Scared to Deaf?

The Granny Gatehouse Project #3

A little while ago I kicked off ‘The Granny Gatehouse Project’ - a series of posts about Granny G - Dads mum - and our efforts to try and understand her unique life story.

As a child I remember being told that she’d been dumped on the steps of a church at 2 years old, or perhaps the steps of a nunnery when she was 3 years old. A profoundly deaf child whose family supposedly couldn’t cope with her. It was all a fairytale.

Please note that we are still in the process of uncovering new information. As anyone who has done any family history research knows sometimes you can find out something that later turns out to be partly or even entirely wrong!

And don’t forget, if you prefer to listen to this post then you can click on the play button to hear my voiceover.

Enjoy!

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Granny G was deaf. That was a fact. That much we know for certain. But what we don’t know is why she had such a profound hearing loss. We don’t even know for definite if - to quote Lady Gaga - she was born that way.

I think that she’d be described as profoundly deaf. If you did a loud, high pitched whistle you could get her attention - if she was in the mood. So she had a little hearing in the highest frequencies, but nothing in the normal ranges. Still she loved music as she could feel it and would tap her hand on her thigh in time with the beat.

We can’t be sure that Gran was deaf from birth - something called congenital deafness. But Dad was convinced that was the case. He had a direct point of comparison as his own father, her husband, was also deaf. However, he had acquired deafness as the result of contracting meningitis when he was around 2 years old. Dad (and others in the family) could see distinct differences in language development and usage between his parents and the positive effect on my grandfather of having been able to hear for the first two years of his life.

So back in 1903 why would Gran have been born deaf?

THEORY #1 - A VIRUS

Dads best guess was that Grans mother had caught rubella (sometimes called German measles) while pregnant - he was quite attached to that theory and I wish I’d asked him more questions about why that was the case while he was still alive.

Rubella during pregnancy, particularly in the first 12 weeks, is highly dangerous and can cause congenital rubella syndrome (CRS) resulting in miscarriage or stillbirth. If the baby survives then in the vast majority of cases they’ll be born with significant challenges such as deafness, cataracts and other eye problems, heart defects, intellectual disabilities, and/or organ damage.

Rubella was certainly a common disease in 1903 when Gran was born. Historical data suggests that rubella was circulating widely within large urban populations like London. So it makes some sense that he could be right.

However, rubella was generally a disease of the young, with many children catching it between the ages of 4 and 9 years. Her mother, Mary Ann, would have been 31 by the time Gran was born. If the disease was that common, and her mother was that old, isn’t there a likelyhood that she herself would have already had rubella as a child?

I then wondered - could her mother have had rubella twice? Apparently it’s rare and extremely unlikely - once you’ve contracted the virus you typically develop life-long immunity.

There are other factors that make me question whether rubella was the culprit. Gran lived to the grand old age of 95 so I think we can assume that her heart was pretty good, until she sat up in bed on that last day. She did indeed have eye problems, however her glaucoma was only in later life. I didn’t find anything other than her deafness that really associated with being a rubella baby. So perhaps it was rubella, but maybe not.

Could it have been another virus caught by her mother in pregnancy that impacted her baby? The simple answer is yes.

There are a number of viruses that can lead to a baby having deficits in their hearing at birth, or that put them at risk of later hearing loss. For example, though as yet unidentified and unnamed by science, congenital cytomegalovirus (CMV) could have been to blame. It’s passed from a pregnant woman to her baby and it would indeed have been circulating in the London population in the early 1900’s. In the present day it is the leading infectious cause of congenital hearing loss in children.

THEORY #2 - GENETICS

The most common cause of babies being born deaf in the UK is genetic factors, accounting for roughly 50% to over 80% of cases. But you’d be wrong if you thought that it was anticipated as their parents were deaf. Statistics from the National Deaf Children’s Society in the UK show that some 90% of deaf children are actually born to hearing parents. Half of deaf children are born deaf and the other half become deaf during childhood.

These days hundreds of genes have been identified that cause or contribute to deafness. But back in the 1960’s (yes, I am that old!) there was no test that would have told Mum and Dad whether I might be born deaf until I arrived in the world. As it tuned out I was born hearing. Then again Kim says I demonstrate truly astounding levels of marital deafness when I feel like it or when he’s trying to talk to me when he can see that I have my noise-cancelling headphones in my ears! I’d suggest he also has similar skills. ;o)

Looking at the family more widely we know that Gran gave birth to two hearing children. One of whom had a hearing child (aka good old me). And there our family ends as I haven’t had biological kids of my own.

