Salivary gland choristoma (heterotopic salivary gland tissue) is a rare condition that occurs at various locutions within the head and neck. We present a 10-year-old boy with u salivary gland choristoma of the middle ear and compare findings with the 15 similar cases published in the English and German languages. Patients typically have a long-standing conductive hearing loss and visible middle ear mass. Operative findings include a lobulated middle ear mass of histologically normal salivary gland tissue attached posteriorly in the region of the oval window, together with absent or malformed ossicles. Frequently the mass is intimately associated with the facial nerve. The constancy of these findings has led to the proposal of an abnormal developmental syndrome. This syndrome will be described and possible explanations for its cause will be discussed.
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