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Mondini Dysplasia

Mondini Dysplasia, sometimes known as a Mondini malformation, is an abnormality in the inner ear that can be the cause of hearing loss.

A person with Mondini dysplasia has a cochlea that is incomplete. A normal cochlea has two and a half turns, a cochlea with Mondina dysplasia has two, one and a half, one, or no turns (or increments, obviously). This results in gradual or even sudden hearing loss that may be profound.

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Mondini dysplasia: A clinical and pathological study (Annals of otology, rhinology & laryngology : Supplement)

Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients
Abstract Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks ...
From Ear, Nose & Throat Journal, 5/1/00 by Rakesh B. Patel
Large internal auditory meatus, inner ear anomalies, and progressive hearing loss
The present paper describes a case demonstrating an abnormality of a large internal auditory meatus (LIAM) together with other inner ear anomalies. The ...
From Australian Journal of Oto-Laryngology, 7/1/99 by Birman, Catherine

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