Tag Archives: Microtia and Atresia

Isla Feels More Confident Than Ever with New Ponto 5 SuperPowers

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Katelyn Harkins tells us how getting fit bilaterally with Ponto 5™ SuperPower processors have helped her eight-year-old daughter Isla blossom socially.

Isla was born with bilateral microtia/atresia. She was adopted from China at 20 months old and had no access to sound prior to coming home. She received Ponto bone anchored hearing processors on a softband as loaners from Oticon Medical the month she turned two and loved them immediately. She even insisted on wearing them to nap!

We are privileged enough to have access to excellent hearing solutions from Oticon Medical, which give Isla a near normal audiogram. We are also lucky to have a school for the Deaf nearby, which gives Isla access to education provided in both spoken English and American Sign Language (ASL).

We were very excited to hear OM was coming out with a Ponto 5 SuperPower! Isla’s hearing loss is severe enough that the extra headroom of the SuperPower allows for access to as much sound as possible, and equally little feedback. Isla was thrilled that the upgrade gave her onboard controls for volume and mute.

Isla’s upgrade included getting bilaterally implanted*. In researching implantation, I understood minimally invasive Ponto surgery (MIPS) was minimally invasive compared to the old “long cut” or “pocket” methods: one hole punched in the skin, one hole drilled and filled with a tiny screw. I brought my research to the Children’s Hospital of Philadelphia (CHOP) ENT and Isla was his first MIPS surgery. He expressed that he loved the process and was very impressed with the how easily it went. In this video, (Isla was still waiting six months to “snap on” her processors because her skull bones are thin).

The Ponto 5 SPs are amazing!  My daughter has loved her devices from the beginning, and we always made them fun by having a bunch of different colored softbands to choose from each morning. She has absolutely blossomed in public and more crowded situations with the upgrade to P5 SPs. She’s more willing to get out there and play or join a group of kids she doesn’t know. Absolutely worth it!

* Ponto surgery is contraindicated for patients under 5 years of age. The subject here is shown wearing two ponto 5 superpowers on a softband but has undergone the surgery stated by the parent. Outcomes and healing times wary patient to patient. Please consult your caregiver.

Craniofacial Conditions and their Effects on Hearing

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Unlike regular hearing aid wearers whose hearing loss is usually the result of exposure to loud noises, ototoxic (literally “ear poisoning”) drugs, or presbycusis (age-related hearing loss) most bone anchored hearing aid wearers are missing parts of their ears. Some are born without the internal components, such as their cochlea or ear canal, or have parts that are damaged. Others only have small portions of their pinna (outer ear) or don’t have an ear at all.

We feel it is important to provide information and insight into these rare conditions and how they affect hearing. Raising awareness will hopefully help those affected feel less alone, encourage understanding and compassion from others, and broaden knowledge of bone anchored hearing as a possible alternative to the hearing loss associated with these conditions.

Microtia (with or without Atresia)

Microtia (literally “little ear”) is a condition that occurs during the first trimester of pregnancy, which is when development of the ears should occur. The pinna (outer ear) is either underdeveloped, only partially present (e.g., just the earlobe), or nonexistent. This condition might only affect one ear or both and is often—but not always—accompanied by another condition, atresia. Typically, atresia refers to having an extremely narrow or missing ear canal.

While microtia, with or without atresia, may occur without any associated or underlying syndrome, it often appears as one of the multiple indications of the following conditions.

Treacher Collins Syndrome

This genetic disorder causes underdevelopment or malformation of various parts of the head, including eyes, cheekbones, jaws, mouth, and palate. People with Treacher Collins often experience hearing loss when the inner and/or outer parts of their ears are affected by microtia and/or atresia. They might also have conductive hearing loss due to issues with their middle ears that limit or prevent sound waves from traveling to the brain.

