Episode Transcript
Krista: Hello everyone. I’m your guest host, Krista Yuskow, and we’re joined today by educational audiologist, Amber Dara. Welcome Amber. Thanks for joining us.
Amber: Thanks for having me.
Krista: You and I have worked together for a while now. How many years has it been, actually?
Amber: I believe it’s been six years where we’ve been direct colleagues, and then pretty much my entire career otherwise, indirectly.
Krista: That’s a long time. In that time, obviously, I’ve gotten to know you a little bit, about your four boys, your U-Pick Farm, your goats, and a little bit about your cochlear implant journey. But before we dive into that, I would love it if you could just introduce yourself and tell us a little bit about your background.
Amber: Sure. As you said, my name is Amber Dara and I am currently working as an educational audiologist. I have been an audiologist since 2004. I worked in a hospital setting, private clinic, and now in schools over the course of my career. I am married with four boys.
When I was pregnant with my second child, I learned that I have Usher 2A syndrome. Until then, the etiology of my hearing loss was unknown and it was thought to be congenital, but I wasn’t diagnosed with hearing aids until I was four years old.
Krista: You were diagnosed at age four, and fit pretty soon after that. Then you did not know you had Usher until you had your first child.
Amber: Second child, yes.
Krista: Second child, okay. Can you tell me a little bit about your journey in coming to your Usher diagnosis?
Amber: As an audiologist, I went to a conference and attended a talk on genetic hearing losses. I was working in private practice at the time, and had often written in reports how families could pursue genetic testing to further investigate the cause of their child’s hearing loss. But I realized after that, that I had no idea what happened. I would just tell them where to go and then I didn’t know the process that they were going through.
I reached out to the local geneticist’s office involved in that testing, and I was connected to her genetic counselor. She agreed to meet with me, and while discussing the process of what families would go through when referred to their clinic, she indicated that families would fill out a questionnaire, and then based on the answers, the child would be referred for either syndromic or non-syndromic testing.
This was an attempt to be more efficient and narrow down the testing so they weren’t just testing for everything. I asked what sorts of questions would suggest a syndromic hearing loss. One example she gave was difficulty seeing in the dark. I said, hey, I have trouble seeing in the dark. So she suggested that I talk to my family physician about a referral to the clinic for myself.
I was referred, underwent testing, and then was diagnosed with Usher 2A syndrome. I was then referred to an ophthalmology specialist to monitor my vision. Usher syndrome can affect hearing, vision, and balance.
My balance is unaffected with Usher type 2. My hearing loss was most likely congenital from birth, but it was initially in the mild to moderate range when I was first diagnosed. I believe my vision loss started in my 20s, where I started noticing a little bit more difficulty seeing in the dark, and that’s when I remember starting to notice that.
I’m lucky, and to date my day vision remains relatively unaffected. I do have early onset cataracts that are thought to be because of my Usher syndrome, but they are being monitored at this point.
Krista: I didn’t know that part about how you got to the genetic piece. That’s really interesting. Do you have any thoughts in general regarding Usher syndrome?
Amber: Yes. I guess when I think about my Usher syndrome diagnosis, initially I remember when I was younger that my mom would always think maybe she had done something wrong when she was pregnant with me and that maybe caused my hearing loss. She passed away five years before I was diagnosed, but I wish she could have known that it was genetic. It wasn’t her fault. She didn’t do anything during pregnancy that caused it.
At the time that I was diagnosed with my hearing loss, my parents were told to expect me to get about a grade six education. I remember my mom making a huge deal out of my grade six report card because I had honors to my audiologist at the time, and now I understand being a parent what a milestone that must have been for her, that we passed together, that like here I am we made it past grade six and we’re going to keep going.
Now that I know that I have Usher syndrome, when I was first diagnosed I really struggled with the word syndrome. I still haven’t really figured out why, and perhaps it meant because that meant there are multiple things that are “wrong” with me.
I think I’ve come to terms with that now that I have this syndrome, it just means there are multiple parts to me, and I’ve come around on that. I know shortly before she died, my mom had said that she felt like my hearing loss made me feel like I always had to prove myself, that I could do anything that anyone else could.
