Mondini's Deafness

Complete Labyrinthine Aplasia: Clinical and Radiologic Findings with Review of the Literature

“…The report also included a detailed description of several associated anomalies. 1 This anomaly was also documented in a few reports (9 case reports describing a total of 12 patients) in the English-language literature, [3][4][5][6][7][8][9][10][11] all describing several associated anomalies. Until recently, the only treatment option for this developmental anomaly was vibrotactile hearing devices.…”

“…[2][3][4][5][6][7][8][9][10] Most of the reported cases of CLA in the literature, 1,2,7,9,10 including the original article by Michel, 1 described bilateral aplasia of the labyrinths. However, as also previously reported by Jackler et al, 2 Isenberg and Tubergen, 6 and Keeney et al, 8 4 (44.4%) of our patients had unilateral CLA (as illustrated by Fig 1B); thus, the presence of unilateral aplasia should not exclude the diagnosis of CLA.…”

“…1,2 This rare anomaly was first described by Michel 1 in 1863 and has only been reported as selected case reports in the literature. [3][4][5][6][7][8][9][10][11] We report the first series of CLA cases consisting of 14 ears in 9 patients, emphasizing their distinguishing and unique features.…”

Inner Ear Malformations in Congenital Deafness Are Not Associated with Increased Risk of Breech Presentation

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Vestibular Abnormalities in Charge Association

“…Other features included a dilated cochlea with absent apical turns (Mondini deformity) 13 and enlargement of the vestibular aqueduct on the right. The bony cochlea was normal on the left.…”