Monday, January 2, 2012

Calling It By Name:

Some people are born inquisitive, analytical, always surveying the world around them and wanting answers to questions life offers them. I've always been one of those; someone who has a hard time accepting "just because" as an answer. That's an answer I don't like; I like "definite". 

And so, of course, one of the things that has most defined my life, my deafness, has always been a complete mystery. frustrating. For a long time it was almost kind of cool that I had this random deafness, this thing that just happened when I was a small child that nobody had any answers for, this total anomaly, but as time went on I became more and more curious. When I had my children, I was fascinated to discover that they were all hearing, and relieved, to a certain degree. But as a person who was born hearing and then became deaf, I had no security that my children would always hear. 

I didn't have Internet when my first three children were born. I didn't get online until 1998, when Rachel was six months old or so. I joined several online communities but none of them were related to hearing that time I was in a fair amount of denial that I had a hearing loss at all and just had no interest in finding others like me. In March of 2006, though, that changed almost overnight. When I had that first sudden loss that began my journey down the road to complete deafness, I didn't walk, but rather, I ran to the Internet in search of people who had experienced cochlear implant surgery. And at this point in time I became intensely curious about the "why". Sudden hearing loss once in a lifetime was possibly of those things that just happened. But I couldn't accept it twice. I was sure there had to be a reason, and I wanted to know what it was. So I pored over the websites that listed genetic causes of deafness. Connexin, LVAS, Marfan, Pendred's, Waardenburg...just a few of literally hundreds of things that it COULD have been. I ruled out many of them as I read. I was aware that there was a possibility that I'd never know what caused my loss, but I continued to search, hopeful that one day I would turn up something that would make sense. 

Along my journey to cochlear implantation, to deafness, through the identity crises, through second and third implant surgeries, through conventions and new jobs and new friendships, I missed very few opportunities to ask people, "what is your story?". That was usually one of my first questions, right after finding out people's names. And the stories were fascinating. I met people who had lost their hearing due to illness, due to genetic disorders, rare autoimmune disorders, suicide bombers in Iraq. It was second nature to ask. Perhaps someone somewhere would have a clue that would give me the answer I was looking for.

And surprisingly enough, a clue came one night a year or so ago in a chat room on Hearing Journey, when several of my cochlear implant friends with Meniere's were talking about their battles with vertigo.  Someone mentioned the "drop attacks", and suddenly, I thought, "I've had those." My first attack was probably eight or nine years ago, and it was totally out of the blue, in the spring of the year. I was walking through the mall, pushing a stroller, and one second I was standing, and the next second, I was flat, and the world was whirling around me. I was aware that people were standing over me and I was able to say, "I'm fine, I'm just dizzy". That was the first of several that I had over a period of a few months. I went in to see the local ENT about them, and he did an MRI, and found nothing that gave any clues about WHY I was having those attacks. I wanted an answer...I wanted TREATMENT...and the doctor could offer nothing more than, "there's nothing to fix, you'll just have to wait it out." Thankfully, the dropping and whirling stopped within a few months. However, I was left with mild vertigo...not constant, but often enough that I could say that it was an ongoing thing. It happened most frequently in the late winter and early spring months. 

Vertigo began in earnest in January of 2006. I wrote on a previous blog about struggles with it. In February, in desperation over the frustration with vertigo, I stopped taking a medication that I was on in hopes that it would resolve the issue. It didn't....if anything, it became worse. And then, in late March, I had the first sudden hearing loss. And I don't remember the vertigo being quite so significant afterwards...I was suddenly very preoccupied with the sudden hearing loss and the insane tinnitus that had suddenly become a huge part of my life. 

Amazingly, it just didn't occur to me that I had Meniere's. I didn't know a lot about it, but what I did know, or thought that I knew, was that people that had it had ongoing, day in and day out, disabling symptoms. And I didn't fit that category. So I dismissed it. Besides, I was looking for something that was related to the hearing loss I had had as a small child, and Meniere's didn't fit, because it was something that came and least, to my way of thinking....and in my case, there had been nearly thirty years between the first loss and the second could they possibly be related?

Between March of 2006 and March of 2007, I had constant tinnitus, occasional vertigo, and hearing that fluctuated as much as several times a week. And then one day at the end of March, 2007, I heard the staccato fireworks that signaled the end of my life as a person with any natural hearing...on that day, for all practical purposes, I became completely deaf. And I still didn't know why.

You know much of the rest of the story. I was implanted, I had a successful right ear, a not so successful left ear. I adjusted. I learned how to hear again, how to live again. I still researched the causes of hearing loss, hoping for an answer. I still came up short. I still asked "why?" to everyone I could think to ask. I stopped short of genetic testing...I didn't want to pay for something that might not give me an answer. I asked my surgeon, who only knew me as "sudden sensorineural hearing loss", "is there any reason why?" and he told me, "your ears are structurally perfect. There's no reason why they shouldn't work. They just don't." 

