Klippel Feil Syndrome: Ear molds and getting a good fit

Posted by maryella7 @maryella7, Mar 26 3:07pm

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

Wow, Mary that sounds very uncomfortable! I also find the mold fitting onerous but grit my teeth and get through it somehow. I hope your new hearing aids are a good fit as a result of your tolerance.
Robin

Liked by maryella7

REPLY
@robinraig

Wow, Mary that sounds very uncomfortable! I also find the mold fitting onerous but grit my teeth and get through it somehow. I hope your new hearing aids are a good fit as a result of your tolerance.
Robin

Jump to this post

Thank you, Robin. Laughing now because my last name is Bird (for real!). I appreciate your supportive message. Mary

REPLY

@maryella7

Hi Mary,

I am also a Mary and have never heard of this syndrome . So I went online to learn something about it and learned that hearing loss is often a result of the syndrome. You do have an ear canal but the shape is making it difficult to fit molds. I am assuming the molds are a soft like acrylic material. Can I assume the outer ear is a normal shape? Who are you getting your aids from, an ENT practice , a private Audiologist or from an audiologist in a clinical setting.

I can imagine your discomfort and molds are so important for the best hearing options. I have had my molds adjusted over the years and I prefer them to reach as much into the canal as possible. The Audi I work with now has sent the last mold back numerous times to be redone until it was the best choice for me. I’ve had molds taken with and without the mouth open which changes the shape.

How much of a hearing loss do you have? The reason I ask is if an open fit molds would be an option or even an CI without molds . I have a friend who has a CI that doesn’t require a mold. I can’t imagine the labs that do the molds have too much experience with “unique” shapes of ear canals. I often wish I could go to the labs that make the molds and, after they do the initial mold, sit there with them and have them either buff certain areas for a better fit or explain face to face what the problem is. Wish they could actually see xrays of the inner ear in addition to have the prototype.

Are there any support groups online with this syndrome where you could connect with other people?

FL Mary

Liked by maryella7

REPLY
@imallears

@maryella7

Hi Mary,

I am also a Mary and have never heard of this syndrome . So I went online to learn something about it and learned that hearing loss is often a result of the syndrome. You do have an ear canal but the shape is making it difficult to fit molds. I am assuming the molds are a soft like acrylic material. Can I assume the outer ear is a normal shape? Who are you getting your aids from, an ENT practice , a private Audiologist or from an audiologist in a clinical setting.

I can imagine your discomfort and molds are so important for the best hearing options. I have had my molds adjusted over the years and I prefer them to reach as much into the canal as possible. The Audi I work with now has sent the last mold back numerous times to be redone until it was the best choice for me. I’ve had molds taken with and without the mouth open which changes the shape.

How much of a hearing loss do you have? The reason I ask is if an open fit molds would be an option or even an CI without molds . I have a friend who has a CI that doesn’t require a mold. I can’t imagine the labs that do the molds have too much experience with “unique” shapes of ear canals. I often wish I could go to the labs that make the molds and, after they do the initial mold, sit there with them and have them either buff certain areas for a better fit or explain face to face what the problem is. Wish they could actually see xrays of the inner ear in addition to have the prototype.

Are there any support groups online with this syndrome where you could connect with other people?

FL Mary

Jump to this post

Hi Mary,
Thanks for reaching out. It seems like you have some common sense ideas about keeping up with ear mold fittings. It would never have occurred to me to get a new fitting every few years. My hearing loss is in the moderate to severe range, so the open fit with vents would not work I believe. I am working with a hearing aid dispenser, HearInc., primarily because I have purchased aids from them before. The BTE will either be Oticon or Resound. Leaning toward Resound. This time I did not test a Phonak. Phonak Marvel is supposed to be the latest and greatest.
Now for my left side where I have Atresia, I am a candidate for BAHA. I found a renowned outfit the Lippy Group in Warren, Ohio. Everything was moving along until the advent of COVID-19. Despite the new waiting time – I haven't received word – I am real excited about the Oticon Ponto BAHA because the simulation was such a success. Thoughts, comments welcome. I haven’t found a syndrome support group yet. Years ago, I think I found a European group and lost track of the information. Have a good weekend, Mary

REPLY

Hi @maryella7, I'd like to also add my welcome and invite fellow members @vickieb876 and @julieannarcand to join the conversation. They have also talked about having Klippel Feil syndrome (KFS).

