When Scott and I decided to have children, we knew there was a very good chance our children would have a hearing loss. Scott has a hearing loss called Otosclerosis (a hardening of the bones in the middle ear) that runs in his family. Usually, this hearing loss does not present itself until adulthood. We never dreamed we would have an infant with hearing loss.
Joseph was in the NICU for 228 days and failed his newborn hearing screening as well as the follow up screening. We were told by the NICU staff this is very common and that once they are out of the hospital, they usually pass the test. I filed that away and assumed that would be the case. We would leave the alarms and beeps and busyness of the NICU and once Joseph was in a quiet environment, he would be able to hear.
Joseph developed a near fatal bout of Necrotizing Enterocolitis (NEC) at five a half months of age (actual) while still in the NICU. After surgery to salvage 41 centimeters of small intestine, we were told that Joseph would need to receive Gentamicin to help fight the infection. Because Gentamicin is an ototoxic drug, there was a very good chance that Joseph would lose his hearing as a result of the drug. It was either he has the drug to have a chance at life and lose his hearing, or he doesn’t have the drug and he doesn’t survive. It was an easy decision for us to make.
Several weeks later, we were allowed to take Joseph to the hospital garden. It was one of the first times that it was just Scott, Joseph, me, and his medicine pole full of bags and machines. There were no nurses or doctors. It was just the three of us as a family – a day we had longed for.
We were enjoying a beautiful spring day in the garden and Joseph was sound asleep in the stroller. A helicopter began to land on the hospital. The sound was deafening and Scott and I both covered our ears. I bent down to cover Joseph’s ears and realized he was sleeping through the incredibly loud noise. He didn’t move a muscle.
Our baby was deaf. He didn’t hear anything. Scott and I just looked at each other and without saying a word we knew he had lost his hearing. We hoped and prayed it wouldn’t happen. We refer to this day in the garden as our “Mr. Holland’s Opus” moment.
We headed back inside to the NICU and neither of us said a word. Our child could not hear. What next? What will we do? We are both musicians and the realization that we, two musical parents, would be raising a deaf child was almost too much to comprehend. Would he be able to enjoy music? Would he be able to sing? Would he even be able to lead a normal life?
We told the nurse what happened as soon as we got back to Joseph’s room. Within the next day, a non-sedated ABR and OAE were performed by the hospital audiologist and the ENT came to see us. Joseph was diagnosed with moderate to severe Auditory Neuropathy.
Auditory Neuropathy is a very frustrating hearing loss. One minute he can “hear” and the other minute he can’t. With Auditory Neuropathy, there is a disconnect between what the ear hears and what the brain understands. There is a “misfiring” in the auditory nerve. I often compare his hearing loss to driving down a rural country road and trying to listen to the radio. The connection keeps fading in and out as you adjust the dial. That is Auditory Neuropathy.
We were told that the prognosis for Auditory Neuropathy is difficult to predict. Some patients are candidates for cochlear implants and some are not, hearing aids often don’t work as they just amplify the “static,” some have to rely solely on sign language – it would all be a wait and see approach. There is no “cure” for Auditory Neuropathy and no set path – every case is different.
Joseph received his first pair of hearing aids just days before his first birthday. A few months later, he began Auditory Verbal Therapy. Eventually, Joseph plateaued and was not making any progress. We made the decision for Joseph to receive a cochlear implant in his left ear. He received his implant a few months after his third birthday.
Today, at the age of four, Joseph attends an oral school for children who are deaf/hard of hearing. He wears a cochlear implant in his left ear and hearing aid in his right ear. He does not use sign language and communicates using listening and spoken language. While his diagnosis of speech apraxia complicates his hearing loss, Joseph is beginning to speak in sentences.
Joseph continues to amaze me. His expressive language is growing by leaps and bounds. He loves listening to music. He talks all the time. Despite our fears after his diagnosis, Joseph will be able to lead a perfectly normal life. We are blessed.