Cape Town - A nine-year-old Cape Town girl born without ears will be the country’s first recipient of a new surgery, the bone anchored hearing aid (BAHA) 4 Attract system, involving wireless technology which will allow her to hear.
Exposing a shaved section of her daughter’s scalp which was covered by a hair band, Cherie Josephs-Dramat, 42, from Kuilsriver, showed where Camryn had a small magnet made of titanium inserted in the bone beneath the skin.
With prior BAHA implants, the patient would only undergo one operation at a time and it usually involved a screw implant which visibly protrudes at the side of the head.
But the new technique, which was performed on Camryn at N1 City Hospital earlier this month, creates an invisible connection between the magnets embedded close to where her ears would have been and equally magnetised sound processors. She will be fitted with the processors in about three months.
Diagnosed with Goldenhar syndrome after birth – a rare congenital defect which is characterised by the incomplete development of the ear – Camryn was certified as “profoundly deaf”. She’s also had two open heart operations to repair holes in the organ.
“It (the syndrome) also causes facial and organ defects,” said Josephs-Dramat, explaining why the left side of her daughter’s body was affected.
Initially, she’d been frightened by the diagnosis, but once she saw her baby girl, she “knew everything was going to be okay”.
The biggest challenge for the family remains funding as medical aid doesn’t cover all procedures, said Josephs-Dramat, who added Camryn still needed ear and jaw reconstruction.
Now in her final year at Tygerberg Hospital’s Carel du Toit Centre for children with hearing impediments, Camryn is completing Grade 3. With a smile, she said “doing maths” when asked what she loved about school.
“She cries when she’s sick and can’t go to school,” her mother said.
She had had to explain to Camryn what sign language was when she asked about it after seeing it on a TV programme.
“She would watch DTV (deaf television) and curiously ask ‘what are they doing mommy?’” said Josephs-Dramat.
She said the centre, which advocates “oralism” rather than sign language, has offered the family tremendous support. After advising Camryn’s family she needed another hearing aid, the school referred them to ear, nose and throat specialist Dr Nathanie Naudè, who’d already been in talks with Australian otolaryngologist Professor Robert Briggs about the new bilateral BAHA technique.
Briggs then travelled to Cape Town to perform Camryn’s surgery, which was observed by more than 20 ENT specialists from across the country.
“To give this to a child is absolutely amazing,” said Carel du Toit audiologist Surida Booysen.
She said once Camryn’s speech processors were attached, sound travels from the skull – known as bone conduction – to the cochlear and auditory nerve. Its wireless connection provides a more hygienic and safer option to Camryn, who can easily remove the devices when she plays, bathes or goes to bed.
“It will be the first time that Camryn will be able to hear surround sound,” said Booysen.
Josephs-Dramat admitted that it was often an “emotional rollercoaster” when her daughter, who she described as a “confident little girl”, had to go for operations. But, the mother’s biggest concern was the fact that Camryn was growing older and becoming more “aware” of her physical disabilities.
She now hopes that the surgery will not only improve her daughter’s quality of hearing, but also help maintain her confidence as she continues into adolescence. “It’s little steps for her to fit in, to becoming a part of mainstream society, but just as long as she’s healthy and happy.”