Hearing Loss in Children With Craniofacial Microsomia.


OBJECTIVE   To evaluate the association between craniofacial phenotype and hearing loss in children with craniofacial microsomia. DESIGN   Retrospective cohort study. SETTING   Tertiary care children's hospital. PATIENTS   Individuals with craniofacial microsomia. MAIN OUTCOME MEASURES   Ear-specific audiograms and standardized phenotypic classification of facial characteristics. RESULTS   A total of 79 participants were included in the study. The mean age was 9 years (range, 1 to 23 years) and approximately 60% were boys. Facial anomalies were bilateral in 39 participants and unilateral in 40 participants (24 right, 16 left). Microtia (hypoplasia of the ear) was the most common feature (94%), followed by mandibular hypoplasia (76%), soft tissue deficiency (60%), orbital hypoplasia or displacement (53%), and facial nerve palsy (32%). Sixty-five individuals had hearing loss (12 bilateral and 53 unilateral). Hearing loss was conductive in 73% of affected ears, mixed in 10%, sensorineural in 1%, and indeterminate in 16%. Hypoplasia of the ear or mandible was frequently associated with ipsilateral hearing loss, although contralateral hearing loss occurred in 8% of hemifaces. CONCLUSIONS   Hearing loss is strongly associated with malformations of the ipsilateral ear in craniofacial microsomia and is most commonly conductive. Hearing loss can occur contralaterally to the side with malformations in children with apparent hemifacial involvement. Children with craniofacial microsomia should receive early diagnostic hearing assessments.

DOI: 10.1597/15-348

Cite this paper

@article{Mitchell2017HearingLI, title={Hearing Loss in Children With Craniofacial Microsomia.}, author={Ryan Michael Mitchell and Babette Siebold Saltzman and S. J. Norton and Robert G. Harrison and Carrie Heike and Daniela Varela Luquetti and Kathleen C. Y. Sie}, journal={The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association}, year={2017}, volume={54 6}, pages={656-663} }