Tag Archives: Cochlear implant

Retinal Implant for Blindness

Short Friday post today. A few months ago I blogged about the future of heading correction, pointing out the similarities between hearing loss and vision loss. In that blog I had mentioned that hearing correction was in some ways ahead of vision correction, but its only a matter of time before vision catches up.

Well wouldn’t you know it today I came across new research on retinal implants, which look in many ways very similar to Cochlear Implants. Below is a good illustration that shows how it works.

retinal_implant

My family has a broad history of vision problems, so when Fiona was diagnosed with profound hearing loss it was a total shock. You never want any harm to come to your child, but if there was ever to be a problem I would have bet on vision problems instead.

While the retina implant still has a long way to go, its fantastic to see research is making progress on this front.

Booth Testing

Tonight the Isham clan braved 2 fantastic hours of rush hour traffic to take Fiona to her first booth testing session. Booth testing is one of the final stages in our journey to prepare Fiona for the Cochlear Implant procedure, and is typically not started until the baby is at least 6 months old. Today Fiona is 6 months and 2 weeks old.

The process is pretty interesting. First you have a soundproof room equipped with speakers and TV flat panels located at strategic locations. Here’s the booth from the outside:

Come on in!

Come on in!

Inside the booth there are speakers immediately to the left and right of the subject, and TV screens located in close proximity to the speakers.

Inside

Inside the booth

During the test, Eliza sat in the booth with Fiona on her lap, facing the front of the room with the speakers and TVs directly to her left and her right. Our audiologist Lauren sat directly in front of Eliza with various hand puppets to distract Fiona and keep her attention focused forward.

On the outside, Dad stood in the dark with Lauren’s assistant Jenny, looking in through the observation window as the test was underway. The goal of course is to keep hidden from Fiona so she does not get distracted by her dad’s incredibly handsome looks.

Somebody's Watching You...

Somebody’s Watching You…

Jenny then proceeded to introduce sounds at different locations in the room, looking for a repeatable response from Fiona. Similar in some ways to the ABR test, she would vary the frequency (high pitch to low pitch), volume (75 dB to 95 dB) and location (left or right). (Check out my earlier post on Frequencies of Familiar Sounds for more information on the sound spectrum).

What makes this fun for children are the TV screens. Whenever the child positively responds to the sound (by looking in the direction of the sound), a short flash of a movie displays on the TV nearest to the sound. In this case, it was good ‘ol Nemo.

Here was the display in the control room (to keep dad entertained during the interludes)

Everyone Loves Nemo

Everyone Loves Nemo

This process is called Visual Reinforcement Audiometry. Here’s a short (1 min 30 second) youtube video that shows this process really well.

To score a positive result, Fiona had to respond conclusively twice to the same frequency/volume/location. While she did have a couple “single responses” at high volume and middle frequencies, she did not have a repeatable result. This was as expected given her prior testing – we did not expect a miraculous change. Still, this level of validation is an important milestone to confirm her candidacy for the CI procedure.

Fiona will be booth tested again at least once or twice more before her surgery on March 7th, but this first test was definitely interesting to watch.

Baptisms, Seminars, and Gadgets Galore

Long day for miss Fiona today. We started off witnessing the baptism of Andrew Cassidy, the son of our good friends Samantha and Brandon (congrats guys!). Andrew was born just weeks before Fiona, so its fun to watch the two of them grow up together.

Fiona had a ball at church and thoroughly enjoyed chewing on the various handouts in our pew. (We kept her away from the bibles, i’m sure there’s a place in hell for those babies ;->).

Hey I know that guy!

Hey I know that guy!

Afterwards we stopped by Chez Cassidy to grab a great lunch and a few most excellent beers. Fiona got into the spirit of things, practicing with an empty cup….

I'm a big girl now!

I’m a big girl now!

Or was it???

