Unilateral to bilateral hearing loss

Bonny was our third daughter in three years. The labour was long but an on-again-off-again affair. She was my smallest baby - 5lbs 13oz - and perfect. Her milestones were a bit delayed. I thought she spent too much time in the baby capsule. Her dummy was her favourite companion (and my saviour) and I thought her speech was slow because the other girls spoke for her and a dummy always plugged her mouth.

I am an occupational therapist by training and always had an interest in development. Before having babies, I had worked at a large hospital where I was the OT at the clinic which followed up all the babies who had needed special care after birth. I always worked with a physio and a speechie and knew a fair bit about normal developmental milestones. By 18 months, I was concerned about Bonny's speech. She was hardly babbling.

We started the rounds of ENT, speech pathologist and audiologist but I was seen as an over-anxious mother. Her speech, however, was delayed and we started seeing a speech pathologist.Her hearing was tested and appeared to be normal.

When Bonny was about 13 months, she developed asthma and required her first hospitalisation. She was always sick. Ear infections, tonsillitis etc etc. By 3 years of age, she had been on antibiotics for almost a year and her tonsils were so large they touched in the middle. She had sleep apnoeia. The ENT decided it was time for her tonsils and adenoids to be removed and grommets to be inserted. A scrawny little thing went into surgery and three weeks later she was putting on weight, talking and no longer had asthma. We thought all our problems were over.

She was talking but she sounded terrible. Very nasal and it grated on our ears. After three years of not talking, we wanted to tell her to be quiet. Back to the ENT.

The diagnosis

He thought I was being histrionic but sent us along to the cleft-palate clinic. One of the causes of hypernasal speech is a sub-mucosal cleft of the palate. We eventually got an appointment and arrived at the Children's Hospital with some trepidation. One of the assessments they do for every child who goes to the Cleft Palate Clinic is a hearing test. Bonny had the hearing test, saw the speech pathologist and then we were called in to see the panel at the clinic and they dropped the bombshell. She didn't have a sub-mucosal cleft but she did have a short soft palate AND she had a significant hearing loss in her left ear. We would need to follow that up at Australian Hearing. She would also need speech therapy to assist with the hypernasal speech.

Back to Australian Hearing. Bonny was now old enough to use headphones, so they could test the hearing in each ear separately. Bonny had no detectable hearing in her left ear. But her right ear was fine. 'No dramas Mother. Children with unilateral deafness do fine at school as long as they sit in the right position. Here are some fact sheets for her teacher. See you again in a year.'

So Bonny followed her big sisters to school. She loved it. She was a social animal and school provided her with every outlet she desired. But she didn't progress academically. She constantly got the wrong message. She couldn't master reading. I grew increasingly unhappy with her progress.

A unilateral to a bilateral loss

On September 23, 1996, the three girls hopped into the car after school. Bonny was 7 and hopped in the front seat. She turned to me and said, 'Mum, everyone sounds like aliens today.' We carried on with the afternoon rush - swimming lessons for one, ballet for the other and then home for homework and dinner. I was testing Bonny for her spelling but she could not hear what I was saying across the kitchen bench. I thought she must have some wax in her good ear so when Bruce came home from work, I took her up to our local medical centre.

The good GP was on and tested her hearing. She had no hearing at 4 and 8kHz. There just happened to be a paediatrician on that night. He saw her and arranged for me to drive Bon straight out to Westmead Children's Hospital. I was terrified. I drove home, shaking like a leaf. I wanted to vomit. We packed her little bag, her favourite toy, Bicky the Bear, and said goodbye to Bruce and the girls. It was the longest drive of my life. Bonny fell asleep, hugging Bicky - oblivious.

One of the major causes of sudden hearing loss in children is enlarged vestibular aquaduct syndrome. There was some evidence that immediate large doses of steroids could help arrest the hearing loss. We arrived at Casualty and Bonny was started on the steroids. The place was crowded and she spent the night on a gurney, while I dozed in a chair.

Bon was as bright as a button the next morning and I felt terrible. Her hearing had deteriorated overnight. She was having troubles hearing normal speech. The registrar organised a hearing test sometime in the morning. He came to see me afterwards. He said Bonny was unlikely to be hearing anything by the next morning. They would need to check the cause but she was an excellent candidate for a cochlear implant.

It was one of those moments when everything seems to slow. I felt I was in a dream. Bonny did ballet. She sang beautifully and only a few days before had been the star of the show in her class assembly. Her whole life revolved around sound. She couldn't go deaf. But she was.

I called Bruce, my Mum and Dad and the girls' school. I wanted Bonny to hear everyone that was close to her before she couldn't hear us any more. She was so oblivious. So unconcerned. While our life seemed to be spiralling out of control, she played happily with her sisters.

Bruce took the girls home. I didn't want Bonny to go to sleep. I told her how much I loved her and thought she would never hear me say that again.When she slept, I phoned Bruce and we cried. But he never looks back - always forward. We needed to be positive about the future and not look back at what might have been. I sat up with the nurses most of the night and talked and cried. They listened and held my hand.

As soon as Bonny stirred the next morning, I was awake. She didn't say anything. I was too frightened to talk. She eventually looked at me and said, 'I can still hear things.'

So began our stay in hospital. They did every test known. She was prodded, poked, X-rayed and scanned but no cause for her hearing loss could be found. Every day the hearing test was worse. After about a week, the ENT decided it was time to do some exploratory surgery. He let us come home for the weekend. It was fantastic to be home with the other girls but it was so strange. Bonny could no longer hear us. She remained completely unfazed by the whole thing.

We returned to hospital on Monday and she was operated on the following day. The ENT found a small leak in the base plate of the stapes - known as a perilymphatic fistula. He grafted the crack and we all kept our fingers crossed.

My mother often reminded me how she had said that it was all over now that they had found a cause of Bonny's hearing loss and I had told her, 'No, it is only the beginning.'

Bonny in 2015

It is hard to believe that it is over 20 years since our journey began. I wish I had a crystal ball in those early days so I could see the person Bonny would become and the life she would forge for herself. There have been ups and downs - the same with all our girls. Although she would prefer not to have lost her hearing, she says that she realises it has made her the person she is and she is happy with that person.  Bonny is now chef and running her own restaurant in London - deaf, 26 and making her own way in the world.