What investigations are needed for a stable newborn with an ear tag on the right ear?

Hearing Assessment is Required for Newborns with Ear Tags

All newborns with isolated ear tags (preauricular tags) should undergo hearing assessment, as they are at significantly increased risk for hearing impairment compared to the general population.

Why Hearing Assessment is Necessary

Ear tags are classified as a craniofacial anomaly and represent a specific risk indicator for hearing loss according to the American Academy of Pediatrics Joint Committee on Infant Hearing 1. The evidence demonstrates:

• 13-17% of infants with isolated ear tags have hearing impairment, which is significantly higher than the general population 2, 3
• Both sensorineural and conductive hearing loss can occur with isolated ear tags 2, 3
• 78% of children with ear tags and hearing impairment have a hereditary tendency for hearing loss 2
• The presence of ear tags, ear pits, or temporal bone anomalies specifically triggers the need for comprehensive hearing evaluation 1

Recommended Investigation Protocol

Immediate Actions:

Hearing Assessment:

• If the newborn passed the initial hospital hearing screening, repeat hearing assessment is still required due to the ear tag being an independent risk factor 1
• If the newborn failed or did not receive hospital screening, refer for comprehensive audiological evaluation by 3 months of age 4, 5
• The evaluation should include diagnostic ABR testing (not just OAE screening), as this can detect both cochlear and neural hearing loss 5, 6

Physical Examination:

• Carefully examine for other craniofacial anomalies including ear canal atresia/stenosis, facial asymmetry, branchial cleft cysts, cleft palate, and dental anomalies 1
• Document any dysmorphic features that might suggest a syndrome (e.g., Goldenhar, branchio-oto-renal syndrome) 1
• Check for white forelock, heterochromia irides, or abnormal pigmentation (Waardenburg syndrome) 1

Family History:

• Obtain a three-generation pedigree focusing on hearing loss, consanguinity, and craniofacial anomalies 1
• Ask specifically about childhood hearing loss in relatives (29% of children with ear tags have positive family history) 2

Additional Investigations to Consider:

Renal Ultrasound:

• Newborns with isolated ear tags have increased risk of congenital urinary tract malformations 7
• This is particularly important as ear tags can be associated with branchio-oto-renal syndrome 1

Cardiac Evaluation:

• Consider cardiac ultrasound as ear tags are associated with increased risk of congenital heart malformations 7
• Obtain ECG if there are any cardiac symptoms or family history of sudden death (to screen for prolonged QT syndromes like Jervell and Lange-Nielsen) 1

Ophthalmology Referral:

• All infants with confirmed hearing loss should have ophthalmology evaluation to screen for associated visual anomalies 1

Genetics Consultation:

• Refer to clinical geneticist if multiple anomalies are present or if hearing loss is confirmed 1
• Genetic testing (including GJB2/connexin 26) may be appropriate if hearing loss is documented 1

Ongoing Surveillance

Long-term Monitoring:

• Even if initial hearing assessment is normal, children with ear tags require ongoing developmentally appropriate hearing screening 1
• At minimum, perform one diagnostic audiology assessment by 24-30 months of age 4, 5
• This is critical because some forms of hereditary hearing loss are progressive or late-onset 1

Common Pitfalls to Avoid

• Do not assume normal newborn hearing screening is sufficient - ear tags are an independent risk factor requiring additional assessment 1
• Do not rely solely on OAE screening - diagnostic ABR is needed to detect neural hearing loss 5, 6
• Do not dismiss isolated ear tags - even without other anomalies, the hearing impairment risk is 13-17% 2, 3
• Do not forget to assess for associated anomalies - 19% have other congenital malformations 3