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August 1991

Mondini Dysplasia Is Not Associated With Meningitis and Cerebrospinal Fluid Fistula

Arch Otolaryngol Head Neck Surg. 1991;117(8):931. doi:10.1001/archotol.1991.01870200125023

To the Editor.—Twelve years ago, we described seven patients with a particular congenital deformity of the labyrinth of the inner ear, two of whom developed a cerebrospinal fluid fistula through the oval window.1 We agreed with Jensen who, in a previous article in the US literature,2 stated that this was not the defect described in detail by Carlo Mondini in 17913 but, rather, a more severe dysplasia. We have now seen 30 patients with this more severe type of dysplasia, 12 of whom have developed a spontaneous cerebrospinal fluid fistula through the inner ear or presented with meningitis. These cases have been described in various publications.4-7 Other European publications have described the difference between the true Mondini and the "pseudo-Mondini" deformity,8,9 although we consider the latter a term to be deprecated.10,11

We are dismayed, therefore, that so much confusion persists in the US