Mary, does KFS cause any other symptoms that you manage?

Liked by julieannarcand

REPLY
@maryella7

Hi Mary,
Thanks for reaching out. It seems like you have some common sense ideas about keeping up with ear mold fittings. It would never have occurred to me to get a new fitting every few years. My hearing loss is in the moderate to severe range, so the open fit with vents would not work I believe. I am working with a hearing aid dispenser, HearInc., primarily because I have purchased aids from them before. The BTE will either be Oticon or Resound. Leaning toward Resound. This time I did not test a Phonak. Phonak Marvel is supposed to be the latest and greatest.
Now for my left side where I have Atresia, I am a candidate for BAHA. I found a renowned outfit the Lippy Group in Warren, Ohio. Everything was moving along until the advent of COVID-19. Despite the new waiting time – I haven't received word – I am real excited about the Oticon Ponto BAHA because the simulation was such a success. Thoughts, comments welcome. I haven’t found a syndrome support group yet. Years ago, I think I found a European group and lost track of the information. Have a good weekend, Mary

Jump to this post

@maryella7

Hi,
Earmolds shrink over time. Molds also stretch out the ear canal over time too. I have 2 Phonaks Nadia’s. One of the best things my Audi did lately is to switch the regular hearing aid hooks to tone hooks. They look the same and both have filters. The sound quality was so dramatically improved I am still amazed. Everything sounded “normal” and I can understand so much better. Each brand of aid has its own hooks (of course) . Ask about tone hooks. I had never heard of them before. I have the greatest Audi in all my 40 plus years of wearing aids. My loss at this point is profound.

I should also mention that the size and strength of the tubing makes a difference too. I have extra thick tubing size 13 that does not vibrate as much as a thin tube. I do my own tubing and change my own hooks when I notice things are not the way they should be. My Audi supplies me with tubes and hooks and I have bought the tools to do the changes. I’ve acquired a lot of gizmos over time.

So….molds and fitting plus tubing and keeping those precious aids in a dry and store are important. For any ear wax that can get stuck in a tube I have a hand held bulb like blower that will blow it away ( remove the tube from the hook first. )
I heard about the Marvels also and was willing to trial them but am so happy with the way things are now that I will stay with the Nadia’s.
I’m not a BAHA candidate nor an open fit candidate. Bug them (gently) about the waiting time if possible. I know how exciting it can be. Let us know.

FL Mary

Liked by julieannarcand

REPLY
@imallears

@maryella7

Hi,
Earmolds shrink over time. Molds also stretch out the ear canal over time too. I have 2 Phonaks Nadia’s. One of the best things my Audi did lately is to switch the regular hearing aid hooks to tone hooks. They look the same and both have filters. The sound quality was so dramatically improved I am still amazed. Everything sounded “normal” and I can understand so much better. Each brand of aid has its own hooks (of course) . Ask about tone hooks. I had never heard of them before. I have the greatest Audi in all my 40 plus years of wearing aids. My loss at this point is profound.

I should also mention that the size and strength of the tubing makes a difference too. I have extra thick tubing size 13 that does not vibrate as much as a thin tube. I do my own tubing and change my own hooks when I notice things are not the way they should be. My Audi supplies me with tubes and hooks and I have bought the tools to do the changes. I’ve acquired a lot of gizmos over time.

So….molds and fitting plus tubing and keeping those precious aids in a dry and store are important. For any ear wax that can get stuck in a tube I have a hand held bulb like blower that will blow it away ( remove the tube from the hook first. )
I heard about the Marvels also and was willing to trial them but am so happy with the way things are now that I will stay with the Nadia’s.
I’m not a BAHA candidate nor an open fit candidate. Bug them (gently) about the waiting time if possible. I know how exciting it can be. Let us know.

FL Mary

Jump to this post

I like your DIY approach and your audi’s cooperation with your requests. I am learning here. Tubing and vibration can affect Sound quality? I will inquire about a tone hook and look it up for myself. And I did receive a confirmation today on my BAHA insurance request. It has been approved. I am stoked! Thanks for your help and information, Mary

REPLY
@colleenyoung

Hi @maryella7, I'd like to also add my welcome and invite fellow members @vickieb876 and @julieannarcand to join the conversation. They have also talked about having Klippel Feil syndrome (KFS).

Mary, does KFS cause any other symptoms that you manage?