From there we drove on to a seminar in Norcross hosted by Advanced Bionics, one of the “big 3” cochlear implant manufactures I’ve talked about in earlier posts. Eliza and I have all but decided to go forward with Advanced Bionics as the implant manufacturer for Fiona’s surgery on March 7th, so today was a fact finding mission to learn more about their latest processor, the Naida CI Q70 and what options we should select.

AB Seminar

AB Seminar…with Free Chocolate Bars!

And oh my the options! Different colors, different batteries, remote controls, cables and even Bluetooth! It felt like car shopping – should we get the rust coating?

Naida Processor

Naida Processors and Accessories

While we’ve seen the new model before, this was our first “hands on” experience. It was great to play around with the implants – feeling the weight, swapping out the batteries, and plugging in the various cables. It helped a lot in our decision making process.

The biggest value of these seminars, though, is talking with other parents who’ve been through before. It’s always uplifting and educational to hear their stories first hand.

Some tidbits I picked up:

  • Fiona is very lucky to get her surgery at 10 months old. In the past, insurance companies would not authorize until 2 years or even older. Without fail, every parent we’ve talked to has confirmed the earlier the better for her speech development. Kids who have received implants at a young age “mainstream” into classes with hearing children much quicker.
  • Several kids at the seminar had graduated from the Atlanta Auditory Verbal Center, the same center we use for Fiona. Nothing but positive mojo about the center and the results on the children. One adorable little girl said she graduated so young she barely remembered even going to therapy. (Her speech was excellent by the way). Remarkable.
  • Interesting tip on CI colors: one parent recommended we NOT get skin or hair color, but rather something bright and noticeable – at least for infants. Why? Because you will constantly lose the implant around the house, outside, etc. and its much harder to locate the skin colored implants. Interesting perspective – we’re still making up our mind on that one.
  • One parent highly recommended getting the product replacement plan with the implant. I almost never get those (can you say “profit center” ?), but he brought up a good point – you don’t want your child to live “in bubble wrap”. If you’re constantly stressing about losing or breaking the implant, you’re going to stunt your child’s exploration of the world. You want them to explore, roll in the dirt and be a kid. I couldn’t agree more.

Anyway, long day for miss Fiona today, but she did great. She just went down to bed, an hour late courtesy of daylight savings. Sleep well!

Now time to watch some Zombies on Walking Dead tonight. Brainnnnssss!

Don't wake the baby!

Don’t wake the baby!

New Algorithm Helps Cochlear Implants Detect Music

Upgrading your hearing in 3...2...1

Upgrading your hearing in 3…2…1

Back in August I posted about one of the major current drawbacks to Cochlear Implants: the inability to effectively pick up music (see Cochlear Implants and Music).

Tonight I read an interesting blog about a new algorithm devised by researchers at the University of Washington in Seattle that “vastly improves the sound quality of existing implants to the point where music sounds like something other than a random clamor”. You can see the full post here.

This is both an encouraging and scary statement. Scary in that music perception is still SO bad, but encouraging in that this problem can be attacked through software upgrades. Pretty crazy if you think about it. Imagine receiving an email asking you to “download the latest upgrade of your implant software to…hear guitar music!”

I have no doubt something like this will be exactly what happens in the future. With the CI implant surgery being so invasive, requiring infant anesthesia and an overnight hospital stay…TWICE (once for each ear), minimizing the need for future surgery is critical.

This emphasizes the value in getting a processor that has room for future software upgrades. While I’m sure Fiona will no doubt need a physical upgrade again later in her life, the advantages of upgrading must be very compelling to go through the surgery process again. Fortunately the processor companies also realize this and have placed the bulk of the processing hardware outside of the implant area itself, allowing for many upgrades without new surgery.

Still, it is inevitable internal upgrades will likely be needed again down the road as the nerve stimulation apparatus becomes more sophisticated. My hope is Fiona’s second round of implants will happen when we’ve finally achieved the “completely internal” implant.

