Cogan's syndrome

[Popple18]

Hello! So I was diagnosed with Cogan's in Oct 23. It all started with severe ear pain after my second COVID vaccine which progressed to hearing and balance issues. This resolved after 3 weeks but I got the same symptoms again after my 3rd vaccine. Again this resolved but returned and gradually worsened, my balance kept going, I would lose my hearing, I had bad tinnitus.

All doctors i saw denied it was vaccine related - 'no evidence'. I was referred to specialist who found permanent hearing loss but overlooked my auto immune history (immunopathic thrombocytopenia and Crohn's disease) and the connection with the vaccine. She said it was a virus.

My symptoms fluctuated but gradually worsened. I lost almost all my hearing permanently in my right ear and now have fluctuating hearing in my left and relapsing anterior uveitis so was diagnosed with Cogan's syndrome. Methotrexate only worked for a few months and now Mycophenolate is becoming less effective. I want to try Rituximab and the Wahl Protocol.

[Oztrax]

Hi was just about to post if anyone has COGANS! I dont have a diagnosis yet , but am leaning towards COGANS for a number of reasons. Have learnt that there is TYPICAL and ATYPICAL types. Can also be associated with vasculitis. I am very interested to learn how your doctors were able to come up with this diagnosis as this is an extremely rare disease and hard to diagnose. What blood tests and markers did you have ?

[Popple18 in reply to Oztrax]

Hi Ortaz, I'm sorry you're heading for a Cogan's diagnosis too - the symptoms are so disruptive. Its a diagnosis of exclusion, there's no test you can do. But I was diagnosed when, in conjunction with my hearing loss and vestibular problems, I also developed chronic anterior anterior Uveitis.

My bloods showed mildly raised inflammatory markers (CRP & ESR).

I had an IAMs MRI of my ears which showed raised levels of fluid (endolymph).

I was also tested for lots of different antibodies but these were all negative (they're sometimes but not always positive in Cogan's).

My left ear hearing loss has always been responsive to intratympanic steroid injections. This is another indicator that the problem is inflammatory in origin.

I've had a full body PET scan and ultrasounds of my major vessels to check for vasculitis but currently all good.

Do you have a good multidisciplinary team looking after you? You need ENT, Audio-vestibular and Opthalmology.

Good luck. Hopefully we'll meet some more Cogan's people on here too.

[Oztrax in reply to Popple18]

I did test positive to HSP70 antibody which can indicate CG. Have had no succes for diagnosis with 4 public ENTs, 1 private ENT specialising in balance, 2 public Rheumatologists and now a public diagnostic physician. Tested ANCA and ANA negetive 2 years ago, so not expecting these to be positve now. Decided on a new approach, concentrate on the vasculitis inflamation in my head and neck, so am seeing and ENDOVASCULAR surgeon, he would know more about blood veesels than these other "hands off" specialists.

[AtopicGuy]

I'd not heard of Cogan's Syndrome before your post dropped into my timeline. This 2023 overview for doctors and scientists covers every aspect of the disorder:

ncbi.nlm.nih.gov/books/NBK5...

[Oztrax]

ATOPIC your link is a reasonable summary, but I did find more info searching and a few youtube medical presentations. Heat Shock Protein is the HSP70 blood test I mentioned and have tested postive for 2 years ago, at that time ANCA and ANA were negetive. Waiting on recent ANCA blood test results ATM. But as you can see vasculitis is not always associated with this, and is multivessel vasculitis when it is present. So symptoms and organ specific issues become hard to LABEL into a specific vasculitis category.

Important thing with CG is the ear and eye involevement, my eye was pretty minor and limited to dryness, grainy and then constant watering. 3 months later the inner ear pain,SNHL,tinnititus and bad vertigo developed. The kind of vertigo where you feel nausea just standing up. I climbed a ladder for a few minutes and ended up vomiting. I didnt see any vasculitis symptoms till 12 to 18 months later when small painful patches started on my scalp, at the same time random local joint pain in my shoulders, elbows , hips and tailbone. By random I mean Left elbow for a month or 2 followed by the other elbow then month later knee etc. Had CT guided steroid injections for shoulders and tailbone which did settle those areas down after a few weeks each time. U/S and CT show bursitis.

My diagnosis will be difficult but with VASCULITIS symptoms and hopefully a positive ANCA test, expecting a result. I am pushing the vasculitis issues as the doctors may be able to diagnose that easier than CG.

I have been on Leflunomide 20 MG for a number of years for PSORIATIC ARTHRITIS before any of these symptoms developed. I find that interesting as this is the secondary medication often used with steroids for CG. Its a DMARD.

In some ways I concentrated too much on the severe fatigue symptoms, which doesnt really help with doctors as its a general systematic symptom from nearly all autoimmune conditions.

Specific symptoms seem to be the best way to progress diagnosis.

[Oztrax]

LINKS:

vasculitisfoundation.org/ed...

vasculitis.org.uk/about-vas...

[Popple18 in reply to Oztrax]

I contacted Vasculitis UK and asked who the best specialist was in the UK for Cogan's. They said Professor David Jayne in Cambridge (NHS). I went to see him and he retested all my antibodies. Still negative but he said this can change, hence retesting. He said the best treatments are Mycophenolate and Rituximab with Rituximab being the most effective. Oztraz, I feel like you need to see a world expert, even if it's an online appointment. Cogan's is so rare.

Also, did you have the COVID vaccine? If so, did it have any impact on your condition? I had 2 x Astra Zeneca then my 3rd as Pfizer.

[Oztrax]

First link is the best info I found

[OldTed60]

I posted this link on Oztrax’s post but thought you too might find it interesting. Good awareness raising for Cogan’s at the time although I felt the emphasis was mostly on the deafness and BSL rather than on the underlying autoimmme disease that’s caused Joe’s deafness: amp.theguardian.com/tv-and-...

[Popple18 in reply to OldTed60]

Thanks so much, I'll watch

[Oztrax]

My symptoms are crazy, vasculitis areas seems to be like GCA, inner ear whilst can occurr with PGA my inner ear issues were ballistic and basically could not stand or sit up for long for maybe 3 months or so. When I saw the Ashton Kutcher's vasculitis on the net, it was said to be "a very rare form of vasculitis" Why couldnt they of said the exact name ? Could help some of us out here battling for diagnosis ( and we are not miliionares who can afford any doctor we choose.) : nbcnews.com/news/us-news/as...