Warning signs?

Following my consultation in London, I took the vasodilators as instructed. These however, made me feel even more light-headed and weak. All my blood tests came back normal with no deficiencies and no evidence of an autoimmune disease.

There is relatively little information on the internet about Cochlear Hydrops; also known as Endolymphatic Hydrops, which is a secondary form of the more documented Meniere’s Disease. I was reading everything I could find about this condition, and was particularly interested in other people’s experiences of it. Often experiences were negative, and the occasional positive ones were stories of how people were noticing a reduction of pressure in their ears, or experiencing less attacks of vertigo (in relation to Meniere’s disease) by cutting out caffeine, alcohol and salt from their diets.

I decided to try eliminating alcohol and caffeine from my diet, and to eat as little salt as possible for a month. After the month I began to introduce things back into my diet to see if they had any effect on the pressure in my ears or the tinnitus. I realized that wine made my tinnitus more loud and lively. However, I was willing to deal with the occasional bout of enraged tinnitus, as I enjoy a glass of wine at the weekends, and especially with a nice meal. My boyfriend reminded me about getting the balance right. OK, I could cut out all these things from my diet, but it there were no real benefits, then why do it? Nobody had given me a definite diagnosis of the cause of my hearing loss or of the Hydrops; if it was in fact Hydrops that I had. Even though the specialist had diagnosed Hydrops, there is no actual test for it: it is a diagnosis based on my symptoms and history that was supported by the results of the electrocochleography (ECoG) test. If I did have Cochlear Hydrops, the specialist had told me that the cause was unknown. I was willing to try things to see if they helped, but I had to be mindful of making sure I wasn’t cutting things out of my diet just for the sake of it. My quality of life was also important. I enjoy coffee on a Sunday morning. I enjoy salty Marmite on toast at the weekend, and I enjoy a glass of wine with spaghetti and pizza. I was already miserable from my sudden hearing loss, why dampen my spirits any more?

As the days and weeks passed, following the ‘diagnosis’ of Cochlear Hydrops, I started to remember events in my life where I had experienced ‘attacks’ of dizziness and tinnitus and fullness in my ears; the main symptoms of this illness. These memories spilled steadily into my thoughts.

There was the memory of collapsing on a flight to Rome, nearly 8 years ago, when I was traveling there with my sister. I had felt a little dizzy and sick, and as I stood up to go to the toilet, my body felt heavy and my legs felt weak, and I fell down to the floor. A few minutes later I found myself sitting at the front of the plane fastened in tightly with a seat belt across both sides of my body; like a child’s car seat. I had vomited, and my sister was with me looking concerned. I felt fine a few hours later, and I put this down to tiredness and the fact that I sometimes have travel sickness. Maybe this had been a sign of Cochlear Hydrops?

There was a time about 10 years ago when I was in a greeting card shop in England. I was on my way out of the shop when my body suddenly started to become really hot. My eyes couldn’t focus and my ears felt stuffy, and I collapsed to the floor. After what I assumed was only a few seconds later, I was able to get up and walk out of the shop and go home. I had consulted my doctor at the time about this, and she had done some blood tests to check my iron levels, but the test results were normal. Maybe this had been a Hydrops attack?

There was even a time around 15 years ago I was walking to university, where I remember feeling overwhelmed by dizziness. My vision was blurring and my hearing became muffled. I fell into a parked car. Also at university, many times sitting in lectures I remember feeling dizzy. It was as though the floor was constantly moving upwards in a curve towards me.  I was having difficulties sleeping at the time, and spoke to my doctor about these things, and assumed these episodes were due to tiredness. Maybe they weren’t due to tiredness; maybe these were the early warning sign of Hydrops?

During the Christmas holidays, 6 years ago, I had 2 ‘attacks’ very close to each other: this was around the same time when my tinnitus (that lasted 3 years) first began. I was extremely stressed in my job and put everything down to stress. I actually thought I was having migraines. I was staying with my sister and her boyfriend, and I was about to make them a lasagna. I reached for a knife and a chopping board, and then all of a sudden everything started to spin and I felt like I was going to vomit. I had to go immediately to lie down. A week or so, just before or just after this attack (I can’t remember), I was staying at my parents’ house, when I experienced the most scary of all ‘attacks’.  I went to the kitchen to make some beans on toast for my sister. Again, I was standing at the kitchen worktop, when I sneezed. At the same time, I felt a twinge in my neck, and then the scariest sensation I have ever felt. I had an awful feeling of dizziness and sickness that came on immediately. My body felt heavy and numb and I felt like things were beyond my reach of control. I vomited and had to go to bed. I slept and slept. Were these Hydrops attacks?

