Anomalies of Auditory Organ in 6p Partial Monosomy -Temporal Bone Report

概要

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Temporal bone histopathology was presented in an five-month-old infant with 6p partial monosomy who demonstrated atresia auris congenita dextra with microtia. The right external auditory canal was completely occluded by a bony atresia plate. The temporal bone showed multiple anomalies of the middle ear, including hypoplastic epitympanum, absence of malleus and incus, columellar shaped stapes with footplate fixation posteriorly and abnormal courses of the branched facial nerve and the trigeminal nerve associated with ectopic ganglions. In addition, there was a wide communication between the saccular and utricular spaces. The vestibular aqueduct and the endolymphatic duct and sac ended blindly in the otic capsule without reaching the posterior cranial fossa. The bony and membranous cochlea appeared normal.
日本耳科学会の論文