By Kathie Dolce, HBCE

**Orginally published in the Fall 2007 Special Needs issue of The Journal of API

When my granddaughter Anna was already over a year old, her parents began to realize she did not hear. Before this, Anna had made lots of sounds, including “mama,” “dada,” and everything else babies usually say. She seemed very tuned-in to everything around her, knew when her mom came home and made the sign to nurse before mom even came into view. But she had never startled to sound of any sort.

Her older siblings played a game of coming up behind her and tapping her shoulder, whereupon Anna would spin around and laugh hysterically. Her parents realized that she did not hear her siblings sneaking up on her. As Anna stopped vocalizing, her parents became concerned that she was losing her hearing.

Her audiogram at 15 months showed Anna to be profoundly deaf and subsequent CT and MRI imaging indicated that she was missing more than two-thirds of her cochlea – inner ear structures that form at six to nine weeks gestation. She was also missing the acoustic nerve on one side. Anna never had hearing. (There is no prenatal testing or imaging that could have shown this.)

The vocalizations Anna had been making were just a developmental stage all babies go through, regardless of their ability to hear sounds. Babies will make sounds for every language in the world, and then imitate the sounds that are repeated or heard when others speak. Eventually, they lose the sounds not used in their family’s language, and in the case of deaf children, they lose interest in vocalizing because they never hear a response. There was no history of deafness in Anna’s family, which is true of 90 percent of all children with hearing loss. Before federally mandated screenings for newborns, the average deaf child was not diagnosed until age three.

New Parents, Request a Hearing Screening for Your Newborn

Federal law mandates hearing screening be available for all newborns, though not all states make it available free of charge. The law where this family lives states that anyone providing obstetrical care, in or out of a hospital, is required to discuss the need for hearing screening for babies with all parents they care for.

The law went into effect July of 2001. Anna was born in January of 2006. Her midwife was unaware of the need for screening and of her responsibility to discuss it with the parents. Had Anna’s parents known, they could have requested free screening for Anna.

The Importance of Treating Deafness Early

Anna was 15 months old before anyone realized that she could not hear. Her parents have chosen to attempt to provide her with a sense of sound. Hearing aids are of no help, and she was 19 months old when she received a cochlear implant. This is her only hope of ever hearing any sound at all. Results are variable. Anna had been totally deaf during the most important phase of normal language development. If and when she did hear at all, she wound need intensive therapy to make up for lost time and missed developmental opportunities.

Babies who are known to be deaf in early infancy receive therapy early, and most who choose cochlear implants have them by one year of age – just when those developmental opportunities begin. In addition, their families can begin learning sign language very early on. It is difficult for Anna’s family to keep up with her voracious interest in learning new signs because they are learning as they go – they have no storehouse of previous knowledge to draw upon.

The Controversial Cochlear Implant
While hearing aids amplify sounds so damaged ears may detect them, cochlear implants bypass the damaged portion of ears and directly stimulate the auditory nerve. They do not restore hearing, and instead provide a useful representation of sounds. Implantation involves a surgical procedure and significant post-implantation therapy. Improved outcomes may result from implantation at a young age because children may experience sound during important developmental times for language acquisition.

However, there is controversy about the use of cochlear implants, and about whether decisions should be made before children are old enough to consider the options. Whether or not parents choose cochlear implants for their children, early detection is important for making informed decisions, and even children with cochlear implants may benefit from early introduction to sign language.

In Anna’s Case
On August 30, 2007, Anna had her cochlear implant inserted and was responding to some sound. She had to learn to pay attention to the sounds she hears and to interpret them.

Please make sure that all parents you know who birth at home are made aware of the opportunity to have a hearing screening performed shortly after birth. Learn how and where homebirth babies can be screened, and make that information available. Do what you can to share Anna’s story with others so that they become educated about early diagnosis. No family should miss the opportunity to consider their options while early intervention is still possible.

For More Information
To locate Early Hearing Detection & Intervention programs by state, visit the American Academy of Pediatrics’ website at www.medicalhomeinfo.org/screening/EHDIstateinfo.html.