Carniofacial Microsomia

Cranoifacial microsomia is an unique clinical presentation of '1st and 2nd arch syndrome' with asymmetrical craniofacial development alongwith conductive hearing loss. A series of 11 patients (4 males, 7 females) is presented which include two patients of 'Goldenhar's variant' with epibulbar dermoids. Two patients had no response on pure tone audiometry (blank audiograms) and underwent CT scan of temporal bone which revealed 'Michel's aplasia' (complete labyrinthine agenesis), rarely reported in the literature.