Craniofacial Microsomia

This is often used as an umbrella term to cover multiple conditions, including hemifacial microsomia, which is characterized by underdevelopment of one side of the head and face affecting the jaw, mouth, and ears and Goldenhar syndrome, which often affects the eyes, ears, and spine. These and similar conditions may be inherited but often simply occur spontaneously without any family history of the disorders. Again microtia, with or without atresia, is common with these conditions, as are issues with damaged or missing middle ear components.

Apert Syndrome

Often caused by a random and spontaneous genetic mutation, this syndrome results when the “seams” between the bones of the skull close prematurely during fetal development. Those affected usually have a pointed or extended skull and malformations of the face, hands, and feet. The condition is often associated with conductive hearing loss in both ears due to fused ossicles (the tiny bones in the middle ear). Chronic ear infections are also often common. Other syndromes related to Apert syndrome involving hearing loss include Crouzon and Saethre-Chotzen.

Velocardiofacial Syndrome

This disorder results when a child is born missing part of Chromosome 22. It is also known by other names (e.g., DiGeorge syndrome, Catch-22 syndrome). Usually there is no family history of Velocardiofacial syndrome, although it can be inherited from either parent. Multiple symptoms may occur (although usually not all at once), including otitis media (chronic middle ear infections). Additionally, conductive hearing loss from a variety of abnormalities in the middle and/or inner ear have been reported as prevalent among people with this syndrome.

Treatment for conductive hearing losses due to syndromes

Hearing loss associated with a craniofacial condition is often conductive in nature, meaning there is a physical cause like a missing or nonfunctional portion of the ear. Some may be surgically reparable, as in a case where an ear canal is present, but the opening is sealed. Others would be best addressed by a bone anchored hearing system like Ponto™, which bypasses the damaged or missing ear entirely to conduct sound via the skull.

Ear Community goes to Washington with Ponto SuperPowers

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Advocacy Day 2019

On February 26, Ear Community was invited to be a part of the 2019 Advocacy Day on Capitol Hill.  This annual event invites advocates on behalf of dental, oral, and craniofacial research to speak directly with federal policymakers about how insurance coverage and research funding would improve the lives of those living with these conditions.

Organizer Melissa Tumblin founded Ear Community and has a daughter, Ally, with Microtia and Atresia who wears a bone-anchored hearing system (BAHS). Melissa was able to arrange the meetings with House and Senate representatives because she sits on the Patient Advocacy Council for the National Institute of Dental and Craniofacial Research (NIDCR).

Melissa and Ally were accompanied by eight other Ear Community families, including one medical doctor, to Washington, D.C. representing the states of Washington, Colorado, Tennessee, Maryland, and Virginia.

Lobbying on behalf of families living with hearing loss and related conditions

On Monday, February 25, Melissa met with directors from the National Institute of Health representing the NIDCR, American Association for Dental Research (AADR), and International Association for Dental Research (IADR) to discuss collaborative efforts regarding children and adults affected by craniofacial challenges due to Microtia and Atresia and Goldenhar Syndrome.

The next day, the Ear Community families met with the following representatives and staff members on Capitol Hill:

  • Staff members for Senator Lamar Alexander’s (R-TN) office
  • Legislative staff members for Congressman Joe Neguse’s (D-CO), Senator Cory Gardner’s (R-CO), and Michael Bennet’s (D-CO) offices.
  • Legislative staff members for Senator Chris Van Hollen (D-MD), and office staff for Congressman John Sarbanes’ (D-MD) and Senator Ben Cardin’s (D-MD).
  • Legislative staff and the press secretary for Congressman Denny Heck’s (D-WA) office and an Appropriations representative for the NIH staff for Senator Patty Murray’s (D-WA) office.
  • Senator Tim Kaine (D-VA), Congresswoman Abigail Spanberger (D-VA), and legislative staff members for Senator Mark Warner (D-VA) and Congressman Ben Cline (R-VA). Sen. Kaine was celebrating his birthday and signed a little girl with Microtia and Atresia named Mazie’s cast. Both Sen. Kaine and Rep. Spanberger tweeted about the Ear Community visit.

