Week 22 – A new diagnosis of Autoimmune Ear Disease?

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.




http://www.ncbi.nlm.nih.gov/pubmed/15505770; http://www.ncbi.nlm.nih.gov/pubmed/7934594;


Week 16 – My very first physio (VRT) and steroid shot

VRT gaze stabilisation exercises

Yesterday I had my first Vestibular Rehabilitation Training session – hallelujah. I was eager for this appointment as so many people have told me they started getting better once they started VRT exercises. I gave up waiting for the NHS because life is just too short to be waiting so long for these essential appointments. It turns out that physiotherapy is actually quite affordable in the UK so I just went private – I so wish I had done this months ago.

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Week 15 – My miserable week

My family wanted to take me out last weekend so I agreed to brave a restaurant to see them. I searched out a place that would be carpeted, spacious and quiet to lessen the impact of background noise on me. Luckily I chose well and the restaurant was quiet and empty. But even still it felt so difficult to hear anyone speak because as soon as more than one person in the room talks I cant hear a thing. It’s like my hearing aids just cant distinguish the voices and mashes all the sound up into one noisy rumble. So much of my understanding now depends on the visual element of communication that I can’t keep up on conversations other people are having because I cant see everyone’s faces clearly. It’s disheartening. I feel so lost in this new world without my natural hearing – everything sounds so distorted, harsh and distant. Its such a struggle to deal with the noise, deafness, tinnitus, imbalance and oscillopsia that I pretty much want to just stay home.

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Week 13 – Neuro-otologists – the specialists

I can’t tell you the relief I felt to finally get my appointment at University College London Neuro-otology Department.  Despite all the effort of travelling into London on the underground with vertigo and new hearing aids,  it was all worth it because finally I was being seen by people who knew what they were doing and who seemed to have time for me.

I was passed through a series of tests specifically designed to assess my balance and hearing. These included rotary chair testing, various audiometry tests and Continue reading

Week 11 – My first techno-ears and the END of the ENT

By the end of the next week I just couldn’t cope without a hearing aid and decided it wasn’t an option to wait a minute longer for the NHS to provide me one. Over the last few weeks I’d gone for free consultations with a few private hearing aid centres in my area and had already gathered info on packages and prices. They all seemed to give money back guarantees so I decided to go for it and spend £3,500ish to help me with this new experience of isolating deaf-tinnitus. I was fitted with my new pair of Phonak Q90’s from Boots and was absolutely delighted Continue reading

Week 9-10 – Brain scans, sudden hearing loss and the NHS

By now I had got myself a Sonido Listener to get by in the world. This is a handheld electronic device which amplifies sound. It has an inbuilt microphone at one end which you point at people to make them feel important, and a headphone connection the other end which you hear them through. Whilst it no way makes up for lost hearing, it definitely improved my ability to hear others in a one-on-one situation; saving them from having to shout. The downside is that you are constantly connected to a machine and wires so its not a long-term solution. I can Continue reading

Month 2 – The Dizzy NHS

I don’t know how to describe the feelings you have as you begin to notice all the ways in which you can’t hear; all the ways in which you’re now excluded from life which is lived interdependently with others. It’s like permanently being the one who didn’t get the joke whilst everyone else is laughing. It’s isolating and if you are going through that, I feel for you and I understand. I’m sorry for your loss.

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Week 3 – Dizzy gets ditched

The second bout of Labs was so horrible I didn’t care as much about being medicine-free and I pretty much begged for the anti-dizzy pills. Only I couldn’t hold them down this time. I was desperate for help but since my GP had told me there was nothing anyone could do, I didn’t think there would be any point going to A&E.. The truth is, that if I had gone, they most likely would have treated my hearing loss and saved my hearing. They may have taken my condition seriously and got me seen by a specialist sooner. So many what if’s.

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Week 2 – Labyrinthitus Take 2

For the next few days I carried on laying horizontal . I slept a lot, researched a lot and found innovative ways of functioning without moving my head from the pillow. Those days that I had to make a bathroom visit, were traumatic because I’d get myself there with my boyfriends help, but then get so disgustingly dizzy that I’d fall to the floor and not be able to move again for hours. I remember him wrapping me up in blankets and sliding me into the kitchen so I could catch some of the sun rays coming in through the window. I must have looked pretty scary laying there half dead and I know it really shook him up to see me like that. But we both had faith in my healing and knew it was a blip that would pass soon.

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