Klippel Feil Syndrome: Ear molds and getting a good fit

Posted by maryella7 @maryella7, Mar 26, 2020

Hi Everyone, I have an ear canal that points up and back and for that reason ear mold fittings can be very trying. For me, the Worst part of purchasing a new hearing aid-which I am doing now-is the ear mold fitting. I guess I’m writing this to feel less alone. I have never met someone with my issue. It is due to a syndrome called Klippel-Feil or Goldenhar depending on the given medical professional’s pronouncement. Give me a shout out. It would really help me out. Thanks, Mary

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Hi Mary; I'm glad you are able to get the things you need for your hearing! But yes, I was wondering how you were diagnosed and does it relate to Klippel Feil? Stay safe out there!

REPLY
@julieannarcand

Hi; thank you for inviting me into the conversation. I have no odontiod process ( the bony pin like structure that sits on the 2nd vertebra and allows the first cervical to rotate. This caused severe pain and numbness (and could have easily caused death) before they screwed C2 up through C1. Other cervical vertebrae were abnormal, malformed or fused requiring 7 spinal surgeries. I had abnormal cervical ribs which caused a painful thoracic outlet syndrome (removing these were the only surgeries that alleviate pain) for which I am thankful. I have what is called a wandering p wave in my heart. I'm wondering if anyone else has this or other heart problems?? It causes tachycardia and a very high pulse at times. I also have abnormal shaped kidneys that I am thankful are not bothering me. I was recently diagnosed with an inoperable brain tumor in the right cavernous sinus surrounding the carotid artery and sitting on the optic nerve ( they do not believe this has anything to do with the Klippel Feil) however I was diagnosed with Klippel Feil at 25 and am now 53. My heart condition is worsening and I wonder if anyone else's is due to age? And my doctors have diagnosed ankylosing spondylitis now due to age and the advanced Klippel Feil which comes with an autoimmune component. Does anyone else have this. Interesting reading about people's ear issues. I was told I had abnormally narrow ear canal due to the Klippel feil but after radiation for my brain tumor I feel like I I literally have to sleep double pillow cases! It seems my right ear is constantly oozing. Sorry to be so gross but I don't know if that is an issue from radiation or Klippel Feil. It has been a journey that's for sure
One other thing that I wanted to ask especially in light of the coronavirus: with each surgery and I've had 10 total I had to be intubated awake because I have no range of motion. I have to wear a medical alert bracelet to warn the medical community that I cannot tilt my head at all. Do you have anything like that??
Thank you and so much love for you all! Stay safe out there and thank you again for including me!!
Julie Arcand

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Hi Julie, My neck rotation is okay turning right, very limited turning toward my scoliosis side. All that you have gone through as an adult gave me great pause. You have been through so much more than I have. I haven’t had medical intervention other than a palate surgery and eye surgery as an adult. My Childhood surgeries were the reconstructive kind on my right ear. I’m sure each family is different. My parents did what they could. My mother refused intervention on my spine. I used to getting racing pulses, but I forget what prompted them. My heart is positioned under my right ribcage. I had a gallbladder surgery scratched due to intubation difficulties. I used to wear a Medic Alert tag around my neck re the situs inversus. You have made me aware of the issue I share with you regarding range of motion. I should be more mindful. Every second counts. How often will you be monitored for the tumor? Is it shrinking? Thank you Julie for sharing your story and current trials with me. I am holding you in my heart. XO, Mary

REPLY
@julieannarcand

Hi Mary; I'm glad you are able to get the things you need for your hearing! But yes, I was wondering how you were diagnosed and does it relate to Klippel Feil? Stay safe out there!

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Hi Julie, I was diagnosed by a spinal surgeon 10 or 15 years ago when I was in my forties.We didn’t get to the treatment plan part. I turned tail and ran. Too afraid to face the prospect of missing work, putting others out. I inherited a workaholic credo from my family. Work over self. Sad. I hope to put that behind me. Mary

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@julieannarcand

Hi Mary; I'm glad you are able to get the things you need for your hearing! But yes, I was wondering how you were diagnosed and does it relate to Klippel Feil? Stay safe out there!

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@julieannarcand
Hi,
Sorry for the misunderstanding but I don’t have Klippel Feil. I was merely responding to the posts about hearing loss and problems fitting molds.

FL Mary

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@imallears

@julieannarcand
Hi,
Sorry for the misunderstanding but I don’t have Klippel Feil. I was merely responding to the posts about hearing loss and problems fitting molds.

