Newborn Hearing Screening

Aka: Newborn Hearing Screening, Hearing Testing in Newborns, Infant Hearing Testing, Auditory Brainstem-Evoked Response, Auditory Brainstem Response, Otoacoustic Emission

1. Definition
   1. Newborn Hearing Screening
2. Types of screening
   1. Automated Auditory Brainstem Response (ABR)
      1. Tests auditory path from ear canal to low Brainstem
      2. Headphones on newborn's ears emit soft clicks
      3. Forehead,neck electrodes measure brain wave response
      4. Computer interpretation results in pass or fail/refer
   2. Transient Evoked Otoacoustic Emissions (OAE or TEOAE)
      1. Tests peripheral auditory system (esp. Cochlea)
      2. Headphones on newborn's ears emit soft clicks
      3. Microphone in ear canal measures Cochlear response
         1. Cochlea hair cells generate echo response to sound
      4. Computer interpretation results in pass or fail/refer
3. Epidemiology: Incidence of Newborn SNHL in United States
   1. Incidence: 5000 infants per year in U.S.
   2. Prevalence: 1 to 3 per 1,000 U.S. newborns
4. Protocol: Initial Testing
   1. Test all newborns meeting testing criteria
      1. See Indications below
      2. Thirty states in U.S. require universal screening
   2. Two-stage testing at birth hospitalization
      1. ABR or OAE repeated twice
   3. Repeat failed tests 2 to 8 weeks after initial testing
5. Protocol: Subsequent Testing
   1. Low-risk children: Hearing screening pre-kindergarten
   2. High-risk children (See Risk Factors below)
      1. Repeat Hearing Testing every 6 months until age 3
6. Efficacy: ABR or OAE
   1. Test Sensitivity: 84%
   2. Test Specificity: 90%
7. Indications for ABR or OAE testing
   1. Neonatal Intensive Care Unit Admission for 2 days
   2. Family History of Hearing Loss
   3. Hyperbilirubinemia requiring Exchange Transfusion
   4. Birth Weight <1500 grams
   5. Bacterial Meningitis
   6. Severe perinatal distress
      1. Low APGAR Scores
         1. Five minute APGAR: <3
         2. Ten minute APGAR: <7
      2. Perinatal Seizures
   7. Congenital infections
      1. Cytomegalovirus
      2. Toxoplasmosis
      3. Bacterial Meningitis
      4. Syphilis
      5. Rubella
      6. Herpes Simplex Virus
   8. Anatomic abnormalities of the head, ear, or Palate
      1. Iris heterochromia
      2. Ear or auricle malformation (e.g. Skin Tag, dimple)
      3. Cleft Lip or Palate
      4. Tympanic Membrane abnormality
      5. Asymmetric facial features
      6. Microcephaly
   9. Syndrome associated with Hearing Loss
      1. Usher's Syndrome
      2. Waardenburg's Syndrome (hypertelorism, pigmentation)
      3. Down Syndrome
      4. Neurodegenerative Disorder (e.g. Hunter syndrome)
      5. Demyelinating disease (e.g. Charcot-Marie-Tooth)
   10. Persistent Pulmonary Hypertension
   11. Ototoxic Medication exposure
       1. Prolonged Diuretics with Lasix
       2. Excessive Aminoglycoside (e.g. Gentamicin) exposure
          1. Prolonged use >5 days or elevated serum levels
          2. Combined with Lasix for more than 5 days
       3. Excessive Vancomycin exposure
          1. Prolonged use or elevated serum levels
   12. Hyperventilation to pH >7.55 for more than 12 hours
   13. Clinical suspicion of Hearing Loss
   14. Maternal Cocaine abuse or Alcohol Abuse
8. Resources
   1. USPSTF Guide to Clinical Preventive Task Force
      1. http://www.ahrq.gov/clinic/uspstfix.htm
   2. National Center on Birth Defects
      1. http://www.cdc.gov/ncbddd
   3. National Institute on Deafness
      1. http://www.nidcd.nih.gov/health/hearing
   4. National Dissemination Center Children with Disability
      1. http://www.nichcy.org
9. References
   1. Cunningham (2003) Pediatrics 111:436-40
   2. Thompson (2001) JAMA 286:2000-10
   3. (2000) Pediatrics 106:798-817
   4. Wrightson (2007) Am Fam Physician 75:1349-52