Ménière’s disease and hearing disorders
At 15, schoolgirl Elizabeth Bova, worn down by violent dizzy attacks and ringing ears, asked her neurologist to destroy a critical part of her inner ear in the hope of regaining some semblance of peace.
Since her first year in high school, the Sydney teen had been struggling with Ménière’s disease, an incurable condition that disturbs the hearing and balance regulators.
“I was at a terrible low after a run of random dizzy and vomiting attacks, and the ringing in my ears was driving me insane because you can never switch it off,” says the now 27-year-old allied health worker.
“I was so fed up I asked my doctor for the injection that kills off your vestibular, but of course he refused because he knew it would have left me completely deaf in one ear.”
Bova, who contracted the disease at 12, is now among the estimated one in six Australians who suffers from a hearing/balance disorder, a statistic that has prompted the federal government to open a national inquiry into hearing health and wellbeing.
The standing committee on health, aged care and sport began sittings in November last year with further hearings scheduled to run until about June in centres across the country.
One of its terms of reference is to assess if the social and economic cost of hearing health justifies it being elevated to priority classification along with nine other chronic conditions including cancer, mental illness and diabetes.
According to Whirled Foundation, formerly Meniere’s Australia, the hearing/balance impaired need urgent help. And the foundation is just one of the many organisations and individuals campaigning for government research funding.
Access Economics in 2005 carried out Australia’s first and only definitive research report on the full economic impact of hearing loss in Australia and put the figure at $11.75 billion, with another $11.3 billion associated with loss of wellbeing.
Whirled Foundation president John Cook says it is likely, in 2017, that that figure will be significantly higher due partly to an outdated Hearing Services Program that was introduced after World War II.
“The thrust of our submission is for changes that give consumers a voice similar to the Living Longer Living Better aged-care reforms,” he says.
Ménière’s disease alone affects about 50,000 people in Australia and strikes mainly those aged over 40.
Symptoms include random attacks of dizziness and vomiting, ringing in the ears (tinnitus), uncontrollable eye movements and gradual loss of hearing that usually affects one ear.
Sufferers, some of whom avoid leaving the house for fear of a sudden attack, describe the sensation as similar to having somebody spin you nonstop for two hours on a roundabout while you are vomiting.
That intensity tones down over the years but sufferers are usually left with diminished hearing or deafness in the affected ear.
Dr Daniel Brown, the head of Australia’s only dedicated Ménière’s research laboratory based at Sydney University, says his team is making steady progress in gaining a better understanding of the inner-ear pathophysiology underlying both the balance and hearing symptoms of Ménière’s.
The inner ear comprises the cochlea (hearing organ) and the vestibule (balance organ) and is filled with two fluids separated by a membrane called the membranous labyrinth.
“It’s called a membranous labyrinth for a reason,” says Brown. “It’s a complex anatomical structure buried deep in hard bone so we can’t simply look at it to see what is going wrong. This makes inner-ear research difficult, and finding solutions to conditions like Ménière’s will not come easily or quickly but we are making inroads,” he says.
Ménière’s symptoms are thought to be caused by an abnormal increase in the fluid volume of the membranous labyrinth, which disturbs the sensitive “hair cells” in our vestibule and cochlea that sense balance and sound.
What leads to this increase is not known but it is thought that it might be caused by any number of pathologies such as autoimmune or hormonal dysfunction, viral infections, genetic disorders or even trauma.
While there are treatments available for Ménière’s, in many cases existing medication is not effective. Brown’s work is focused on trying to correct the fluid imbalance as a targeted treatment for Ménière’s.
He is also interested in developing better diagnostic techniques for a condition notoriously difficult to pinpoint.
“I know it’s frustrating for sufferers who want answers now. Even though I’m not a clinician I get regular calls from desperate people who are pleading for help,” he says.
One of the experts Brown regularly brainstorms with is eminent otologist Professor William Gibson, a recognised champion of the hearing impaired.
Gibson came to Australia from England in the early ’80s, inspired by the work of Melbourne’s Professor Graeme Clark who with his team developed the world’s first effective cochlear implant, also known as the bionic ear.
Gibson expanded the program to New South Wales, attracting controversy when he performed an implant on a five-year-old with congenital deafness in 1987.
“Opponents, some of whom were from within the medical profession and even from the hearing-impaired community, considered it politically incorrect to take these kids away from sign and to make them into what they called pseudo hearing kids,” Gibson recalls.
“The little girl’s name was Holly and the principal education officer at her school went around to see her parents to try to stop the procedure on the grounds it would fail and this child would lose out.”
The parents gave their consent and Holly went on to excel at school and eventually become a lawyer.
“A pile of mail would arrive before every operation on a child during that period but most of our kids did brilliantly, in stark contrast to what they would have achieved with only sign language,” says Gibson.
Amid that early hostile environment Gibson learnt what it meant to scratch around for every health dollar, so he supports the push to make hearing impairment health a priority, and considers it long overdue.
“It is still an invisible disorder and is largely underestimated by the community despite the fact it can be a big disadvantage in life,” he says.
“With blindness, for example, we can shut our eyes and realise how dreadful that is, but with hearing disorders people tend to think, ‘Oh, they’ll be alright because they can lip read or use a hearing aid.’”
Elizabeth Bova, along with many sufferers who have made submissions to the inquiry, agrees public awareness is still sadly lacking.
“When I was about 19, a bouncer at a bar I was at tried to throw me out because he saw me staggering to the toilet to vomit during an attack and thought I was drunk,” she recalls.
“Even a night out at a restaurant can be frustrating because salt can bring on an attack, but it is so difficult to try to convey that message without sounding like a whinger,” she says.
Bova says she has finally reached a stage in the disease where attacks are fewer and less intense. But the tinnitus has never left her.
“I had to learn that on the bad days you just go with it and on the good days you make the most of it,” she says.
“Looking back, it was tough. I missed a year of school and a year of university but I finished my degree and I cling to that success because it gives me hope for the future.”
The Whirled Foundation’s John Cook says the government’s failure to act on its own recommendations for improvements in 2010 and 2012 still stings, but he is cautiously optimistic the latest inquiry will result in action.
“We can only hope,” he says.
This article was first published in the print edition of The Saturday Paper on Mar 4, 2017 as "Starting to listen".
A free press is one you pay for. In the short term, the economic fallout from coronavirus has taken about a third of our revenue. We will survive this crisis, but we need the support of readers. Now is the time to subscribe.