Lisa Kelly: My experience of being ‘fixed’ (BSL)

The recent news that pioneering gene therapy has restored a girl’s hearing has been met with consternation in the Deaf community, who fear it is another attempt to wipe out Deafness and its associated rich culture centred on sign language.

The British Deaf Association (BDA), which represents the Deaf signing community in the UK, responded to the news by highlighting that the critical intervention necessary for the Deaf children to thrive is not gene therapy – but the opportunity to learn sign language.

“Deaf children are not broken; what they need most of all is access to sign language,” it said.

The gene therapy, which replaces DNA causing a type of inherited deafness, was given to Opal Sandy before her first birthday. She is part of a trial for the treatment, made by Regeneron and a professor is quoted as saying he hopes gene therapy can be used to restore hearing, so children don’t have to have cochlear implants.

It seems the medical profession is always looking for something to eradicate deafness and thereby the Deaf community – be it hearing aids, cochlear implants or now gene therapy. Rarely is the option of sign language and an introduction to the Deaf community offered alongside these so-called cures.

It is understandable if hearing parents or carers feel pressured to opt for a ‘cure’ because they are not aware of sign language or Deaf culture. There is no obligation on the part of audiologists to learn British Sign Language (BSL) or its history of oppression cemented by the Milan Conference of 1880 when hearing attendees voted to ban sign language from schools with devasting consequences for the Deaf community.

I was shocked when my first BSL teacher told the class how she was punished for signing at school and had her hands tied to her chair if she was caught communicating with her Deaf friends in her natural language. It is easy to look back and condemn this level of cruelty, but a news story which heralds gene therapy as a ‘cure’ for deafness is a continuation of this cruelty. Deaf people – who have fought and won to have their language recognised with the passing of the British Sign Language Act in 2022 – should not portrayed as needing to be ‘fixed’.

I know because it happened to me as a child. And I had no say in it because I was a child. My deafness was diagnosed around the age of seven after the clues added up. Castigated for ignoring teachers and rudeness; I also told my parents, I was undisturbed by their loud parties because I could sleep on my right side and enjoyed telling people about the ‘magic’ of holding the telephone receiver to my left ear and not being able to hear the caller speak. This led to the realisation I had single-sided deafness. It also led to an endless round of medical appointments – first to the local GP surgery, then High Wycombe Hospital and finally the ENT specialist in Upper Wimpole Street. The excitement of travelling by train to London quickly wore off in the audiometric booth. There, I was subjected to hearing tests, where the sound level was turned up so high, the vibrations caused excruciating pain.

I was never offered British Sign Language (BSL) lessons or introduced to the Deaf community, and its rich history and culture. My parents were offered no information by the audiologists and medical experts. My brother-in-law is an audiologist and talking with him, I’m not sure if the situation has improved. Finance is a big problem. BSL classes are expensive.

The National Deaf Children’s Society campaign, Right from the Start, highlights the need for the Government, local authorities and health bodies to work together to make a commitment to ensure high quality support is in place as soon as a child is diagnosed as deaf. Surely, given the opportunity to become bilingual is empowering rather than deafness solely treated as a medical challenge.

Unfortunately, parents are faced with paying thousands of pounds to learn sign language far from home, rather than it being provided locally and fully funded. More worryingly, 25% of parents reported not getting any general advice on language and communication development, following diagnosis. With 90% of deaf children born to hearing families who are less likely to know BSL, this is a lamentable gap in provision.

There is also a lamentable gap in providing child-centred outcomes that don’t prioritise medical professionals’ ideas about fixing deafness. I remember coming round from an operation to remove a bone in my ear, which after ruling out glue ear, was thought to be the cause of my deafness. Although it did not show up in my medical records, I realise the doctors misdiagnosed otosclerosis, and I underwent a stapedectomy to remove the stapes bone.

As a child you believe adults and doctors and that any operation is necessary and will work. I expected to hear in both ears, so when I came round from the anaesthetic, apart from experiencing pain, I thought I would be able to hear as the ear healed. I remember my mother bought me a doll I’d craved as a get-well present. I also remember pretending I could hear something – anything – to not disappoint her.

Looking back, as a protective mother, she was more vested in my hearing being restored than me. But once in the grips of the medical profession, it wasn’t long before my pretence was found out with subsequent hearing tests. At least the diagnosis by the ENT specialist of mumps-induced deafness put a stop to further investigations. I had sensorineural hearing loss, and the auditory nerve was dead; a hearing aid at that time would deliver no benefit.

