Ask the Audiologist

Infants at High Risk for Hearing Loss

Certain newborns and infants are considered to be at risk for having a hearing loss.

At risk simply means there is a greater than average chance that a newborn or infant may have a hearing loss.

This web page describes the current protocol by which these newborns and infants at risk can be identified and then further evaluated to confirm the presence of a hearing loss.

It is important to identify these newborns and infants who do have a hearing loss as early as possible so that appropriate care and management can be initiated.

A protocol was established to initially screen all newborns and infants utilizing the High Risk Criteria established in 1982 by the Joint Committee on Infant Hearing.

Using the updated 1994 risk indicators, in a questionnaire form, infants or newborns having one or more of these indicators are considered at risk for having a hearing loss. All such newborns and infants should have their hearing tested within three months. It doesn't matter at what age the newborn or infant has been identified because hearing can be tested accurately at any age! Therefore don't delay your request for audiological testing if a child is at risk or a hearing loss is suspected by the child's parents.

Even if newborns or infants at risk pass an initial hearing test, it is important that follow-up hearing tests be conducted to rule out delayed onset hearing loss. Certain risk factors (i.e. cytomegalovirus (CMV), prolonged mechanical ventilation, Waardenberg's Syndrome) are associated with delayed onset hearing loss.

Testing Methods & Techniques
The following is a summary of various testing techniques which can be used to evaluate the hearing of newborns and young children.

Auditory Brainstem Response (ABR) Testing:
The ABR test is reliable, objective, noninvasive and painless. Brain wave activity in the auditory centers of the brain is recorded in response to a series of clicks presented to each ear. Thus, the ABR test indirectly estimates the level of hearing in the peripheral auditory system (middle ear and inner ear). Usual age: From birth.

Otoacoustic Emmissions (OAE)
Sound is presented at the ear by a small instrument which then listens for an "echo". If the echo is present within expected limits, then the mechanical parts of the ear are working. If the echo is not present, the instrument instructs the technician to refer the patient for further testing.

Behavioral Observation Audiometry (BOA):
Trained observers watch for repeatable changes in a child's behavior in conjunction with the presentation of sound. Types of sound used may vary from toy noisemakers to standard pure tone stimuli. Usual age: Birth to 3 years.

Visual Reinforcement Audiometry (VRA):
Sound and lights are presented together. Once the child has learned that there is a relationship between the lights and sound, the light is withheld and the sound is presented alone. The child will look for the lights in response to the sound, and the light is then presented as a reward. Usual age: 3 months to 3 years

Play Audiometry:
The child is taught to play "listening games" using blocks, pegs, rings, etc. Such games maintain a child's interest better than standard testing, which requires pushing a button. In this way, it is possible to obtain precise measurements of hearing acuity. Usual age: 2-6 years

Impedance (Immittance) Audiometry:
This is an objective test which uses a headset equipped with a soft but airtight plug inserted in the entrance of the ear canal. Special equipment measures how well the eardrum moves, middle ear pressure and the presence or absence of acoustic reflexes. Usual age: From 6 months

If Your Child Is At Risk:
Any high risk infant should have a medical evaluation (pediatrician, ENT specialist) in conjunction with a hearing test. This team approach is critical in the successful identification of hearing loss.

Neonates (Birth to 28 days of age)

  1. Family history of hereditary, childhood sensorineural hearing loss.
  2. In utero infection such as cytomegalovirus, rubella, syphilis and toxoplasmosis.
  3. Craniofacial anomalies including those with morphological abnormalities of the pinna and ear canal.
  4. Birth weight less than 1500 grams (3.3 pounds).
  5. Hyperbilirubinemia at a serum level requiring exchange transfusion.
  6. Ototoxic medications including but not limited to the aminoglycocides used in multiple courses or in combination with loop diuretics.
  7. Bacterial meningitis.
  8. Severe depression at birth with Apgar scores of 0-4 at one minute or 0-6 at five minutes.
  9. Prolonged mechanical ventilation five days or longer (e.g., persistent pulmonary hypertension).
  10. Stigmata or other findings associated with a syndrome known to include a sensorineural and/or a conductive hearing loss.

Infants (29 days to 2 years of age)

  1. Parent/caregiver concern regarding hearing, speech, language and/or developmental delay.
  2. Bacterial meningitis and other infections associated with sensorineural hearing loss.
  3. Head trauma associated with loss of consciousness or skull fracture.
  4. Stigmata or other findings associated with a syndrome known to include a sensorineural and/or a conductive hearing loss.
  5. Ototoxic medications including but not limited to chemotherapeutic agents, or aminoglycocides used in multiple courses or in combination with loop diuretics.
  6. Recurrent or persistent otitis media with effusion for at least 3 months.

Delayed Onset Indicators
for use with infants who require periodic monitoring of hearing (29 days to 3 years of age):

  1. Family history of hereditary childhood hearing loss.
  2. History of in utero infection such as cytomegalovirus, syphilis, rubella or toxoplasmosis.
  3. Neurofibromatosis Type II, and neurodegenerative disorders.
  4. Recurrent or persistent otitis media with effusion.
  5. Anatomic deformities and other disorders which affect eustachian tube function.
  6. Neurodegenerative disorders.