Ossicular fusion and cholesteatoma in auriculo‐condylar syndrome: In vivo evidence of arrest of embryogenesis1. (2nd August 2012)

• Record Type: Journal Article
• Authors: Propst, Evan J.; Ngan, Bo Y.; Mount, Richard J.; Martin‐Munoz, Daniel; Blaser, Susan; Harrison, Robert V.; Cushing, Sharon L.; Papsin, Blake C.
• Is Part Of: Laryngoscope. Volume 123:Number 2(2013:Feb.)
• Page Start: 528
• Page End: 532
• Publication Date: 2012-08-02
• Subjects: Otolaryngology -- Periodicals; 617.51005
• DOI: 10.1002/lary.23492
• Languages: English
• ISSNs: 0023-852X

Abstract

<abstract abstract-type="main" xml:lang="en"> <title>Abstract</title> <p>Auriculo‐condylar syndrome (ACS) is a rare condition affecting first branchial arch structures. The types of hearing loss and temporal bone findings in ACS have not been reported. We describe a 14‐year‐old male with constricted pinnae, mandibular dysostosis, glossoptosis, a high‐arched palate, hearing loss, and cholesteatoma. Computed tomography imaging demonstrated malleoincudal joint ankylosis. The fused malleoincudal complex was removed during mastoidectomy for cholesteatoma. Electron microscopy and histopathology of the joint suggested the fusion was congenital. This is the first report of ossicular fusion and cholesteatoma in ACS and the most detailed in vivo evidence of disruption of embryogenesis during malleoincudal joint formation. Laryngoscope, 2012</p> </abstract>