I don’t know if I would want to change that part about myself. I don’t know even if I could change all this, if I would. It depends on the day, how I feel about that (I think) if were to ask me. I know I am worried or a little bit nervous. We’re scared, really, about what happens when the vision loss starts to occur and the loss of independence that could come with that.
When I was first diagnosed, I did cry and grieve for a little bit about that diagnosis. But for now, I’m just sort of, when I cross that bridge I’ll deal with that. One other thing is that often I think about that if I was born now, my family likely would have found out about the hearing loss and Usher syndrome right away, because what I was diagnosed with, even if they went for genetic testing at the time, they wouldn’t have identified that gene yet.
I’m actually grateful that no one knew until I was an adult because many people with Usher syndrome do lose their daytime vision much younger, but I’m still doing well, even in my mid forties. So I wonder If I had been told younger about the vision loss part would I have not pursued the same interests or career or things like that because I would have thought at some point I’m going to be losing my vision, maybe I can’t do that, and I’m glad that I didn’t have that thought of not being able to do anything.
Krista: That makes sense. Interesting about the grade six piece, because not only did you make it through grade six, but you have your audiology doctorate, and you’re one of the most brilliant people I know. That piece about just that determination, you are also one of the most self determined people I know. It is just such a pleasure working with you.
I just think you’re awesome, Amber. You’ve gone through so much, and it’s made you who you are.
Amber: Thank you so much. I really enjoy working with you, and I feel the same way about you.
Krista: You’re just such a strong person, and, oh my gosh, your boys. She’s four boys, guys. She’s just an amazing mom and an amazing audiologist. I learned from you like every day you make me a better audiologist. So thank you for that.
Obviously, you were diagnosed initially with mild-moderate loss, and it’s obviously progressed to the point that last year you became a candidate for a cochlear implant. What were some of the deciding factors in pursuing the CI?
Amber: I was initially diagnosed with hearing loss in the mild to moderate range, but slowly over the years it gradually declined. I am lucky that when I was a child, I was seen every year for a hearing test, and was able to monitor that. Year to year, the results showed that there was no significant change. But if you were to look over a few years span, then you could see that there were changes happening.
I know when I first got my hearing aids, my mom said that I loved them. I put them in right from day one. I never resisted wearing them. From day one, they said that I had to put them in, and I did every day.
I know she said that there was a moment where I said like, stop, I hear the birds and then she cried. She told me that I always found it hard to believe, but she swore up and down. It was true that my hearing loss was initially diagnosed because I was playing in a water trough with my siblings. My parents yelled to us to stop. My siblings did stop and I didn’t, and I was apparently usually quite obedient. So they knew then that something was wrong because I didn’t stop.
Initially, my doctor told my parents that I probably wasn’t talking as much because I had an older sister, but my parents trusted their gut and pushed for a hearing test anyway. Then I was diagnosed.
In my 20s and 30s, I started to experience more significant decline in my hearing. I had been seen for a cochlear implant assessment a couple of times prior to when I was actually approved, but was determined not to be a candidate at that time. At that time, the assessment criteria looked at what your better ear was hearing like. So my better ear was hearing too well to be a candidate in my poor ear.
The time that I was actually approved—it’s kind of embarrassing—what happened was that I get frequent migraine headaches, and I had been laying down in my bedroom, just trying to have it quiet. I thought I need to spend some time with the kids. I was lying on the couch in the living room with a magic bag on my head while my kids were playing. I had my hearing aids out, though, because of the migraine. I just stuck them on my stomach. Then after a while, the magic bag wasn’t hot anymore. I’d also laid it on my stomach, forgetting about the hearing aids.
I asked my then six year old if he could take the magic bag and warm it back up for me for two minutes in the microwave. I passed it to him, he ran it to the microwave, started it, came back to play, and I asked him to go grab it for me. He started screaming that the magic bag was on fire.
I jumped up, went to the microwave, and sure enough there were flames. I quickly threw the magic bag in cold water in the sink. Then I went back to grab my hearing aids, and then that’s when I realized I only had one hearing aid. I ran back to the kitchen and sure enough, the magic bag hadn’t started on fire, but my hearing aid had.