Jump to this post

Hi Colleen, For me, KFS has presented in the following ways: Situs Inversus (internal organs are reversed); Scoliosis – again reversed (left not right); two missing neck vertebrae; slight cleft palate, small jaw; myopia; blephritis (sp?); microtia; and, maybe stunted growth. The situs inversus since it wasn’t treated may have led to a twisted or flattened diaphragm. I have bronchiectasis which is monitored by lung function tests and year-round meds and inhalers I take on an as-needed basis. It would be nice to hear from those members you mention. Thanks! Mary

Liked by julieannarcand

REPLY
@colleenyoung

Hi @maryella7, I'd like to also add my welcome and invite fellow members @vickieb876 and @julieannarcand to join the conversation. They have also talked about having Klippel Feil syndrome (KFS).

Mary, does KFS cause any other symptoms that you manage?

Jump to this post

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

REPLY
@imallears

@maryella7

Hi,
Earmolds shrink over time. Molds also stretch out the ear canal over time too. I have 2 Phonaks Nadia’s. One of the best things my Audi did lately is to switch the regular hearing aid hooks to tone hooks. They look the same and both have filters. The sound quality was so dramatically improved I am still amazed. Everything sounded “normal” and I can understand so much better. Each brand of aid has its own hooks (of course) . Ask about tone hooks. I had never heard of them before. I have the greatest Audi in all my 40 plus years of wearing aids. My loss at this point is profound.

I should also mention that the size and strength of the tubing makes a difference too. I have extra thick tubing size 13 that does not vibrate as much as a thin tube. I do my own tubing and change my own hooks when I notice things are not the way they should be. My Audi supplies me with tubes and hooks and I have bought the tools to do the changes. I’ve acquired a lot of gizmos over time.

So….molds and fitting plus tubing and keeping those precious aids in a dry and store are important. For any ear wax that can get stuck in a tube I have a hand held bulb like blower that will blow it away ( remove the tube from the hook first. )
I heard about the Marvels also and was willing to trial them but am so happy with the way things are now that I will stay with the Nadia’s.
I’m not a BAHA candidate nor an open fit candidate. Bug them (gently) about the waiting time if possible. I know how exciting it can be. Let us know.

FL Mary

Jump to this post

This is extremely interesting to me!! I was told that I had abnormally small ear canals due the the Klippel Feil; but due to a recent ( I think but now am wondering) brain tumor and radiation, I have a great deal of ear wax coming especially from my right ear ( this may be because it is the side I lay on most. It seemed to have worsened after radiation. Where are you getting the tubing and how was it diagnosed?

REPLY
@maryella7

Hi Colleen, For me, KFS has presented in the following ways: Situs Inversus (internal organs are reversed); Scoliosis – again reversed (left not right); two missing neck vertebrae; slight cleft palate, small jaw; myopia; blephritis (sp?); microtia; and, maybe stunted growth. The situs inversus since it wasn’t treated may have led to a twisted or flattened diaphragm. I have bronchiectasis which is monitored by lung function tests and year-round meds and inhalers I take on an as-needed basis. It would be nice to hear from those members you mention. Thanks! Mary

Jump to this post

This is very interesting. I have what is known as mirror movement in my hands. I can actually use both hands to write my name and if you put the 2 papers together they match up: one hand is writing backward the left while the right is writing normal!!! They always said it was from Klippel Feil. Fortunately, I can control my hands and arms from simultaneously moving however not when I was more intricately tested for my brain tumor. Both hands just did the same thing. This surprised the neurosurgeons until they looked into the many anomalies that accompany Klippel Feil syndrome!

REPLY
@julieannarcand

This is extremely interesting to me!! I was told that I had abnormally small ear canals due the the Klippel Feil; but due to a recent ( I think but now am wondering) brain tumor and radiation, I have a great deal of ear wax coming especially from my right ear ( this may be because it is the side I lay on most. It seemed to have worsened after radiation. Where are you getting the tubing and how was it diagnosed?

Jump to this post

@julieannarcand
Good morning
I’ve gotten extra tubing from my Audi or ordered it online from places such as Westone or Oaktree. There are many online suppliers of hearing aid accessories and batteries and you can shop around for good prices. I’m not sure if you are asking me if how my hearing loss was diagnosed.

FL Mary

REPLY

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

Jump to this post

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!

Jump to this post

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

REPLY
Please login or register to post a reply.