Mondini Syndrome Is Just Deafness Like RA Is Just Arthritis. Not.

I was home for 3 hours today in between doctor appointments for my Tiger and I spent that time reading up on his diagnoses. As I drove him to 3 doctors in 2 days, thoughts swirled around and I decided to share some of it with you. This is not about RA, but there are some relevant concepts for those with any chronic illness or who are parents or care givers. I’ll leave out as many details as possible to shorten it as much as I can.

Once upon a time

Deaf child at play signWhen my older daughter Mary Khris was little, I suspected hearing loss and was dismissed repeatedly that she was fine after ear “cleanings.” At four, her loss progressed quickly and she finally saw an audiologist. When she was completely alone in a silent world before I could get her some loner hearing aids, I shed quite a few tears for her but, always after she was asleep.

She had excellent care and sophisticated testing by a pediatric ENT who I’ve written about before as being a saint. Really. Yet, no one could tell me why Khrissy could not hear. I made the best decisions I could for her. We did twice a week speech therapy when she forgot how to speak. I taught her to read and write and continued home speech therapy.

When Khrissy was 8, her baby brother Tiger got his first cold. And it was painfully obvious to me that he could definitely not hear.

The pediatrician (and everyone else) started the familiar run-around: “Don’t worry, Kelly. He’s fine.” But this time, I was unshakable. I was developing a mother-of-a-patient voice.

I went around the doctor to a pediatric audiologist who measured Tiger’s hearing loss and fitted him with powerful hearing aids.

(That’s a long story made very short, but you can read a bit more of our story on epatients.net.)

When Mary Khris was a teenager, I read that it was possible to get CT scans of the inner ears to possibly diagnose the cause of the hearing loss. My pediatrician (nicknamed so far as fantastic nurse practitioner – we need to do better, I’ll work on that) helped me get both of the kids scans and referrals to an academic center. (Translation: hassle.) But we were finally given a word for what we experienced: Mondini. I wrote about Mondini syndrome in a WEGO Health article if you’d like to know more.

That was the end of that road, I’d thought. Mondini causes deafness. End of story.

My incredible kids continued to excel and refused to let their disability be a barrier to accomplishments. They were blessed with a couple of women who were true guardian angels, helping ensure that hearing tests were done and constantly repairing hearing instruments: Ellen, when they were little, and Cynthia, the last few years. Cynthia does have a nick-name, but she doesn’t know it until now: Fairy godmother who magically makes things work. She really can do miracles.

Dr # 1: Yesterday

Yesterday, we’d been sent to an ENT to deal with impacted wax and do current testing. When the doctor heard Tiger wants to play football, he informed us that it’s not safe for anyone with Mondini syndrome to do contact sports. “One hit could decimate his ear and cause leakage of spinal fluid.”

What? What happened to the top academic pediatric ENT’s in 2 states telling us “It’s just deafness”?

Can I turn the clock back one day to “just deafness.” Like most people with RA would love to have “just arthritis.”

The doc told me to “Go online and find out whether the odds are 1 in 100 or 1 in 10” so we can decide whether it’s worth “rolling the dice.” He added, “Stay out of chat rooms.” At least he thinks patients should use the internet! When I couldn’t find any such odds today, I called his office to ask whether they could help with the search. I hope they can help us because he seemed to care what happens.

There are several similar issues with Mondini syndrome to those we find with Rheumatoid Arthritis:  there is misinformation, outdated information, and debating about proper terms. For years, there was discussion about whether the condition occurs with a genetic link or is merely the fault of some event. Here’s one fascinating article: What is a ‘Mondini’ and What Difference Does a Name Make?

Dr # 2: Back pain

It was a re-run today. Cruel to a person with RA: a 7 a.m. alarm clock. But we got out the door and arrived on time. Then we waited 90 minutes to meet the orthopedist, but it seemed worth it. He listened carefully and answered questions. The answers on the other hand were not as pleasant.