Still a long way off, but I’m sure it will happen in her lifetime.

We Have a Surgery Date!

Cochlear Implant Illustrated

Cochlear Implant Illustrated

After weeks of relative quiet on the hearing front, today we had another big day!

Today, Eliza, Fiona and myself visited Fiona’s Cochlear Implant surgeon for the first time. Armed with a well researched and thoroughly documented list of 10+ questions (printed in hard copy mind you), we were ready to rock this appointment. The doctor didn’t leave disappointed .

Doctor Brian Hermann is one of the most experienced CI surgeons in the country, and (word of mouth has it) the top CI doctor in Atlanta. He’s been performing CI procedures over 10 years, and has “high hundreds” of CI procedures under his belt. His demeanor was very professional, patient and accommodating so we were well at ease with the discussion. We learned quite a bit, but more importantly we were able to talk through Fiona’s upcoming timetable.

Dr. Hermann has done procedures on infants as young as 10 months, sometimes younger if medical conditions warrant. With infants, the younger you get them implanted, the better the chance they have of normalized speech development – but if you go too young, the bone density of their skull is not hard enough to safely support the procedure.

FDA approval in the US is currently at 12 months, but many doctors have been safely performing the surgery in the US and abroad as young as 9 months old for many years now. Dr. Hermann sees 10 months as his sweet spot.

Eliza and I have long been in favor of “earlier is better”, provided its medically safe to do so. All the research and discussions we’ve had indicate 10-12 months is desirable. Given this discussion with Dr. Hermann, we were able to go ahead and book a surgery date. Fiona will be 10 months old on March 1, 2014, so we chose the first opening after that…

Drumroll….

Friday, March 7, 2014!

For safety reasons (disruptive to inner ear balance, infant under anesthesia a long time, etc.), Dr. Hermann strongly recommends only implanting one ear per surgery, so we’ll be doing the first ear on March 7th, and the second ear about 2 months later in the May 2014 time frame.

About 1 month after the first surgery (April 2014 timeframe), her implant will be “activated” and she will hear her first moments of sound at that point. You bet your ass I will be filming and blogging that moment. (see my old post “waking up to sound” for a taste of that experience).

Of courese we are told not to expect miracles out of the gate – advances will start out slow and ramp up as they increase signal over the months to come. Still, its very exciting to think about the possibilities!

Some other “lessons learned” from our chat with the doctor today:

  • We will need to select a Cochlear Implant model by early February. I’ve blogged about this topic quite a bit, but its still a big decision. Apparently the latest “Cochlear” model (see my last blog) has had some problems so they are not implanting that model. We are leaning towards Advanced Bionics because it has a waterproof version, but haven’t 100% made up our mind yet. More research to come.
  • The surgery lasts about 2 hours, but Fiona will need to stay in the hospital overnight. For the next month, risk of ear infection is very high so we need to watch that closely. After the skin heals properly, the activation event will occur. This will happen before her second implant procedure is done. (most likely in April 2014 timeframe).
  • The new Advanced Bionics model also has a version of the implant that is less destructive to the existing auditor nerve, meaning the procedure does not knock out as much of the existing hearing. Since Fiona doesn’t have much existing hearing this is likely a non-concern, but its still good to know in case our understanding of hearing changes down the road, which we all know is very possible. I may do a more detailed blog about that down the road.

All in all an exciting day and we’re looking forward to the future. And since I’m overdue for a recent baby picture, here’s Fiona today (at 5 1/2 months) waiting in the lobby…

Fiona waits for the doctor...

Fiona waits for the doctor…

“Cochlear” Releases new CI Sound Processor

Nucleas 6

Nucleus 6

The wave of innovation continues, Yay capitalism!

If you’ve been following my posts for some time now, you’ll recall I’ve referred to the Big 3 Cochlear Implant companies: Advanced Bionics, Cochlear and Med-El. Back in August I posted about the new release by Advanced Bionics of the Naida CI Q70 implant.

