I am a rare phenomenon! or Huh? What did you say? Excuse me?

I have significant hearing loss.  Not just any hearing loss, but like everything else I have wrong with me, I have a type that is rare.  So rare, that I have searched the whole internet and can’t find statistics about just how common (or uncommon, as the case may be) that it is.

It is called cookie bite hearing loss and it’s almost always in both ears. (Bilateral)  Also sometimes called pool or soup bowl or “u-shaped”.  It is named thus due to the shape of the graph when you plot your thresholds in Hertz and Decibels.   Hertz is the measure of pitch or frequency, and of course decibel is the measure of intensity or loudness.  The full scientific name for my  kind of hearing loss is Bilateral autosomal dominant, nonsyndromic, midfrequency sensorineural hearing loss (with tinnitus).  I did find studies that went into the genetics behind this, which having been an RN, I am somewhat able to decipher, however, the genetic specifics don’t tell me that much.  I also have some very  Menière-like symptoms at times,  of “spinning”  or the world spinning around me, or feeling like I am pitching in an exaggerated manner in whatever direction I mean to move.  It can literally result in falls, incoordination and overall clumsiness as well as nausea (motion-sickness). All I have deciphered is that those two factors (type of hearing plus equilibrium facet) point to not just one, but two different genetic mutations.  Yipee!  And autoimmune factors play a role.  Hair cells die and other structures in the ear are mis-shaped etc.

cookie bite on audiogram

A “cookie bite” result on audiogram

Aging, noise exposure, infections, lots of things diminish hearing over time.  But in a cookie bite situation, we hear some sounds just fine, and blank out other sounds, so that speech can sometimes sound like someone fiddling on the volume control while you are trying to watch a television prgram.  There is distortion of the things you do hear, plus gaps, (accounting for sounds you can’t hear at all), and hearing aids can’t help much with these issues.  Turning the sound way up lets me hear the “quieter” things, but where the louder, or high or low frequency sounds are thrown in there, those can be painful to me when the volume is set high enough for me to hear the softer sounds.

Hearing loss can be devastating to one’s social life, not to mention ability to work, sing, and even keep oneself (and your kids) safe, in some instances.  It has definitely effected my quality of life greatly.  Basically the spectrum of sound I am missing, is the one into which “normal volume” speech falls.  What does that mean for me in terms of socializing?  Isolation.  Even in a crowd, (actually if the whole crowd is chattering, such as in a restaurant, it is worse, because I can’t decipher much of anything) so there I sit with conversation flowing all around me, and I can’t participate.  People tend to get snippy when you ask them to repeat something more than twice.  And most of the time, it takes three, four, maybe five repetitions for me.  It helps if you rephrase what you say, using alternate words.  It helps if you say it in a higher pitched voice, even.  I also can hear lower pitch sounds than most people with normal hearing.

Our brains automatically fill in blanks when any sensory systems malfunction.  Therefore it is possible for me not only to not hear what you did say, it is also very likely I may hear something entirely different from what you actually did say!  It can be comical, and I try really hard to have a sense of humor about it, but over time, the fact is,  it makes me sad.

When I go to the Christmas get-together with my brothers, if it is at my younger brother’s house, the ceilings are vaulted and the floors are hard wood, so it’s very echo-y.  Everyone spreads out for seating, and there are always multiple conversations at once, and probably a television going, and I can’t hear ninety percent of what anyone is saying.  It is also wearying, the literal effort I have to exert in trying to comprehend.  I may ask for someone to repeat something once, maybe twice, and finally I will make some nondescript gesture and mumble that I hope will cause the other person to think I finally heard, or feel so awkward themselves that they move on to other topics or, better yet, someone else to converse with.,

Having those things be a constant factor is bad enough.  But I also have tinnitus.  And it’s not just one sound.  It is multiple.  I have a constant screech/squeal that sounds like the old dial-up connection for internet, or that sound when you dial a fax number on your landline.  Behind that I have what I describe as my “standing on the tarmac by an airplane” sound.  Like the sound of their engine as they taxi away from the gangway.  Sometimes, especially when I first wake up, there is also a whooshing sound.  And adding to my repertoire, in the last couple of years I have started to have musical tinnitus.  Not nice recognizable music, nooo, that would be not so bad.  What I hear is the saound like a child banging out randomly on a cheap electronic keyboard.  I am not sure Chinese water torture is any more madness-inducing than the sounds I hear 24/7.

