Risk factors for permanent hearing loss in infants

Acquired pre- and perinatal factors

Need for NICU care for >5 days

Very low birth weight (<1500 g)

Perinatal asphyxia or neonatal encephalopathy

Congenital infection (CMV, syphilis, toxoplasmosis, rubella, HIV, Zika virus)

Postnatal CNS infection (bacterial meningitis, HSV encephalitis)

Exposure to otoxic medications (aminoglycosides*, diuretics)

Severe hyperbilirubinemia requiring exchange transfusion

Mechanical ventilation >5 days

Required ECMO support

Family history

First- or second-degree relative with permanent hearing loss in childhood

Syndromes associated with hearing loss

Examples include Alport syndrome, Jervell-Lange-Nielsen (long QT) syndrome, neurofibromatosis, Pendred syndrome, Usher syndrome, and Waardenburg syndrome.

However, numerous genetic syndromes (well over 100) are associated with early hearing loss.^¶

Craniofacial anomalies

Cleft lip and/or palate

Abnormalities of the pinna or ear canal

Temporal bone abnormalities

This table summarizes factors that are associated with increased risk of permanent hearing loss in infancy. Infants with ≥1 of these risk factors generally warrant formal audiologic assessment at least once by age nine months, even if they pass the newborn hearing screen. Formal audiology should be performed sooner in infants who fail the newborn hearing screen. For further details, refer to UpToDate's topic on newborn hearing screening.

NICU: neonatal intensive care unit; CMV: cytomegalovirus infection; HIV: human immunodeficiency virus; CNS: central nervous system; HSV: herpes simplex virus; ECMO: extracorporeal membrane oxygenation.

* Certain genetic predispositions appear to increase an infant's risk of aminoglycoside-related hearing loss.

¶ Some of the more common syndromic causes of hearing loss are listed here. However, this list is not comprehensive. For additional details, refer to separate UpToDate content on the etiology of hearing loss in children.

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Risk factors for permanent hearing loss in infants