More immediately, last winter, every time I went for a walk in the city, I would feel dizzy and tired and weak.

Was it possible that these were all signs of Cochlear Hydrops?

The specialist in London had said that, the fact that I had tinnitus for three years, was probably a sign of the presence of Cochlear Hydrops. Doctors and physiotherapists at the time had told me that it was a Pulsatile Tinnitus; probably due to an ongoing problem I have with my neck. In actuality, the tinnitus subsided suddenly one day when I had returned home from a run, and stretched my neck really far to the right (the opposite direction of ear with the tinnitus – my now deaf ear). Hence, a problem with my neck, being the cause of my tinnitus, seemed very plausible.

Maybe these were all warning signs? Maybe my body had been trying to warn me for years that I would finally experience the sudden hearing loss in my left ear? But maybe not. I still didn’t have any definite information regarding a cause of my hearing loss. I wanted to keep trying to find the reason for my hearing loss. For peace of mind, if there was something I could do to prevent any further damage happening, in order to protect my right ear – my only hearing ear – then I wanted to know what I needed to do.

A second opinion (and lots of medical terminology)

It was early in the new year, and my boyfriend and I had traveled to London for a second opinion about my condition. We arrived at the Ears Nose and Throat clinic, and were asked to complete a basic information form and to wait in the waiting room. The waiting room was enormous. There were plush sofas around the perimeter, and an elaborately decorated Christmas tree that dominated one side of the room. At the end of the room on the left, was a drinks machine that served all types of teas and coffees; all free of charge. We had done our research to find a specialist with lots of experience and with expertise in some of the issues I had been having. We were hoping to gain some more information and some understanding regarding my hearing loss.

When we first met the specialist, my immediate thought was that he looked older than the photo on the clinic’s web-page. He was smartly dressed; wearing a dark suit jacket, white shirt and a tie. His hair was dark with a generous scattering of flicks of silvery grey; additions to his jet black hair that had not been captured in his professional photo. As we entered the consultation room, he greeted us by shaking our hands. He dropped his pen as he walked towards his desk, and stooped down to pick it up. He had the slightly awkward air of a genius. He immediately starting asking questions and scribbling down information on his notepad. He was eager to see some previous medical notes, and grabbed at what I had brought. He continued to scribble down information. Whilst writing, he kept looking up at my face, and commented more than once telling me that he thought I was very pale. He had the nature of an accomplished professional, who seemed to be trying to find an answer to my problem, at an accelerating pace.

He started with some unusual tests. He asked me to stand up and I accompanied him to the corner of the room. He commented on my size saying, ‘there wasn’t much of me’. I was asked to walk in a straight line; stand still and balance; and to close my eye, put my hands together in front of me and march on the spot. When he asked me to stop marching, I opened my eyes and found that I was no longer looking at the same part of the wall as when I had started marching; I had rotated about 45 degrees. My boyfriend found this amusing. This test showed that I was somewhat off-balance. He also asked me to sit down and look him in the eyes, as he flung me from side to side. He asked me to lie down on a chair in a small room that was attached to the main consultation room, and again I had to look at the bridge of his nose as he flung me from side to side. This left me quite dizzy, and when he let go of me I swayed slightly to the left. Next I went to have some hearing tests. These were the usual tests that I had done so many times, and also tests of the middle ear, including a tympanometry and Eustachian tube function test.

After a short wait, we went back into the consultation room. The doctor told me directly, that I had lost 90 percent of the hearing in my left ear. He told me that there was also a small hearing loss in my right ear. There was also evidence of significant inefficient Eustachian tubal function in both ears; marked on the right side.  I had never been told so clearly the extent of my hearing loss, although I was very aware that it was severe. Nobody had told me that I also had a hearing loss in my right ear, and this came as a shock to me. He said that as the hearing loss in my left ear was so severe, it would be unlikely that a hearing aid would help me. This was disheartening. The specialists in Spain had given me some hope regarding some kind of aid. I appreciated this specialist’s candidness, even if it was difficult to receive this information. He then commented again about the paleness of my skin. He recommended that I get my Vitamin D levels checked, along with some other blood tests. I said ‘yes’ to all of his suggestions. He also suggested that I carry out a speech discrimination test of my (good) right ear, to see how well I understand speech.

I had my blood taken. Soon after, I was sitting back in the hearing test room, listening to an audio of someone saying words at different volumes. All I had to do what say what I could hear. It reminded me of a test teachers might give to young children or to children who are learning English as a second language. I found this test OK, until the quiet level, where I ended up saying word endings or just a single letter-sound that I could distinguish in the words. Everything happened at such a fast pace.