Looks like now it’s Cochlear’s turn with the release of their new “Nucleus 6” sound processor. Here’s the press release.

As with Advanced Bionics, the trend continues towards smaller implants, better compatibility with wireless devices, and more upgrade room for the future. The last part is especially important since you don’t want to have a new surgery every time you upgrade.

This press release has a lot of “forward looking” statements which makes me wonder if its “not quite ready” yet, but i’m sure time will tell.

Cochlear will continue its history of breakthroughs in innovation with future upgrades to the Nucleus 6 Sound Processor. The new chip is designed for the next generation SmartSound processing, which leverages new algorithms that focus on speech and reduce background noise, as well as third generation wireless accessories that do not require any neck worn loops.

We’ll definitely have a tough decision next March. Competition is good.

How Hearing Aids Actually Sound (Video)

This is a clever video Eliza found recently, prepared by the Vermont Center for the Deaf and Hard of Hearing. In this video, they recorded the actual sound received by a hearing aid under 4 different scenarios:

  1. Close by with no background noise (the “ideal”)
  2. Far away with no background noise
  3. Close by with background noise
  4. Far away with background noise

This is a great way to “walk in the shoes” of the listener. Scenario 4 is very common in the classroom setting, and very challenging to cope with. I have no doubt it’s exhausting!

After the exposition, they then repeated the same 4 scenarios using an FM transmitter, showing significant improvements. Good stuff.

Here’s the video, keep your eye on the student with the red hat – an Oscar winning performance…

While this video demonstrates hearing aids, I do know many of the Cochlear Implants out there now offer similar integration with transmitter systems. I’m expecting (perhaps wrongly?) a similar experience for CI users.

Regardless of the technology, clearly the teacher and school system have to be on board with this for it to succeed, so we’ll definitely need to weigh our options on schools when Fiona gets to the appropriate age. Fortunately we still have a few years there.

If anyone reading this has firsthand experience on this topic they’d like to share, we’d love to learn more. Especially with school systems in the Atlanta area.

Advanced Bionics Firefighting Fun

Chicks Dig Firetrucks

Chicks Dig Firetrucks

Early this morning we packed into the car for a 30 minute drive south to Clayton Country Fire Station 4, just south of Hartsfield Jackson airport. What a beautiful day for a drive: 65 degrees, zero humidity and not a cloud in the sky. Good weather always feels better on a Saturday.

Advanced Bionics was hosting an event to meet Kelli Duncan, a Cochlear Implant recipient who works at the fire station and graduated at the top of her class. It was also a great opportunity to meet other parents with CI children and to see the new Naida Cochlear Implant.

Kelli was truly inspiring, a very capable and respected member of the force. Her parents and sister were also in attendance, and you could see nothing but pride in their eyes. Truly inspiring and you couldn’t help but get caught up in the emotion.

Kelli Duncan

Kelli Duncan

And of course fire stations are like candy crack to kids. They couldn’t enough of it. Fire trucks, coloring books, fire hats, hot dogs, sunny weather, more fire trucks. Kid heaven.

Fiona was by far the youngest there, so much of this was lost on her. Still, she was a crowd favorite and we did our part to embarrass her properly.

Firetrucks...Yawn

Oh a fire station…yawn…

Group Photo

Who Can Resist a Picture with a Fireman?

Of course the little boy in me couldn’t resist some of the fun. In this short video one of the fire fighters demonstrated how fast they get dressed when the alarm sounds. (look for Kelli as the firefighter on the right side in the foreground).

Eliza and I really enjoyed talking with the other parents and children with Cochlear Implants. Many of the children already had an implant and were doing fantastic in such a noisy environment. This was by far the largest concentration of implant wearers we had ever seen in one place.

The tone around the room was optimism and excitement. CI’s are really a game changing invention, and you could hear nothing but pride and excitement about the possibilities this device has made for everyone in the room. We made several new friends, and shared contact info with many that we hope to keep in touch with.

