World First Gene Therapy Trial

World First Gene Therapy Trial

A deaf girl has been cured and heard her mum’s voice for the first time following a new gene therapy trial.

Opal, from Oxfordshire, was born deaf from the condition auditory neuropathy: the disruption of nerve impulses travelling from the inner ear to the brain.

After just one procedure, Opal can hear again at almost normal levels.

Professor Manohar Bance from Addenbrooke’s Hospital stated that he’d been waiting his whole career for this moment, and the results were even better than he expected, adding that it seems to be a potential cure for all future patients.

The procedure involved a faulty gene being swapped for a healthy gene to enable the nerves to begin sending signals back to the brain.

After the surgery, Opal turned her heard to the direction of a loud clap. Her parents thought a light had caught her eye and couldn’t believe it when the same thing happened after repeating the clap several times.

The good news continued, just 24 weeks after the surgery, Opal could even hear softer sounds such as whispers.

A second child has undergone the same low dose surgery with similar results.

Soon, a high dose of the genes will be tested, and rolled out to a larger set of children if successful.

Currently, cochlear implants are the best treatment for auditory neuropathy, but this is looking likely to change in the coming years.


Why it’s important to always seek help with any hearing loss

This story about BBC radio broadcaster, Helena Merriman’s hearing loss journey encapsulates why it’s key to seek out an audiologist when you suffer any hearing loss.

There are numerous reasons why you might notice a change in your hearing and many of them are nothing to worry about, but Helena’s story shows that seeking professional help can both provide a diagnosis and supply ways to treat the problem.

Helena suffers from otosclerosis, which is relatively common problem with one to two percent of the population suffering from it. However, it is particularly prevalent in women who have recently given birth. This is because of the effect that the pregnancy hormones have on the female body, which fast-tracks the disease.

While Helena’s hearing is not completely back to normal getting help has made her be able to manage the issue. 

Whether it is surgical intervention, hearing aids or therapeutic help there is always something that can be done to support someone who is suffering with hearing loss. There is not always a quick fix but at Clifton Audiology we will give our patients the tools and advice they need to continue enjoying life regardless of their diagnosis.   

Following Helena’s diagnosis, she now presents a regular podcast on BBC Radio 4, in which she interviews people who – like her – were changed by a diagnosis.

The emotional power of hearing

Being able to hear clearly again can be a joyful and emotive experience

This video shows babies hearing their mother’s voice for the first time after being fitted with a hearing aid and fully encapsulates the delight that comes with regaining such an important sense.

Fiona Watts, Clifton Audiology, Clinical Director, says:

“We all know how amazing it is to see a loved one for the first time in a while, but we often forget how much emotion is conveyed by hearing a familiar voice clearly again. At Clifton Audiology, we recognise how momentous getting the right hearing aid can be not just for our patients but for their families too. As this video shows, hearing and communicating is a key part of all of our behaviour and getting it back can bring sheer joy.”

Watch the video here.

Video of babies hearing their mother’s voice for the first time

The Sound of Metal Review by Jonthan our Audiologist

The latest endeavour from Amazon Prime, the film Sound of Metal, touches on a subject close to my heart; musicians and hearing loss. It is an excellent film, and well worth a watch. Without overly analysing the story, I feel there are some key take-home messages embedded in the narrative which apply to our patients.

The film follows a heavy metal drummer, Reuben, whose life falls into free-fall as he starts to suffer from hearing loss whilst on tour. He goes through a number of emotions, including anger and denial as he comes to grips with his situation. You watch as he is supported by a small deaf community and comes to terms with his new reality.

Having worked with a lot of musicians, the emphasis needs to be on wearing hearing protection in loud environments. I cannot stress this enough; if you are a live performance musician, you need to have custom moulded hearing protection. Whether it be custom in-ear monitors, or a custom moulded passive flat frequency response decibel filter, a ‘generic’ fitting solution simply will not suffice. This very topic matter was highlighted in another film A Star is Born with Bradley Cooper and Lady Gaga (also great viewing).

It’s worth noting that noise-induced hearing loss tends to be gradual unless caused by acoustic trauma from an exceptionally loud instantaneous sound in close proximity to the ear. This is not what happened in the case of this film’s protagonist (Ruben), it seems. Noise-induced hearing loss is often accompanied by persistent tinnitus, which Ruben suffered with initially but in his case, it quickly subsided.

It is more likely he suffered what’s called an idiopathic sudden sensorineural loss (SSNHL). The most widely accepted explanation for SSNHL is an autoimmune event, but it is still quite poorly understood and affects a small percentage of the population. Sudden sensorineural loss, if caught early, can sometimes be rectified with a steroid injection. If hearing drops suddenly then seek urgent audiological assessment and medical intervention, as the time-sensitive treatment window could mean the difference between retaining much of your hearing or going deaf as Ruben did.

