Keratosis Obturans

[manxie17]

My 6 year old daughter has been having ear problems for the last two years.  

Her problems started two years ago when I noticed her hearing was reduced and she had a nasty smell coming from her ears.  She was found to have polyps which were removed.  

She continued to have problems with her ears becoming blocked, causing pain and a reduction in hearing.  She had her ears cleared out twice under general anesthetic.

Last year a new ENT diagnosed her condition as
being Keratosis Obturans which I understand is rare in children.  We now go monthly to have her ears suctioned out to remove the keratin plug.  Before our visits she experiences pain and has recently developed a sensitivity to noise and struggles with the classroom environment.

I would just like to know if there is any treatment for this condition other than the management we are currently undertaking.  Also what is causing the sensitivity to noise.  What is the future likely to bring is she likely to have to have monthly visits for the rest of her life?

[Thehearinglessone]

Hello, I am a 34 year old male with the same problems. (It gets worse as we get older) I have had 7 sets of tubes, bilateral stapedectomy with revision on right (didnt hold), bilateral tympanoplasty, BAHA (bone anchored hearing aid) on right ear, mixed hearing loss bilaterally, Chronic myringitis, granulation tissue, chronic OM, and keratosis of the ear. Some days my life ***** but other times it is awesome! My only advice to parents with these conditions, find the best ENT Doctor you can and stay with them. I was in the military for ten years and it seemed like I changed ENT Doctors every year. I deployed to Iraq 4 times which was a mistake because of all the sand and junk that gets into ears. It made everything worse. I wore hearing aids for about 2 years but living in the desert with my ear conditions is was difficult to keep my ears from producing moister and it continually cause more problems. Then the VA got involved and I demanded to see the best ENT Doctor in Tucson Arizona and the did, to my surprise. He fitted me with the BAHA a year ago and it has Been wonderful since.

Parents: Your children have probably already learned to read lips. But this is a lifetime condition that doesn't get better. Teach them sign language. Teach them to not be afraid to ask for help. Teach them about self esteem and confidence. Make damn sure the schools are on the same page. Hearing loss affects everything. Social skills will diminish as they reach high school because some high school kids are jerks. Your children will learn less if they are not able to hear, so the size of class makes a huge difference. Hearing aids and the BAHA allow you to hear, but the downside is you hear everything. I cannot talk to my wife when the tv is going and the kids are talking, it drive me completely nuts. To help with reading, watch tv with captions on. They can hear the sounds and begin the lip reading course at home. (I started doing this as an adult. I was not able to read or write until I was 16 when I was tired of being "dumb")

Last thing, the vacuuming of the ears does not get better as we age. It seems like the old I get, the more it hurt. And the hearing test? I can pass it without trying. That's how I got in the military. But it only hurts you in the long run. Your children with learn as well so they don't have to wear the hearing aids or so they can stop getting their ears cleaned regularly. I hope all is well and look forward to hearing back! (Not hearing hearing haha) hope this helps.

[JoAus]

Hi, My son suffers from Keratois Obturans as well as cholesteoma and I have stuggled to find any support groups or forums about this condition and the effects is might have on our children.  My son was diagnosed 2 yrs ago at age 4 and has hearing loss in both ears and wears hearing aids but what we are finding is the hearing aids are clogging up his ears and this is causing infections.  We currently visit the ENT once a month and he has a operation every 3 months to keep his ears clear,  We are also struggle with the fact that he has a very limited diet and refuses to even try new foods.  We think it is because of all the infections and how this changes the taste in his mouth?  His behavior is also pretty bad and we think this is also connected to everything else.  I have been trying to find others with this condition but our ENT said that it is rare.  We live in Australia but so far I have found anyone else?  Thank you for updating us on your daughter. Jo

[oeaimom4]

Just checking this again, it's been three years and my daughter is now 10.  I'm so glad this information is still here.  I think it's important to communicate about keratosis obturans in children, there do not seem to be any other support forums out there.  

I am sorry for your son and his battles with his ears, allergies, and asthma and the debridements under anesthesia you've endured together.  I completely understand how difficult that is, frustrating, and scary when it is such a rare condition and there are no absolute answers for parents.

As a mother of four with food and environmental allergies, and having food and environmental allergies myself, I feel there is an extremely important connection between allergies, ears and sinuses.

Here is an update regarding my daughter with keratosis obturans.  In June 2013 my daughter, then age 8, had a surgery to try and reconnect her bony middle ear (ossicular chain repair) in her left ear.  The middle ear had been damaged by her very young (age 2) experience with the wax and skin compaction of keratosis obturans.  After almost 2 years, we are still waiting and watching to see how much her hearing in her left ear will improve.  She is showing improvement in the low frequencies, but she still has her high frequency hearing loss.  She took a break from wearing her hearing aid, but is now set to get a new hearing aid in her left ear as her doctor does not think her hearing will improve much more than it has since surgery.  This is heartbreaking for me as her mother, to be honest.

I really want to share with you all how much improvement we saw in her skin after the removal of dairy (cow's milk products) from her diet, with her pediatrician's approval.  I myself have a dairy and wheat allergy, and we have completely removed both from our daily diets.  She has not had a sinus or ear infection since the removal of dairy and wheat in her diet (and neither have I, nor have my other children!).  We also try to eat a 'whole foods' diet, one free of processed foods.  She no longer has any symptoms of eczema as well, and she's been able to stop use of her dexamethasone drops between her Otology visits, every 8 weeks, with little buildup in her ears.  She also no longer needs to use Nasacort for her sinuses, and hasn't needed antibiotics in over 2 years.  It has been amazing the improvements we've seen in her keratosis obturans with the change in diet - it is something to seriously consider.