I had to check some of my audiology colleagues that have put my hearing aids and say, I fried my hearing aid in the microwave. It was so embarrassing because that’s not something an audiologist is supposed to do.
As part of getting new hearing aids to replace the fried one, I needed an updated hearing test. That test showed I really wasn’t hearing very much out of my poor ear, especially for speech understanding, and that my better ear had come down a little bit as well. They re-referred me to be assessed for a cochlear implant, and that time I was approved.
Krista: I just love that story, and a couple of things come to mind when I hear you tell those two bits. Just the importance of serial audiograms. You said initially, that difference wasn’t noticed from year-to-year but when you compared over the course of a few years how the audiologist was noticing a drop, so just how important those regular audiologic assessments are.
So many parents worry about—and I know kids worry about—what if my hearing aids get wet or broken, or things happen to them? Even as an audiologist, you can say, yup, I put them in the microwave.
Amber: My cochlear implant journey probably would have started a lot later. Maybe it happened for a reason.
Krista: Right. I love that. That story always makes me smile. With your CI, we’ve been working together. We were working together when you became a candidate, and I was there when you went, not at your bedside, but we were in the same office as you went through the process. What in that process was probably the most surprising or the most unexpected?
Amber: There’s been, to be honest, a few things that have surprised me. Just first as a disclaimer, even though I’ve worked in lots of areas in my career, I’ve never actually worked with cochlear implant assessments or activations or adjustments. I’ve worked with people who have cochlear implants in other capacities, primarily using a remote microphone in conjunction with their cochlear implant.
For me, the surgery part was luckily very smooth. I didn’t have any nausea or vertigo. My biggest challenge at the beginning was sleeping at night because I could only lay one way and it wasn’t the way I usually sleep. I would wake up with pain in my neck, and then I’d have a hard time falling back asleep.
I went back to work around seven days, but I feel like around 10 days was when I was starting to feel like I could sleep comfortably again. That’s when I was working probably those three days before I was quite exhausted.
I know when I first got activated, I was really surprised that there was a lag between my cochlear implant ear and my hearing aid ear. The cochlear implant, everything would just come in a little bit slower than the hearing aid ear, so it almost sounded like an echo. I was pretty nervous about that because I thought this isn’t going to sound great long-term if it stays like that.
I can’t remember exactly how long it was until that change, but I think it was longer than six months. I really had to be patient with that to get that where it sounded like one signal going in and it does now. It just took a little while for that to happen.
Another thing that surprised me was that I knew with my cochlear implant that I would have a hard time probably finding helmets that would be comfortable for biking, just because I have extra stuff on the outside of my head.
I didn’t anticipate with where the internal part is behind my ear that I actually had a hard time finding sunglasses that were comfortable because it would be painful for me behind my ear once I wore them for a little while. I wear contact lenses almost exclusively. Probably with normal glasses, I would have the same experience that I would need to wear the glasses for a couple of hours before I would know if they’re comfortable.
I know with my cataracts, it’s very important that I need to wear my sunglasses very consistently to help reduce the likelihood of them growing. It took a lot of experimenting for me to find sunglasses that would fit comfortably. That’s not something that I anticipated was going to be something I would need to do.
One other thing was that after my surgery, the cartilage on my ear was really floppy compared to before surgery or compared to my other ear. I’ve never had problems keeping my hearing aids in my ear. They would stay on no problem. But when I first had my cochlear implant, the processor would just always falling off and then it’d be dangling by the magnet on the back of my ear.
I have no affiliation with, but I ended up getting a cool product that is made by a company called Deafmetal. It’s got a retention ring and a holster. Everybody just thinks it looks like an earring, but it actually keeps it so my processor never falls off at all.
That was another learning curve for me that I didn’t realize that that would [...] I just thought my ear cartilage was my ear cartilage. I didn’t realize that part would change.
Krista: Is there anything that the audiologist or the surgeon (I suppose) told you could happen? I’ve actually never heard of that occurring.
Amber: No. I think it’s not so much at the cartilage change, but just when they did the surgery behind the ear, it just changed the angle of my ear. Probably not so much that the cartilage itself changed, but just the angle of the way everything’s sitting behind there. It just made it so it doesn’t just tuck in as easily. It just hangs there. Then because it’s just hanging there, I’d be at work and it would just fall off multiple times.
