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.

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