Hemifacial microsomia and bilateral mondini dysplasia: A rare clinical presentation

Abstract

We present a rare association of bilateral Mondini dysplasia with hemifacial microsomia (HFM). HFM is a clinical spectrum of malformations involving the orbit, mandible, ears, facial nerve, and soft tissues of the face. It occurs in about 1 in 5600 births and requires a multidisciplinary approach to management.[1] We report a case of HFM in an 11-year-old girl who presented with recurrent discharge from the nasal dorsum, and facial asymmetry since birth but was otherwise asymptomatic. The hearing evaluation revealed bilateral sensorineural hearing loss. High-resolution computed tomography scan of the temporal bones showed bilateral Mondini dysplasia of the inner ear which to the best of our knowledge has not been previously reported in HFM. We conclude that all cases of HFM regardless of symptomatology should undergo hearing evaluation. This will facilitate rehabilitation of deafness if present.

Keywords

• craniofacial anomalies
• hemifacial microsomia
• mondini dysplasia