Cogan's syndrome, which primarily affects children and young adults, causes eye inflammation, vasculitis, and hearing loss.
Cogan’s syndrome is a rare autoimmune-mediated rheumatic disorder of interstitial keratitis (inflammation of the cornea) that develops in children and young adults shortly after their recovery from an otherwise unremarkable respiratory infection. First reported in 1945, Cogan’s syndrome causes symptoms that are similar to those of syphilic keratitis although there is no evidence of syphilis infection in Cogan’s syndrome.
Who is Affected
Cogan’s syndrome primarily targets children and young adults in their 20’s and 30’s. Cogan’s syndrome frequently occurs after an influenza-like infection. Although no definitive infectious agent has been established as a precursor of Cogan's syndrome, several studies show an association with a previous Chlamydia infection.
Symptoms
Cogan’s syndrome causes eye inflammation, including conditions such as keratitis, iritis, scleritis, or conjunctivitis, associated with balance problems, dizziness and ear ringing (tinnitus) and other symptoms that resemble Meniere’s disease. These vestibuloauditory symptoms can progress to rapid bilateral deafness due to inflammation affecting the 8th cranial nerve.
Other symptoms include vasculitis, nausea, vertigo, poor balance, fever, fatigue, weight loss, vomiting, light sensitivity, and a bilateral blurring of vision. Rarely, patients may exhibit enlarged lymph nodes, rash, chest pain, night sweats, arm pain, cardiac involvement and shortness of breath.
Systemic cardiac manifestations of Cogan’s syndrome include aortitis, aortic valve insufficiency, pleuritis, pericardial effusion, coronary arteritis, and possibly myocardial infarction.
Vasculitis in Cogan’s syndrome can cause major organ damage, and in rare instances it can be fatal. Hearing loss is often progressive and can lead to permanent deafness within two years without early diagnosis and treatment intervention.
Diagnosis
The erythrocyte sedimentation rate and the C-reactive protein tests show mild to marked elevations. Vasculitis, if present, can be detected with imaging tests and confirmed with tissue studies. While there are no formal diagnostic criteria for Cogan’s syndrome, it is diagnosed in patients recovering from respiratory infection with rapid-onset sensorineural hearing loss, eye inflammation, and symptoms of vasculitis. In a few cases, Cogan’s syndrome has been associated with Chlamydia infection, with patients showing high titers of antibodies to Chlamydia.
Treatment
Anti-inflammatory eye drops are used in cases of mild eye symptoms. If signs of infection occur, antibiotic eye drops can be used. In severe eye disease, oral corticosteroids or immunosuppressive medications such as cyclosporine or cyclophosphamide may be used. In cases where the eye’s blood vessels are damaged, surgical intervention and corneal transplant may be required.
If excess fluid in the inner ear affects balance, diuretics may be used to reduce fluid. Antihistamines such as diphenhydramine (Benadryl) are also used for problems with imbalance. Cochlear implants are used in cases of hearing loss.
Resources:
James Grant, Otolaryngologic Manifestations of Systemic Disease, UTMB Grand Rounds, 2001.
Cogan’s Syndrome, National Association for Rare Diseases
S Van Doornum, G McColl, Prolonged prodrome, systemic vasculitis and deafness in Cogan’s Syndrome, Annals Rheumatic Disease, 2001,
The copyright of the article Cogan's Syndrome in Autoimmune Disease is owned by Elaine Moore. Permission to republish Cogan's Syndrome in print or online must be granted by the author in writing.
I am 31 and was diagnosed with Cogans 2 years ago.I have had 3 strokes and
cochlear implants. I constantly have inflammation in my eyes.
May 9, 2008 8:15 AM
Guest :
I am 60 yr. old,just a few weeks ago I had the answer to what happened to
me more than 40 yrs. ago.At that time (1967) no one had answers for me.I
lost over half of my hearing and have worn hearing aids since but never new
just what happened to me.One visit to an otologist (looking into a cochlea
implant)and I had my answer.I can't tell you what that was like.Now I
continue to research this.
