A “Refer” Result on Newborn Hearing Screening: What Parents Should Know

The issue

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Before the advent of universal newborn hearing screening 15 years ago, parents would often not learn of a childhood onset hearing loss until around 2 years of age.  Fortunately, since newborn hearing screening has become universal across the United States, hearing loss can be detected very soon after birth.  Currently about 97% of newborns in the United States are screened for neonatal onset hearing loss.  Most children are tested in the newborn nursery by volunteers using an automated test that detects the brain’s response to clicks presented to the sleeping newborn’s ears.  It is highly accurate in detecting all degrees of hearing loss, but only gives 2 results: “pass” and “refer”.  A “pass” requires no further testing in an otherwise healthy infant without any other risk factors for hearing loss. A “refer” result is not a diagnosis of hearing loss, but simply a flag that requires further evaluation.

What we know

The rate of clinically significant hearing loss in otherwise healthy infants is estimated to be approximately 2-3 out of 1000 newborns.  The “refer” rate of automated newborn screening is approximately 10-12 in 1000, so many of the children who get referred for further testing will eventually be found to have normal hearing (called a “false positive”). This can be due to presence of debris or fluid in the ear canals or middle ear spaces, which is a temporary problem.  Depending on how long the child is in the hospital after birth, re-testing can often be carried out before the child even goes home.  If needed, the child is brought back for further automated testing as an outpatient.  If the child still refers on these follow-up automated tests, the child is likely to be referred to a pediatric audiologist for further testing.  This is typically in the form Auditory Brainstem Response (ABR) or Brainstem Auditory Evoked Response (BAER) testing.  These are more in-depth auditory tests, again measuring the brain’s electrical response to auditory signals.  Not only do they determine the presence or absence of hearing loss, but they can also determine the approximate level of hearing loss.  Up until about 4 to 6 months of age, this can be reliably done under natural sleep.  After 4 to 6 months of age, the child will often wake up because of the auditory stimulation, and an accurate test cannot be performed.  At that point, further ABR testing is often done under light sedation.  The results of this testing are critical in not only confirming the diagnosis of hearing loss, but also helping to determine the best mode of intervention based on the degree of hearing loss and which specific frequencies are affected.

What we don’t know

In some hospital settings, when child gets a “refer” result on automated testing, further testing is arranged automatically.  Unfortunately, in many situations this is the sole responsibility of parents to follow-up on.  According to recent statistics, almost 35% of children who referred on newborn hearing screening never got subsequent definitive testing for hearing loss.  That’s when what we don’t know CAN hurt the child.  Untreated hearing loss during early brain development can lead to delays in speech, language, and cognitive function.  These delays can lead to lifelong problems with language acquisition, reading comprehension, social development, and cognitive development.  Many hospitals provide information about where to seek out further audiological testing, but in some areas (particularly rural settings) the parents must seek this information out on their own.  The parents should seek out a hearing health professional known as a “pediatric audiologist” who can help direct further workup.  More often than not they can be found in university hospital or teaching hospital settings.

Implications

When the child is confirmed as having early onset hearing loss, avoiding the complications of hearing loss is typically very simple: early and consistent use of amplification technology otherwise known as hearing aids.  Hearing aids can be fit in infants as young as 2 weeks of age, and in the majority of cases will remedy all but the most severe levels of hearing loss.  In cases of very severe to profound hearing loss, cochlear implant technology may be considered to help allow the child access to sound.  Consistent use of amplification technology along with early childhood intervention will help ensure that the child’s auditory brain develops normally, and the child will be poised to fulfill his or her full potential, regardless of the hearing loss.

 

Posted on Jan 5, 2017 by
Theodore Mason
Ear, Nose & Throat Surgeons of Western New England LLC
tpmason1 {at} comcast.net

 

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