The ultimate Christmas gift: 6-month-old hears mom and dad for the first time

Francesca is first patient treated for hearing loss after early virus detection through world-leading newborn screening test

On December 10, 2018, Francesca Jones had her cochlear implants activated, often referred to as being ‘turned-on’, allowing her to truly hear for the first time. The moment the first implant was activated, it was apparent she could hear her parents talking to her. Her mother, Julia Tirabasso, said “Ciao Francesca!” and Francesca looked right into her eyes and smiled.

Francesca is the third youngest infant to receive cochlear implants — electronic devices surgically implanted to partially restore hearing — at The Hospital for Sick Children (SickKids). She had her cochlear implant surgery at just five months of age and the activation took place when she was six-and-a-half months old.

“The past six months presented us with an extreme amount of uncertainty. Seeing Francesca look up and smile at us as she hears our voices for the first time is the ultimate Christmas gift. We have so much to be thankful for this year,” said William Jones, Francesca’s father, shortly after SickKids audiologist Susan Druker had activated the cochlear implants, one side at a time.

In July, just weeks after birth, Francesca was diagnosed with congenital cytomegalovirus (cCMV) infection — the most common cause of non-hereditary hearing loss in children. She is the first infant to have the virus detected from a new targeted newborn screening test introduced earlier this year by Newborn Screening Ontario (NSO), based at CHEO in Ottawa, and Ontario’s world-leading Infant Hearing Program (IHP).

Funded by the Ministry of Children, Community and Social Services and the Ministry of Health and Long-Term Care, this new screening aims to identify many more babies like Francesca to ensure early intervention strategies are available.

Newborn screening is a test done in Ontario shortly after birth, where a small sample of blood is taken from the baby's heel. The sample is then tested for a variety of treatable diseases. Every newborn in Ontario also has their hearing tested through the IHP. When Francesca failed her hearing screen, her parents consented to testing her blood spot sample for cCMV, a virus that can cause progressive hearing loss. Early detection is critical because a child’s development is tied to hearing and learning language. With cCMV confirmed and after further auditory testing, physicians treated Francesca with antivirals and determined she could benefit from cochlear implants.

“We typically aim to have cochlear implants activated by the time a child is one year old, to limit the effects on development. By determining the cause of Francesca’s hearing loss right away, we were able to accelerate this process,” says Dr. Sharon Cushing, Otolaryngologist and Director of the Cochlear Implant Program at SickKids. “This will ensure that Francesca is no further behind in development than other infants her age.”

Each year, around 925 infants are born with cCMV in Ontario and only 10 to 15 per cent of these infants show symptoms at birth. It is unknown why the virus causes hearing loss in some children but not others. 

“Everyone here at NSO in Ottawa is really happy for Francesca and her family,” says Dr. Pranesh Chakraborty, Medical Director of Newborn Screening Ontario and a pediatrician at CHEO. “Optimizing a child’s ability to communicate is crucial for all aspects of child development. We hope and expect to see many more children benefitting from earlier access to services and treatments thanks to Ontario’s leadership.”