Collaboration across hospitals returns Hannah from the sound of silence

Pictured above: Dr Dayse Tavora-Vieira with Hannah McPierzie, pictured below: Hannah McPierzie post surgery and with her husband Kukame McPierzie.

Hannah McPierzie, now aged 35, was born with neurofibromatosis type 2 (NF2) - a rare genetic condition that causes multiple benign tumours on the brain and spine, affecting hearing and balance nerves.

Even after experiencing gradual hearing loss this wasn’t something Hannah knew she had until the shock diagnosis on her 29th birthday following an investigative magnetic resonance imaging (MRI).

After this diagnosis Hannah underwent routine monitoring, including regular scans and chemotherapy, to try to suppress the growth of the tumours on her auditory nerves.

In 2019, Hannah was informed she needed surgery which was undertaken at Sir Charles Gairdner Hospital (SCGH) to remove a tumour on the right side of her brain and insert an auditory brainstem implant (ABI).

Hannah was an ideal candidate because an ABI can help to provide hearing to people with hearing loss who are unable to benefit from a hearing aid or cochlear implant due to a non-functioning cochlear nerve, but unfortunately, it did not work and Hannah was left with only one functioning ear.

Hannah said for most of 2020, she worked on improving her Auslan skills and relied on a hearing aid on her left side, but gradually her hearing started to deteriorate.

“Unfortunately, in January 2021, my MRI showed significant growth of another brain tumour. This time on my left side,” Hannah said.

Hannah was given two choices and she faced a decision she never thought she would have to make.

“If the tumour was left, I would lose the ability to walk and gradually deteriorate further, losing more capacity as I went.” Hannah said.

“However, if the tumour was removed, I would lose my remaining hearing and become completely deaf.”

Hannah made the tough decision to remove the tumour, undergoing surgery again at SCGH in March 2021.

The 18-hour surgery was a tremendous collaborative effort led by Dr Jafri Kuthubutheen and Dr Arul Bala from SCGH as well as a team from the newly formed state-wide audiology service at Fiona Stanley Hospital (FSH) led by Dr Dayse Tavora-Vieira.

The FSH audiology team played a crucial part in the surgery, guiding the surgeons with the placement of a new ABI.

Hannah said it was strange going into surgery knowing she was about to lose full hearing.

“You could feel the energy in the theatre reflect the graveness of the situation,” Hannah said.

“I awoke completely deaf as I expected but it also affected my facial nerves and I had a lot of weakness in the left-side of my face.”

Hannah spent the next two months in SCGH recovering in complete silence, something she had never experienced before.

“Recovery was tough, it impacted my mental health and I lost a significant amount of weight – 15kgs to be precise,” Hannah said.

Finally, three months after surgery, Hannah’s new ABI could be switched on by the FSH audiology team, within a SCGH theatre because of the risk of cardiac disturbances during stimulation of the implant.

“I was very nervous as I hadn’t had any luck with the first ABI, I remember lying on the bed just staring at my heartrate on the monitor, in complete silence,” Hannah said.

“Dr Dayse Tavora-Vieira switched on the electrodes one at a time and the first ‘beep’ I heard was magnificent.

“I could hear Dr Dayse Tavora-Vieira and Dr Jafri Kuthubutheen discussing the results and they were amazed that I was able to understand them; albeit they did sound tinny and robot-like.”

Thanks to the successful placement of her second ABI, Hannah was able to understand speech straight away as long as she could see the person’s mouth as they formed words.

It took a few months and regular sessions with Dr Dayse Tavora-Vieira at FSH to map the frequency of the electrodes to get more accurate sound.

Dr Dayse Tavora-Vieira said that Hannah went from completely deaf to getting eighty per cent speech understanding scores in three to four months.

“What makes Hannah’s case very special is that she was able to understand speech within just a few weeks after surgery. This restored her ability to communicate with her loved ones and meant she no longer had to rely on a writing pad.”

Hannah says the treatment she received changed her life. She is now teaching again, she can cross the street and hear oncoming traffic, and she can even detect when someone has an accent.

“Being able to hear the voices of my family and friends again was surreal, I never thought I’d hear my husband’s voice again – there were many tears and hugs,” Hannah said.

“I am incredibly grateful for our public health system here in WA and the amazing team that treated me, with special thanks to Dr Dayse Tavora-Vieira, Dr Arul Bala and Dr Jafri Kuthubutheen.

“It is no exaggeration to say that I owe my life to them and that they will always hold a special place in my heart.”

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