FL Mary

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@julieannarcand
A PS to the above…there is another Mary @maryella7 ….that I believe you have me confused with. And I thought I was the only Mary ……😄
FL Mary

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@imallears

@julieannarcand
A PS to the above…there is another Mary @maryella7 ….that I believe you have me confused with. And I thought I was the only Mary ……😄
FL Mary

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@julieannarcand This Fl. Mary is a wine conesuer

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@maryella7

Hi Julie, My neck rotation is okay turning right, very limited turning toward my scoliosis side. All that you have gone through as an adult gave me great pause. You have been through so much more than I have. I haven’t had medical intervention other than a palate surgery and eye surgery as an adult. My Childhood surgeries were the reconstructive kind on my right ear. I’m sure each family is different. My parents did what they could. My mother refused intervention on my spine. I used to getting racing pulses, but I forget what prompted them. My heart is positioned under my right ribcage. I had a gallbladder surgery scratched due to intubation difficulties. I used to wear a Medic Alert tag around my neck re the situs inversus. You have made me aware of the issue I share with you regarding range of motion. I should be more mindful. Every second counts. How often will you be monitored for the tumor? Is it shrinking? Thank you Julie for sharing your story and current trials with me. I am holding you in my heart. XO, Mary

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Hi Mary; so good to hear from you. I was raised in upper Michigan by an alcoholic marine Corp father!! Work work work was the order of the day. It wasn't until I was married and had to have a c section with unknown complications giving birth. Thankfully, I gave birth at the University of Washington's medical school and they looked further into the complications and found the severity of the Klippel Feil. I was immediately put in a halo brace ( FULLY AWAKE!!!!!) and had multiplie surgeries! I'm actually in the text books as such a rare and severe case.

I truly am thankful that it was found later in life because there simply were no hospitals or physicians capable of doing the delicate surgery in the upper mid west and I had parents as you described that basically said I'll give you something to complain about!! So I just stopped complaining of the severe headaches and inability to move my neck in certain ways that caused what I can only describe as electrical pain. Now we know that having no odontoid was causing the electric type pain and any fall or car accident could have killed me immediately.

I have been through radiation for thr tumor however it has not shrunk at all. It is an atypical meningioma ( that is what they think as it is in such a difficult location that I cannot even get a biopsy). Normal meningiomas can be dangerous due to their location as mine is; however typically they grow very very slowly. If they grow so slowly they call them benign. Mine is growing faster than the typical meningioma but not so fast ( thankfully) that I still have some time before it invades the entire cavernous sinus and covers my carotid artery. There is a newly developed 3d surgical procedure that was approved in late 2019 that only 3 hospitals in the United states have the equipment. I have had my records sent to Pennsylvania but can do nothing until the coronavirus is completely over. It is difficult to live in pain all the time but I really try to look for positive things like my grandchildren ( I have 2 and absolutely adore them). Yes, I often cry myself to sleep because there are so many things that come with Klippel Feil and I've seen so many specialist who obviously want to be helpful but they simply don't know enough about Klippel Feil and the sequelae. My brain tumor went undiagnosed for so long because they thought 5he KFS was just getting so severe that it was turning into ankylosing spondylitis and that was causing the cluster headaches and swollen eye and right side of my face. So I do try to keep positive when I can!! Cry when I need to and take it day by day! Please tell me more about your upbringing…I spent 25 years in therapy. Yes, 25 for PTSD…but it truly was a blessing because I could not have coped with all of the surgeries if I had not had the support of therapy. Living with an abusive alchohol marine was probably way worse than any medical condition that I've been through! Much love and thought of kindness and strength to you! Your fellow warrior, julie

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Hi Mary: I have the same problem. Tried several places but they just don't fit in
my ear canal. They just keep poping out. Cant participate in conversations as I cant hear what is being discussed. Mary Lou

REPLY
@imallears

@julieannarcand
A PS to the above…there is another Mary @maryella7 ….that I believe you have me confused with. And I thought I was the only Mary ……😄
FL Mary

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Sorry about that but I still send my love and well wishes!! Stay safe out there; warmest regards, Julie

REPLY
@tardi400

Hi Mary: I have the same problem. Tried several places but they just don't fit in
my ear canal. They just keep poping out. Cant participate in conversations as I cant hear what is being discussed. Mary Lou

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That is unacceptable. The hearing industry should have a solution for you. But I understand you can only tap into the resources around you. I hope someone reads this and has a suggestion for your dilemma. Take care, Mary

REPLY
@julieannarcand

Hi Mary; so good to hear from you. I was raised in upper Michigan by an alcoholic marine Corp father!! Work work work was the order of the day. It wasn't until I was married and had to have a c section with unknown complications giving birth. Thankfully, I gave birth at the University of Washington's medical school and they looked further into the complications and found the severity of the Klippel Feil. I was immediately put in a halo brace ( FULLY AWAKE!!!!!) and had multiplie surgeries! I'm actually in the text books as such a rare and severe case.

I truly am thankful that it was found later in life because there simply were no hospitals or physicians capable of doing the delicate surgery in the upper mid west and I had parents as you described that basically said I'll give you something to complain about!! So I just stopped complaining of the severe headaches and inability to move my neck in certain ways that caused what I can only describe as electrical pain. Now we know that having no odontoid was causing the electric type pain and any fall or car accident could have killed me immediately.

