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Ossicular fusion and cholesteatoma in auriculo‐condylar syndrome: In vivo evidence of arrest of embryogenesis
Author(s) -
Propst Evan J.,
Ngan Bo Y.,
Mount Richard J.,
MartinMunoz Daniel,
Blaser Susan,
Harrison Robert V.,
Cushing Sharon L.,
Papsin Blake C.
Publication year - 2013
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.23492
Subject(s) - medicine , cholesteatoma , mastoidectomy , condyle , temporomandibular joint , anatomy , histopathology , hearing loss , ossicles , ankylosis , middle ear , surgery , pathology , audiology
Abstract 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