Sen. Mark Werner and Ear Community FamiliesRep. Spanberger with members of Ear Community

Melissa also scheduled phone meetings with Congressman David McKinley (R-WV) and Congressman Michael Thompson (D-CA), as they both serve as co-chairs of the Congressional Hearing Health Caucus. Rep. McKinley is a cochlear implant user, and his grandson wears a bone-anchored hearing aid.

Each family left behind a detailed packet of information with the story of the Ear Community organization, an explanation about the need to wear bone-anchored hearing systems, and a sample of anaudiogram along with other helpful facts and figures. Each family explained to legislators and their staff that a bone-anchored device is their only option, because they don’t have the same hearing challenges that qualify others to wear cochlear implants or benefit from traditional hearing aids. Therefore, wearers need lawmakers to work toward mandating bone-conduction hearing device coverage by insurers nationwide. Otherwise, insurance companies are effectively discriminating against thousands of children and adults with hearing loss by not covering these medical devices.

A Stranger Things star gives back

Gaten Matarazzo poses with Ear Community kids for Advocacy Day 2019.

The Ear Community families also received an exciting treat: thanks to the NIDCR and AADR, they were given the opportunity to meet actor Gaten Matarazzo from the hit TV show Stranger Things! He was part of the Advocacy Day non-profit organization lobbying to bring awareness to Cleidocranial dysplasia (CCD), a rare genetic mutation affecting the growth and development of teeth and bones.

Melissa expressed her appreciation to Oticon Medical for lending her two Ponto 3 SuperPower BAHS on soft bands. During her visits, representatives tried on the Pontos and listened to her talk during their meetings, so they could experience for themselves what it’s like to hear through bone-anchored devices.

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Ear Community hosts a series of picnics where Microtia and Atresia families can learn more about Oticon Medical’s Ponto 3 SuperPower. Please click through this link for the 2019 picnic schedule.

Ready to try your first Ponto BAHS or upgrade to our latest model? Click below to get in touch with an audiologist in your area who can help you choose the best option for your hearing needs.

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Becky Zingale shares how her daughter navigates Treacher Collins Syndrome and conductive hearing loss with her Ponto 3 through her blog

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You might be familiar with the blog “The BAHA Mama”, a well-known resource for parents and families who have children with hearing loss and Treacher Collins Syndrome (TCS). The blog, which was created by Becky Zingale, follows her family’s journey of navigating TCS, hearing loss, speech therapy, sign language, and parenting.

Becky began writing around 10 years ago to document her travel experiences. Later, when she and her husband learned that their second daughter, Ariasha, had TCS, Becky focused her blog on sharing knowledge and personal stories with other parents.

“My husband is a marketing consultant and has empowered me to be a thought leader. I’m a great success story for what happens when you follow the advice of your marketing consultant (husband),” she says.

Ariasha, also known as “Little Lioness” and “PipSqueak”, was born with Treacher Collins Syndrome and microtia and atresia, just like her father Duane. She underwent cleft palate surgery in 2016 when she was sixteen months old, which is when they discovered her conductive hearing loss. Thinking Ariasha would follow in her father’s footsteps and wear the same bone anchored hearing device as him, she trialed his device first but experienced issues with feedback.

Through online Facebook groups focused on hearing loss like this one and this one, Becky discovered the Oticon Medical Ponto Bone Anchored Hearing System. All the information they received convinced her that Ponto was the right choice for her daughter.

They trialed the Ponto and Ariasha now wears bilateral Ponto 3’s on a softband. During the first few weeks of her wearing her Ponto, Becky noticed just how much it was making a difference in her ability to communicate.

“She had her Ponto for two weeks and I tried dropping her off at childcare. I thought the noise might be overwhelming so I decided she shouldn’t wear the Ponto, and she was really upset. It turned out that she had become so used to being able to hear for those two weeks, that she preferred dealing with a loud environment than not knowing what was going on. She has also become more social since getting her Ponto. Beforehand, when people would come over, she wanted to go to bed. We thought she was really introverted. But with her Ponto, now she wants to interact with people since she can distinguish sound, hear different voices, and has an idea of what’s going around her.”