Click HERE to hear what symphony orchestra music sounds like for me, and notice how difficult it is to even tell what pitch or key the music is playing in.

Click HERE to hear what tinnitis with moderate hearing loss sounds like.

Click HERE to experience some of the many different sounds tinnitus sufferers endure non-stop.  In my case, there is not just that cringe-inducing high-pitched sound, but always three or more other sounds along with.  My sounds are at 1:03, 1:48, occasionally I get the one at 2:03 that sounds like a heartbeat, also 3:40

The ones at 1:33 and 2:28 scare the bejeebers out of me!!!  Glad I don’t have either of those! Some people hear people talking, and that as well as the musical tinnitus, are technically auditory hallucinations, but not the schizophrenic or psychotic kind.  Just the brain trying to catagorize, evaluate and filter odd “sounds” and organizing them into something that makes sense.

The parts of speech that are hardest to hear:  Vowel sounds are lower pitched but generally spoken more loudly than the consonants.  Consonants are higher pitched but spoken at lower volume.  The consonants and combonations of consonants s, f, v, sh, th, ch, are pretty significant in distinguishing the words someon is speaking, but these can all sound the same for me.  Sat, fat, vat, that, chat, all the same.  Fish, dish, wish, swish, ditch, witch, all the same. Goat, note, moat, dote, all the same.

“I sat in the ditch with a goat”, comes out as “A cat in a thick winter coat” or “is that gonna fix the remote?” As you can see, I heard the right vowels, but the consonants were a total “crap shoot”.  Yeah, people with my kind of hearing loss experience a lot of non-sequetors.  When we respond to what we thought we heard, we contribute non-sequitors of our own.  Add that to memory loss, fatigue and fibro fog, and I feel more seventy than fifty-two.

It hurts to have someone get irritated and snappy with me when I ask them to repeat themselves.  This is even worse in settings such as court or a doctor’s office, where staying on scehdule is important.  Court is nerve-wracking and intimidating for anyone, whether it’s just to get your kid’s drivers license, or a speeding ticket.  Doctor’s offices are also very stressful and guess what?  My tinnitus is worse when I am stressed.

With my own family and friends, the ones who KNOW I have pretty significant hearing loss, I get snippy with them sometimes in my frustration, because it does not come natural for them to speak loudly and they forget to do so multiple times in a single interaction.  I understand they can’t help it, they understand I can’t help it, but it’s still an irritant.

Since I have narcolepsy (rare), with cataplexy (even more rare) also, which effect perceptions anyway, you can just imagine how much fun it might be to, say, work in a busy E.R., or cafeteria, or a sales floor on Christmas eve.  Angry customers who think you are ignoring them, when in reality they’re standing on the side of your “bad ear” (in my case, worse ear) and you just don’t hear them.  But when I am straining already to hear one person, I do tend to “tune out” other sounds or voices as best I can.

Hearing loss, unless in babies who haven’t learned to speak yet, is not considered a health issue (by Medicare, Medicaid, and Health Insurances),  therefore hearing testing and hearing aids are generally not covered.  Hearing test are offered free by those who want to sell hearing aids. But hearing aids start around three thousand each, on up to 7 grand depending on the “bells and whistles, (pun intended).  That makes shopping for a hearing aid daunting and subjects you to the dreaded sales tactics of high-pressure salespeople.  I looked for a local place to have one of my hearing aids repaired, and when I got there, it only took sixty seconds of looking at the ream of papers the lady handed me, “before the hearing test” to figure out that “hearing aid repair” was just the pretext for getting people in there.  They would do a hearing test, and then proceed to try and sell a new hearing aid, or preferably two.

Getting hearing aids is not a panacea.  It actually was very hard to tolerate.  It amplifies the speech sounds I struggle to hear, but it also lets me hear the hum of the fridge, the tick of the clock, the scratching dog, the dripping faucet, the birds and crickets, etc.  I rearely could tolerate wearing them for more than an hour or two.  Even though mine were designed and programmed to minimize “background noise” they didn’t do so consistently.  The sensory overload was just as exhausting as the struggle and strain to hear and decipher without them.

That, is my sad saga of hearing loss.  Only one of several conditions that contributes to my “disablement”.

The upside is that the older I get, the more I value silence!  Silence is to the mind, what sleep is to the body.  My silence isn’t actually silent though.  What with the squealing, roaring, whooshing, and cacophany of the orchestra warming up and all.  <Heavy sigh>