Healthcare

After failing his newborn hearing tests, Maxime had hearing aids fitted at just eight weeks old but although this gave him access to sound at low frequencies, he was still profoundly deaf.
Maxime was referred to GOSH in September 2014 and was subsequently diagnosed with Connexin 26, the most common genetic cause of deafness in children.

After carefully assessing Maxime the team at GOSH, led by Dr Kaukab Rajput, felt he was likely to benefit from bilateral cochlear implants in both his ears. These electronic systems stimulate the auditory nerve directly, bypassing the hair cells in the cochlea that usually facilitate hearing. Cochlear implants can give a sensation of sound to profoundly and totally deaf children unable to hear even with the most powerful hearing aids. The effects can transform patients lives, as they gradually understand the sounds around them and many learn to talk and the majority follow speech without needing to lip-read.
Two weeks after his first birthday Maxime was admitted to GOSH for a six-hour operation. The surgeon made an incision behind one ear before drilling through the bone to the cochlear to create a bed for the implant, which was then inserted using a microscope. The same process then took place for the other ear. Maxime quickly recovered from the surgery and was discharged home the next day.

It has been an exciting but anxious time for his mum Giulia and dad Julien since Maximes surgery was first scheduled. Giulia said:
Weve faced a real rollercoaster of emotions over the past year, from the uncertainty of not knowing what was causing Maximes hearing problems to the news that he might be suitable for cochlear implants.

This operation will be life-changing for Maxime, as he will be able to learn to speak like a hearing child. Im from Italy and my partner is French so were hoping he might even become bilingual, which would be simply unthinkable without cochlear implants.

Two weeks after surgery, Maxime returned to GOSH for an important appointment: the switching-on of his new cochlear implants. Audiologist James Sharp gradually activated each implant to establish Maximes level of hearing, then programmed the processor so that the volume of sound would be comfortable and not too overwhelming. Maxime looked on curiously as a toy was banged on the table and touched each of his ears as they began to gently tingle, a sure sign that the implants were working.
It was an emotional moment for Guilia: Weve waited so long for this day and now its real. It was incredibly exciting to see Max react to sound. Its the first step on an exciting journey for all of us.
Dr Kaukab Rajput, lead of GOSHs Cochlear Implant Department, said:
As the 1000th patient to receive cochlear implants at Great Ormond Street, Maximes surgery represents a huge milestone for the hospital. Things have changed dramatically since our first procedure in 1992. Since newborn hearing screening tests were introduced in 2006, we can identify and treat deaf patients much sooner. This means we can perform cochlear implant surgery before children even reach their first birthday, giving them the best chance to learn to speak like a typically developing child. If there is such a thing as a miracle in medicine, then cochlear implants must surely be one of them.
Maximes parents have now been trained in how to use the cochlear implants, and Maxime will follow a programme of speech and language therapy to ensure he can adapt and make the most of this technology.

GOSH is one of the largest paediatric centres in the UK to provide cochlear implants. When the service started in 1992, the audiology department hoped to fit 12 cochlear implants a year. Today, around 100 procedures are carried out each year.

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