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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Hearing loss and otitis media in velo-cardio-facial syndrome. A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: Our findings, which have already been described by others, shows that there can be also middle and inner ear malformations, along with malformations of vestibule and semicircular canal.

Mondini dysplasia – Wikipedia

Support Radiopaedia and see fewer ads. Recently, middle and inner ear malformations have been described in VCFS. This is the case of 12 years-old girl who was attended to at the pediatric and otorhinolaryngology service of “William Soler” university pediatric hospital because she presented with three meningoencephalic infections from which Streptococcus pneumonia serotype 19F was isolated.

What is a ‘Mondini’ and what difference does a name make? Timpanometry was of type B and the acoustical reflex was disllasia bilaterally. The neuropsychomotor development was normal. Abnormal carotid arteries in the velocardiofacial syndrome: Some publications have suggested a link between Mondini malformation and spontaneous CSF fistulae and mobdini however, this appears to be only the case with more severe forms of cochlear hypoplasia, and not with a true isolated Mondini malformation 1,4.

Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early mndini life.

displasia de Mondini – English Translation – Word Magic Spanish-English Dictionary

Languages Deutsch Polski Edit links. Oblique CT reformation with evidence of deformity of the posterior crus of the left stapes arrow. Views Read Edit View history. Audiometric moncini, computerized tomography of displaaia temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome.


Although considered as one of the most frequent syndromes associated with cleft palate 6 kondini, it is not frequently recognized in the pediatrics practice. CT showing bilateral inflammatory otomastoidopathy and a common cavity between the vestibule and the lateral semicircular canal at right arrow inA.

Ossicles had a normal configuration. Mondini dysplasia has been associated with thalidomide and rubella embryopathies as well as a number of syndromes:. CT of temporal bones shows a bilateral displasia of the lateral semicircular canals, which are shorter in comparison with the posterior and superior semicircular canals, globosity of vestibules Picture 2 and 3 and mild pericochlear radiolucent foci Picture 4.

Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the displasua with the use of reformations based on multislice acquisitions on of the computerized tomography of the temporal bones.

As the basal turn of the cochlea is intact, high-frequency hearing is generally preserved. In both, analyses of DNA sample with markers of 22q11 region were performed. This deformity was first described in by Mondini after examining the inner ear of a deaf boy.

The other child showed a congenital middle ear malformation with fixation of the malleus on the left annulus tympanicus, and a common cavity bilaterally between the vestibule and the lateral semicircular canal Mondini dysplasiaalso known as Mondini mnodini and Mondini defectis an abnormality of the inner ear that is associated with sensorineural hearing dosplasia.

Unsourced material may be challenged and removed. Further studies are necessary to establish whether this is a consistent morphological trait in VCFS.

Addicional eye findings in a girl with the velo-cardio-facial syndrome [letter]. Middle and inner ear malformations in velocardiofacial syndrome [letter]. Log in Sign up. One of them showed a Mondini type cochlear malformation, an abnormal shape of mondnii ossicles, with fusion of the malleus with the incus and a monopodal stapes.


Patients have sensorineural hearing loss, which is usually bilateral. Primary pulmonary dysgenesis in velocardiofacial syndrome: The first reference to a primary defect of the ossicular chain was made inin a child with signs of the VCFS and 22q The displqsia is often used inappropriately to describe any cochlear abnormality, rather than a specific type of cochlear hypoplasia.

Some children may pass newborn hearing screen to lose displasla in infancy but others present with a hearing loss at birth. Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula.

This article aims at describing and characterizing the middle and inner ear malformations found in two Brazilian boys with the VCFS.

Check for errors and try again. Approved on March 19, Middle and inner ear malformations were foud in two children with VCFS.

Mondini dysplasia

From the radiological point of view, oblique reformations with zoom parallel to the stapes are very helpful in detecting mild deformities or incomplete crus. From the radiological point of view, attention should be given to the use of high quality techniques for the tomographic study of temporal bones. Retrieved from ” https: Otolaryngological manifestations of velocardiofacial syndrome: This finding was only detected after an oblique reformation parallel to the stapes about 30o to 45o.

Case 3 Case 3. Audiological evaluation, including pure tone audiometry, timpanometry, acoustical reflex, and Computerized Tomography CT of temporal bones. This article about a disease of the ear and mastoid process is a displlasia.