Beginnings, Part I

I was born hearing with Hirschsprung’s, a disease that makes you stop pooping. In slightly more scientific terms, part of your intestine stops working. This means your body can’t eliminate waste, so it just builds up. Without treatment, it can be fatal.

So I was in and out of the hospital for the next two years, and underwent five surgeries. On the last one, at eighteen months old, I got an infection. I became profoundly deaf from the antibiotics they gave me to treat it. My mom thinks it was the kind of thing you can’t know about until it goes wrong. Twenty-some years later, I took a college class on genetics, did some research (read: very intense googling), and now I figure I carry a genetic defect that made me more susceptible to ototoxic medication. This is the long, boring explanation that I wrote in my final paper:

The most common of these mutations is the A1555G variant on the MTRNR1 gene, accounting for 1% of hearing loss in Caucasians and higher percentages in other ethnic groups. The A1555G mutation substitutes an adenine base for a guanine base, interrupting mitochondrial protein synthesis and impeding OXPHOS production. Though A1555G does not always directly cause hearing loss, it does make affected individuals more susceptible to the ototoxic effects of aminoglycoside, which is used in several medications. Kokotas et al. speculate that the A1555G mutation changes the 12S rRNA gene so that it resembles the 16S rRNA gene, which is a target of aminoglycoside action; this may explain aminoglycoside’s pronounced effects on the A1555G mutation (384, 387, 388; Guan 1792).

Anyway. The diagnosis. My parents were stunned. My grandma wanted to sue the doctor and the hospital; my mom didn’t see the point in it: “Money won’t bring back her hearing.” Now me, I’m glad we didn’t sue. The doctor who treated me died of cancer a year later and left behind a wife and two children. Had he lived, I think I’d have liked to go visit him, and tell him I was never angry at him. I didn’t forgive him, because there was nothing to forgive.

The truth is, I can’t miss what I never really had. I grew up deaf. It’s what I know, and it helped shape who I am, and how I see the world. At the same time, it doesn’t define me. I’m me, and that’s all there is to it.

At the time, my parents couldn’t have known that. They knew nothing about sign language, Deaf culture, auditory-verbal therapy… my mom’s only experience with deafness was a deaf girl in college that she barely understood (then again, my mom is terrible with accents). Their introduction to Cued Speech would come a few years later. In the meantime, they had a newly deaf baby, and no idea what to do with her.

A Croaking Dalek with Laryngitis.

What’s up with the name?

Long story short, I am deaf. I got a cochlear implant when I was ten. No, my parents didn’t ask me for my input. No, I didn’t and don’t resent them for it. No, it’s not a cure, and yes, it does help.

The title comes from a late-deafened British member of Parliament, Jack Ashley, who got a cochlear implant in his 70’s. He described the sound as “a croaking dalek with laryngitis,” and the phrase stuck with me. Coming up with unique URL names is ridiculously difficult, so I’m copping this one while it lasts.

No, I haven’t seen Dr. Who yet, and yes, I plan to watch the series.

OK, so what’s up with this blog? 

Well. Most deaf kids are raised with sign language or spoken language– which are often referred to as manualism or oralism respectively (quit snickering)– or a combination of both. Now me, I grew up with Cued Speech. Because it’s not terribly commonplace, there are a lot of misconceptions out there about it, so this blog is my attempt at sorting it out.

Cued Speech? What’s that?

Cued Speech is one of those things that is just difficult to explain because nobody has a frame of reference for it; it doesn’t neatly fit into any one box. The way I try to explain it, whilst floundering all over myself (“no, it’s not sign language, yes, it uses the hands but it’s not sign language, no, it’s not visual phonics, I don’t know why they’re different, they just are, no, you don’t need to voice it, yes, it represents sound but you don’t need to SAY it…”), is this:

Cued Speech is a system of visually representing the sounds of spoken language in conjunction with lipreading. It’s got eight handshapes, with about three consonants per handshape, and four movements/placements with three or four vowels per movement/placement. They’re arranged so that sounds that look the same on the lips are assigned to different handshapes– for example, /m/ goes on handshape 5, while /b/ goes with handshape 4 and /p/ with handshape 1. As you mouth/voice the words, you put the cues together like a puzzle, and presto! Cued Speech.

There are plenty of sites out there that explain it far better than I ever could, and they have video too. The National Cued Speech Association is a good place to start: www.cuedspeech.org. CueEverything has an excellent collection of damn near every Cued Speech video out there, at www.cueeverything.com.

What I’ll be doing here is sharing my experiences and observations with Cued Speech, as well as forwarding research or news related to it, on a regular basis (approximately once a week is my plan) until… I run out of things to say, I suppose. Down the road, I’d like to publish brief vlogs in both Cued and sign language. Whichever way it goes, I’ll post all about it right here.