May 11, 2008 12:25 PM
Guest :
Sep 6, 2008 6:23 PM
Guest :
I am 26 years old and i have cogan syndrome now since 2 years (august
2006). My second "attack" was last april this year (2008). They
threated me with a lot of prednison. After a while my sight is coming
back. Still less sight at my left eye because of the cornea etc. I am not
deaf, i have little hearing problems, since almost 2 weeks i have poor
balance. The rest of the signs like vomiting and weight loss were in 2006
but did not return. Till now they checked the other organs but still no
signs. I hope there is somebody with the same disease who will tell me some
more. greetings
Sep 14, 2008 5:07 PM
Guest :
i was 18 at the time and was suffering severe fever and sweats i loss
hearing in both ears to the ponit where i was deaf. and my eyes where blood
shot.it took toronto sick kids three months to determain what it was i'am
now 25 and have no symptons of the disorder.i was the first case at toronto
sick kids in 150 years.
Sep 20, 2008 9:54 AM
Guest :
I'm a 47 year old male, and was diagnosed with Cogan's Syndrome Friday the
13th of February, 1987 (I still remember the date).
I had light
sensitivity and very sporadic dizziness issues for 2 weeks prior. All of a
sudden within 24 hours I was terribly ill with vertigo and violent
vomiting, and lost the hearing in my left ear overnight. My retinas were
bleeding I was told as well.
After 2 weeks in the hospital
receiving IV Solumedryl, I was released and recuperated at my parents home
for a few months. I was told that if my physician had not recognized the
symptons and took action that next morning, I would have been blind and
deaf by the end of the weekend.
For years I was always worried
about it reoccurring every time I was slightly dizzy, but it's now 21 years
later and I never worry about it. I got away with a loss of most of my
hearing in the left ear - my eyes and right ear are fine. I am incredibly
lucky.
I owe my life to a set of doctors here in NY who I
haven't seen in years; Dr. Sandy Friedman, Dr. Joel Sugar, Dr. Harry Spira
and Dr. Sarah Johnson. I hope all are well.
Mark H., New
York.
Sep 21, 2008 4:18 PM
Guest :
Wow, after reading these comments, I feel compelled to write. I run a
support group for people with cogans syndrome. www.coganssyndrome.info and
can be reached at uscogans@juno.oom. it would be a delight to have any
coganite write me and learn how we survived the treatments, social impacts
of the illness and deafness and alternative treatments as well. Ever want
to meet someone just like you?? this is the place. Don't be a stranger.
A. Yuppa, Founder/Administrator. CCN since 1989.
Sep 29, 2008 11:45 AM
Guest :
iam 32 year old i had diagnosed since i was 26 years old i lost slowly one
ear and the other one also the other one 2 got problem with my eye the
left the first time and the next time again both eyes but the left was
extremely bad i whent to the eye doct she said you have cogans and later in
3 years i did cochelear implainted it whent well the right ear i take
steroids and eye drops and anti immune system medicines i hope in the
future to go the infaction
Oct 17, 2008 5:29 AM
Guest :
Amazing, that I found this web site and all of these comments! Eleven
years ago,on April 1st (yes, April Fools Day) when I was 48 yrs old I woke
up with my entire left side of my face totally numb with complete hearing
loss on that side. That started on a 3 year journey trying to figure out
what was my problem. After being treated with "lethal" doses of
dexamethasone for a very long time, some of my hearing did come back but
during this time loud sirens and numerous other tinnitus sounds became a
part of my life. When I started falling over and had horrible vertigo I
had to quit my job as a dentist. I fell over 4 times in one day in one of
my operatories. Then when I got eye involvement with painful anterior
scleritis, I was rererred to The Cleveland Clinic where a rheumatologis,
Dr. Karen Rendt said it was completely plausible that I had Cogan's
Syndrome. Going through 2 days of balance testing showed definitive
vestibular loss as well as having the hearing loss. I am now on private
disability due to my imbalance which continues and my rheumtologist is
treating me with methotrexate which defininitely has helped the pain in my
eyes. Now after 9 years of therapy I am no longer depressed. I do
continue to have problems.
