Emmerdale couple share son’s rare health diagnosis: What is Usher Syndrome?

Watch: Emmerdale couple reveal that their children are living with a rare genetic condition

A couple, who both appeared on TV in Emmerdale, have revealed that their two children are both living with a rare genetic condition called Usher syndrome.

Mark Jordon and Laura Norton, who played Daz Spencer and Kerry Wyatt in the ITV soap, have two children together. Their son, Jesse, now two, was born in 2021 and daughter Ronnie followed a year later.

Both children have been diagnosed with the condition, which causes deafness or hearing loss along with vision issues due to an eye disease called retinitis pigmentosa (RP).

The couple explained that they first thought something was different not long after son Jesse was born in 2021 when he failed his newborn hearing tests and was diagnosed with moderate hearing loss.

However, he was only diagnosed with Usher syndrome after having further tests during Norton's pregnancy with their second child.

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Mark Jordon and Laura Norton have revealed their children are living with Usher syndrome. (Getty Images)
Mark Jordon and Laura Norton have discussed their children's diagnosis of Usher syndrome. (Getty Images)

Jordon added: "It’s weird because that in itself felt like a big change to the dynamics you imagine as a parent, but instantly you go into defence mode.

"That’s fine – he’ll just have really funky hearing aids and we’ll master everything, we’ll make sure his speech is fine."

Speaking as a guest on the panel of ITV's Loose Women, Norton explained that Usher syndrome is a very rare condition that affects hearing, vision and balance.

Jesse was diagnosed by blood tests taken when he got his hearing aids – but the family didn’t find out the diagnosis until a year later.

"It took a year [for the results] to come back, so I think the moment had gone and we just thought it was a weird little anomaly – he’s got hearing loss, it’s part of who he is now, he’s thriving, it’s great we’ve learned so much about it," Norton recalled.

The couple are sharing their story in the hope of raising awareness of the condition, while also campaigning for funding.

They have previously discussed their son's diagnosis, with Norton revealing she was initially "heartbroken" at the news particularly after learning Jordon and Norton were both carriers of the Usher’s Syndrome gene.

"Knowing that we'd passed this on to our son was heartbreaking," Norton told Hello! magazine, who was subsequently told that her children will develop night blindness in their teens, before peripheral vision loss and tunnel vision.

"To watch our little boy enjoy the world around him yet know so much will be taken from him, was painful.

Jordon added: "Although it was devastating when Ronnie was diagnosed with the same condition as her brother, it was comforting that neither of them will go through this on their own, that they've got each other.

"As they grow up, Jesse will be the best influence and support for her."

The family are now coming to terms with their children's diagnosis, but say that they are currently both "thriving".

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Usher syndrome is a rare genetic condition that impacts hearing and vision. (Getty Images)
Usher syndrome is a rare genetic condition that impacts hearing and vision. (Getty Images)

What is Usher syndrome?

Usher syndrome is a genetic condition that affects hearing, vision and balance.

The condition is inherited, meaning it is present from birth and caused by changes in specific genes you inherit from your parents.

The condition is rare, affecting between four and 17 people in every 100,000 worldwide.

While people who have the syndrome are born with it, they are often only diagnosed in childhood or as teenagers.

What are the symptoms of Usher syndrome?

According to the charity Sense, there are three types of Usher syndrome and each type has its own set of symptoms.

Those with Type 1 experience profound hearing loss or deafness at birth, loss of night vision by the age of 10, sever loss of their vision by midlife, and suffer from balance problems that interfere with activities like walking and sitting up.

People with Type 2 have moderate to severe hearing loss in early childhood, loss of night vision by teenage years, with severe vision loss by midlife, but have normal balance.

Lastly, those with Type 3 Usher syndrome have normal hearing at birth, with hearing loss starting in childhood. They will likely experience loss of night vision by the time they’re teenagers, with severe vision loss by midlife. Around half of people also experience some problems with balance later in life.

'Atypical Usher' is when someone has a range of symptoms that do not fit into one of the three types above.

The condition impacts eye sight. (Getty Images)
The condition impacts eye sight. (Getty Images)

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What is the treatment for Usher syndrome?

At the moment there is no cure for Usher syndrome, but research continues into the genetic causes of the condition and potential new treatments.

There are some early treatments that can help those living with the condition in terms of their hearing and sight.

When it comes to hearing problems, hearing aids, assistive-listening devices, and cochlear implants are some of the treatments used to help patients hear better, depending on the type of Usher syndrome the patient is living with.

For vision problems, treatment for retinitis pigmentosa focuses on relieving symptoms and making the best of someone’s remaining sight.

This could include the use of low-vision aids and vision-rehabilitation services, including regular monitoring of eye function or including the prescription of glasses, if needed.

Children may also be encouraged to learn sign language and Braille to help provide them with further methods of communication.