As Ariasha continues her hearing loss journey, Becky documents everything they learn and answers common questions from parents online through informative blog posts and personal stories. One such post discussed a common question posed by parents – how to show a child to place their BAHS (Bone Anchored Hearing System) in a safe place to avoid it getting lost or broken.

“Sometimes Ariasha decides to change her clothing randomly in the middle of the day and her Ponto will end up in a pile of clothes. So, we created “Ariasha’s BAHS Box”, a safe place to put her Ponto. The other day she went upstairs to play and then came downstairs wearing a new outfit. ‘Where is your BAHS?’ I asked. She responded, in a very sassy teenager-ish tone, ‘In my BAHS box’.”

Learning about Bone Anchored Hearing Systems and advocating for Ariasha has definitely become a family affair. Ariasha’s sister Chloe, also known as “Monkey” or “Big Sis” on the blog, helps to check whether her sister’s Ponto needs new batteries and event sports matching headbands with her.

To parents trying to figure things out, Becky’s advice is to find a community. She says the online community has been very helpful with discussing things like speech therapy and how to get your child to keep their BAHS on their head.

“Hope is what the name Asha means. It’s my hope to inspire and encourage mamas in their journey to raise healthy, thriving families in spite of whatever challenges life brings.”

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CardioThoracic nurse, Iris Leak, shares how her Ponto 3 SuperPower makes a difference at work and at home

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Working as a CardioThoracic nurse for 27 years, Iris had become accustomed to noisy environments. However, after the removal of a glomus tumor and cholesteatoma, she found herself with conductive hearing loss and challenged to perform her work duties. She began searching for a solution and found the Ponto 3 SuperPower.

The effects of her hearing loss
Iris had been relying on her “good” left ear for some time, but as her hearing loss became more severe it also became increasingly difficult to hear and understand her co-workers and family.

“People at the hospital would have to throw things my way to get my attention because I couldn’t hear them anymore. It was getting more and more difficult to hear in my work environment,” she says.

In social situations, she struggled to keep up with conversations and found her hearing loss isolating her from family and friends. “I’m a talker naturally and I love being around people. But with my hearing loss, I found myself becoming more isolated because I couldn’t hear the conversations and couldn’t participate. I wasn’t myself.”

Finding the right solution
Iris started doing her research. She found support and resources through online Facebook groups like this one and worked with an audiologist who helped her understand her options. She demoed different bone anchored hearing devices and ultimately chose the Ponto.

In November of 2017, she had the Minimally Invasive Ponto Surgery (MIPS) and received her Ponto 3 SuperPower in January of 2018. To ensure her abutment was placed in the best position to accommodate her reading glasses and CPAP (Continuous Positive Airway Pressure) mask she brought her glasses and mask to her surgeon to mark the abutment site. When she received her Ponto on the abutment for the first time she immediately put it to the test. “I went out to eat afterward, and I picked the noisiest place I knew—the mall. It was amazing! I love hearing and I feel like myself again.”

As Iris has grown more accustomed to her new world of hearing, she’s started using Ponto’s accessories like her Oticon Medical Streamer, which she describes as “game-changing”. “I hadn’t listened to music in years, but now it’s possible with my Ponto 3 SuperPower and Oticon Medical Streamer.”

Iris’s advice for those looking at a hearing loss solution is to remember nothing is perfect. Do your research to find the solution that is best for you.

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Click the button if you want to learn more about our Ponto bone anchored hearing systems or arrange a trial.

The Ponto 3 SuperPower ignites a love for music for Talia

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Three-year-old Talia Jarvis was born with unilateral microtia and atresia, and bilateral moderate-severe hearing loss. When the Jarvis family adopted Talia in March of 2017, they took her to an ENT right away to begin her hearing journey.

