Looking deeper, learning more

Samantha Provenzano visits with Big Bird inside the University of Michigan C.S. Mott Children's Hospital eight months after undergoing surgery to remove a brain tumor.

Doctors evaluating then-6-year-old Samantha’s hearing problems found something else – a tumor in her brain.  Now she’s back on track after surgery, finishing first grade this spring and sporting a colorful set of hearing aids.

Like most 7-year-olds, Samantha Provenzano is always on the move.

She loves rollerblading, riding her bike, playing baseball and practicing Tae Kwon Do with her younger sister, Gabriella.

Anyone meeting the Livonia first grader may find it hard to believe that about a year ago, surgeons at the University of Michigan C.S. Mott Children’s Hospital removed a tumor from her brain.

Samantha’s parents, Lee Ann and Eugene “Bud” Provenzano, came to Mott last year seeking a second opinion on their daughter’s hearing loss. As U-M doctors looked for the source of the problem, they found another – a tumor pressing against her brainstem.

“If it wasn’t for the team of U-M doctors who worked together to find the source of Samantha’s hearing loss, we wouldn’t have known about the tumor,” says her mother. “We’re so thankful. They saved our little girl’s life.”

Samantha had failed a routine hearing screen at her school and the Provenzanos were looking for answers.

Based on a friend’s recommendation, the couple made an appointment with Mott pediatric otolaryngologist Marci Lesperance, M.D., and pediatric audiologist Katie Kuboushek, Au.D.

Samantha’s hearing test suggested a more complex reason behind the loss than recurrent ear problems.
“The moment we spot hearing loss that appears to be progressing it’s crucial that we do something sooner rather than later because there can be so many implications that could impact a child’s language, learning and social development,” says Kuboushek. “At Mott, we focus on the big picture. If there’s a problem, we want to find its source.”

The Provenzanos were able to see Lesperance the same day they were referred to her. A CT scan confirmed Lesperance’s initial suspicion: an enlarged vestibular aqueduct on Samantha’s right side seemed to have caused the hearing loss.

The vestibular aqueduct is a narrow channel that travels from the inner ear to inside the skull. When the vestibular aqueduct is abnormally large, it may indicate that the inner ear did not develop properly and that the child is at risk for further hearing loss. Lesperance says that Enlarged Vestibular Aqueduct Syndrome (EVAS) is one of the most common abnormalities she sees on a CT scan.

Samantha Provenzano spends time with a special visitor as she recovers in the Pediatric Intensive Care Unit at the University of Michigan C.S. Mott Children's Hospital. Samantha underwent a nearly 10-hour surgery to remove a tumor pressing against her brainstem.

What isn’t common is finding a tumor. The CT scan revealed a mass on the left side of Samantha’s brain, which Lesperance says wasn’t associated with her hearing loss.

“When I see an incidental finding on a CT scan like I did with Samantha, I walk across the hall to our neurosurgeons and say ‘Before I talk to the family and tell them what it shows, let me start working on addressing this issue for them,’” says Lesperance.

Lesperance turned to Cormac O. Maher, M.D., a pediatric neurosurgeon at Mott, who immediately scheduled a follow-up appointment and MRI.

Maher told the family that although the tumor was most likely benign, it needed to be removed due to its location and possible damage to cranial nerves. In April 2010, Samantha spent more than 10 hours in surgery.

Ten weeks later, she walked across her school’s stage to receive her kindergarten graduation certificate.

Samantha is proud of her scar, which the boys in her class thought “was cool,” although it’s no longer visible under her golden locks. She now wears bright blue hearing aids with pink, white and blue ear molds to correct her hearing loss.

Samantha’s parents say they are thankful for the Mott doctors who listened to their family’s concerns and helped heal Samantha.

“The University of Michigan doctors are very good at what they do,” Lee Ann Provenzano says. “They made a very scary situation easier.”

Written by Heather Guenther

Editor’s Note: Today is the one-year anniversary of Samantha’s successful 10-hour surgery to remove a brain tumor.  Congratulations to Samantha and her family on a great recovery!