18-month-old girl hears for first time after medics switch on a cochlear implant remotely

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Moment 18-month-old girl hears for the first time after medics switch on a cochlear implant remotely during lockdown

  • Margarida Cibrao-Roque was born deaf because of Ushers syndrome type one   
  • University of Southampton audiologists activated her hearing device remotely
  • It’s the first time a cochlear implant has been switched on over the internet

By Sam Baker For Mailonline

Published: | Updated:

An 18-month-old girl has been given the gift of hearing after her cochlear implant became the first in the country to be turned on remotely.

Margarida Cibrao-Roque has been deaf since she was born because of Ushers syndrome type one and a cleft palate which can also affect the ability to hear.

She was given her hearing by audiologists at the University of Southampton who switched on her hearing device over the internet.

Margarida’s mother Joana Cibrao said: ‘The Southampton team, they were amazing. I cannot praise them enough really, the effort of the team – they were just brilliant and they made it happen.

18-month-old Margarida Cibrao-Roque, pictured with father Paulo Roque and mother Joana Cibrao, was given the gift of hearing after her cochlear implant became the first in the country to be turned on remotely

‘The possibility of Margarida calling me Mummy one day would mean the world.

‘We will be able to speak with our daughter, to play with her, she will be able to watch TV, things that you take for granted she doesn’t have, so you know, this is a victory really.’

Margarida’s father, Paulo Roque, added: ‘We are trying to give the best that we can for her and so we’ve opened a big window for Margarida now. All we need to do is take time, step by step and we will get there, definitely.’

Normally the cochlear implants are activated in person at the university’s clinic, but lockdown rules means that an alternative method was thought up.

Margarida was born with Ushers syndrome type one which leads to hearing loss because of abnormalities in the inner ear

Margarida was born with Ushers syndrome type one which leads to hearing loss because of abnormalities in the inner ear

The cochlear implant is a device that uses microphones to pick up sound and send electrical signals to a second device placed in the inner ear.

These electronic signals are interpreted as sound by the brain.

When the implant is activated its sensitivity is set low and gradually increased so the audiologists can monitor the patient’s reaction to the electrical signals.

This is especially important for young children who cannot communicate if the sound is too loud. 

Professor Helen Cullington works in the university’s auditory implant service and she turned on the device from her home while Margarida and her parents were at their own home in Camberley, Surrey.

To remotely activate the hearing device Professor Cullington video called Margarida so that she could monitor her reaction to the gradual increase in test electronic waves.

Professor Helen Cullington of the University of Southampton turned on the hearing device from her home while Margarida and her parents were at their own home in Camberley, Surrey

Professor Helen Cullington of the University of Southampton turned on the hearing device from her home while Margarida and her parents were at their own home in Camberley, Surrey

She said: ‘Usually we do the switch-on of a cochlear implant at our clinic at the university but, with some technical creativity and some advice from colleagues in Australia, we were able to do everything necessary over the internet.

‘The session went really well and everyone was thrilled with the outcome.

‘At switch-on, a child begins wearing their processors for the first time, and they are able to hear what is around them.

‘However, it takes a long time to get used to this and – especially in babies and children who have never heard before – the brain has to learn to understand these sounds.’

Ushers syndrome leads to hearing loss because of abnormalities inside the ear. 

A university spokesman said: ‘With some ingenious thinking, the team worked out a way to conduct their tests over the internet – connecting two computers, utilising specialist software and hardware and monitoring progress via video link.’

What is Ushers syndrome? 

Everybody with Usher syndrome experiences the condition in a different way and it changes over time. 

The main symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa (RP).

If you have Usher syndrome, you may also have balance problems.

There are three types of Ushers syndrome:  

Usher type one: People with Usher type 1 are usually born with profound hearing loss in both ears.

Balance problems are also common. Babies may show delays in sitting and walking, and experience disorientation throughout their life, particularly as their sight changes.

Usher type 1 is also associated with the development of nightblindness. This occurs in the first 10 years of a child’s life, progresses to tunnel vision and further sight problems associated with RP.

A person’s primary communication method is typically British Sign Language (BSL), though this may change as their sight deteriorates.

Usher type two: People who have Usher type 2 are usually born with a mild to severe hearing loss in both ears. This is typically within the higher frequency ranges.

Type 2 is not associated with balance problems, which is a characteristic that distinguishes it from type 1.

Usher type 2 is characterised, in most cases, by the development of retinitis pigmentosa (RP) in the teens or early twenties.

A person’s primary communication method is usually speech, often assisted by hearing aids and lipreading.

Usher type three: Usher type 3 is characterised by gradual sight and hearing loss, which occurs later in life after a person has learnt to speak.

Some people may develop severe hearing loss while others may not.

People with Usher type 3 typically have problems with their balance. It’s common for people to be misdiagnosed as having type 1 if they demonstrate balance problems and severe hearing loss.

A person’s primary communication method is usually speech, often assisted by hearing aids and lipreading.

Not everyone who has Usher syndrome fits into these three clinical types. People may have varying symptoms that are difficult to diagnose as a specific Usher type. Cases like this are called atypical.

Source: sense.org