Aug 2, 2021 · Mondini malformation is a historical term used to describe an incomplete partition type II anomaly with large vestibular aqueduct. The term is often used inappropriately to …

Feb 16, 2023 · Often accompanying the Mondini dysplasia is abnormal communication between the endolymphatic and perilymphatic spaces of the inner ear and subarachnoid space. It is usually caused by a defect in the cribriform area of the lateral end of the internal auditory canal.

We retrospectively reviewed the audiologic, genetic and anamnestic data of 66 patients with NSEVA/PDS followed by our audiology service.

Jan 23, 2025 · First described by Mondini in 1791, the Enlarged Vestibular Aqueduct (EVA) or alternatively "Large Vestibular Aqueduct syndrome" (EVA or LVAS) consists of enlargement of the bony compartment (vestibular aqueduct) that contains the endolymphatic duct (see detailed figure below) that connects the endolymphatic compartment (blue above) to the ...

Mar 21, 2023 · Low-frequency hearing loss is a relatively rare condition in which a person has a decreased ability or inability to hear deeper or low-pitched sounds. These occur in the frequency of 2,000 hertz (Hz) or lower, like an airplane passing overhead or a running appliance.

Enlarged vestibular aqueduct (EVA) is the most common inner ear malformation associated with earlyonset sensorineural hearing loss. 1 It was first described by Mondini 2 in 1791, when it was found in conjunction with cochlear malformation in human temporal bone specimens.

LVAS may be associated with Mondini’s Syndrome or it may occur by itself. This proves that the resulting hearing loss is caused by the large vestibular aqueduct and not by any other abnormalities.

Mondini dysplasia describes failure of complete cochlear development and appears radiologically as a single basal cochlear turn with a distal sac. From: Otolaryngologic Clinics of North America, 2002

Aug 25, 2017 · A high-resolution computed tomography (CT) of the temporal bone was performed. It revealed bilateral enlarged vestibular aqueducts (EVAs) as well as bilateral incomplete partition type II (Mondini dysplasia) of the cochlea. The …

The combination of hearing impairment, EVA or Mondini malformation, and eventually goitre/thyroid dysfunctions is the element that leads to the suspect of PDS as the underlying aetiology of a patient’s HL.