Oct 31, 2008 5:11 PM
Guest :
I am 37yrs old and have just been diagnosed with Cogan's Syndrome. I
woke up one morning and my left eye was so painful i couldn't open it.
After a visit to the opthamologist it was discovered I had ulcers on my
cornea as well as thinning of my cornea. We tried topical steroids,anything
actually to try and control the severe eye issues. I was refered to a
rehumatologist and started he started me on cyclosporine.I have severe
hearing loss as well.This has been a very long 3yrs me and my family. I am very lucky though as I have a fantastic team of doctors!!!! Support
is so very important from both family and doctors. I think that
depression is something that most definately inevitable with this
disease.How could it not be? It's alot to deal with quickly and all at one
time! I'm glad to have found this web site and I give good wishes to
everyone!
Nov 18, 2008 5:47 AM
Guest :
I am the same person from 26 years old that wrote tekst at sept 6, 2008. I would like to know which persons where diagnosted with chlamydia and
which persons were haven other problems (like herpes simplex or herpes
zoster). I have still problems with my left eye. Sight of 30 procent. Does
anyone know an other treatment than prednison to help me? Or is having
other treatment to get higher immunity system? thanks for reactions.
greetings p
Nov 21, 2008 6:53 PM
Elaine Moore :
Hi, chlamydia is reported to be a trigger of Cogan's. See
http://www.ncbi.nlm.nih.gov/pubmed/15904966 I'm not sure what the
percentage of people with chlamydia is. In this article I mention several
other treatments that are used besides corticosteroids. Best, Elaine
Dec 29, 2008 7:52 PM
Guest :
My daughter was diagnosed with cogans when she was 6. She started out with
a fever for 8 days and from there she had inflamation on her corneas. Had
balance problems, aorta symptoms, vomiting and a high sed rate for 5
months. When we noticed hearing loss she was diagnosed. Started on
Predisone and methotrexate and eventually started Homeopathy which lead to
getting off the drugs. Was in remmission for 3 years and is now back on
same drugs. ENT doctor suggested shooting steriods in her ear to get her
off the oral Predisone. Has anyone ever heard of this working?
Jan 8, 2009 3:47 PM
Guest :
I am 26 yrs. old and was told a week before christmas (So only about 3
weeks ago) that I have cogan's. I have been battling hearing loss for the
last 5 yrs. I am deaf in my left ear and slowly losing my right ear and
have had eye problems throughout the last 5 yrs. I have been on and off
steriods for the past 5 years and now the doctors are going to try other
drugs to see if they can save my hearing. I have been doing my research but
boy this syndrome is scary. I dont even know where to start any comments
from anyone about how they deal with this would help I am supposed to go
back to work in less then a month but I really just want to sit in my room
in bed all day and cry! all the best to everyone!
Jan 8, 2009 3:47 PM
Guest :
I am 26 yrs. old and was told a week before christmas (So only about 3
weeks ago) that I have cogan's. I have been battling hearing loss for the
last 5 yrs. I am deaf in my left ear and slowly losing my right ear and
have had eye problems throughout the last 5 yrs. I have been on and off
steriods for the past 5 years and now the doctors are going to try other
drugs to see if they can save my hearing. I have been doing my research but
boy this syndrome is scary. I dont even know where to start any comments
from anyone about how they deal with this would help I am supposed to go
back to work in less then a month but I really just want to sit in my room
in bed all day and cry! all the best to everyone!