I have been through radiation for thr tumor however it has not shrunk at all. It is an atypical meningioma ( that is what they think as it is in such a difficult location that I cannot even get a biopsy). Normal meningiomas can be dangerous due to their location as mine is; however typically they grow very very slowly. If they grow so slowly they call them benign. Mine is growing faster than the typical meningioma but not so fast ( thankfully) that I still have some time before it invades the entire cavernous sinus and covers my carotid artery. There is a newly developed 3d surgical procedure that was approved in late 2019 that only 3 hospitals in the United states have the equipment. I have had my records sent to Pennsylvania but can do nothing until the coronavirus is completely over. It is difficult to live in pain all the time but I really try to look for positive things like my grandchildren ( I have 2 and absolutely adore them). Yes, I often cry myself to sleep because there are so many things that come with Klippel Feil and I've seen so many specialist who obviously want to be helpful but they simply don't know enough about Klippel Feil and the sequelae. My brain tumor went undiagnosed for so long because they thought 5he KFS was just getting so severe that it was turning into ankylosing spondylitis and that was causing the cluster headaches and swollen eye and right side of my face. So I do try to keep positive when I can!! Cry when I need to and take it day by day! Please tell me more about your upbringing…I spent 25 years in therapy. Yes, 25 for PTSD…but it truly was a blessing because I could not have coped with all of the surgeries if I had not had the support of therapy. Living with an abusive alchohol marine was probably way worse than any medical condition that I've been through! Much love and thought of kindness and strength to you! Your fellow warrior, julie

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Hi Julie, Born and raised in Pennsylvania. I deal in dark humor, so I knew it was okay to laugh about your comment about your father versus your medical condition. I am a product of a father who conquered alcoholism after us five kids were out in the working world. I wouldn’t describe him as abusive. His drinking might have been hereditary. Both his father and grandfather were alcoholics. His father left the marriage when he was two so fortunately he didn’t pick up any nasty learned behaviors on how to mistreat children. Now for my mother who is still living. Mom is bipolar and Was prone to low lows and high highs. It was tough. There were times when one parent was up and the other was down. They remained faithful to each other and rode out the bad stuff. Sometimes mom committed herself to get away and be with like minded individuals and understanding helpers. Other times my mother’s family would encourage him to make her get help. Naturally it was hard to be heard in the family. When I would mention weird abnormalities to my mom, she would brush them off. I think I would benefit from therapy. I am easily manipulated. I don’t like confrontation. Which hospital in PA is it? Children are the best. I never married and snuck into a profession-public librarianship-that lets me see kids grow up and move on. Thank you for your kind words. Now I need a Viking name! Ha, ha, Mary

REPLY
@maryella7

Hi Julie, Born and raised in Pennsylvania. I deal in dark humor, so I knew it was okay to laugh about your comment about your father versus your medical condition. I am a product of a father who conquered alcoholism after us five kids were out in the working world. I wouldn’t describe him as abusive. His drinking might have been hereditary. Both his father and grandfather were alcoholics. His father left the marriage when he was two so fortunately he didn’t pick up any nasty learned behaviors on how to mistreat children. Now for my mother who is still living. Mom is bipolar and Was prone to low lows and high highs. It was tough. There were times when one parent was up and the other was down. They remained faithful to each other and rode out the bad stuff. Sometimes mom committed herself to get away and be with like minded individuals and understanding helpers. Other times my mother’s family would encourage him to make her get help. Naturally it was hard to be heard in the family. When I would mention weird abnormalities to my mom, she would brush them off. I think I would benefit from therapy. I am easily manipulated. I don’t like confrontation. Which hospital in PA is it? Children are the best. I never married and snuck into a profession-public librarianship-that lets me see kids grow up and move on. Thank you for your kind words. Now I need a Viking name! Ha, ha, Mary

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I'm sorry to hear all that you went through!! It had to be extremely difficult to have a mother who had extreme highs and lows. I think children need that security or we will end up being walked on; dismissed and unable to say no. I definitely want this virus to go away but truthfully, for me personally, I've never felt so free!!! Because of how we were raised I feel like I can never say no; responsible for things that I haven't even been asked yet; so having the excuse of not being able to go anywhere and do anything feels incredibly freeing to me!! I hope you will look into therapy. It isn't for everyone perhaps, but I don't think I would be here now without it. It really is eye opening to see the physical things I've been through and to know without a doubt that they were much easier than the emotional. The dr I am hoping to see is a dr Lee in Pennsylvania Pittsburgh university hospital? Or Pennsylvania university hospital. John Y K Lee; a neurosurgeon. I have enjoyed talking with you very much!! Let's keep it up. Stay safe and healthy!! I'm glad you get to enjoy kids from afar. I love my 2 grandkids but I know my own daughter was affected by the many surgeries I had one every year for 10 years. She is now 30 with 2 kids ( I only have 1 child) and I think she understands more now that she is a parent. Of course guilt makes me want to do more than I really am capable of but right now since she is happily married and 2 hours away; I can't go over there. Life is difficult!! Ive never found it easy and try to stay as positive as possible but it is not always possible. You are a warrior!! Much love, Julie

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