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 second 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.”
About The Hospital for Sick Children (SickKids)
The Hospital for Sick Children (SickKids) is recognized as one of the world’s foremost paediatric health-care institutions and is Canada’s leading centre dedicated to advancing children’s health through the integration of patient care, research and education. Founded in 1875 and affiliated with the University of Toronto, SickKids is one of Canada’s most research-intensive hospitals and has generated discoveries that have helped children globally. Its mission is to provide the best in complex and specialized family-centred care; pioneer scientific and clinical advancements; share expertise; foster an academic environment that nurtures health-care professionals; and champion an accessible, comprehensive and sustainable child health system. SickKids is a founding member of Kids Health Alliance, a network of partners working to create a high quality, consistent and coordinated approach to pediatric health care that is centred around children, youth and their families. SickKids is proud of its vision for Healthier Children. A Better World.
Dedicated to the best life for every child and youth, CHEO is a global leader in pediatric health care and research. Based in Ottawa, CHEO includes a hospital, children’s treatment centre, school and research institute, with satellite services located throughout Eastern Ontario. CHEO provides excellence in complex pediatric care, research and education. We are committed to partnering with families and the community to provide exceptional care — where, when and how it’s needed. CHEO is a founding member of Kids Health Alliance, a network of partners working to create a high quality, standardized and coordinated approach to pediatric health care that is centred around children, youth and their families. Every year, CHEO helps more than 500,000 children and youth from Eastern Ontario, western Quebec, Nunavut and Northern Ontario.
Newborn Screening Ontario (NSO) has tested nearly 2 million newborns for rare diseases that are treatable. Since NSO started at CHEO in 2006, over 3,000 babies with these diseases have been diagnosed through newborn screening. NSO is the most comprehensive newborn screening program in Canada, and is one of the largest and most modern programs in the world. NSO is funded by the Ministry of Health and Long-Term Care.
Funded by the Ministry of Children, Community and Social Services, the Infant Hearing Program identifies children born with permanent hearing loss across Ontario and provides audiology assessment and intervention services to help these children develop language and literacy skills. Ontario is one of two provinces that have an EHDI (Early Hearing Detection and Intervention) program in Canada and its evidence-based program protocols for screening and assessment have been adopted by jurisdictions worldwide. Last year, more than 125,000 newborns received a hearing screen through the Infant Hearing Program.
The Hospital for Sick Children (SickKids)
416-813-7654 ext. 228728
The Hospital for Sick Children (SickKids)
416-813-7654 ext. 201436
CHEO and NSO
613-737-7600 ext. 3536
Ministry of Children, Community and Social Services