Perhaps there’s not enough evidence to rule it out the possibility that Gran was deaf from a faulty gene, but I think it’s enough to make it seem less likely.

Image caption: one from the family archives of Granny G circa 1974

THEORY #3 - SOMETHING ELSE

If it wasn’t a virus affecting her mother during pregnancy. And it seems unlikely to have been due to some genetic issue. Then why was she deaf?

In her school records it stated that Granny G was deaf as the result of ‘fright’. Yep, you read that correctly - FRIGHT. Generally defined as a sudden, intense feeling of fear, terror, or alarm, often triggered by a shocking, unexpected, or perhaps traumatic event of some kind. Someone somewhere in the family suggested it was related to a fire. But what fire? When was this fire? Did it relate in some way to her being removed from her mother and her stays in hospital? Or is the fire just another convenient family fairytale?

When I started to write this post with the title “Scared to Deaf”, I was playing with words, and it was definitely tongue-in-cheek. It seemed absurd to even think that someone could indeed end up deaf from such a thing as fright.

BUT… just before hitting publish on the original (very different) version of this post I thought I should check if it was a possibility. And when I went to Google I got a surprise.

It seems that intense fright, shock, or severe acute stress can cause deafness or sudden hearing loss, though it’s most frequently temporary. It is a recognized physical reaction to extreme anxiety or trauma. However, it seems to be in only very rare cases that it would lead to permanent deafness.

But back in the early 1900’s medical understanding of the reasons for deafness were still evolving, and psychological trauma was thought, in addition to infectious diseases like meningitis and scarlet fever, to be a factor. It was thoughts that “maternal fright” experienced by a mother while pregnant could cause her child to be born deaf or it could be caused by the “fright of the child” where sudden deafness was attributed to severe emotional shock experienced directly by that child.

An 1880 article described a 12 month survey by Professor Stanich who had looked at reasons for deafness in the population of Sydney, Australia. By far the most common one (around 40% of cases) that he noted was a cold and what I think we’d now assume to include ear or sinus infections. He also listed other diseases that were recognized as being associated with deafness such as scarlet fever, rheumatic fever, mumps, and typhus. But he didn’t stop there and he included some other reasons that today seem incredible such as ‘mental excitement’, ‘sorrow’, and ‘deficiency of sleep’!

Gran was indeed in hospital for extended periods (in both case a couple of months) in 1908 and again in 1909. It’s a period of her life that we plan to investigate further - currently we don’t know what was wrong with her, though we’ve guessed it might be due to something like scarlet fever and perhaps later rheumatic fever. Both of which could be associated with loss of hearing. However, she’d have been 5 or 6 years old by that stage and if she had been hearing up until that time then we assume that her language capabilities would have been far more advanced than they turned out to be.

The mention of colds and ear infections in that Australian survey got me thinking though. As a small child I was hit hard by nasty ear infections on several occasions. Mum still remembers me asking her to go and ask the noisy kids outside to play more quietly. But the noise was all in my head from the infection. Is there something about my family that made us more prone to ear infections as children?

What if the answer to all this is very simple? What if Gran had some sort of cold and ear infection as a baby? What if that infection destroyed her hearing? It’s unlikely that her single mother could have afforded any form of medical care. And there would have been no effective treatment as it was decades before the advent of antibiotics. By coincidence my other grandmother - Granny P - worked on the first penicillin production lines at Glaxo in the 1940’s.

Ultimately we’ll most likely never know why Gran was deaf. And it probably doesn’t matter. She managed to make a good life for herself - married - had children - enjoyed playing with a grandchild - and gave a wide variety of dogs, cats, and other animals (often against their will!), big, squelchy, loving, kisses and cuddles. I have to admit that I definitely inherited the gene for that last part!

I’ll never know if I do carry a gene for deafness as I’m the end of the line when it comes to our genetic pool. But then again, maybe I’m wrong. Gran did after all have an older brother…

To be continued.

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If you missed the previous The Granny Gatehouse Project posts then you can find them both via the links below:

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If you liked this post then I’d love it if you would click on the heart and add a comment about what resonated for you – it means a lot to me to hear from each of you.

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In case you missed it, I published my book - Hold My Hand: A Journey Back to Life - here on Substack last year. If you’d like to read it then you can find each chapter by clicking HERE and it will take you directly to the webpage dedicated to the book where you can read or listen to each chapter.

If you would also be kind enough to share it I would be eternally grateful as it will help more people learn about these deadly infections. Maybe one day that knowledge will save a life.

Thank you!