Tiger was diagnosed with bilateral sacralized lumbar vertebrae (aka “batwing deformity” – yes, I realize how insensitive that sounds, but that’s what doctors call it). The L-5 vertebrae fuse to the sacral segments. His looks severe so now we know the cause of his sacroiliac joint pain, worsened by exercise and why he was unable to do certain exercises in the spring football training last month.

We will get tests, look at options, and try physical therapy, whatever it takes. And then our family will decide. Tiger has wanted to play football all his life. And while I was passing on my love for football, I’ve taught him to not give up, so he’s determined.

Dr # 3: Toenail surgery

Late this afternoon, Tiger saw the podiatrist for an infected toe caused by injury and an ingrown toenail. The straightforward solution was a familiar simple outpatient surgery. And hopefully an end to barefoot basketball in the driveway.

If you’ve heard my story, you know a decisive role was played by a certain podiatrist who was the first person who believed my joint symptoms were real. He helped me get to a rheumatologist and get treated. His compassion was even more meaningful while other doctors were dismissive due to my perpetually normal sed rate. [Yes, I took Tiger to the same guy.]

Unanswered questions

How can a mom know? I wish I’d been wrong when Mary Khris was a baby. I wish I’d been wrong when Tiger couldn’t hear either. I was certainly wrong when I believed that hearing loss was the only real “problem” with Mondini. Hearing is not the only problem with Mondini any more than pain is the only problem with RA.

We obviously have more questions than answers right now. Is football more dangerous for Tiger? Mary Khris and I want to know whether or not it’s safe for them to fly? It causes pain. What does that mean? How can we find out? What can be done?

Being an empowered patient or parent is a tough job some days. Searching for information. Reading conflicting answers. Finding trusted doctors. Making hard decisions.

[Edit 0833 am: Corrected typo. Added comment.]

Recommended reading

Kelly Young

Kelly Young is an advocate providing ways for patients to be better informed and have a greater voice in their healthcare. She is the president of the Rheumatoid Patient Foundation. Kelly received national acknowledgement with the 2011 WebMD Health Hero award. Through her writing, speaking, and use of social media, she is building a more accurate awareness of Rheumatoid disease aka Rheumatoid Arthritis (RA) geared toward the public and medical community; creating ways to empower patients to advocate for improved diagnosis and treatment; and bringing recognition and visibility to the Rheumatoid patient journey. In 2009, Kelly created Rheumatoid Arthritis Warrior, a comprehensive website about RA of about 950 pages and writes periodically for other newsletters and websites. Kelly served on the Mayo Clinic Center for Social Media Advisory Board. There are over 42,000 connections of her highly interactive Facebook Fan page. She created the hashtag: #rheum. Kelly is the mother of five, a home-schooler, Bible teacher, NASA enthusiast, and NFL fan. You can also connect with Kelly by on Twitter or YouTube, or LinkedIn. She has lived over nine years with unrelenting Rheumatoid disease. See also http://www.rawarrior.com/kelly-young-press/

21 thoughts on “Mondini Syndrome Is Just Deafness Like RA Is Just Arthritis. Not.

  • May 31, 2012 at 8:27 am
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    I’m so sorry! It’s hard enough to go through all that ourselves; when our kids are dismissed, it’s over the top. I was just informed earlier this week by a renowned specialist (rheum) that my back and hip pain have nothing to do with RA…really??? All she was concerned about were number of joints flaring, and since I could not demonstrate swollen joints in my hands that morning, I don’t think she even believed my hands and feet were really a problem…. sigh. I am grateful for my local rheumie, who is kind, compassionate, and most importantly, believes me and takes me seriously. The specialists at academic centers have a checklist mentality (Do you wear your seatbelt? check!) …the local doctors in private practice care more about the folks who are their neighbors and friends as well. Hoping for the best for you and your children.