I would love for this to be a success story for you, but it's definitely an improvement story.  Best wishes to you and your son!

[sodaniel]

Wow. So glad to have found this. My son is 5 and battling ears since 1yr of age. He too have sinus problems, severe allergies and asthma. I have always wondered if all this is related. After getting his diagnosis two yrs ago we have felt utterly hopeless about it. He had undergone so many debridements under anesthesia....what complicates things is the fact that he also has T tubes and this limits what can be safely put in his ears. We just had another procedure today and I felt so alone. This condition is so rare the doctors don't know what to do fir it and that is hard to deal with. I'd love to hear a success story on this forum.

[oeaimom4]

Hello again,
I wanted to update you about my 7 year old daughter with keratosis obturans.  Her regular cleaning schedule with the otologist she sees is every 6-8 weeks, or sooner if she is having symptoms of itching and ear fullness, and/or pain.  At each visit he also now places Triamcinolone topical ointment in each ear which seems to help as well.  She has had three CT scans, which after reading this article:  (1958) "Wax Keratosis in Children's Ears' by Black and Chaytor http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2026403/?page=1
I asked the Otologist to review her CT scan, which included her sinuses, and the radiologist noted that she had chronic sinusitis.  She has done several rounds of antibiotics and her ears have cleared dramatically.  We have also started her on a dairy free diet, which has cleared her eczema that she would get on her scalp and behind her external ears.
We continue use of the Dexamethasone drops, once daily in each ear, and she also uses Nasonex steroid nasal spray once daily in each nostril.  We have also removed all carpeting from our home, for her dustmite allergy, and use a dust mite cover on her mattress.  We are hoping that this will help with her sinusitis.
She still uses a hearing aid for her hearing loss in her ear, and other than the sinusitis and the eczema, has no other medical health problems.
Thank you and I hope you and your daughter are doing ok and that you have found some answers.

[manxie17]

Thank you so much for your post.   I am so sorry that your little girl is also suffering with this condition.

It is wonderful to be finally in contact with someone else who is also dealing with this problem.  How often does your daughter get her ears cleaned?  We seem find that four weekly visits keep on top of things, any longer and it is much harder for the ENT to remove the plug.  The drops the ENT gets us to use if her ears look red and wet are Sofradex which are a combination of  antibiotics and the dexamethasone.  

I have read of the connection between sinusitus and also bronchitis.  My daughter has lots of problems with a persistent almost constant chronic cough in the winter months.  On the advice of a respiratory dr we have had her adenoids removed which dosent seem have had any affect.

Does your daughter have any other medical health problems?  My daughter developed a very strange rash last year, under her eyes and around her mouth the rash looked like little bubbles.  We saw two different dermatologists who could not determine the cause (apparently yet another rare condition!)  Thankfully it has almost resolved by itself.  My daughter also had hypotonia (low muscle tone at birth) and the cough as described above.  

I too hope for an answer - the thought of monthly ENT visits for the rest of her life just seems incredible

[oeaimom4]

Hi,

I too am searching for more information regarding keratosis obturrans.

I know what you are going through as my 7 year old daughter was diagnosed last year with the same condition.  At age two she suffered a hearing loss in her left ear due to the severe compaction of ear wax and skin that had led to the erosion and seperation of her middle ear bones.  She has undergone several procedures under anesthesia for ear tube placement and debratement of the continuosly rebuilding debris.  Both of her ears are affected although her hearing loss remains in her left ear and she now wears a hearing aid.

Now that she is getting a little older, she has been able to tolerate the cleanings at her Otologist's office not under anesthesia.  However, it is very uncomfortable for her and painful, and a difficult thing to go through as a parent watching them remove large chunks of painful gunk from your child's ears.  It has been five years so far.

My advice is to keep up with your schedule of regular visits for cleaning.  We have found with our daughter that if her ears do not get regular cleanings, it makes matters worse.  Also, the one topical treatment that was prescribed to her was Dexamethasone - steroid drops normally used for opthamology use.  We have found that they improved things slightly, short-term, but she still needs regular cleanings.  We are also looking into the possible connection between chronic sinusitus and keratosis obturrans.

I just can't say enough on how I know how difficult and frustrating this has been.  To watch your otherwise normal child with normal ears suffering from a rare skin condition with little anwers and little treatment is not fun.  I hope that someone out there has a real solution to help our kids.

[Paderla Anitha, DNB]

Hello mam and hope you are doing well.

Keratosis Obturans is likely to cause ear pain because of the keratin plug. It could also cause conduction hearing loss. Keratinolytic agents can be used topically to reduce the keratin plug formation. Please discuss with your doctor regarding the usage of these agents.

Hope this helped and do keep us posted.

[manxie17]

Thank you so much for taking the time to respond to my question.

I was just wondering what topical medication is usually used for treatment?  
Also do you know what the long term affect is likely to be on my daughters hearing.

Many thanks again for your answer.

[Paderla Anitha, DNB]

Hello and hope you are doing well.

Keratosis Obturans is the building up of epithelial layer of the skin of the ear canal and the formation of a keratin plug. It requires regular life-long micro-suctioning of keratinous debris because of the failure of the normal migratory mechanism. It is treated conservatively by debridement of the external canal and application of topical medication. This is likely to cause tinnitus, which is perception of abnormal ear or head noises.

Hope this helped and do keep us posted.