By Donna V.
Once upon a time there was a family of four — a mother, a father, and two children ages 10 and 4.
The oldest child, Ashley, had exceeded childhood milestones in many ways — walked at 9 months, ran at 12 months, climbed jungle gyms like a monkey at 18 months, spoke in full sentences by the age of 18-19 months, and rode a bike without training wheels at 35 months.
It became apparent the youngest child, Sarah, was very different. She appeared to be "delayed" compared to the first daughter. Sarah crawled at 10-12 months and walked at 18 months but could not walk more than two or three steps without falling. She often bumped her head when she fell. Although she was talking and expressing herself with a few select single words, the connected speech and audibility of those words were not clear.
She received speech therapy through early intervention for a while but then Sarah's parents were told at age 2 she didn't qualify for continued speech services because "testing" put her speech in the average range. Just before age 3, early intervention conducted their periodic assessment and asked "what happened to her speech?" and they started speech therapy two months before she aged out of early intervention. The parents supplemented with private speech therapy.
Meanwhile, over a period of one year, Sarah had three audiological assessments conducted by a world-renowned medical center. All assessments were inconclusive and incomplete. Sarah's frustration in trying to communicate with others was evident. She would throw "fits," cry, and lash out at people. Everyone who tried to communicate or work with Sarah was frustrated. Sarah's parents encouraged Sarah to "show" them what she wanted. This was working, to a degree, but Sarah's family needed answers. The owner of Sarah's speech therapy practice encouraged Sarah's parents to seek yet another audiological exam at Children's Hospital in Lexington. By now, Sarah had just turned 4 years old.
I think you can guess where this story is heading. On the first visit to Children's Hospital, Sarah was diagnosed with bilateral sensorineural moderate to severe hearing loss. Given her diagnosis and the severity of her hearing loss her two speech therapists as well as her parents were shocked given the "advancement" of her speech. Although people could not comprehend Sarah's connected speech 80% to 90% of the time, she had enough speech that did not support that level of hearing loss. As Sarah had passed her newborn hearing screening , her parents, and the medical professionals, needed even more answers.
Sarah was now under the care of Children's Hospital Deaf and Hard of Hearing department located in Waltham. Children's Hospital "fast tracked" Sarah through multiple levels of medical care given her late diagnosis. The first step was to obtain hearing aids for Sarah. Many factors contribute to why people have hearing loss but the hearing loss technology available today helps people lead a productive and quality life.
Let's fast forward to Sarah's diagnosis of Enlarged Vestibular Aqueduct Syndrome (EVA). A devastating (for at least her parents), life-long unstable condition that's inoperable. This condition causes coordination problems, any bumps to the head could cause additional hearing loss and could progress to profound deafness, Ah, ha — "bumps to the head" — that was the key. Remember? Sarah was falling and bumping her head all the time.
Her parents were told Sarah should never participate in any contact sports for the rest of her life. This includes games such as kick ball in gym class, playing basketball at recess, climbing rock walls, avoiding monkey bars in the playground, etc. Anyone have bubble wrap for a very active 4-year old who absolutely loved to do all those things and more? After seeking consultation with Sarah's new Ear, Nose and Throat (ENT) specialist, Sarah's parents decided to take one day at a time and evaluate each situation as it presented itself. Sarah and her sister Ashley are learning sign language through a DVD program with support from their mother who had learned sign-language years ago (who is also trying to teach Sarah's father) all in preparation for the future. However, if Sarah were to go deaf, she would be eligible for a Cochlear Implant.
I am pleased to report that Sarah, my daughter, is doing great! She loves wearing her hearing aids, which she's had for six months, and doesn't like to be without them, even for a couple of minutes. With her hearing aids and continued speech therapy she is progressing and is a pure joy to listen to. As a matter-of-fact, we can't keep her quiet and she continues to hear new sounds for the first time! Her frustrations have significantly decreased and her behavior has significantly improved. She has a great sense of humor and has a never ending smile that will brighten your day. She has many hurdles ahead of her but with the support of family, friends, her audiologists, and Children's Hospital the hope is that she will progress nicely in the years to come.
If you, or someone you love, is having trouble hearing, please seek the guidance of an audiologist or other medical professional. Your ears depend on it!! An actor once said:
"You should HEAR what you're missing."
- Better Hearing Institute
- National Institute on Deafness and other Communication Disorders
- National Dissemination Center for Children with Disabilities
- Enlarged Vestibular Aqueduct Fact Sheet