Choristoma of the middle ear: A component of a new syndrome?

Case Report: Salivary choristoma of the middle ear is a rare entity. The au thors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the uric: capsule and facial nerve in ways not yet reported. Our patien t presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the lite rature revealed salivary choristomas of the middle ear to be Frequently ass ociated with branchial arch abnormalities, most commonly the second, as wel l as abnormalities of the facial nerve. Review of the Literature: All 25 cases were reviewed and the results report ed with respect to clinical presentation, associated abnormalities, operati ve findings. and hearing results. It has been proposed that choristoma of t he middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of t he facial nerve. Conclusion: Eighty-six percent of the reported patients with choristoma hav e three or four of the four criteria listed to designate middle ear salivar y choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.