Mondini dysplasia associated to recurrent bacterial meningitis-a clinical and imaging correlation. Rev Cubana Pediatr [online]. , vol, n.1, pp Mondini dysplasia is a type of inner ear malformation that is present at birth . Villamar M, del Castillo I, Moreno F. Sensorineural hearing loss and Mondini. Mondini malformation is a historical term used to described incomplete partition type II anomaly with large vestibular aqueduct. Terminology The term is often.

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Middle and inner ear malformations in velocardiofacial syndrome [letter]. TBX1 is required for inner ear morphogenesis. Patients with hearing loss associated with otitis media and with external ear malformations are described Ddisplasia Learn how and when to remove this template message.

Mondini dysplasia

The first reference to a primary defect of the ossicular chain was made inin a child with signs of the VCFS and 22q Loading Stack – 0 images remaining. Audiological evaluation, including pure tone audiometry, timpanometry, acoustical reflex, and Computerized Tomography Displzsia of temporal bones. This deformity was first described in by Mondini after examining the inner ear of a deaf boy.

What is a ‘Mondini’ and what difference does a name make?

This article needs additional citations for verification. From the clinical point of view, we consider it to be highly important to carry out a thorough evaluation and the monitoring of the hearing evolution, as well as the occurrence of symptoms related to the vestibular function, already described in children with the VCFS Log in Sign up.


Mondini dysplasia: recurrent bacterial meningitis in adolescence :

Timpanometry was of type B and the acoustical reflex was absent bilaterally. A Toshiba Aquilion-slice thickness 0. Languages Deutsch Polski Edit links.

As the basal turn of the cochlea is intact, high-frequency hearing is generally preserved. We consider it to be highly important to carry diaplasia a thorough evaluation and monitoring of the hearing evolution, as well as the occurrence of symptoms related to the vestibular function in patients with velocardiofacial syndrome.

He had a minor delay in his motor development and a heart murmur dysfunction that was monitored by a cardiologist. Synonyms or Alternate Spellings: The audiological evaluation shows a conductive hearing loss, with air thresholds around 40 to 50dB HL on the right side and 25 to 30dB HL on the left, with normal bone thresholds, between 0 and 10 dB HL bilaterally.

While the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. Case 2 Case 2.

Otolaryngological manifestations of velocardiofacial syndrome: To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Please help improve this article by adding citations to reliable sources. From Wikipedia, the free encyclopedia.

He has a third degree cousin from his mother branch, which bears a cleft palate. Mild pericochlear radiolucent foci arrows in CT coronal view. Braz J Med Biol Res.


Patient 2 is a 4. There are mmondini 1. Patients have sensorineural hearing loss, which is usually bilateral. The DNA sample analyses from the patient and his parents, with five markers, revealed the delection of, at least, one marker in the 22q Views Read Edit View history.

Displasia de Mondini asociada a meningitis bacteriana recurrente, correlación clínico-imagenológica

A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: Velocardiofacial syndrome with facia and pinna asymetries. Some children may pass newborn hearing screen to lose hearing in infancy but others present with a hearing loss at birth. This paper underlined the importance of the clinical suspicion of Mondini disease, and the scientific mondkni of the computerized axial tomography of the temporal bone in order to early diagnose the congenital fistula in the inner ear mondjni to recurrent bacterial meningitis.

Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula. It is considered that the dizplasia of this malformation results from the disruption of the embryonal development on the 7th week of gestation when the cochlear development ceases.

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