Mondini dysplasia

Mondini Dysplasia

Mondini Dysplasia (pronounced: mon-dee-nee dis-play-zhuh) is a rare congenital malformation of the inner ear that is present at birth. The term "Mondini" is derived from the Italian anatomist, Carlo Mondini, who first described this condition in the 18th century.

Definition

Mondini Dysplasia is characterized by an incomplete development of the cochlea and the vestibular system, the parts of the inner ear involved in hearing and balance. This condition is also associated with an enlarged vestibular aqueduct, a small, bony canal that runs from the inner ear to the skull.

Symptoms

Individuals with Mondini Dysplasia often experience hearing loss, which can range from mild to profound. They may also have recurrent ear infections and problems with balance. In some cases, individuals with this condition may also have other abnormalities of the inner ear.

Causes

The exact cause of Mondini Dysplasia is unknown. However, it is believed to be due to a disruption in the development of the inner ear during the early stages of embryonic development. Some cases have been associated with genetic mutations, but the condition can also occur sporadically, with no known genetic cause.

Treatment

Treatment for Mondini Dysplasia is aimed at managing the symptoms and may include hearing aids, cochlear implants, and therapy for balance problems. In some cases, surgery may be required to treat recurrent ear infections.

Related Terms

External links

Medical encyclopedia article on Mondini dysplasia
Wikipedia's article - Mondini dysplasia

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Mondini dysplasia