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December 29, 2002

Whisper of hope in toddler's world of silence

From: Independent Online, South Africa - 29 Dec 2002

By Liz Clarke

The one-in-a-million case of a 13-month-old South African toddler born with both her inner ears missing is to be presented to an international forum of the world's leading surgeons in Austria next month with an appeal for help.

If brain-stem implant surgery on the little girl goes ahead, she will be the youngest yet to have undergone such a procedure.

The story of baby Jennifer McMillan, from Port Shepstone, which was featured in The Sunday Independent last week, has prompted a South African cochlear implant surgeon to take her case to a world forum in the hope that an answer can be found to improve the toddler's quality of life.

Dr Maurice Hockman, a member of an international team of surgeons who recently performed groundbreaking brain-stem implant surgery on a girl called Melissa, a Howick teenager with multiple tumours that led to loss of hearing, said Jennifer's inner ear condition was so rare that in his opinion it needed to be discussed and monitored at an international level.

"It is difficult to say what exactly can be done for her," the Johannesburg-based surgeon said, "but the best chance we have is to discuss her case with the world's best surgeons."

The lively blonde toddler at the centre of the debate has a profound congenital defect of her inner ear region, where the hearing and balancing mechanisms are normally located.

The snail-shaped cochlea that receives sound vibrations is missing, making Jennifer profoundly deaf. She also lacks the semicircular canals that help maintain balance.

One of the options is for her to be fitted with a "modified implant" electrode, similar to one fitted to Melissa and which resulted in an encouraging hearing response.

Hockman said that while a magnetic resonance image scan would determine whether an auditory nerve was present, only a surgical procedure could determine whether a nucleus of the nerve sufficient for a successful implant was intact.

"In Austria I will be meeting the same surgeons who came here for Melissa's operation. I hope to speak to the parents and specialists treating her before I leave, so I am armed with all the up-to-date information."

Among the doctors with whom he will be discussing Jennifer's case are leading cochlear implant surgeon Dr Joachim Muller from Wurzburg, Germany; and Dr Robert Behr, a world-renowned neurosurgeon from Cologne, Germany. Others include cochlear implant specialists from Nottingham, England, one of the biggest such centres in the world.

During the delicate operation to help restore hearing where the cochlea is damaged or void, a device containing 12 electrodes is fitted to the bed of the eight nerves where the nucleus rests. So far about 200 such operations have been performed in the world with varying degrees of success, but none so far on a young child. One of the challenges that will be discussed next month is how to programme the electrodes once they are in place to elicit an auditory response. "In an older person they can tell you whether they are hearing high or low sounds. In a young child this may be more complex, but not insurmountable."

Hockman said that if an operation of this nature was required for Jennifer there should be no problem in flying out a team of top German and British surgeons and audiological technicians to work with the South African implant team.

"This is a very interesting case," he said. "If we can help Jennifer, we could help other similarly affected children."

Jennifer's parents, Justin and Maggie McMillan, were on holiday with family in Kimberley when they heard of the new developments.

"Obviously we don't want to get too excited," said her father, "but this sort of exposure is certainly something we have dreamed of."

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