Craniofacial, temporal bone, and audiologic abnormalities in the spectrum of hemifacial microsomia

Objectives: To evaluate the clinical, audiologic, and temporal bone compute d tomographic findings in patients with hemifacial microsomia and to use th e OMENS (each letter of the acronym indicates 1 of the following 5 dysmorph ic manifestations: O, orbital asymmetry; M, mandibular hypoplasia; E, auric ular deformity; N, nerve involvement; and S, soft tissue deficiency) gradin g system to assess possible correlations between the severity of dysmorphic features with the type of abnormalities in the temporal bone and with degr ee of hearing deficit. Design: Retrospective study. Setting: Tertiary care children's hospital. Patient: Ferry patients with hemifacial microsomia. Result: Mandibular hypoplasia and auricular abnormalities were the most com mon clinical manifestations, present in 39 patients (97%) and 38 patients ( 95%), respectively. Conductive hearing loss was noted in 35 patients (86%) and sensorineural hearing loss in 4 patients (10%). Facial nerve weakness w as present in 20 patients (50%). Twenty patients had unilateral aural atres ia, 12 patients had unilateral aural stenosis, and 7 patients had bilateral anomalies. Moderate hypoplasia or atresia of the middle ear was noted in 3 6 patients (90%) and ossicles were malformed in 30 patients (75%). Hypoplas ia of the oval window was the most common inner ear abnormality. Conclusions: Severity of craniofacial features (total OMENS score) signific antly correlated with the degree of temporal bone abnormality, but no corre lation was noted with the degree or type of hearing loss. We recommend the following. (1) use of the OMENS classification system for documentation and analysis of dysmorphic finding in hemifacial microsomia; (2) complete audi ologic evaluation in all patients with hemifacial microsomia regardless of the type of craniofacial abnormalities; and (3) temporal bone computed tomo graphy for further evaluation of hearing deficit.