I have several friends in my "inner circle" who have followed my journey for a long time....some longer than others. I'm lucky enough to have a few special girl friends who love each other dearly, and we all stay connected here and there, mostly on Facebook. Two of them have Meniere's. And as the weather this fall started to turn, and their symptoms started to flare up, I started realizing how much I related to what they were saying. Then on HearingJourney a few weeks ago, the topic of those awful drop attacks came up again, and that was enough for me....I wanted to know more about Meniere's. So the research began on every website I could find, and every website that I looked at reinforced my suspicions: I have Meniere's Disease. The websites answered every question completely: Drop attacks are uncommon in any other vestibular disorders. Children can have Meniere's, although it's rare. Meniere's can go into remission for years without a flare-up before rearing its ugly head. And one person's severity of symptoms will not match everyone else' it's perfectly reasonable that I have mild symptoms sporadically when other people are disabled by it. Not fair, exactly, but reasonable. 

After one night of voraciously reading websites into the wee hours of the morning, my mom came over to bring lunch and visit. I told her that I had been researching, and she said, "Interestingly enough, when you were a small child, based on the symptoms you had, the doctors said that it appeared that you had Meniere's. But they ruled it out." I asked her, "Why did they rule it out?" and she responded, "Because, they said, children don't ever get Meniere's; it's something that happens to people later in life." I told her, "Based on what I read last night, children DO have Meniere's. It's rare, but they do." She told me then that they had used the words "endolymphatic hydrops" to describe what they were seeing. That term is synonymous with Meniere's today. 

I took all of this information to the local ENT who first saw the "drop attacks" several years ago on December 22nd, and his face lit up in a "Eureka!" expression. "That's it," he said. "I have no doubt. You meet all the criteria for a definitive diagnosis, and your MRI ruled out any other cause of the vertigo. You have Meniere's Disease." I told him what my mother had said about the doctor's findings way back in 1976, and he agreed with us that juvenile Meniere's was probably the cause of my deafness back then. We have no 100% solid proof, but we have a good circumstantial case. He is forwarding all of my information to Vanderbilt in case my surgeon is interested in doing any farther testing for a true clinical diagnosis, but as far as we are concerned, the mystery is solved. 

I'm overjoyed. In one sense, it's not the best possible news in the world...I still have symptoms. I'm already completely deaf, so there's good news there: I won't become deafer than I am now! My tinnitus is manageable at this time...I occasionally hear fireworks (my mother said that I heard the same sound as a little girl) and I occasionally hear high pitched tones and trains (I remember hearing those as a little girl). I never hear them when I have my processor on, and the noises don't invade my quiet time unmercifully like they do some of my other friends (although the fireworks startle me occasionally when they're loud). My biggest problem is the vertigo and the pressure in my ears. True swirling vertigo isn't a huge issue for me but I have a sense of being "off" some days, especially on bad weather days in the late winter and early spring. The pressure in my ears usually accompanies those bad days, and I just have this overall full head, off balance sensation that's more annoying than disabling, although some days it really gets under my skin. It is what it is. It's always been very manageable, and I'm grateful. And it's truly a relief to suddenly be able to see it for what it is, to realize that the sensations and the pressure are all part of the package deal, pieces in the puzzle. 

A diagnosis doesn't answer my questions as to whether or not my children will be deaf. Meniere's can occur randomly...I may be the only person in my family who ever has it. However, it can also run in families....we go to church with one family where the father and son are the fourth and fifth generation of Meniere's in their family. In that sense, I won't ever have any real will happen or it won't. At the moment, the children all hear reasonably well. There are no tests to determine if they have it while they are still hearing. So we continue to wait, but we are no better off and no worse off than we were before, and at least now we know what we're looking for. 

I have a great amount of peace at having an answer for myself. One of my friends commented on Facebook that only about ten percent of people ever know "why". I'm really grateful that I have been given an official diagnosis, a name for the disease that changed my life. As my friend Tom said, it doesn't change the outcome; that remains the same, and I can live with that...I have long since accepted my deafness for what it is. But there is much peace in being able to call it by name. 


Anonymous said...

I'm much like you, Jennifer. I like definitive reasons and certainties. "I don't know" has never set well with me - especially for medical things.

Although I would never wish Meniere's on any person, I'm glad that some answers are more clear for you now that you have an official diagnosis. Because I write about Meniere's so much, I have people write to me about their own symptoms and initial diagnosis. They are confused about why we can have the same thing but our symptoms are so different. Every medical journal article I have ever read about the disease is clear that no two person's symptoms are the same, nor do they have the same triggers. It is, I believe, why doctors are so stumped about it and why there is so little they can truly do for a person with the disease. Like you though, I have found a lot of "hope and help" from simply educating myself and finding people who can commiserate because they LIVE IT. I wouldn't go so far to say that "misery loves company", for in truth we are no longer miserable simply because we've discovered we are not alone. After finding support from others I have fully accepted who I am now and am at a place I'm OK being "me". :-)

Like you, I do worry about my own children - specifically my daughter. From my own research when these things do run in families it is usually to same sex offspring. We pray and hope - and in the mean time strive to be the kind of positive role models "just in case". Amen?


Jeffrey Swartz said...

Very interesting. I actually have pseudo-seizures due to pain in my ears and I pass out for a few seconds until my brain or body heals or gets back on track. Nobody understands. I don't have Meniere's and all neurological test are fine. I am so new to this world of silence. Sometimes scary and lonely and sometimes nice just to have silence.