Archives for March 2019

By Mah-rya Proper, South Denver Parent Guide

I will never forget that almost-spring day…the day we finally ended up at the audiologist through Child Find.

My oldest son, Augie, was three-and-a-half years old. He had been having meltdowns and tantrums, and was distracted and impulsive. He wasn’t learning his alphabet, even though his 18-month-old sister could recite it easily. He wouldn’t sit and play games or listen to books like he used to. He didn’t seem to listen to me or, especially, my husband. Yet, one-on-one, he could talk forEVER about his passion of the moment. He actively participated in our silent signing meals. We couldn’t quite put our finger on what was wrong, but it was time to find out what was happening.

Hence the perfunctory appointment with Child Find. Before they could do language testing, they would do a hearing test. A former audiology classmate from my undergrad program greeted me. She would be doing his testing. With my younger daughter in the baby carrier, I followed Augie into the booth and the familiar testing began.

All my experience in sound booths, being a friend of Deaf and hard of hearing people, and a practicing sign language interpreter…all of it came flooding over me. I saw my son’s (non) response to the very first tone, and I realized he couldn’t hear. He was Deaf. I just didn’t know “how” deaf he was at that point. I stepped out of the room so my reaction didn’t show or skew his results. I walked outside and cried. I didn’t cry because he was Deaf; I cried because I had woken up that morning with a Deaf son, and the world STILL didn’t know sign language.

I took some deep breaths, composed myself, and went back in. While the audiologist explained the results, I could see the audiogram and his moderate to severe rising bilateral hearing loss. I’d seen audiograms before, many of them. Cookie-bite. Sloping. Pure tone. Aided. Unaided. This one was different; this one was Augie’s.

The Unknown Age of Onset

I flashed back on his early weeks; he referred on his newborn hearing screen in the hospital. The nurses thought it was “just fluid” since he was born by C-section. In the follow up testing at two weeks old, I sat uncomfortably, still recovering from my C-section, nursing a newborn with a pinching latch. Hours later, when the audiologist finally finished, she said he was “fine”. If I had future concerns, I should follow up with my pediatrician. We left, putting aside any notion that Augie might be Deaf.

Until this day. Until this evidence, in black and white. On an audiogram. I had so many questions. How had he passed his screen, but had this loss?  Our audiologist suspected a progressive loss since he had speech sounds that didn’t match what he could now perceive. I thanked and hugged my former classmate, and walked out.

My head was spinning. When, and why, and how did this happen? How did I not notice?  I had an undergrad degree in this stuff. I had worked with Christie Yoshinaga-Itano on pediatric research published in audiology journals, and here my own kid was late-identified. HE had lost so many opportunities. How long had it been since he had heard us say we loved him?  How long since he had access to language? While I was thankful that I could and had signed fluently with him early on, I mourned the paucity of ASL compared to hours of only spoken English in our household. I mourned my husband and parents missing out on learning ASL when he was a baby–it would be hard for them to get ahead of the game now. I began signing full force, all the time, and my husband began to learn.

Simultaneously, we searched for an audiologist and sought out copies of his newborn testing. I called two major clinics and was told there was a month or more wait for an appointment. With years of lost time already, Augie couldn’t wait. I finally found a former colleague still in early intervention who recommended an audiologist, and I shamelessly name-dropped on her voicemail, sharing our story. She got us in quickly and confirmed the bilateral sensorineural loss. We reluctantly agreed to a sedated ABR, and our wise audiologist recommended making his first earmold impressions while he was under. (He previously suffered through a botched wax removal that made him loathe for anyone or anything touching his ears.) Finally, the infant hearing test results came. About 75% of his OAEs were absent. He should’ve been referred for more testing, but the audiologist wrote her own reason for his results: “small ear canals.” I was livid. He most likely had undiagnosed mild/moderate hearing loss at birth. So much time had been lost.

We hurried to meet with a CO-Hear (regional infant hearing specialists in Colorado) and were set up with the Colorado Home Intervention (CHIP) services. At the time, CHIP services were still available past age three if a child’s late identification and birthday warranted additional support through the summer to get ready for preschool. A Deaf mentor came to read teach us how to share books with him.