Jan 15, 2009 9:37 PM
Guest :
I'm 32 years old and was diagnosed with cogan's syndrome in late 2006. It
all started on June 11, 2006 on a graveyard shift at 2am, where i started
hearing a high pinched tone in my left ear. A couple of hours later, I
stumbled forward as if someone had pushed me from behind. When I woke up
that day around 3:30pm I had severe vertigo and was floored literally,
along with this intense tinnitus and hearing loss. I experienced vommiting
the day after, tinnitus, balance loss and vertigo. Two weeks later it hit
my right ear with roaring sounds, high pinch tone combinations along with
vertigo, balance issues and also hearing loss. It was in September of that
year when my eyes where affected, with painful redness in my left eye, then
my right a few days later and reoccured 2 to 3 times in each eye. Luckily
my eyes are normal so far with a little blurriness in the left, but
otherwise no problems since then. I was fitted with hearing aids in
November 2006 with about 50% hearing loss. I'm on the medications
prednisone, cycosporine along with remicade infusion at different doses. In
the last 6 months they've tried self injections with embral with didn't
seem to help me (8 injections over 8 weeks). Between the end of July to end
of September 2008, they tried direct injections in my left ear of
dexamethsone over a 9 week period, once a week, with very little success in
restoring my hearing. In the last 2 months they've added methotrexate in
hopes that they'll find the right combination of meds to keep me from
lossing my hearing completely. I've been positive and keeping up with the
meds and trying all the combinations the docter's give me in hopes of
possibly getting my hearing back. I told my docter's that i'm willing to
try anything, even if it's a small chance, to try to get back my hearing.
Thankful today that in the last 8 months i've got my balance back
completely and that my sight is near normal, where on the flip side my
hearing continues to decrease where my hearing aids aren't working to well
anymore. Still fighting the good fight to try to end this. Glad to find
this site and have the opportinity to share my story. Anyone else's hearing
change at all, like good days and bad days vice versa? Does anyone have
tinnitus 24/7 and the sounds change or increase/decrease in volume? Would
love to know what worked for treatments in others and will post if/when i
get out of this. Think positive, have hope, good luck to all who's dealing
with this syndrome.
Jan 16, 2009 9:05 AM
Elaine Moore :
Hi, Thanks for taking the time to share your experience. I'm glad your
vision is responding well to treatment and hope your hearing improves. Ask
your doctor about low dose naltrexone. It's definitely worth a try. My
book, The Promise of Low Dose Naltrexone Therapy came out last month and
explains how LDN works in autoimmune diseases. It would need to be used
off-label, side effects are minimal (I work graveyards and take it before
work); and it's inexpensive. Best, Elaine
Mar 25, 2009 6:18 AM
Guest :
I am 29 and was just diagnosed this past week. My hearing still hasn't
improved and the vertigo is still there. I really don't know what to
expect from my life at this point. I'm just thankful I live next to the
Mayo Clinic and have great family support. The more I read the more scared
and depressed I feel. Right now I am on disability and am taking
prednisone meds for my eyes and ears. It looks like my eyes will be fine.
The worst parts of this disease is the absolute suddenness and that fact
that you can't prevent it at all.
Mar 25, 2009 10:16 AM
Elaine Moore :
Hi, A sudden diagnosis can be difficult to take in all at once. If you
look in about the 4th post down, you'll see some links that I think you'll
find helpful. Often, people with autoimmune disorders get some of their
best information from fellow patients. You're fortunate to have a good
support system and a close proximity to Mayo Clinic. Best, Elaine
Sep 23, 2009 2:02 PM
Guest :
Hi I am 28 yrs old and have posted on this page before. I have
cogan's and am deaf in my left ear and have 40% of my hearing in my
right. I have started a facebook page called "Cogan's
syndrome" So far there are 13 member most whom have cogans. It is a
great place to chat and get help also to give support I ask all those who
have cogan's, friends and family to sign up it is free. All the best
to everyone
Sep 23, 2009 2:58 PM
Elaine Moore :
Hi, Thanks for sharing your Facebook Cogan's Syndrome information.
It'll be a help for others. Best to you, Elaine
