Hearing loss in infants with craniofacial anomalies.

Results of auditory brainstem response (ABR) evaluation of 145 infants, ages 6 months and younger with craniofacial anomalies (CFA), were examined to determined predicted degree and nature of hearing loss. Approximately 50% of infants demonstrated at least mild bilateral hearing loss. Presence and degree of hearing loss varied by CFA. All infants with bilateral aural atresia exhibited at least a moderate bilateral hearing loss; less than 20% of infants with isolated external ear anomalies (ear tags, isolated microtia) exhibited any degree of hearing loss. In 92% of infants with hearing loss, results of ABR were consistent with primarily conductive dysfunction. Implications for early identification and audiologic management of infants with CFA and hearing loss are discussed.