What I Know Now

While all these medical and audiological appointments proceeded, I went to work as a VRS interpreter, pondering his future between calls. He was not yet four, and I was wondering where he would go to college. Gallaudet?  RIT? Who would he marry? Would she be Deaf?  Hearing? An interpreter? I felt like his world and options had shrunk to the subset of America who signed. Thankfully, a fellow interpreter and a CODA (Child of Deaf Adults) helped restore my perspective and reframe my thinking. She listened, and reminded me that his world had also enlarged. He now had sisters and brothers in every country around the globe who shared a bond and a deep level of understanding with him. Hands & Voices connected me with another parent of a late-identified child. My friend was right – I needed to focus on the “Deaf gain” versus the “hearing loss”. He gained a community and a first and second language. I gained a support network. We were ready to move forward.

My husband and I enrolled our son at Rocky Mountain Deaf School, a bilingual charter school. Based on his audiogram, I figured he would be a signing kid. While it was easy to find speech therapy, I didn’t know of another place that provided “sign therapy.” He attended RMDS for preschool (in what I call “remedial ASL” for two years) until he came to me and said in spoken English, “Mom, I don’t want to go to a signing school; I want to go to a talking school.” I would’ve never forced my Deaf kid to speak, but here I was forcing him to sign, and he didn’t want to anymore. That was the moment when “what” I knew as a professional was replaced with “who” I knew. As his parent, I’ve been learning about him ever since.

We changed school placements. We did some Listening and Spoken Language therapy with one of the best therapists in Denver (something I would never have pursued under my previous, professional “expert” opinion). We kept signing. We used an FM system without an interpreter. We tried a captioned telephone and VRS. We transferred to a small out-of-district charter with a 504 Plan. In fifth grade, it was clear auditory access alone wasn’t enough. Now he attends a different school with an IEP and an educational interpreter. Through all of this, I tried to expose him to Deaf people, sign, speech, and options. We talk about pros and cons. He gets to make decisions about his access. Last year, he began using an educational sign language interpreter and the Roger touchscreen. This year, he decided to stop using the Roger system but kept the interpreter, when he chooses to pay attention, instead of reading his always-present book.

Next year?  Who knows?

Now I know he is going to be fine, well, as fine as any parent of a thirteen-year-old boy imagines their kid will be. He is smart, kind, passionate, and determined. Good Lord, is he determined. I am no longer trying to project what his future will be; I walk with him as he creates his future. He has given me so many gifts: the gift of parenthood, the gift of parenting a Deaf kid, the gift of parenting HIM. I wouldn’t trade this journey for anything.

My husband and I feel very lucky to feel that we are able to accomplish most anything when we set our minds to it. We decided to marry in 2002 and start our family of four kids soon after.  We did exactly that and we had our first child in 2004.  I left my career as a special education teacher when our first child was born and we were blessed enough to expect our second child in 2006.

The pregnancy was seemingly healthy and normal. The only concern was that he was beginning to be quite a big boy and since his grandfather was 13 pounds at birth, we kept a close watch on that.  Towards the last month, I noticed that baby Noah, a pretty laid back little guy, started to move less and less. We had a few non-stress tests in which he was less responsive than the doctor preferred to see, but there were no glaring concerns. However, the doctor decided the non-stress test coupled with the concern for Noah’s size was enough to decide to induce him a little early.

A Few Concerns Leads to Induction Day

Being induced was quite a bit different than when we had our first baby. Each night I remember going to bed in the last days of my first pregnancy asking myself “Is this it?  Is this the night we will have our baby?” but with Noah, there was no ambiguity. I knew that we would go to sleep Friday night, wake up Saturday morning and welcome our baby.  We went to the hospital well rested that early morning, and excitedly checked in. We preferred a more natural approach, so my doctor agreed to break my water in an attempt to jump-start labor. After four strong contractions, we thought we were on our way… until the contractions came to a stop. So from 9 am until 5 pm, our doctor allowed me to try to augment naturally until finally, she dropped the “P-bomb”.  Yep– Pitocin was needed to speed up my labor.

I didn’t really progress quickly with my first so I told my family and friends who were waiting to leave for dinner. After all, I was in labor for 36 plus hours with our first as she, just like Noah, preferred the occipital-posterior position, fondly known as sunny side up. I had to push for three and a half hours so this seemed totally logical to have everyone leave, right?  Wrong. Shortly after they all left, the nurse came in to check on me and asked how I was doing.  I announced to her that I was great but the baby must have moved and perhaps he was on a nerve because he was now putting pressure on my rumpus.   This is apparently the universal code for ABOUT READY TO DELIVER ANY MINUTE.