    Reply
    • June 3, 2012 at 8:26 pm
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      You know, Donna, this is exactly why I am starting a campaign to encourage those of us with RAD to PHOTOGRAPH our joints when they are swollen. Digital cameras are commonplace – if you don’t have one, get your friends to take pics with their phonecams. Then, take the pics in to your rheum doc (and every other doc you see!) to show what’s going on with your joints.

      It really worked for me. I have a great Rheum doc, but we were having some problems understanding each other at a visit. I whisked out my smartphone and showed him the picture of my hands from the month before. His response, “O that’s just NOT RIGHT!” And he changed my meds, gave me another Medrol dosepak, and a PT referral.

      So, now I’m taking pics of my feet, my hands, my unreplaced knee, and my husband is taking pics of my hips (and my buttocks) when they flare or swell. He says he’s going to take a picture of me when a fibro attack hits me with all the nausea, diarrhea, muscle pain, and malaise. He’s going to take pics of my swollen muscles and my skin over them covered with Biofreeze (to heck with Calgon, Biofreeze, take me away!).

      I believe that photos (and vids) taken on our digital cameras will bring a new dimension to being well-informed e-patients.

      Cheerio!
      Elizabeth

      Reply
  • May 31, 2012 at 8:46 am
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    Kelly,

    Thank you for sharing this very personal story. My heart goes out to you and your entire family. Your children sound like fighters, or more appropriately “warriors”. A trait they share with their mother no doubt. The fact that you are able to summon so much strength to advocate for your children in the face of your own struggle with RAD is impressive and admirable.

    Reply
    • May 31, 2012 at 11:59 am
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      Thank you Marie. They truly are warriors and stoic about any suffering. Tiger was silent and still with his toe surg. yesterday same as I always am with procedures.

      It’s hard to explain without sounding like just “complaining” so I end up not saying much, but I really appreciate your comment that makes me feel understood about summoning strength. It is so hard to drive & sit in a chair 90 minutes with screaming knees and pretend it’s all good. And bend to see the screen to discuss the xrays with the dr, as if it doesn’t hurt or isn’t hard, etc, etc. Every move is hard from turning off the alarm forward and you can’t take a pain med cuz you’re driving… But I’m his mom & wouldn’t give anything for that!

      Reply
  • May 31, 2012 at 10:13 am
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    Hi Kelly,

    Thanks for sharing your story about your children’s hearing loss. I’m a certified teacher of the deaf/hard of hearing and a speech-language pathologist. I was/ am working on my PhD in Deaf Education and was slammed with RA this spring semester. You are an amazing human being and I’m sure your children are just as wonderful !!! I enjoyed the story. Kind regards.

    Reply
    • May 31, 2012 at 11:50 am
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      Hi Helen, I remember reading your letter. I hope you’re able to keep going. One of my dear friends is a teacher in deaf educ. and she has RA also, Nancy. You might enjoy the video I just posted in my reply to Roselynn.

      Reply
      • June 3, 2012 at 8:58 pm
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        Kelly, have you seen the blog “Hearing Elmo” http://hearingelmo.com/ ? It’s by a woman with adult-onset deafness with inner ear loss of balance. She has a cochlear implant (CI) that she decorates with “bling” for each holiday / season. She teaches sign to high school students who are not deaf. It’s an amazing chronicle of her illness and her adaptations to it – and she is assisted by her children and husband as much as you are! Very positive blog!

        You are to be commended – nay, extolled! – for your faith, trust in God, and your determination to let NOTHING stand in the way of your children having the same opportunities as other children of their ages, and let NOTHING stand in the way of finding an adequate and helpful treatment for your RAD. You are a shining example for us! I love you!

        Cheerio!
        Elizabeth

        Reply
        • June 3, 2012 at 10:05 pm
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          thank you Elizabeth. I will check her out!
          Love you too! You are such a brave warrioress as well!!

