Ethan's Story

Our little boy was born with Goldenhar syndrome. Even though he was not officially diagnosed until after his first birthday, we were pretty sure he had it thanks to our internet searches. For Ethan, Goldenhar syndrome looks like atresia of one ear—his ear is completely closed, skin tags on the cheeks by both ears, and hearing loss.

Our son was transferred from the hospital he was born at to one with many specialists. He spent 2.5 weeks in the neonatal intensive care unit (NICU), where he had every organ checked and what seemed like, every test done. Throughout that time, as we observed and interacted with him, it seemed obvious that he either couldn’t hear or there was something cognitive going on. He didn’t react to sounds or talking; he only reacted to touch or light changes. 

Ethan had his first brainstem auditory evoked response (BAER) test a couple days before he was discharged and the test results came back inconclusive. We were told he would need a follow-up BAER test but that his ear with atresia was more promising than the other. I started learning American Sign Language (ASL) right away and felt pretty comfortable with the alphabet before we left the NICU. 

Even though we didn’t know much about what Ethan could hear, we were okay knowing that he hears differently. For me it seemed that if he just couldn’t hear, he could still have a promising future. But other people were not as okay; they tried to reassure me that Ethan really could hear. 

At 2.5 months old, Ethan had his repeat BAER test and was diagnosed with a conductive hearing loss from the atresia in one ear and auditory neuropathy in the other. We understood atresia—sound was unable to enter his ear—but we did not understand how much, if any, he could hear with auditory neuropathy. But it felt reassuring to have a diagnosis.

Around the age of 6 months, Ethan got his bone conduction device. He didn’t have any big response like kids in videos hearing for the first time. I felt a bit discouraged, but as time went on, it became evident that there was definitely a difference of response from Ethan, with and without his device.

I continued learning ASL and implementing it where I could. I used some ASL apps, took online courses, looked up words as needed, and even did one on one ASL lessons with our DHH provider.

Ethan got started with speech therapy, first working on eating/swallowing, and later working on communication of speech and sign. 

At age 3, Ethan started preschool in a DHH classroom. After just 6 months in school, he went from communicating very minimally, mostly just basic needs, to having conversations and chattering most of the day.

We do our best to give our son whatever means of communication best suits him. He favors spoken words but will also sign. Ethan is 3.5 now. He is not yet understood by everyone, but I am so proud of him for how far he has come!