The association of facial palsy and/or sensorineural hearing loss in patients with hemifacial microsomia.

Hemifacial microsomia (HFM) is a common craniofacial disorder that is known to be etiologically heterogenous. Phenotypic differentiation of the various subgroups remains unresolved. A review of 50 patients with HFM has yielded data that may help explain different pathogenetic processes. Of particular interest is the association of facial nerve palsy, sensorineural hearing loss (SNHL), or both in a higher percentage of patients than expected. Twenty-two percent had evidence of facial palsy of varying degree. Thirty-three cases had microtia or anotia, and all instances of facial palsy were associated with auricular malformation. Sensorineural hearing loss was found in 16 percent. All patients with microtia and sensorineural hearing loss had facial palsy. Ear tags or pits were found in 21 patients, only two of whom had facial palsy. In all but one case the palsy was found on the more hypoplastic side of the face. In the single exception, both sides of the face were hypoplastic.