          Reply
      • June 9, 2012 at 11:02 am
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        Hi Kelly,

        I would enjoy connecting with Nancy, if that is ok with her and with you. I’ve sent you an email from my email account today about the discontinuation of methotrexate injections by the manufacturer. I’ll send another email to you with the subject line SLP_TDHH connect with Nancy. If you ever want a listening ear about your children, I’d be happy to be in touch. You give so much to others it would be my pleasure.

        Reply
        • June 9, 2012 at 4:18 pm
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          Thank you Helen. & I’ll reply to your email as soon as I can.

          Reply
  • May 31, 2012 at 10:59 am
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    whoa… must have been an overwhelming day for you and Tiger. Hope you find the answers you need (and that Tiger gets to play football).

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  • May 31, 2012 at 11:10 am
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    The only thing that hurts more than RA is our children having pain. Mary Kris and Tiger are so lucky to have you as their mother as well as other parents with children with “Mondini Syndrome” will learn to and appreciate Kelly. I’m so sorry for what Tiger is going through and hope that the outcome is that he can play some kind of football for I believe that even Tag Football is growing as a true sport.

    It is devastating to me how we trust what Doctors say after we have our children and then it turns out to be more and you feel helpless for the time that was wasted. My son Austin always walked on his tippi toes and I expressed concern at the age of 2 and was told it would all be okay. Turns out his ankle area is unaveragely smaller and his achillius tendon is tight and it caused us to have to put him in physical theraphy as well as having to wear the medal bracelets for three months. He is still having issues and it causes him to run different there for not being a fast runner and having pains and falls with it.

    Just wanted to share this experience in understanding your frustration and sending you support in all the fight ahead of you to get help for Tiger not just for his love of football, but for his hearing and other issues with this syndrome in his life ahead.

    Let me know if there’s anything that I can do in supporting your Mondini Syndrome awareness move.

    With much love,
    Diana

    Reply
    • May 31, 2012 at 11:48 am
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      Thank you, Diana. I hope Austin will be able to find a solution so he can walk and run without pain. It is harder when we see them suffer – I remember how my heart ached for each one when I learned they could not hear.

      Reply
      • June 1, 2012 at 8:59 pm
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        Wow, look at the spelling errors. It must of been tired, blurry vision, enough pain meds (lol).

        *achilles tendon
        *metal braces
        and I hope that was it.
        Sorry!

        Reply
  • May 31, 2012 at 11:10 am
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    Hi Kelly, There is so much I’d like to share, but I’ll keep it short as I can. I started learning to sign when I was a church youth group leader over 20 years ago, and before I became a nurse practitioner. I now have many Deaf friends, and it’s a culture that I continually learn about. One of my friends from my latest church (I’ve only been there about 7-8 years) commuted to NTID and graduated this past Friday(google it, it’s a fantastic school) and is now an Interpreter. The Deaf/HOH can do anything—one of my colleagues is a doctor who is Deaf. Do your children sign? Are they part of the Deaf community? If not, you can go to Deaf Chat Coffee online to find a social group near your home. Interpreting is very challenging, I get very tired at times, you know how that RA can be! My son is autistic, and I wish that he had learned to sign, because it would have helped his communication, even though he does hear. One of the friends I’ve met at Deaf Chat has RA also, and I believe it helped her to be able to talk to someone who could emphasize—health care providers can’t understand that a Deaf person who cannot sign because of painful swollen hands are SILENCED! Anyway, all the best to you and your children—and remember, don’t blame yourself—you had no more to do to cause your children’s Deafness than your parents did to cause your RA. When I was at NTID last Friday, it was an incredible, joyful celebration of accomplishment!

