Mondini dysplasia

Mondini dysplasia
Synonyms	Mondini malformation, Mondini defect
Pronounce	N/A
Specialty	N/A
Symptoms	Hearing loss, vertigo, Tinnitus
Complications	Sensorineural hearing loss, Recurrent meningitis
Onset	Congenital
Duration	Lifelong
Types	N/A
Causes	Genetic mutation
Risks	Family history
Diagnosis	CT scan, MRI
Differential diagnosis	Other inner ear malformations
Prevention	N/A
Treatment	Hearing aids, Cochlear implant, Speech therapy
Medication	N/A
Prognosis	Varies
Frequency	Rare
Deaths	N/A

Mondini dysplasia is a rare congenital abnormality in the inner ear that can cause hearing loss and balance problems. It is named after the Italian anatomist Carlo Mondini, who first described it in 1791.

Symptoms

The main symptom of Mondini dysplasia is sensorineural hearing loss, which can range from mild to profound. This type of hearing loss is caused by damage to the cochlea, the part of the inner ear that converts sound vibrations into nerve signals. Other symptoms can include vertigo, tinnitus, and a feeling of fullness in the ear.

Causes

Mondini dysplasia is caused by an incomplete development of the cochlea during the early stages of embryonic development. The exact cause of this abnormal development is not known, but it is thought to be due to a combination of genetic and environmental factors.

Diagnosis

The diagnosis of Mondini dysplasia is usually made through a combination of audiometry (hearing tests) and imaging studies such as MRI or CT scan. These tests can show the characteristic abnormalities of the cochlea that are seen in this condition.

Treatment

There is no cure for Mondini dysplasia, but the symptoms can be managed with hearing aids or cochlear implants. In some cases, surgery may be needed to correct the structural abnormalities of the inner ear.

See also

• Sensorineural hearing loss
• Congenital abnormality
• Cochlear implant

References

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