The temporal bone findings from two patients with Mondini's genetic aplastic deafness are described. Profound sensorineural deafness was present in both cases and audiology and speech studies were available for the first patient. Cochlear changes which were similar for both, consisted of a flattened cochlear capsule, 1½ instead of 2½ cochlear turns, missing interscalar septum, between middle and apical turn forming a scala communis or cloaca, organ of Corti lesions more marked in the upper turn, spotty aplasia of the stria vascularis and spiral ligament, and reduced spiral ganglion cells and dendrites. An unusual finding in the first patient was the complete absence (aplasia) of the three semicircular canals, the utricle, and the vestibular nerve. An abnormal saccule and a markedly enlarged endolymphatic duct and sac were also present.
Paparella MM, ElFiky FM. Mondini's Deafness. Arch Otolaryngol. 1972;95(2):134–140. doi:10.1001/archotol.1972.00770080222009
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