The film covers the grieving process well, as sudden hearing loss is just that – a loss. And you follow the protagonist’s journey through the stages of grief to acceptance. It also paints a very accurate picture of the limitations of cochlear implants (CI). It is much harder to accept the sound quality offered by CI if you have experienced natural hearing, and success starts with comprehensive management of expectations as to such. Ruben probably should have had this explained to him more thoroughly! However, on the whole, the film is compelling and opens a vivid window into the world of hearing loss, shining a light on this silent condition which is hugely in need of public awareness as always.

You can watch The Sound of Metal on Amazon Prime.

The Sound of Metal poster

Hearing benefits available for people with hearing loss

Did you know that there are several benefits available to people with hearing loss?

Matthew Allsop, The Hearing Guy, has a new video that explains the hearing benefits available to people with hearing loss or who are deaf in the UK. They range from discounted travel to access to work support and a personal independence payment.

Fiona Watts, Clinical Director here at Clifton Audiology, says:

“It can be exceedingly difficult being Deaf, deafened or hard of hearing, when travelling or when at work. This video highlights some of the areas you can get both funding and support if required. At Clifton Audiology, we think about every aspect of our patient’s journey, not just when they are being assessed but also how they will cope in their relationships, and with everyday communication and tasks.”

Watch the video to find out the benefits that might be available to you and the details of how you could qualify.

Hearing benefits available for people with hearing loss video

The Impact of COVID-19 on our hearing and balance

The recent news that the first man in Britain has suffered permanent hearing loss* after developing COVID-19 is undoubtedly worrying.

The coronavirus family is not new to us, and through our understanding of its other virus family members, we can tell that it is likely to enter the central nervous system in a similar way (1). This is evidenced by COVID’s trademark symptoms of impairing smell and taste (2), and so it is not a far leap to question whether the disease can cause balance or hearing issues as other viral infections have been proven to (3).

Indeed, up to 12% of confirmed COVID patients since the initial outbreak in Wuhan have reported dizziness as an identifiable symptom (4), and other studies have found over 10% suffered from lasting hearing issues (5).

In the current climate-driven by COVID-19 a more sedentary lifestyle is becoming commonplace, and the vestibular system (the motion-detecting organ of our inner-ear) is therefore going to be less stimulated for a growing number of people. When impairment of our inner-ear happens quickly, often through a viral infection, the symptoms can be severe – comprising rapid hearing loss, tinnitus, nausea, vomiting, and vertigo lasting for days on end.

It is still too early to prove whether the coronavirus itself can directly affect the inner ear. Armed with this initial information, and foreseeing our less mobile lifestyle, it is highly likely we will see an increase in the number of inner-ear related balance and hearing disorders in people over the coming months.

At Clifton Audiology, we are prepared to help and deal with any consequences of viral illnesses, like COVID-19, on hearing loss and balance disorders.

We have several services and are using the latest state of the art equipment to help diagnose and then recommend treatment or rehabilitation.

Please contact us to find out more.

*BBC News Article


Hearing loss: Tips for better communication

Communicating with hearing loss or to the hearing impaired can sometimes be difficult, but here are some simple communication tips you can use, to help the person understand you.

1. Attract the listener’s attention before speaking

It’s important to get the listener’s attention before you begin talking. For example, by saying his or her name. This simple action will prepare the person to listen and allow them to hear the first part of the conversation.

2. Face the person you are communicating with

Your facial expressions and body language add vital information to your overall communication. For example, you can “see” a person’s pleasure, frustration, or excitement by watching the expression on the persons face.

Sit where there is good lighting so that you can see more easily and where possible stand or sit no more than 2 metres (6 feet) away from the person you are communicating with.

3. Avoid covering your lips and mouth

Most listeners make use of lip-reading. Lip-reading helps improve recognition of more difficult speech sounds, especially in difficult listening situations such as background noise. Make sure the person you are talking to can see your mouth where possible, and if you are using a face mask or covering try to select one with a clear panel over the mouth.

4. Speak naturally and clearly

Speak clearly and at a natural level, but you do not need to shout. Shouting can distort the words and will hinder effective communication. If you tend to speak quickly, try taking more time over your words at a relaxed pace. Use pauses, rather than slowed speech, to give the person time to process what you have said.

5. Rephrase rather than repeat

If the listener has difficulty understanding something you said, find a different way of saying it. If he or she did not understand the words the first time, likely, he or she will not understand them a second time. So, try to rephrase it. For example, if the person did not understand when you said “what time did you get here?” change it to “when did you arrive?” instead.

6. Reduce background noise where possible

Turn off the radio or television. Move to a quieter space away from the noise.

The charity RNID has designed this card which you can download with similar tips.