I would be talking to people, and I wouldn’t realize till I got to the car, then it’d be hanging in my hair. It was still on, I was still hearing, and then I’d have to pop it back in my ear. Sometimes I did notice, but it was just something I hadn’t anticipated.
Krista: It’s interesting about the Deafmetal retention piece. I was familiar that you had that, but I didn’t know it was for retention. I thought it was a cool cochlear implant jewelry, how kids bling up their hearing aids and we encourage students to go big and proud. So I just thought it was that piece. I didn’t actually realize that it had a function. Very cool.
Amber: Yes, it has a function and it’s pretty at the same time. The last thing I would say—going back to the initial question about what surprised me—was just that when I had my surgery, sometimes you will still have some remaining hearing lock, but for me, I ended up losing all my residual hearing. When I don’t have my cochlear implant on, I’m not hearing anything out of my left ear.
I was activated in February 2022, so I’ve had it 1½ years now. Even though it’s been 1½ years, I still struggle with the sensation when I first put my cochlear implant on. There’s something about just going from completely deaf in that ear to hearing in such a short time frame is completely overwhelming every single time I have to put on my cochlear implant.
Usually three, four, or five minutes, it feels totally normal and doesn’t bother me at all. But I actually still dread that sensation when I first put it on every day, even though it’s been a year-and-a-half and I wear it every day.
It isn’t painful. It’s a hard sensation to describe. The closest analogy I can think of is when you’re driving home, when you’re playing your radio, and you make it louder. You keep turning it up, then you turn the car off. The next day you go to get in the car, you’re getting blasted by the music, and you’re like, whoa, how did I listen to that? You have to quickly turn it down.
It’s the same sensation except that you can’t turn it down because that’s what you need. You just persevere with it. Then after a few minutes, it sounds normal and like a good volume again.
I think in my ideal world, I wish the cochlear implant over five minutes gradually turned on from a very soft level to the level that I need it at. But I get the reason it doesn’t because probably there are some situations you need to put it on and hear immediately. That would be my wish and something that I didn’t anticipate happening, but then also makes me a little more sympathetic when somebody is having a hard time getting used to their cochlear implant because that initial hearing does feel loud and overwhelming.
Krista: ACI manufacturers, if you’re listening out there, that would be an option. Amber would be very grateful.
Amber: Yeah.
Krista: I know you’ve spoken to other people and audiologists with cochlear implants. Have they also mentioned that, or do you feel that’s unique to your experience?
Amber: I haven’t really asked anybody else. Right now most of the people that I have talked to a cochlear implant for kids, but I haven’t really asked that. That’s a good question I should ask if that’s something that more people experience, to see if it’s unique to me or a need that’s out there greater than me.
Krista: I think that’s an interesting piece. Gosh, you have so many interesting insights. Do you have anything that’s been super memorable or a cool experience since you’ve had your CI, like a listening experience that you have felt is pretty awesome and memorable? Or maybe not awesome. Maybe the opposite.
Amber: I’ve had a couple, one that’s less awesome maybe I’ll start with. Like most people, I struggled at the beginning with choosing which cochlear implant manufacturer to go with.
Ultimately, I chose one that I felt like was going to have the capacity for more upgrades in the future, and that allowed two electrodes to respond at one time, because I felt like that would be more what typical hearing is like, that not just one hair cell responds at a time.
I also wanted one that was directly compatible with Roger Personal DM systems. Those are my rationale for the one that I chose.
When I was going through my cochlear implant process, we switched to the processing strategy where two electrodes could be firing at one time because that was part of my reason for choosing that cochlear implant. But when that happened, I struggled with facial stimulation, especially in response to high frequency sound.
If my kids banged a spoon on a bowl, or we have this gymnastics mat, if they jumped on it, it made my left eye twitch to the point that I’d be like, okay, you’re doing my face. They thought it was funny sometimes. I thought that I just needed to persevere and get used to it.
I didn’t let my cochlear implant audiologist know for a while. But at the same time, there was other sounds that I wanted louder and I wanted it turned up. That’s contradictory to want some stuff not aloud, and other stuff louder. Eventually it wasn’t getting any better.