“I wasn’t familiar with all the different devices. Our audiologist said they were all pretty much the same. They didn’t provide the Ponto so we took home a different bone anchored hearing device to trial and it didn’t reach our expectations.” – Laurie Jarvis

Talia’s mother, Laurie, began doing her own research because of the severe feedback issues Talia was experiencing. She began speaking to other users online in Facebook groups like this one and this one that helped her find the Ponto 3 SuperPower and halt her current bone anchored hearing system order until Talia trialed the Ponto. “I told my doctor that if they didn’t let us trial the Ponto device we would be switching to someone else, ” she explains.

Since receiving her Ponto 3 SuperPower Talia’s world has expanded and is happy to wear her device all day. She is now learning language and her mother says she can hear the differences in sounds. 

“With the Ponto, she cried the first time I took it off at night. The next morning she pointed to it on the dresser and every day since then it’s the very first thing she asks for in the morning.

Before it was hard to get her to sit still to read a story. Now she will ask for book after book after book. She wants to learn new vocabulary and is constantly asking me what this and that is. That interest was not there before.” – Laurie Jarvis

One of the greatest differences her family has witnessed is seeing her discover a love for music. At home, Talia now enjoys listening to her sister play the piano, a sound that until recently was something she had not heard.

 

“My oldest daughter was playing the piano one morning and Talia was sitting in her highchair eating breakfast and she looked at me and she pointed to my daughter who was playing and she signed ‘piano; It was obvious that she was hearing something different.” – Laurie Jarvis

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Click the button if you want to learn more about our Ponto bone anchored hearing systems or arrange a trial.

Advice from 8 Ponto Parents

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Finding the right hearing solution for your child’s individual hearing loss can be difficult. While research is an important aspect of finding the right solution, we also believe there is power in stories and listening to what others have to say about their experience.  Below we’ve brought together advice from eight parents who chose Ponto for their children.

Research, research, research

“I just want to make other people aware that there are other options. Check other resources to make sure you are making an informed decision right away. I quickly learned that no one is going to give me all the information I need.” – Caroline Mannato

“Research those (Facebook and other groups) boards. Don’t allow yourself to be saddled with one device because your audiologist doesn’t want to try something different. Fight for your children’s rights. Make sure you know you have options.” – Jillian Shelton

Social media groups can not only provide community but valuable information

“Look to social media support groups on Facebook, like this one and this one, to help research options and talk to adults and who are wearing the devices you are interested in. – Liz Anderson

“Ultimately, what helped was knowing there are people just like her out there.” – Theresa McCorkell

“The stories that adults and older children were telling were the stories we needed to hear.” – Erin Bullock

Trial other options

“Insist on trialing all options. It’s definitely worth the time it takes.” – Laurie Jarvis

“Try all your options, on your child and yourself, to help you make the most informed decision for your family.” – Heidi Jeffs

“If you have an audiologist who is not open to trying a different company, push or go elsewhere – just do your research.” – Liz Anderson

“Advocate, because our children need us.” – Shannon Gordon

Interested in learning more about these Ponto families? Click here: User Stories

 

Learn how Heidi Jeffs chose the Ponto 3 for her daughter Rosalee and became an advocate in the hearing loss community

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We hear time and time again from parents of Ponto users that doing research into all the hearing options available is the best way to make the most informed decision. For Heidi Jeffs, research into different hearing groups is what led her to not only find the best hearing solution for her five-year-old daughter Rosalee but become an advocate in the hearing loss community.

Rosalee was born with microtia and atresia on her left side and fit with a bone anchored hearing device on a soft band given to her by her audiologist.

“We met with our audiologist and wanted her to have a device. We didn’t know any different or know of other options.”

Shortly after, Heidi learned about Ear Community, a non-profit organization that offers a supportive community for individuals born with microtia and atresia, and other varying degrees of hearing loss.