    Reply
    • May 31, 2012 at 11:36 am
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      Thank you for sharing those wonderful stories, Roselynn. My son is learning to sign with another scouting family who has a deaf son. He’s very reliant on his aids & when they are not working, it shows how important other skills especially lip-reading can be! I agree that deaf people can do almost anything, but they are not accepted into some places such as military careers (non-combat) currently and it’s something I hope changes. We saw this video recently – very inspiring Keith Nolan: http://www.youtube.com/watch?v=gtKTcPq7XBs

      Reply
  • May 31, 2012 at 1:21 pm
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    It always hurts so much more, when it is our kids being afflicted. I always believed that is why as a mom with RA and other AI issues, that I should model proactive behavior about it, yet not let the RA define me, nor overshadow my kids’ lives. They should be able to have as normal as lives as possible, without unneeded worry for their mom or what is going on with her. And by seeing that their mom or parent is handling disease by being a proactive and informed patient, not dwelling on fear, but finding the positive in life; then when they are afflicted with life’s problems, whether it is a disease or any type of problem, they might be fearful but do what needs to be done, adapt , and continue to live life as fully as possible.

    I am not saying I achieved those goals, since there were periods of frequent hospitalizations for several years and obvious periods of ill health, but my husband and I always tried to make life as normal as possible for them. When the home nurses made their visits, my youngest son, would look at it as a great playtime. While the nurse was treating me, he was busy in his play kitchen, preparing just the right dish to serve the nurse at the end of the visit. And the sweet nurses played along.

    Kelly,I hope your kids can also fight the disease they have, and also live life as fully as they possibly can.I hope they find the right help to enable them to do that.

    Reply
  • June 1, 2012 at 11:06 am
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    Kelly, as always, I am amazed at what you can handle. First of all , your strength in handling your own ailments is unbelievable, and then you still manage to comfort and inform your fellow warriors. But, most of all, and I know this only too well, you’re also now facing and handling the toughest job of all–health issues of your family. I know you have much experience in researching different theories, treatments, problems of so many illnesses, but, as you have said, the hard part is getting answers for which options to follow, which are best and safest for your children, and which specialists are best to facilitate the path chosen. Wish so much that we could help you as much as you have helped us….but we will pray that someone qualified will help guide you in the direction you need to follow, to help you make the decisions you need to make for your children. Gentle hugs to you all!

    Reply
  • June 11, 2012 at 5:21 pm
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    Hi Kelly,
    My husband, in-laws and all five kids are deaf. My husband does well with hearing aids but would jump at a choclear implant if his hearing got worse. My kids are all adopted, four from China, so the reason for their deafness varies. High school football is BIG in the deaf community. Their school, Maryland School for the Deaf, has been national champs for many years. Hope you can get a clear answer about your son and football.
    Judith

    Reply
    • June 23, 2012 at 9:00 pm
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      HI Judith! Thanks so much for speaking up! What a special mom you are – with all 5 kids being deaf! What a wonderful family you must be.
      I did not know that about football – I’ll look that up. I wonder if anyone knows how great the risk is with Mondini. Still looking for information.

      Reply
  • June 24, 2014 at 10:18 pm
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    Wow!! I experienced the very same things with my youngest. He was born in Newport Beach, CA with the best pediatricians, yet no one would listen to me when I told them Jack had a hearing problem. I even demonstrated the startle technique. Nothing. We moved to Houston when he was 6 weeks old, and I got the same response, there. Then we moved to Hamilton, MT, and SAME.. I enrolled him in a preschool for speech delay. Low and behold, when the audiologist went there for routine visit, she called and said, “Jack has a significant, permanent hearing loss”. I said, “yep”, and she asked why I hadn’t gotten help. He was 3 years and 3 months old. Had we found this out PRIOR to the three year mark, we would have had assistance from the state. Instead, after $32,00 in tests, MRI’s cat scans, blood work, and hearing aids….ultimately, bankuptcy since insurance considered it cosmetic, we found out it was Mondini Sydrome. Fortunately, he is doing very very well. A wonderful gift of reading people and animals. an amazing feel for rhythm and music, perfect pitch, and many other qualities we call his “extra-abilities”.

    Yes, NO contact sports. We have him on the swim team, but wonder if this is causing problems. Any insight would be appreciated.

    Thanks

    Suzanne

    Reply

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