I made a follow-up appointment and we tried multiple things, like turning off some electrodes, turning it down. The only thing that work to stop the facial stimulation was going back to the processing strategy where one electrode fired at a time.
Initially, that was hard for me because that was one of my reasons for choosing this one, so it was hard for me to let go of that, but I am doing very well with the processing strategy that I have right now.
Krista: Does your CI audiologist think that at some point you can try the dual channel stimulation again?
Amber: I don’t think so. To be honest, I don’t think that I would want to switch. Once you get used to one processing strategy, that’s what your brain gets used to. It’s like being reactivated all over again to switch processing strategies. It takes quite a bit to get used to. I don’t think we would go down that road again.
Then a positive experience was, once I was upstairs and I could hear the toilet running downstairs. I live on an acreage and we have to haul in water. If a toilet’s running, that means we’re going to run out of water, which is not fun.
Normally, I don’t hear the toilet running until I just happened to be in the bathroom and see like, oh, the toilet’s running. Crap, we’re going to be out of water. That was exciting to be, I hear sound. I think the toilet might be downstairs, and I was able to stop it before it became a problem.
Krista: That’s something very functional. Who would have thought that getting a CI would allow you to not have to haul in water?
Amber: I still have to, but not if someone flushes it and walks away and it’s still running, not as often.
Krista: I’m assuming there might be people listening to this podcast who have CIs or newly implanted, or thinking about getting a cochlear implant, parents considering for children. Is there a valuable lesson or a piece of advice that you have that you’d like to share?
Amber: Initially, when my cochlear implant was activated, every sound I heard sounded to me like a hearing aid squealing or feeding back. Everything just sounded like that annoying sound that you or your child wear hearing aids that nobody likes. As an audiologist, I’d known to expect things not to sound great at the start.
Most simulations were like it would be a robotic voice, or it would sound like the adults in Charlie Brown, that wah-wah-wah. But everything just sounded like a hearing aid squealing to me. I couldn’t tell if it was speech, I couldn’t tell if it was an environmental sound. It just was that squealing sound, and I couldn’t really differentiate.
You join Facebook groups and things like that to see what other people’s experiences are. Then there are some people that post I can hear right away on the first day and I can understand speech the same day that I was activated. I wanted to start to and be like, yes, I can hear speech right from the first day, so I was feeling discouraged at the beginning.
Also I was experiencing that lag between the hearing aid and the cochlear implant ear, which sounded like an echo. Initially when my cochlear implant was on, I was hearing worse with it on than it was with just my hearing aid on its own, because I had this other sound that was interfering with my hearing aid. That just made it harder to keep going.
Initially, my plan had been, I will wear both of them at work, then when I get home from work, I’m going to take off my hearing aid and just wear my cochlear implant. But then, my kids and my husband have struggled with me not being able to understand them, and I just felt like it wasn’t working out because it wasn’t very fair that I couldn’t communicate with them at home either.
I did end up just wearing both of them at the same time. That might have slowed down my adjustment to the cochlear implant than if I had just worn the cochlear implant on its own. But I did get used to both of them, and now the sound is coming in as one signal.
I actually have better speech understanding in my cochlear implant ear than my hearing aid ear now. It’s been through testing we’ve been able to confirm that I have better speech understanding, but I also notice it just day to day as well that I can hear better with my cochlear implant ear than my hearing aid ears.
My biggest advice would be, I know everyone says this, but you really do just have to persevere and just keep wearing it every day, even when you don’t want to. It does get better. Right now, I use a cochlear implant and a hearing aid. Maybe one day I would have two cochlear implants, but right now I love having the best of both worlds with having one cochlear implant and one hearing aid.
Krista: I love your story, Amber, your journey and how you can articulate. You have that unique experience of being an audiologist with a cochlear implant. You have the academic background with the personal experience. You can just articulate that so well, and comment on things that maybe people aren’t aware of or have assumptions about.
I just love your stories and really appreciate you spending some time here with us today. It was really exciting. I just want to say thank you and I’m grateful for joining us here at The Future Is Sound, and I’ve really enjoyed our time together.
Amber: Thank you likewise.
Krista: Thanks, and listeners we’ll see you next time. Bye.