Of course, being part owner of a small technology startup, the business man in me also had to marvel at the flawless marketing prowess of Advanced Bionics in hosting this event. Provide real value to the parents, make it a fun time for the children, give out cool freebies with the AB logo, and showcase off your new technology to anyone interested. Well played Advanced Bionics!

Of course I ate it all up, and was glad to have a chance to check out the new processor. There was definite excitement in the room for the new technology: the new processor is smaller then anything on the market, and more powerful (more clarity, more features, better quality, etc.). AB has invested a lot of R&D integrating the new CI to the digital world: BlueTooth, FM receivers, seamless integration with cell phones, you name it. This is a completely different level of excitement then waiting for the next iPhone or xBox version, these advances are real game changers to the hearing loss community. Great stuff.

All in all it was a fun event. We learned something, met new friends, and enjoyed a few laughs. Not a bad way to spend a beautiful Saturday morning.

Now if only Fiona would wake up…

Yawn...What Fire Station?

Yawn…What Fire Station?

The Genetic Tests Are In!

Close View of a DNA Strand

It’s hard to believe, but it’s been over 3 months since we first learned of Fiona’s deafness. We’ve come so far.

When this journey first began, we had 2 big fears: would Fiona be eligible for the Cochlear Implant and would this prevent us from having a second child?

A month later, our first fear was laid to rest: Fiona was pronounced eligible for the Cochlear Implant. I blogged about this on July 16th.

Yesterday we received the answer to our second fear: Fiona’s deafness does not appear to be genetic.

This is great news as it lays to rest our concerns about having a second child. It’s also good news for adult Fiona as she likely will not pass on hearing loss to her children (if she so chooses to have any).

We couldn’t be happier!

Yippee!

Yippee!

How Did They Test?

About two months ago, Fiona’s doctor drew blood to conduct the genetic testing. Apparently there are 3 known genetic mutations that are common to infant profound hearing loss.

Before I go further, i’ll put out the disclaimer that this information is very technical and difficult to understand. To make matters worse, the websites I found were very heavy in “Doctor-talk” so was hard for me to follow. I did my best to understand the high level concepts, but it’s very possible I missed some key details.

Connexin 26 and Connexin 30

Connexin 26 and 30 are 2 proteins used by the inner ear to communicate from cell to cell. A defect in the “GJB2” gene (good ‘ol GJB2!) can cause these 2 proteins to disrupt communication across the cells in the ear, leading to the inability of the ear system to function properly. Here is one of the better articles I found on the topic from the California Ear Institute.

For two hearing parents to have a deaf child with the Connexin mutation, both parents must have the Connexin mutation. This means the mutation is passed recessively to the child (since neither of the original parents were deaf). Fiona’s testing did not indicate this mutation, clearing her and her children of this possible cause.

Mitochondrial DNA Defect

Mitochondrial DNA defects are the other common genetic cause of hearing loss. Mitochondria are cellular structures that help “power” the activity of cells in your body. A specific genetic defect in the Mitochondria can lead to premature death of some of the cells specifically used for the process of hearing. For a medical layman this is somewhat hard to follow, but here’s a good article I found here: Yale Study.

And here’s a good but technical 4 minute video on the topic:

The mitochondrial DNA defect is only passed on maternally, meaning it can only come from the mother. Fiona did not have this defect either.

So What’s Next?

It should be stressed that medical research is still evolving quite a bit in this area, so its very possible there are other genetic factors at work here of which we’re simply not aware. We can’t know for sure, but as of today all known genetic factors appear to be clear.

At this point it’s unlikely we’ll ever know the root cause of her hearing loss, but we’ve been told that’s the case more then 50% of the cases. Perhaps medicine will shed some new light on this in the years to come, but at least for now we know the odds are low in a recurrence in future children.

Drinks all around!