Preauricular skin tags and ear pits are associated with permanent hearing impairment in newborns

OBJECTIVES. Our goals were to (1) study the prevalence of hearing impairment in a large cohort of infants with preauricular skin tags or ear pits and compare it with that among all other newborns participating in our universal newborn hearing screening program during the same period and (2) evaluate the effectiveness of transient evoked otoacoustic emissions as a hearing-screening tool in this population. PATIENTS AND METHODS. During the study period of 7.5 years, 68 484 infants were screened for hearing impairment, of whom 637 (0.93%) had preauricular skin tags and/or ear pits. The population was divided into 3 groups: (1) a low-risk group for hearing impairment; (2) a high-risk group for hearing impairment; and (3) a very high-risk group for hearing impairment. The screening results and audiological follow-up for these infants were examined retrospectively. RESULTS.A significantly higher prevalence of permanent hearing impairment was found among infants with preauricular skin tags or ear pits (8 of 1000), compared with infants without tags or pits (1.5 of 1000). In the low-risk group, the prevalence was 3.4 of 1000, compared with 0.5 of 1000 in infants with and without preauricular tags or pits, respectively. In the high-risk group, the prevalence was 77 of 1000, compared with 20 of 1000 in infants with and without preauricular tags or pits, respectively. The odds ratio for hearing impairment associated with preauricular skin tags and/or ear pits after adjusting for level of risk group was 4.9. All infants diagnosed with permanent hearing impairment, with the exception of 1 with late- onset impairment, were detected by in-hospital transient-evoked otoacoustic emissions screening. CONCLUSIONS. Infants with preauricular skin tags or ear pits are at increased risk for permanent hearing impairment. Transient-evoked otoacoustic emissions were found to be an effective hearing-screening tool in this population.