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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Mild pericochlear radiolucent foci arrows in CT coronal view.
Support Radiopaedia and see mondinj ads. Vestibular function is also often affected. 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.
Check for errors and try again. How to cite this article. Retrieved from ” https: Views Read Edit View history. To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome.
Mondini dysplasia associated to hearing loss and recurrent bacterial meningitis was described in this paper. See classification of congenital cochlear anomalies.
While the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. 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, displazia well as the occurrence of symptoms related to the vestibular function, already described in children with the VCFS Velocardiofacial syndrome with facia and pinna asymetries.
Some publications have suggested a link between Mondini malformation and spontaneous CSF fistulae and meningitis; 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.
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 On the other hand these primary middle and inner ear malformations in VCFS leads to the studies about the role mmondini the genes TBX1, in the morphogenesis of middle and inner ear Patients have sensorineural hearing loss, which is usually bilateral.
The Mondini dysplasia describes a cochlea with incomplete partitioning and a reduced number of turns, an enlarged vestibular aqueduct and a dilated vestibule. Sometimes the routine axial images do not show completely the stapes and reformations based on multislice acquisitions are of high quality. High-resolution computerized axial tomography of the ear axial and coronal planes evinced the cochlear and vestibular malformation, with tissue of the middle ear communicating directly with the inner ear of the left size at the oval window.
He had a dksplasia delay in his motor development and a heart murmur dysfunction that was monitored by a cardiologist.
Mondini dysplasia – Wikipedia
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Log in Sign up. From the radiological point of view, attention should be given to the use of high quality techniques for the tomographic study of temporal bones. Diseases of the ear and mastoid process Congenital disorders of eye, ear, face and neck Disease dixplasia.
The term is often used inappropriately to describe any cochlear abnormality, rather than a specific type of cochlear hypoplasia.
Primary pulmonary dysgenesis in velocardiofacial syndrome: Audiometric evaluation, computerized tomography of the temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome. Case 2 Case 2. The syndrome of congenital shortened velum and dual innervation of the soft palate. He has a third degree cousin from his mother branch, which bears a cleft palate.
University Diploma in Phonoaudiology. Oblique CT reformation with evidence of deformity of the posterior crus of the left stapes arrow. dispkasia
This is the case of 12 years-old girl modnini 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. The mondinii loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth.
Mondini dysplasia has been associated with thalidomide and rubella embryopathies as well as a number of syndromes:. Edit article Share article View revision history. The neuropsychomotor development was normal.
This finding was only detected after an oblique reformation parallel to the stapes about 30o to 45o. Timpanometry was of type B and the acoustical reflex was absent bilaterally.
You can help Wikipedia by expanding it. The displzsia evaluation shows a conductive hearing loss, with eisplasia 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.
CT showing bilateral inflammatory otomastoidopathy and a common cavity between the vestibule and the dsiplasia semicircular canal at right arrow inA. Mondini malformation is a historical term used to described incomplete partition type II anomaly with large vestibular aqueduct.
April Learn how and when to remove this template message. Regarding the labyrinth, a three-dimensional reconstruction is an interesting tool for a global analysis.
TBX1 is required for inner ear morphogenesis. Born by cesarean he developed respiratory infection, hyperbilirubinemia and hypoglycemia in his 4th day of life, treated for 10 days. One of them showed a Mondini type cochlear malformation, an abnormal shape of the ossicles, with fusion of the malleus with the incus and a monopodal stapes.
Mondini dysplasiaalso known as Mondini malformation and Mondini defectis an abnormality of the inner ear that is associated with sensorineural hearing loss. It is considered that the occurrence of this malformation results from the disruption of the embryonal development on the 7th week of gestation when the cochlear development ceases.
Axial and 3-D reconstruction showing displasia of the lateral semicircular canal arrowheads in A and B in comparison with the posterior semicircular canal double small arrows in B and globosity of the vestibule long arrows in A and B.
A Toshiba Aquilion-slice thickness 0. Further studies are necessary to establish whether this is a consistent morphological trait in VCFS.