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;


Guest Post – Sue Stevens (age 60) Labyrinthitus in 2014, East Sussex

In January 2014 I was heading for a shopping trip with my daughter in her car. It was a normal day and we were chatting away. However, in a second things became very different. Completely out of the blue I became dizzy. This is a bit of an understatement because it was more like being on a fairground ride where I didn’t know where I was – the right way up or upside down. I was spinning at 100 miles an hour. I thought I was dying or having a stroke at least. I remember saying to my daughter ‘help me’ and she asked me what on earth was wrong. I was then violently sick in her car. I couldn’t get out or even open the window because my body just didn’t seem to know what to do! This was alarming to her and me and she wanted to rush me to the nearest hospital. My home was about the same Continue reading

The sound of music upon my injured ears

So I’m sitting here at home on another summers evening. Keeping busy as usual. Not thinking of what I might have been doing had my life not fallen to pieces from this stroke of difficulty that fell upon me. Not thinking about Glastonbury or all the festivals that I might have danced at had my body not been so drastically injured by this passing invisible unknown thing that, randomly, deliberately or cruelly chose me.  I’m not thinking about how long this might go on for or how I will live a life I want to live without my hearing or inner ear balance or with this awful metal on metal screeching sound in my head.

I decided today that since my hearing has fluctuated, I should not waste any opportunities to listen to music (just in case there is any further decline in future).

If you had asked me before what pastime gave me the most pleasure I would have said music. I was born singing and as soon as I was old enough was playing with records and tapes, recording sounds over and over each other to create music. I learnt flute at school, taught myself guitar as a young teen and then ditched them both for turntables once the dance music scene took me over. I’ve always loved singing but my voice got pretty ruined by 20 years of smoking. I’d always promised myself that if I could quit smoking I’d treat myself to singing lessons.By the time I quit I felt I was too old to start from scratch.

It’s always been one of my biggest regrets that I didn’t do anything with my love of music. About 2 years ago I treated myself to the most beautiful and sweet sounding Breedlove guitar. I fell in love with this particular guitar as soon as I heard it. I yearned for it for a whole summer and went to visit it in Denmark Street so many times that eventually the shop keeper took mercy on me and let me have it with a discount. Unfortunately years later it’s been sitting untouched on its stand. Life got busy with work, boyfriends and other necessities and I’ve been waiting for a quiet time to devote to it.

I’m certainly no songbird, and never really honed skills in any instruments…but nevertheless, music and singing is a pleasure to me without comparison. And so as my hearing deteriorated over the past 4 months, and sounds have became increasingly distorted, I’ve become scared of listening to music and it sounding really awful as then I’d have to face the loss of something so deeply precious to me.

And so I decided tonight for the first time in 4 months, to be brave, and try to listen to some music.

Although the quality is lost, I can hear music when it’s streamed straight into my hearing aids. I cried a lot – because I feel both blessed to be able to hear music at all, but also because I’m so sad that the quality is so awful. Because I am profoundly deaf in the high frequencies, music is distorted and compressed. I’ve lost the quality, the roundness, the smoothness. What I hear is probably a bit like the sound that comes out of speakers you’d buy in the £1 shop – crackly, edgy, tinny. … But I can still hear enough to enjoy the song if it’s straight into my aids.

But I don’t see how I’ll ever enjoy listening with other people as the music would have to be really loud for me to hear it through speakers.  I worry it will sound like the TV – fuzzy, undefined, noise that I cant quite make out. Every situation where there is music playing in the background is going to be uncomfortable for me as I will be excluded from the mutual enjoyment of it and instead have to tolerate its distortion. I didn’t try playing music through my speakers tonight as I have no sense of volume and don’t want to wake the street. I’ll try tomorrow with a box of tissues and update here.

I know there is a lot worse that could have happened. There are far more people that have more significant things to be sad about. But to me, who’s life is now monk-like, unable to go out and be with people because I either hear too much or not enough, my loss of hearing and my loss of music is my sadness tonight. Tonight I play the blues for all that has been lost these past few months – for my partner in crime who left without even so much as a goodbye, for my desirability, freedom, ability to walk, drive, run, dance, hear, listen, respond, communicate, mix and merge in the world.

I’ve done so so well. People I meet compliment me on how well look and how positive and chirpy I am despite whats happened. I really feel I’ve coped amazingly. Tonight I allow myself a little crumble. There is only so long I can be in denial of how much I have lost. My life as I knew it has been taken from me and I’ve no choice but to start again…learning how to live with my poor injured ears in a world.

Here’s a song for us. Ray Lamontagne soothes my soul and I hope he soothes yours to.

I’d really love to hear from others with sudden severe hearing loss. There must be ways of managing to still enjoy music? So far there haven’t been many deafened people respond to my blog and I certainly haven’t come across any people who have experienced it as quickly as me in both ears. Anyone else out there been/going through this experience?

Invitation to guest blog your experience with labyrinthitus, hearing loss or vestibular issues

I’d like to open up a space on this blog for other people to share their experience with vestibular issues, labyrinthitus and hearing loss. It can be so isolating as the people around us don’t understand what we are seeing, feeling and hearing. Personally I so appreciate connecting with others who have had similar experiences and so understand. Its particularly uplifting to connect with people who have succeeded in getting back to ‘normal’ life.

You are invited to write your story here which I will publish as a Guest Blog Post. Continue reading

Week 18 – On a quest for support, equipment and balance

Last week I had my final intratympanic steroid injection. I’m glad that’s over as neither me nor my family enjoyed travelling an hour to hospital to sit there and wait for 5-6 hours each time. It was exhausting and it’s not exactly a comfortable thing to have a needle pushed all the way through your ear drum, squirting liquid into a place that it doesn’t normally go.

But I’m pleased to say that it was all worth it because it lifted the hearing in my right ear. Yippee. Its not as good as it was 3 weeks ago but its good enough now for Continue reading

Small things to remember when you are ill

1. This is not forever

It feels like this has been going on forever. It feels unbearable – the pain, discomfort, restriction, isolation, helplessness and loss of who we were and life as we knew it. The truth is that we don’t know the future nor how long our healing will take, but its not likely to stay this way forever.

We have to be patient and allow the healing process to take place. This is just today, not for the rest of our lives. Lets just get through this day and this week as best we can and not try to prophesize or catastrophize the whole of the future.

As Shakespeare said “There is nothing either good or bad, but thinking makes it so”. Try to pull back from the thoughts which want to distract us from the present moment.

Continue reading

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.

Continue reading

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.

Continue reading

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