Temporal Bone Study of Down's Syndrome

Temporal bone histopathologic findings in partial trisomy 13 and partial trisomy 14

“…Stapes abnormalities previously reported in trisomy 13 include bulky stapes, thick crura and footplate, and more central attachment of the anterior and posterior crura to the footplate [5]. The facial nerve canal dehiscence which was observed in our case also occurs in trisomy 13 and other chromosomal aberrations such as trisomy 21, trisomy 22, and 4pÀ [5,[8][9][10][11][12][13][14][15]. Other middle ear abnormalities including small facial nerve, persistence of the stapedial artery, wide angle of the facial genu, and absence of the pyramidal eminence described in trisomy 13 were not observed in the present case.…”

Inner Ear Dysplasia is Common in Children With Down Syndrome (trisomy 21)

“…Schmidt 19 evaluated cochlear neuronal populations in a series of ears with developmental defects, including one patient with DS, and profound hearing loss and found a significantly decreased neuronal population. Bilgin 20 more recently evaluated 16 DS temporal bones and 10 control temporal bones, and measured the width of the vestibular–LSCC complex, the bone island of the LSCC, and the size of the bony LSCC lumen confirming Igarashi's findings of hypoplasia in DS. They also confirmed overall shorter cochlea in six, shortened organ of Corti and spiral ganglion, and a lesser spiral ganglion cell population in the DS temporal bones, and described a Mondini cochlea in four ears and semicircular canal abnormalities in five.…”

Down syndrome: An electrophysiological and radiological profile

“…In our series, external ear abnormalities consisted mostly of a significantly underdeveloped EACs. No middle ear malformations were radiologically discovered; this is surprisingly inconsistent with the histopathological findings reported by many authors . In their article, Balkany et al stated that the most common ossicular anomalies were confined to the stapes superstructure and the crural curvatures.…”