We called everyone back quickly and we were ready for business. The doctor sat down and I asked if I should push since I was contracting. She said I could and had one hand in “birth-ready” position and another setting up tools until she abruptly spun around and had both hands engaged in a catching position. I was still anticipating a more than three-hour delivery saga. All of a sudden I was pushing a second time and on my third push, she held up what looked to be a baby. My head was spinning. I asked aloud “was that the baby… the whole baby?  Did I just have the baby?”  WOW.  I was shocked – it happened in a blink of an eye- how marvelous!!!!  They handed the sweet little man to me and I was over the moon and then suddenly my whole life changed in one small, graceful movement of my hand as I gently cupped it around my newborn son’s delicate little head and made a heart-wrenching discovery… he was missing an external ear.

Time Stops on the Road to a Diagnosis

Contrary to the blur of frenzy with the quick delivery, the next five minutes were a blur in an entirely different way. They seemed to creep by with the most stifling slowness as I watched my husband and mother-in-law walk with the nurse to another part of the room with Noah, all examining him with questioning eyes. All I could hear in my mind was the echo of my last words ringing in the air, “He is missing an ear… I think he doesn’t have an ear.”  I heard the reassurance of the doctor saying “Oh it is fine. It is probably folded down from his birthing position; it happens all of the time.”  She meant well and believed it just as I wanted to. I couldn’t believe though because I knew that wasn’t true.  I felt it and watched it in my husband’s eyes as he just looked down on our little angel wondering, as I did,  “how could this happen?”

The next few hours were painful as we waited for information. Noah seemed to check out alright physically but no one could tell us anything so we waited.  A doctor finally came in at our persistent requests and ordered tests but didn’t indicate to us what these were for. We entered into the longest, most horrific night of our lives.

At 6 a.m., they came in and took our little innocent baby and did an x-ray, shortly after an ultrasound and finally an MRI. There is something so wrong about having to watch your newborn, not even a day old, in that machine undergoing diagnostic testing that many go a lifetime without.  Finally, our pediatrician came in to see him. He walked in like a brave knight rides in on his horse and told us the things we so dearly wanted to hear- ANSWERS.

The Unknown has a Name: Goldenhar Syndrome

Armed with literature, he shared that our son has a syndrome, Goldenhar Syndrome, and that it is a clinical diagnosis and has varying characteristics.  He described a facial asymmetry of Noah’s in an underdeveloped jaw bone and torticollis.  He has microtia, a missing external ear. He said that Noah appeared to have been spared other trademark characteristics such as spinal fusion, missing or malformed kidneys and growths on or abnormalities of the eye.  Hence the x-ray, ultrasound, and MRI.

I had held my angel all night and wondered… is this really happening?  We both feared the unknown but once we knew what we were up against we felt we could handle it — all of it. We were going to face it all and raise this amazing little boy. We didn’t know yet how all this impacted him, and some areas we learned about much later as he hit or missed developmental milestones.  We quickly learned that Noah was the important force, not any of his labels. He was our exceptional little boy.

I mentioned earlier that I was a special education teacher, with half of my credits towards an Early Childhood Special Education endorsement, all but one class in my Severe Affective Endorsement all on a Moderate Needs Special Education Masters that I hold… but none of that covers Deaf Education. So I have armed myself these last three years with all of the knowledge I can find to help guide Noah in his journey.  The greatest thing that has helped us was connecting with agencies such as CHIP and Foothills Gateway to receive support and services for Noah.  He has had physical therapy and speech therapy since he was three months old.  We have been so grateful to have these resources and connections to assist Noah and our family as we feel they have a critical role in his success to date.

By the time he was three, he was amazingly skilled in fine motor skills but was behind in gross motor skills. With his severe to profound hearing loss in his right ear (or “little ear”) and normal hearing in his left ear, he used a bone conduction hearing device on his hat, and he still loves it, most of the time.  We had believed he had a heart murmur but learned that it is really a VSD, which is a hole in his heart. We continue to monitor his vision and many other facets of his health and he will have to as he grows older. He decided in his late elementary years to ask us to look into reconstructive surgery for his little ear, and that was quite a journey. My husband and I feel fortunate to feel that we can accomplish anything if we set our minds to it. Noah has been through many surgeries and reconstructions in his lifetime. We are constantly grateful for the remarkable son. We don’t see him so much as the little boy with Goldenhar Syndrome but instead as a charming, adorable, tenacious, determined, funny, thoughtful and bright young man that we know will be even more amazing as he grows.

“Children are not things to be molded, but are people to be unfolded.”   ~Jess Lair