Risk Indicators

The Joint Committee on Infant Hearing (JCIH) recommends that infants who have a risk factor for late-onset hearing loss should have at least one comprehensive audiologic evaluation by 24 to 30-months. The JCIH Risk Indicators Associated with Permanent Early Onset and or Late Progressive Hearing Loss in Childhood include:

  • Parent or caregiver concern regarding hearing, speech, language or developmental delay.
  • Family history of permanent childhood hearing loss.
  • Neonatal intensive care greater than 5 days, which may include extracorporeal membrane oxygenation (ECMO) assisted ventilation, exposure to ototoxic medications (gentamicin and tobramycin) or loop diuretics (furosemide/lasix), and hyperbilirubinemia requiring exchange transfusion.
  • In-utero infections such as cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis.
  • Craniofacial anomalies, including those involving the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies.
  • Physical findings such as white forelock, associated with a syndrome known to include sensorineural or permanent conductive hearing loss.
  • Syndromes associated with hearing loss or progressive or late onset hearing loss such as neurofibromatosis, osteopetrosis, and Usher syndrome. Other frequently identified syndromes include Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson.
  • Neurodegenerative disorders, such as Hunter syndrome, or sensory-motor neuropathies, such as Friedreich's ataxia and Charcot-Marie-Tooth syndrome.
  • Culture positive postnatal infections associated with sensorineural hearing loss, including confirmed bacterial and viral (especially herpes and varicella) meningitis.
  • Head trauma, especially basal skull/temporal bone fracture requiring hospitalization.
  • Chemotherapy.