Ausnew Home Care | Hannah faced a 'deaf future', until an auditory brain stem implant changed her life

Hannah faced a 'deaf future', until an auditory brain stem implant changed her life

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At the age of 31, Hannah McPierzie faced a decision she never thought she'd ever have to make: leave a tumour in her brain and lose the ability to walk, or remove it and become completely deaf.

Ms McPierzie was born with a rare genetic condition called neurofibromatosis type 2 (NF2), which causes multiple benign tumours to grow on the brain and spine, affecting hearing and balance nerves.

She didn't even know she had the condition until she noticed hearing loss in her 20s.

"I'm a teacher so I noticed it more every day in my work, hearing kids in class got harder and I noticed I needed captions when I was watching TV," she said.

Following a shock diagnosis on her 29th birthday, the now 35-year-old underwent years of routine monitoring, including regular scans and chemotherapy, to try and suppress the growth of the tumours on her auditory nerves.

Hannah in hospital on her birthday surrounded by balloons and holding a glass of wine
Ms McPierzie spent years in hospital as specialists tried to suppress the growth of the tumours on her auditory nerves.

Due to a non-functioning cochlear nerve, Ms McPierzie would not have benefited from a hearing aid or cochlear implant but was instead identified as an ideal candidate for an implant, which goes all the way into the brain.

In 2019, she underwent surgery at Sir Charles Gairdner Hospital to remove a tumour on the right side of her brain and to insert an auditory brain stem implant (ABI).

However, it failed to work, and she was left with only one functioning ear.

"I got told that it takes a while for the nerves to learn how to process sound so I wore the processor every day, waiting to see if I could hear anything, but I never did," Ms McPierzie said.

An unimaginable choice

Ms McPierzie spent the next year working on improving her Auslan skills and relying on a hearing aid for her left ear, before that also started to deteriorate after she developed another tumour.

Hannah with 'I am deaf I sign in Auslan' written on a bandage around her head
She worked on her sign language skills as her hearing deteriorated.(Supplied)

She was given two choices – leave the tumour and potentially risk losing more motor skills over time, or have an operation which risked total deafness.

She made the tough decision to remove the tumour, undergoing surgery again in early 2021.

The 18-hour procedure at Sir Charles Gairdner Hospital was led by Dr Jafri Kuthubutheen and Dr Arul Bala, with the assistance of Dr Dayse Tavora-Vieira and a specialist team from the newly formed state-wide audiology service at Fiona Stanley Hospital.

Dr Dayse Tavora-Vieira sits at a desk with Hannah as Dr Jafri Kuthubutheen observes
Dr Dayse Tavora-Vieira doing a frequency test with Ms McPierzie as Dr Jafri Kuthubutheen observes.(ABC News: David Weber)

"We were very anxious that we may not be able to give Hannah a sense of hearing back after removing the tumour," Dr Kuthubutheen said.

"But we felt that we had to give it a go, we owed it to Hannah … she was such a strong person, she has been so brave throughout the whole journey, and we felt like we had to give it give it our best shot.

"It was really a team effort, and we couldn't have done it with all the team, from the neurosurgeons to the anaesthetists, to the nursing staff, to everyone at the clinic."

Dr Jaffri Kuthubutheen examines Hannah's auditory canal
Dr Jafri Kuthubutheen and his team restored Hannah McPierzie's hearing. (ABC News: David Weber)

Ms McPierzie spent the next two months recovering in complete silence.

"I knew I would be deaf, but it was just really strange," she said.

"It was a really surreal experience … mixed with recovery."

Returned from the sound of silence 

Three months after her surgery, Ms McPierzie's new auditory brain stem implant was switched on.

A close up of the implant
Hannah had started to get used to the idea of being deaf before the implant was switched on. (ABC News: Dave Weber)

"When we came in for a switch on, I didn't have high hopes because I didn't want to get disappointed again," she said.

But that all changed when she heard the first 'beep'.

"They turned on each of the twelve electrodes and I heard every single one," she said.

"I could hear people talking, but it didn't sound like a normal person … it sounded very strange."

It took time for Ms McPierzie's brain to interpret and process sounds, but due to the successful placement of her second ABI, she went from being completely deaf to getting 80 per cent speech understanding scores in under four months.

'I owe my life to them'

Her condition soon improved to the point where she could do everyday things like ordering a coffee and crossing the street, and it also allowed her to go back to teaching.

"She actually holds a special place in our heart because the progress that she did in so short a period of time is amazing, amazing, absolutely amazing," Dr Tavora-Vieira said.

"It was very difficult for us because of the first one not working, we were all very aware that if this doesn't work, then she's going to be there [deaf] forever.

"So we were aware and very emotional about it, that this [time] it needed to work.

"You have good or bad outcomes and Hannah's is an example of both … one didn't work but the other one did, above any expectation from any professional."

Ms McPierzie said the treatment she received had changed her life.

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

"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."

Source: ABC

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