Music to a Little Girl’s Ears

Penelope Grand had normal hearing from the day she was born. She loved to dance to music and she was easily woken by noises. Her parents, Luci and Paul, anxiously awaited her first word. Then, when she was about 10 months old, something changed.

“We really knew there was something wrong when I went to get her out of her crib after her nap one day,” mom Luci says. “She was calling out and fussing, she wanted out of there, and I came into the room but she wasn’t facing the door. I stood right in front of her crib and I called out her name, I clapped, but I got no response from her.”

Genetics are the cause of roughly half the cases of prelingual deafness, even when symptoms don’t appear at birth, as in Penelope’s case. Hearing tests and an auditory brainstem response (ABR) test revealed that Penelope had developed profound hearing loss in both ears. She had become essentially deaf.

“We were devastated,” Luci says. “I was preparing to hear ‘mild to moderate,’ not ‘profound.’ The air sucked out of my lungs. I wondered why. She is so sweet and loving, why did she have to lose her hearing?”

cochlear implant

Penelope’s audiologist prescribed hearing aids, but she had no benefit from them. That made her an ideal candidate for a cochlear implant, a new treatment offered at CHOC Children’s in close partnership with Providence Speech and Hearing Center. A cochlear implant is a small electronic device that is surgically implanted under the skin behind the ear, and has an electrode that extends into the cochlea in the inner ear. The implant’s electrode directly stimulates the cochlear nerves that perceive sound.

Dr. Nguyen Pham, CHOC pediatric otolaryngologist and head and neck surgeon, performed two outpatient surgeries on Penelope to place a cochlear implant on each of her ears.

“A cochlear implant can be life-changing for a child,” Dr. Pham says. “Children who would have been in the deaf community are now joining the hearing community because of cochlear implants. It is essentially restoring nearly normal hearing in these children.”

A Sense of Sound Restored

Oct. 13, 2015, was a memorable day for the Grands. That’s the day Penelope’s cochlear implant was activated, amidst her family and a crowd of supporters from CHOC and Providence.

Penelope begins to hear at 0:06.

“The first moment we knew she could hear was, well, it’s hard to describe what that felt like: joy, pride, happiness, excitement,” Luci says. “The look on her face was beautiful. We must have rewatched that video a hundred times.”

A cochlear implant does not translate sound perfectly. Those who wear an implant hear sounds with a robotic, mechanical quality. Following the implant’s activation, Penelope’s team of audiologists at Providence fine-tuned the frequencies of the electrodes—a process called mapping—until they produced the optimal sounds for her ears.

Now, she is undergoing comprehensive speech and language therapy, a crucial step in helping her interpret the sounds she hears and learn to communicate. She also uses American Sign Language.

“We are in awe of her, how much she’s already progressed,” Luci says. “She responds to her name now when we call out to her. She finally said her first word, bye bye, and she can now say mom. She has begun to dance again. She also has started singing in the car with me along to the music. We realized that she is so smart and so very happy, and her potential is endless. We are happy and grateful every day that she had this opportunity.”

Learn more about the CHOC Children’s Cochlear Implant Program.

 The Cochlear Implant Program is generously supported by Wildcard Army, in memory of Nixon Wildcard Hunter. 

Related articles:

Advice to Jiu Jitsu Parents: How to Prevent Cauliflower Ear in Your Child

By Dr. Nguyen Pham, Pediatric Otolaryngologist

In recent times, many parents have turned to martial arts to empower their children against the threat of bullying.  Many of these parents view Brazilian Jiu Jitsu as the ideal self-defense method, as it teaches children to use leverage and guile to protect themselves from larger attackers without the need for excessive violence such as punching or kicking.

Unfortunately, like any martial arts activity that involves physical contact, Brazilian Jiu Jitsu increases the risk of a child developing a cosmetic deformity called cauliflower ear.  However, contrary to popular belief, cauliflower ear is completely preventable.

Cauliflower ear is caused by direct impact and shear force to the outer ear. This can happen when a child is accidentally struck in the ear by an opponent’s head or elbow. A common result of this injury is damage to the perichondrium on a child’s ear. The perichondrium is a thin layer of tissue that surrounds the cartilage on the outer part of the ear. It is important because it provides the cartilage with its necessary blood supply and nutrients. Injury to the perichondrium also damages the perichondrial blood vessels, resulting in blood filling the space between the perichondrium and the cartilage. This pool of blood creates a problematic condition called an Auricular Hematoma. If this hematoma is not urgently drained, permanent damage to the cartilage will occur. The cartilage will then become thickened and scarred and start to look like the cauliflower vegetable; hence the term cauliflower ear.

Fortunately, there are two effective ways to protect your child from developing cauliflower ear. The first way is through preventive techniques, and the second way is through recognizing the signs of an auricular hematoma.

