Posted on Thursday 5 October 2017
Charlotte Wright had a pioneering procedure to improve her hearing
Charlotte Wright is the youngest person in the UK to benefit from a pioneering middle ear implant.
A little girl who is the youngest person in the UK to have a middle ear implant has experienced life-changing benefits from the pioneering surgery.
Charlotte Wright from Teddington in south-west London was three when she had major surgery at Guy’s and St Thomas’ NHS Foundation Trust in London to implant the cutting-edge device. She was born with microtia, a congenital deformity where the outer ear is underdeveloped, and atresia, absence of the ear canal. The conditions, which are uncommon, typically occur together and mean sound cannot travel to the inner ear on the affected side. Experts say patients with other more common conditions may also benefit from the implant.
Sophie and Simon Wright, who also have a four-year-old daughter Emily, were shocked when Charlotte was born and they saw her right ear had not grown properly. They hoped it would not affect her hearing and development because her left ear was normal. However, early hearing tests showed that she had glue ear (a common childhood condition where the middle ear becomes filled with fluid) in her left ear, meaning that she could not hear well on either side.
Sophie, 43, said: “Charlotte’s hearing difficulties meant that her speech was delayed, when she spoke no one could understand her and she had behavioural problems because she was so frustrated at not being able to hear.”
Under the care of St Thomas’ Hearing Implant Centre’s specialist microtia and atresia clinic, one of the only clinics of its kind in the UK, Charlotte started using a bone conduction hearing aid on a headband at age three. This sends vibrations directly through the skull to the cochlea, the hearing part of the inner ear, bypassing the blocked ear canal.
Sophie, who runs a business consultancy, said: “It helped but the box on the headband needs to be kept in the correct position and Charlotte always moved it so it didn’t make a big difference.”
Then doctors found that, unlike some children with microtia and atresia, one of Charlotte’s ossicles – the three tiny bones in the ear which transmit sounds to the cochlea – was not malformed. Because the little bone, known as stapes, is the key one of the hearing bones it meant she was suitable for a Vibrant Soundbridge middle ear implant. While it had been used on patients with hearing loss before, it had never been implanted into a child as young as her in the UK.
The device replaces the function of the middle and outer ear. An external processor with a microphone, which sits behind the ear, picks up sound. This connects magnetically to the receiver, which is implanted under the skin during surgery. Signals are transmitted from the processor to the receiver which, in turn, causes the part of the implant that sits in the stapes to vibrate. The vibrations travel to the inner ear, where the normal hearing process can take place.
The middle ear device is implanted under the skin so unlike some other hearing devices, it does not leave an open wound, which needs a lot of care and can be problematic due to risks of infection. At the Hearing Implant Centre, ear surgeons work closely with plastic surgeons to ensure the operation does not compromise the option to reconstruct the outer ear when the child is older.
Charlotte had the procedure in July 2016, just before her fourth birthday, and the device was turned on 12 weeks later. Sophie said: “It was incredible when it was turned on. Charlotte heard things she had never heard before and she could finally hear the teacher properly at school. It made a big difference straightaway.
“The audiology team at St Thomas’ were meticulous when adjusting the settings to get the sound right for Charlotte. For example, it was reprogrammed so she could hear noise from different directions and individual voices over background noise. By early this summer the settings were perfect for her.”
Professor Dan Jiang, consultant otolaryngologist at Guy’s and St Thomas’, says other young children could benefit from the implant.
He said: “A proportion of children with microtia and atresia have relatively normal stapes which can be accessed by surgery. They may be suitable for the device, as well as children with other types of hearing loss, especially those who have problems using hearing aids, and those with chronic ear infections or tumours.
“This is a world-leading intervention. The beauty of it is the receiver is under the skin and young children can have it so their hearing loss can be rehabilitated early in their life. It also won’t have negative implications for the future reconstruction of the outer ear.”
Charlotte, now five, has experienced dramatic improvements in her speech and behaviour and is flourishing at school. Sophie said: “I’m proud she is the youngest child to have the implant in the UK. It was a big thing to go through and she coped incredibly. We are very pleased she had it – it’s been amazing.
“When she is a bit older she will have reconstruction surgery if she wants it. We used to worry she would be embarrassed about her ‘little ear’ but she’s so proud to tell everyone about it.”