Then the specialist told me that he recommended me to have an Electrocochleography (ECoG) test of my (good) right ear. This test measures the electrical potentials generated in the cochlea—a part of the inner ear—in response to sound stimulation. He wanted to see if the test would show any evidence for the reason of the small loss of hearing I had in this ear. He stressed the importance of taking care of my right ear – my only hearing ear. So he squeezed anesthetic cream into my ear and we went downstairs to the waiting room for about half an hour while my ear became numb. Yet again, I had electrode pads stuck to my head. He rubbed my forehead harshly with the sandpaper, and was surprised at the sensitivity of my skin. I then lay back on the chair and I was attached by the electrode pads to the computer. An electrode was also fed deep into my good ear. Yet again I had the same feeling as when I had the steroid injections: the scratchy discomfort, deep in my ear and in the back of my throat. Yet again, a series of clicks were played into my ear.

The specialist explained the results of this test. He told me I had a form of a condition called endolymphatic hydrops in my (good) right ear. Endolymphatic hydrops (also known as Cochlear hydrops) is thought to be an early form of Ménière’s disease. Basically, it is a problem with the fluid in the inner ear. This diagnosis explained the pressure and feeling of fullness in my ears; the tinnitus; the hearing loss; and dizziness I had been experiencing. The specialist thought it was likely that the hydrops was also the underlying pathology affecting my left ear six years ago, when I had begun to experience tinnitus that had lasted three years. He felt I had probably had Cochlear hydrops in my left ear all these years, and it had subsequently resulted in my sudden sensorineural hearing loss.

After five hours of tests and consultations, we had a lot more information and a bit of a plan. I was to wait for the blood test results. I was going to take some more strong antiviral medication for 5 days, in case of the hearing loss being a result of a virus. After completing the anti-viral medication I was then to start inner ear vasodilator treatment by taking a medicine called Serc 16mg, for 4 weeks, to see if this had any effect on reducing the pressure in my ears.

Testing testing

Eight weeks after experiencing sudden hearing loss in my left ear, I was back at the hospital to have a test called a Brainstem Auditory Evoked Potential (BAEP). I had read that this test involves monitoring responses to a series of ‘clicks’ using electrodes positioned on the scalp. In my case the BAEP could be used to assess conduction through the brainstem and auditory nerve pathways that are not as accessible to other testing procedures. In other words; the results could give the specialists more of an idea as to why I couldn’t hear anything in my left ear.

I arrived at the hospital early in the morning, slightly before the time of my appointment. I was on my own, and feeling somewhat nervous about what was going to happen. I watched as people went into the room where they do the hearing tests. I watched people who arrived after me, go in before me. I guess that due to the amount of time the test was going to take, they saw the people first who were going to have the, less time consuming, routine hearing tests.

My name was called, and a man explained to me what was going to happen. He cleaned my forehead and behind my ears with what I assumed was alcohol, and then used what felt like a small piece of sandpaper to rub the same areas in order to help the electrode pads stick to my skin. Then he stuck two pads in the middle of my forehead, one above the other, and also one behind each ear. My face stung slightly as the alcohol permeated my newly exfoliated skin. The electrode pads were white circles of thin plastic with a silver metal circle and raised bit in the middle; where wires would be attached. They looked like small targets on my head. I asked one of the staff members who, after my many hospital visits, I was now well acquainted with, and who always greeted everyone with a smile and a joke, for something to tie my hair up with. He shortly emerged from one of the rooms and handed me an elastic band. So there I was, on my own; hair roughly tied up with an elastic band and white targets on my head. I waited for the next step of the process. I was sitting in a doorway. Nurses and hospital staff kept passing by, taking a look at me and making little comments such as, “Pobre!” or “Pobrecita!” –  Literally translated as “Poor person!” or “Poor dear!” Every time someone came in or out of the room of the doorway in which I was sitting, the people in the waiting room opposite were presented with me, the ‘pobrecita’, with the electrodes stuck to her head. When the doors slid open, there I was for all to see! I waited for over an hour. They were carrying out the test that I would be having, on a young child, and it must have been taking longer than they had planned for.

When it was my turn, I was shown to a small room with white walls, two chairs, a desk and a computer. I was asked to sit on a chair that was covered with a white sheet. Wires were then connected to pads on my head and behind my ears. I put on a pair of headphones and I was told to relax and listen to the ‘clicks’. My brain was going to do the work for me; I just had to sit there. I was also told that sometimes people fall asleep during the test. Throughout the process I could hear lots of ‘clicks’. Some that were played into my good ear were almost painfully loud. I have no idea how anyone could possibly fall asleep; sitting in a small room with a stranger, with electrodes attached to their head with clicks being randomly played at loud volumes! I had the thought that, what if my brain was picking up the signals from me hearing my tinnitus noises and confusing them with the real sounds that were being played into my ears. Would this show on the results? I’m sure this isn’t the case, as most people with hearing loss also have tinnitus. But it was a thought that intrigued me. The test took about an hour. The electrode pads were removed and I was free to go home.

