeld, cochlear implants are used widely throughout South Africa, with over a 1 000 having been done throughout the country over the years. He said the Tygerberg Hospital Programme had done over 600 implants since the first one was performed on November 4 1986.
This is what a cochlear implant looks like
A cochlear implant is used to bypass the inner ear or cochlea as a transducer of sound, explained Dr Wagenfeld.
"There are tiny hair cells in the cochlea - about 30 000 of them - which perform this transduction function, which is the conversion of the physical sound waves in the air into an electrical current in the nerve, which then conducts this information through to the hearing centre in the brain where you then perceive the sound."
He said there were a number of causes for loss of hair cells, and in Neave’s case it was some unexplained inflammatory process of her inner ears which had probably been there since birth.
"Electrical testing has shown that when you put sound into her ear on the outside, no electrical current is generated in the nerve and thus we know her transducers aren’t working."
Dr Wagenfeld said they then implanted a series of electrodes into the cochlea near the little 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. It stimulates them according to the code which has been worked out beforehand.
"Our experience has been that this supplies patients with enough sound information to enable them to develop normal speech and language communication skills which are after all what it is all about," he said.
Watch: How a cochlear implant works
Dr Wagenfeld indicated that he is positive about performing this surgery on little Neave.
"Her bone is very thin, her middle ear space and the mastoid in particular is still reasonably underdeveloped and so gaining access to the cochlea can be particularly hazardous. I would say that, rather than making one anxious, it makes one just maybe even more watchful than one would normally be and might add some time to the surgery.
"This entails actually gaining access to the cochlea deep in the temporal bone via the mastoid and the middle ear space, something which normally takes us in the region of 1½ to 2 hours."
He said the real hazards involved were potential damage to the delicate structures one encounters on the way through to the inner ear, but this was something surgeons were trained to do and so it should be accomplished without too much danger.
"I imagine the surgery could take about 2 hours, and if we have to do both ears it would take naturally double the time. This is, in fact, the major hazard of surgery, in that one tends to get more complications the longer the child is under anaesthesia and that is something that we will try and limit as far as possible."
Neave will have to stay in hospital afterwards just until her fluid dynamics have been stabilised and she is well, however, it shouldn’t be necessary for her to stay longer than 24 hours.
In the days after the surgery she would have a bandage over her head to prevent fluid collection under the flap, but there would be no stiches to remove.
"We really hope that by performing this surgery on Neave at this early stage we will be supplying her with the wherewithal to develop normal communication skills and normal speech and thus play an active and full part in the community throughout her life. Without this technology and had the diagnosis been delayed this would not have been possible."
The implant itself, which came from Australia, will only be activated some 2 to 3 weeks after the surgery.
Magic - waiting to happen
"Any response to sound is going to be magic", said Mia. "It is amazing and very emotional to think that she will be able to hear us for the first time," added Mark.
The implants and the processors - which are the things that go on the ears - cost just over R500 000. The family's medical aid will contribute towards this, but there is a funding gap of R110 000. The operation costs, including ongoing costs, are also expected to be reasonably significant.
The family has signed a contract to raise funds for cochlear implants through the University of Stellenbosch trust and anyone wanting to make a donation specifically for the cochlear implant devices can use the following details:
Reference: Project Neave Barrett
University of Stellenbosch
Branch code: 050610
Account number: 073006955
For more details, mail Joan Weyers who is managing the donations.
Update: During surgery it was revealed that there was no ossification, which was the concern and the reason behind the urgency of having the operation while Neave was so young.
The cochlear implant was only done on the right ear and the left ear will likely happen when Neave is about 7 months.
"Thankfully there is no evidence of ossification so we revert back to standard protocol, which includes a behaviour response to sound test when she's about 5 or 6 months and then probably the implant at 7 months," Mia told Health24.