I cant believe its been 5 months since I became ill. Time is passing so fast and all I’m doing is going to appointments back and forth trying to get my life back on track. There is so much I want to write about but I just haven’t had the time unfortunately as I’ve had changes in my home and family that have needed my attention not to mention all the stuff of life which constantly needs sorting.
I guess the key thing to mention as its been a while since I updated, is that the intratympanic steroids (week 16) did work to lift up my hearing in my left ear after the drop in May (week 15). This just demonstrates how vital it is to get steroids immediately. How much better might I be now had I got them all the other times I needed them? Shame shame on you NHS for your lack of patient care and the poor training of all your medical staff.
Three weeks ago I had my appointment with the Lupus Unit at Guys Hospital to eliminate the possibility of my illness being an autoimmune condition. The consultant asked me tonnes of questions, pushed and prodded me and did lots of tests on me. He even tried to stick bits of paper in my eyes and leave them there for FIVE MINUTES!!! (no way Jose! Noone sticks anything in my eyes! especially now I am so dependent on them). He concluded that I have no symptoms of any systemic autoimmune disorder but he took a long list of blood tests to double check anyway. I breathed a big Phew and await the results which I will get next week.
But then yesterday I had my appointment with my Neuro-otologist at UCLH. I was just asking her a few questions and I mentioned Labyrinthitus and she said “but you don’t have Labyrinthitus”. ….!!!????? So I asked her with my jaw on my lap “So what exactly do I have?”. She explained that she thinks I have auto-immune ear disease (AIED) or some other genetic disorder on the basis that:
1. Labyrinthitus only ever attacks once
2. I responded well to steroids
3. Labyrinthitus never affects both ears, and doesnt normally cause fluctuating, rapidly progressing hearing loss
5. Labyrinthitus doesn’t cause as much damage to the vestibular function as in my case (mine is completely buggered).
Nice of her to tell me now, in passing, I thought!
I’m confused…. plenty people have labyrinthitus more than once in fact one of the first things the GP said to me is that once you have Labs you are susceptible to getting it again in your life. And lots of people respond well to steroids, that’s why its given for Labyrinthitus. The truth is, the viral cause of Labs has never been confirmed or identified either. So whats the difference?
But one thing cannot be denied…most Labyrinthitus sufferers don’t have hearing loss in both ears. I haven’t encountered anyone yet. And also I haven’t encountered anyone with fluctuating or progressive deterioration of hearing loss, (apart from in Meniere’s sufferers). This is likely the biggest reason she has diagnosed AIED.
She explained that even though I have no symptoms of systemic auto-immune disease (e.g. Lupus) it’s still possible to have an auto-immune attack only to the ear (referred to as primary AIED). There is no blood test for it so even if my blood results come clear it doesn’t mean I’m clear of it.
The drugs they give for AIED suppress the entire immune system and can produce severe side effects. She said she doesn’t want to give them to me yet and instead wants to wait and see what happens. What she means is… we have to wait and see if her diagnosis is correct… and I lose even more hearing.
I left in tears…because I know that AIED can lead to complete deafness. I think I would prefer the diagnosis of a terminal illness over one which isolates me from people forever. I cannot even contemplate this possibility. It’s beyond my ability to bear.
So tonight I started my research. Because it’s such a rare condition and the inner ear cant be biopsied, there is very little research being done. The accessible scientific research papers are too technical for me so I’m going to have to learn more about immunology to understand, but here is basic info I have found so far:
Auto-immune Ear Disease
Systemic autoimmune diseases which can cause sensorineural hearing loss and dizziness:
- Polyarteritis nodosa (PAN), Cogan’s syndrome, relapsing polychondritis, Wegener’s granulomatosis, polyarteritis nodosa, systemic lupus erythematosus (SLE), (PAN), Sjögren’s syndrome, and Behçet’s disease.
- Some infectious conditions, such as Lyme disease and syphilis can cause hearing loss by triggering autoantibody production.
Classic symptoms of primary autoimmune ear disease:
- Hearing loss occurring rapidly over weeks to months (+30 dB)
- Bilateral in most patients (80%+).
- Can fluctuate and stabilize at a certain level, or can progress without fluctuation.
- The overall course is progressive deterioration in auditory function.
- Accompanied by dizziness and imbalance in about 50%.
- Tinnitus and aural fullness which can fluctuate in severity in 25-50%
- Rare – less than 1% of all cases of hearing impairment or dizziness. Precise incidence is controversial as they think 16% of people with bilateral Menieres and 6% of people with any type of Menieres may be due to immune dysfunction.
- One of few forms of treatable inner-ear disorder with a good response to immunosuppressive therapy.
- Options are limited, with steroids being the only validated treatment option.
- Conflicting research as to benefits of Methotrexate.
- Cyclophosphamide is restricted to patients willing to endure the risks.
- The administration of toxic medications to preserve hearing at all costs is a less desirable option than cochlear implants upon total loss.
So far I’ve had the diagnosis of Labyrinthitus – go home and rest (GP); Menieres – take these drugs for the rest of your life (ENT); and now AIED – we’ll put you on seriously toxic drugs when your hearing deteriorates (Neuro-otologist).
I’m a bit speechless right now. Its a tough call to make – to try to save the hearing using drugs which damage the rest of the body. Would you choose your ears or your fertility? Your hearing or the possibility of cancer? I’m pretty anti-medication so the thought of having to take these drugs is a difficult one. Then again I think I would do absolutely anything to save my hearing.
My plan is to do everything I can to naturally heal myself and prevent any further deterioration. I’d love to hear from anyone in the 1% group that have AIED….and welcome any info on healing approaches for autoimmune illness etc.
I’ll update this post as I learn more.