Auditory and facial nerve dysfunction in patients with hemifacial microsomia.

BACKGROUND: Hemifacial microsomia (HFM) is a common craniofacial disorder characterized by a wide spectrum of anomalies, including conductive hearing loss due to external and middle ear deformities. However, the prevalence of sensorineural hearing loss (SNHL) as well as facial nerve dysfunction is underappreciated.

OBJECTIVE: To determine the frequency of auditory and facial nerve dysfunction and its relationship to more severe forms of bilateral HFM.

DESIGN: Retrospective medical record review to characterize the clinical severity of HFM and the prevalence and nature of the associated auditory and facial nerve dysfunction.

SETTING: Center for Craniofacial Anomalies at the University of California, San Francisco, Medical Center.

PATIENTS: Ninety-nine pediatric patients with HFM evaluated at the University of California, San Francisco, Medical Center.

MAIN OUTCOME MEASURES: The prevalence of SNHL and facial nerve dysfunction in this patient population and any associations between these 2 characteristics.

RESULTS: Hearing loss was present in 74 (75%) of 99 patients, with a conductive component in 73 patients. Sensorineural hearing loss was present in 11 patients ( 11%), with mixed hearing loss in most patients. Fourteen patients required rehabilitation with auditory amplification. Nearly a quarter of the patients (22 [22%] of 99) had facial nerve dysfunction, but only 1 patient had facial palsy on the same side as the SNHL. There was a statistically significant association between having auricular abnormalities and conductive hearing loss or SNHL (P = .30 and .80, respectively). However, there was no statistically significant association between bilateral HFM and the occurrence of either SNHL or facial paralysis, nor was there an association between auditory and facial nerve dysfunction.

CONCLUSIONS: Sensorineural hearing loss and facial nerve dysfunction are common in HFM. These findings have important implications in the treatment of patients with HFM.