The community, which was started by Melissa Tumblin whose daughter Ally was born with microtia and atresia, provides support through their online Facebook group as well as annual picnics around the country.

Through her involvement with Ear Community, Heidi began meeting other parents and bone anchored hearing system (BAHS) users and found that feedback was a common issue.

“We were having a lot of feedback issues. Some moms were commiserating about it and then there was one mom whose daughter had bilateral Pontos and she was like ‘what are you guys talking about?’ The woman said her daughter could lay on a pillow with her Pontos on and it wouldn’t squeal. So I started looking into the Ponto devices.”

The Ear Community picnics were where Heidi became familiar with Oticon Medical and her daughter was able to try on a Ponto device for the first time.

“It didn’t sound echoey. It was just clear. I was flat out convinced. Then the more and more that I learned about the company I was even more impressed. It really felt like Oticon Medical stood by their motto ‘People First’.”

When it was time for Rosalee to upgrade, she tested the Ponto 3 on a softband and said yes to switching to the Ponto. Heidi notes that before, it was difficult to get Rosalee to wear her device, but since switching she hasn’t complained once about the softband being uncomfortable or sore, making the transition very easy and enjoyable.

“Between the quality of products and the integrity of the company, it is so far above the others out there.”

Since beginning this hearing care journey with her daughter, Heidi has served on the board of Ear Community for three years and is now a full-time staff member. Additionally, she served on the board for the Idaho Council for the Deaf and Hard of Hearing and was chosen as a parent representative for Idaho to attend the EHDI conference – an early hearing detection and intervention event. To all parents looking to learn about hearing options for themselves or their child with hearing loss, she recommends trying all your options, on your child and yourself, to help you make the best decision for your family.

 

Are you interested in learning about the Ponto 3 SuperPower and how it can provide early support for children and adults with hearing loss? Click below to be connected to a representative from our team.

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“We’re Just Trying To Do What’s Best for Him” – A Parent’s Perspective

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Davin is 8 years old and was born with bilateral microtia and atresia. In the video below, Davin and his mother, Shannon Gordon, talk about his hearing journey with his Ponto’s and their experience with the new Ponto 3 SuperPower.

“Davin’s hearing is one of the most important things in our life and we’re just trying to do what’s best for him.” – Shannon

Interested in learning more about the Ponto 3 SuperPower? Click the button below to be connected to a representative from our team.

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The Gift of Hearing Travels to Africa

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We’d like to introduce you to someone very special!

Meet Raheeg Abdelrahman, a four-year-old girl living in Sudan with her younger twin siblings, her mother, and her father, Eltahir.

Raheeg was born with bilateral Microtia and Atresia, and when her family couldn’t find a suitable hearing device in Sudan, they traveled to Egypt to meet with an audiologist who they hoped could help them find a solution. Unfortunately, the family was unable to find an affordable solution and were forced to return home without a device for Raheeg.

That’s when we heard about Raheeg and her family through our friends at the Ear Community and partnered with them to provide two Oticon Medical Ponto Plus hearing devices for her.

The devices were sent to Egypt where Raheeg was fitted with her devices with great results:

 “She can answer our call easily and surely this will help her to receive her lessons like other kids…it is difficult to get words to express my thanks but I say God bless you!

Please convey my deepest heartfelt thanks and appreciation to all of Oticon Medical staff for the help and support they provide to everybody in our Ear Community, and special thanks for Mr. Alan Raffauf, Tove Rosenbom, and Claus Frees Horneman…THANKS to YOU dear friends for giving me hope that my daughter can go to school, hearing sounds and practice her life like other kids.  Thank you for making my impossible dream become real!”

– Eltahir Abdelrahman

We’re honored to have had the opportunity to help provide the gift of hearing to another individual with hearing loss and are thankful for our friends at the Ear Community for sharing Raheeg’s story with us!

If you’d like to learn more about Raheeg and Microtia and Atresia, the Ear Community blog is a great resource!