Imaging Findings of Bilateral Mondini Malformation Associated with Enlarged Vestibular Aqueduct: A Case Report
Year: 2026
Type: article
Study design: Case Report
Abstract: Mondini malformation, also known as incomplete partition type II (IP-II), is one of the most common congenital inner ear malformations associated with sensorineural hearing loss. It results from arrested embryological development of the cochlea during the seventh week of gestation, leading to incomplete cochlear differentiation. Imaging plays a key role in diagnosis, classification, and preoperative evaluation before cochlear implantation. We report the case of a 26-year-old female presenting with bilateral progressive post-lingual sensorineural hearing loss referred for cochlear implant evaluation. High-resolution computed tomography (CT) of the temporal bones demonstrated bilateral incomplete cochlear partition characterized by reduction of the cochlea to one and a half turns, absence of the modiolus, cystic dilatation of the apical turn, enlarged vestibule and enlarged vestibular aqueduct. Magnetic resonance imaging (MRI) confirmed the presence of intact cochlear nerves without associated intracranial abnormalities. This case highlights the essential role of CT and MRI in the diagnosis of Mondini malformation and in guiding surgical planning before cochlear implantation.
Institution: Centre Hospitalier Universitaire Ibn Rochd
Language: English
Subfield: Genetics (clinical)
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7203966438