Mondini dysplasia--late complications.

Area of Science:

• Otolaryngology
• Genetics
• Neurology

Background:

• Mondini deformity is the second most frequent congenital genetic deafness cause.
• It presents with diverse inner ear bony and membranous abnormalities.
• Associated risks include early-onset meningitis and bilateral profound hearing loss.

Observation:

• Six cases of Mondini deformity were analyzed.
• Sequelae observed included meningitis, vertigo, and progressive hearing loss.
• Endolymphatic sac decompression was performed in three patients.

Findings:

• Mondini malformation significantly increases the risk of meningitis and hearing loss.
• Head trauma, even minor, poses a considerable danger to affected individuals.
• Clinical presentation varies widely due to morphological and functional inner ear abnormalities.

Implications:

• Early diagnosis of Mondini deformity is essential for timely intervention.
• Comprehensive patient counseling is vital to manage risks and prevent complications.
• Understanding the risks associated with Mondini deformity can guide preventative strategies and treatment.