His advice that nothing could be done and that my parents should forget about my deafness was brusque, but not uncommon. A friend with single-sided deafness says her parents received similar advice and she has recently had a cochlear implant. But in many ways, I am grateful that hearing aids and cochlear implants were not a realistic option for single-sided deafness in the 1980s, as my parents might have thought it was a good idea, and I might not have started learning BSL in later life.

The technology for hearing aids has improved with bone-anchored and bone-conduction hearing aids offered for single-sided deafness. In 2016, I tried a Contralateral Routing of Signals (CROS) hearing aid provided by the NHS, which picks up sound from the side with no hearing and feeds it into the hearing ear to give the sensation of hearing in both ears. I was curious to know what binaural hearing felt like. I learned how to clean it and insert the batteries, and was happy to wear it, but I never realised the sensation of hearing in both ears.

I did try the phone trick – this time with my mobile to my deaf ear – and I could hear the caller’s tinny-sounding voice, but it did not compensate for sitting in an Italian restaurant and my hearing aid picking up the background muzak to such a level that it drowned out any conversation.

I took it out and never put it back in. Perhaps, my brain needed more time to process this new way of hearing, or perhaps the hearing aid needed adjusting. Either way, it sat on my desk for a while gathering dust, and then got shoved to the back of a draw. I am not alone is my rejection of hearing aids.

A fifth of people with hearing aids do not use them, according to a study led by University of Manchester researchers in 2020. The research showed that 20% of adults do not use their hearing aids at all, 30% use them some of the time and the remaining 50% most of the time.

Reasons for non-use vary from lack of perceived benefit (the box I ticked) to handling difficulties (the box my father ticked). I think back to how he felt increasingly isolated in his 80s because of his hearing loss. He found his prescribed hearing aids fiddly and refused to wear them. It was too late for him to learn how to handle hearing aids, and it was too late for him to learn BSL.

Recently, I requested my medical notes to find out more clues about my single-sided deafness and the treatments I received. The care-coordinator my doctor’s practice unearthed an old letter from the Ear Nose and Throat (ENT) specialist, with its Upper Wimpole Street address, dated 27 May 1980.

It refers to my deaf ear as a ‘dead’ ear, which I found upsetting, but it is symptomatic of the language which frames deafness as undesirable. It went on to recommend I sit at the front of the class and to “make sure her right ear is turned towards the teacher” and “to not warp the child in cotton wool”. I did get on with it until I started learning BSL which led to many new experiences and opportunities.

The term Deaf Gain was invented in 2005 by Aaron Williamson, a British performing artist visiting Gallaudet University in Washington, D.C, the global leader in education for Deaf and Hard of Hearing students. Aaron became deaf in later life and took issue with doctors repeating that he had ‘hearing loss’ and flipped the coin to ask, ‘Why hearing loss and not gaining deafness?’

If it was not for my deaf ear, or as the medical profession might have it, my dead ear, I might not have found a creative aspect to my life, including my love of reading in quiet spaces, that I can directly attribute to my hearing loss.

I would not have started my journey learning sign language, after co-editing the Deaf issue of Magma magazine with Raymond Antrobus; would not have written my first collection of poetry, A Map Towards Fluency, with its focus on my BSL learning experience; would not have written my second collection, The House of the Interpreter, with a deeper investigation of Deaf culture and would not have met many Deaf creatives; and would not have co-edited an anthology, What Meets the Eye: The Deaf Perspective with Sophie Stone for Arachne Press.

I am sure there will be further Deaf Gain. An individual’s experience of Deaf Gain is unique, but the critical point is that positive framing can encourage pride in Deaf identity, finding opportunities, and new pathways. Gene therapy that promises to ‘solve’ deafness is not part of that journey.

Lisa Kelly’s first collection, A Map Towards Fluency, was published by Carcanet in 2020. Her poems have appeared in Stairs and Whispers: D/deaf and Disabled Poets Write Back (Nine Arches Press) and Carcanet’s New Poetries VII. Her pamphlets are Philip Levine’s Good Ear (Stonewood Press) and Bloodhound (Hearing Eye). She is Chair of Magma Poetry and runs poetry workshops at the Torriano Meeting House, Kentish Town. Lisa has single-sided deafness and is also half Danish.