Goldenhar syndrome with external auditory canal stenosis complicated by canal cholesteatoma and first branchial cleft cyst

SummaryThis report features a case of Goldenhar syndrome with congenital left facial nerve paralysis, microtia and external auditory canal stenosis complicated by an external auditory canal cholesteatoma and first branchial cleft cyst. The workup and surgical management of this rare constellation of defects is detailed. The features of this case are instructive as they provide insight into abnormal ear development. Otolaryngologists should retain a high index of suspicion for the presence of congenital cholesteatoma, branchial cleft anomalies and facial nerve abnormalities when evaluating patients with Goldenhar syndrome.