In terms of preventing cauliflower ear, parents should require their children to wear ear protection at all times during any type of Jiu Jitsu or wrestling activity. Most sporting goods stores sell grappling or wrestling ear guards that provide children with solid protection against any type of ear trauma.

Parents and martial arts instructors should also learn how to immediately recognize signs of an auricular hematoma, which looks like a soft bulge of skin on the front surface of the ear. When pressed, this soft bulge feels like a moderately filled water balloon. The overlying skin on this bulge is often red or purple in color but can also be normally colored.

If an auricular hematoma is recognized, the next step is to have it drained by a surgeon. After drainage occurs, it is important to closely monitor the ear for the re-accumulation of blood, which can happen if the perichondrium does not heal properly after the injury. It is important for the perichondrium to heal so that it can reattach to the cartilage and resume providing the cartilage with its necessary blood supply and nutrients.

To ensure that the perichondrium heals completely, a compression dressing should be applied to the injured ear for one week following the drainage of the hematoma. In addition, the child should avoid engaging in Jiu Jitsu training for at least two weeks. These steps will allow the ear to completely heal.

Related articles:

  • How to teach sportsmanship to your kids
    With summer sports in full swing, now is a great time to teach kids about the importance of good sportsmanship. It’s easy to get caught up in a game and ...
  • Improving an Athlete’s Mental Game
    With the school year and spring sports season winding down, now’s the time when young athletes might need an extra edge over their competition. Additional drills and practices can help, ...
  • FAQ: Hydration for Young Athletes
    By Shonda Brown, RD, CNSC, CSP, clinical dietitian at CHOC Children’s  Water is the most essential nutrient for athletes, yet it’s often forgotten when discussing adequate nutrition for physical activity and ...

Kids and Ear Infections

Pic of Boy Covering EarsEAR INFECTION SYMPTOMS
An ear infection is an acute inflammation of the middle ear caused by fluid and bacteria behind the eardrum. “Usually it starts with a cold, so the child will have a runny nose and a cough. Colds can lead to  ear infections in susceptible children,” says Dr. Nguyen Pham, an ear, nose and throat specialist at CHOC  Children’s. “Older kids will pull on their ears and tell you their ears hurt. For infants, symptoms can include  fever, irritability, or changes in their eating and sleeping patterns. A pediatrician can look at the eardrum  to diagnose an ear infection.” Generally, ear infections are treated with oral antibiotics.

PROTECT LITTLE EARS
“The best thing families can do is to have really good hand hygiene,” says Dr. Pham. “Everyone should wash hands constantly. Encourage children to not touch their faces with their hands or rub their eyes,” he says. Colds and the flu can frequently lead to ear infections, so children should be protected against colds and get a flu vaccine, Dr. Pham advises.

HEARING LOSS HELP
“An acute ear infection can lead to temporary hearing loss because of the fluid behind the eardrum. That type of hearing loss will get better over several weeks. If it doesn’t get better, that’s the time to go to the pediatrician or a specialist,” says Dr. Pham. If you suspect your child has hearing loss, ask for an audiogram,  which is a formal hearing test. A pediatrician can perform this test or refer the child to a specialist such as  an audiologist or otolaryngologist.

IS THERE AN EFFECTIVE, PREVENTATIVE SURGICAL TREATMENT FOR CHILDREN WITH FREQUENT EAR INFECTIONS?
Children who have four or more ear infections per year meet the criteria to have ear tubes inserted into the  eardrum. Ear tubes create a drainage pathway for bacteria behind the eardrum to get out, so infections don’t  form. This is a commonly performed surgery in the U.S. and is very effective in preventing ear infections.

FAST FACTS

  • Number of babies born in the U.S. with permanent hearing loss: 3 in 1,000
  • Percentage of children in the U.S. with some hearing loss: 10%
  • Percentage of children who will have at least one ear infection by their second birthday: 90%

View the full feature on Kids and Ear Infections

Dr. Nguyen Pham
Dr. Nguyen Pham
CHOC Ear, Nose and Throat Specialist

PHYSICIAN FOCUS: DR. Nguyen Pham

Dr. Pham specializes in pediatric otolaryngology – head and neck surgery. Dr. Pham completed his residency at the  UC Davis Medical Center and a fellowship at Stanford University. He practiced advanced surgical techniques in airway reconstruction, otological surgery and the treatment of congenital defects at Stanford’s Lucille Packard Children’s Hospital. Dr. Pham has participated in many humanitarian endeavors, including a medical mission to perform cleft lip and palate surgeries in the Philippines and helping patients in New Orleans after Hurricane Katrina. Dr. Pham speaks fluent Vietnamese.

Dr. Pham’s philosophy of care: “My philosophy is to truly listen to my patients and to provide compassionate  care based on the best possible scientific evidence.”

EDUCATION:
UC Irvine School of Medicine

BOARD CERTIFICATIONS:
Otolaryngology

More about Dr. Pham

This article was featured in the Orange County Register on February 24, 2014, and was written by Amy Bentley.