Three weeks later I was back at the hospital, with my boyfriend, for a consultation with an Ears Nose and Throat specialist. We were there to talk about the results of the BAEP test; whether the steroid tablets and steroid injections had helped me; whether my hearing had improved; and how I was feeling in general. My boyfriend and I had unintentionally placed a lot of hope in this appointment. We hoped the specialist would be able to tell us some more information, or give us a plan of what to try next. We walked into the specialist’s office with all of our questions written down, so I wouldn’t forget to ask them. Things always feel a bit rushed in the hospital, and they are always so busy. My heart sank when I realized it was a specialist whom I hadn’t previously met. I had hoped to see one of the many specialists who were familiar with my story. I had done a hearing test before I went into the consultation room, and the specialist explained that the test results were the same as when I first lost my hearing. He told us that the MRI results were normal: I already knew this as I had received these results weeks ago from my GP. He also told us that the BAEP test had shown the same as the hearing test – that I couldn’t hear. The test results weren’t explained in any more detail.  He said that sometimes there are cases of sudden hearing loss where, within a 6 month period, a patient’s hearing comes back naturally. He told me to wait for three more months and then come back for another consultation. Maybe a hearing aid could help me if there was no change in my condition.

Deflated, we went outside of the hospital and sat down on a bench in a park across the road.  I cried. They hadn’t told me anything I didn’t expect or already know.  I didn’t really expect them to have any more answers to tell us. But I couldn’t help but raise my hopes for a solution. I didn’t want to stop trying things that could help my situation.

We needed to put our minds at rest. We decided to seek a second opinion.

 

Sounds of Madrid

There are days where my unilateral hearing can make me feel so vulnerable. Going outside can be such a challenge, and one that I have to mentally prepare myself for every day. I live in a city, and as soon as I open the apartment block door, I walk out into a mess of noise. Loud deep roars of traffic and the chatter of people forces me into a bubble of isolation. When I am surrounded by sound I cannot sense when there is something approaching my deaf side. I do not realize when someone is walking next to me, or if a bike or car is about to pass me by. When there are background noises, it is difficult for me to focus on conversation; I can’t hear individual sounds clearly, and my ear and head fill with pressure from the sounds, which also adds to the difficulty in understanding speech. If I am outside by myself, I try to keep my deaf ear to the wall, or next to the traffic, so that my good ear has a better chance at hearing noises of significance. When I’m outside with my boyfriend or friends, I have to keep them on my hearing side, and find myself constantly looking at them, to check if they have said something to me.

Traveling on the metro is uncomfortable. The loudness of the train approaching and the screech as it nears the platform can be almost unbearable. Once on the train, there is a bell sound and an announcement of each stop. This bell can be piercingly loud. Before arriving to a station, a recorded announcement says: “Próxima estación [name], correspondencia con línea [number]”: “Next station [name], transfer with line [number]”. Some metro lines have announcements to mind the gap between the train and the platform. Some trains have a whistle sound when the doors close. The metro is a challenging bombardment of sounds. I worry when I’m traveling alone that people might talk to me, and I won’t be able to hear them, or even worse, not even realize they are speaking to me, if they are on my deaf side. They don’t know I can’t hear them. I always worry about seeming rude if I don’t respond to people.

Madrid is a city that is bursting with the music of performers and entertainers. Every part of the city has its own soundtrack. People hop on and off of metro trains; wheeling their portable amplifiers, and various instruments: panpipes, guitars, violins, even miniature pianos. In metro stations, and in fact anywhere in the city, you might encounter a man playing his violin to the soundtrack of Titanic. You might encounter someone playing drums, made of pots and pans or other recycled materials, to a fast backing track beat. You might encounter another violinist, this time without a backing track, and passionately playing a well-known piece of classical music. You might encounter guitarists strumming guitars wildly and singing with raw passion. When walking into town, you can hear a Spanish guy sitting on the side of the street playing his guitar, singing Bob Marley songs in his own unique style; short high-pitched staccato sounds.

This intrinsic creativity, that is present in every part of Madrid, is enchanting. But it is also something that now presents me with a challenge. Now I am trying to enjoy the sounds of Madrid again. Now I am trying not to focus on the tinnitus that the loud sounds of music can bring, and not to focus on the perpetual worry of missing a strangers words. Instead I want to focus on learning to enjoy this wonderfully noisy city that I live, and that I am trying to love again.