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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We consider it to be highly important to carry out 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 has a third degree cousin from his mother branch, which bears a cleft palate.

As the basal turn of the cochlea is intact, high-frequency hearing is generally displadia. The syndrome of congenital shortened velum and dual innervation of the soft palate. He was born by cesarean, after a week pregnancy. Int J Pediatr Otorhinolaryngol. 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. The oral examination shows a hypoplastic uvula, a short palate with reduced elevation movements.

The Mondini dysplasia can occur in cases of Pendred Syndrome and Branchio-oto-renal syndrome and in other syndromes, but can occur in non-syndromic deafness. displaia

It’s caused by delection of the long arm of the se 22 22q Loading Stack – 0 images remaining. This article aims at describing and characterizing the middle and inner ear malformations found in two Brazilian boys with the VCFS.

Mondini dysplasia

The DNA sample analyses from the patient and his parents, with five markers, momdini the delection of, at least, one marker in the 22q Axial and mondino 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.


He showed a submucous cleft palate, which was operated when he was 2.

He had a delay in language development and, currently, shows a hypernasal voice and compensatory articulation errors. Thus, most would favour not using the term, except perhaps in cases where the findings exactly match those described by Mondini.

Sometimes the routine axial images do not show completely the stapes and reformations based on multislice acquisitions are of high quality. The neuropsychomotor development was normal.

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

Patient 2 is a 4. There are only 1. CT showing bilateral inflammatory otomastoidopathy and a common cavity between the vestibule and the lateral semicircular canal at right arrow inA. 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.

Born by cesarean he developed respiratory infection, hyperbilirubinemia and hypoglycemia in his 4th day of life, treated for 10 days. Articles needing additional references from April All articles needing additional references Infobox medical condition new All stub articles.

This article about a disease of the ear and mastoid process is a stub.

Mondini malformation | Radiology Reference Article |

University Diploma in Phonoaudiology. By using this site, you agree to the Terms of Use and Privacy Policy. Regarding the labyrinth, mondinj three-dimensional reconstruction is an interesting tool for a global analysis. While the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. A Toshiba Aquilion-slice thickness 0. This finding was only detected after an oblique reformation parallel to the stapes about 30o to 45o.

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.

Timpanometry was of type B and the acoustical reflex was absent bilaterally. Otolaryngological manifestations of velocardiofacial syndrome: New phenotypic alterations of this syndrome have been described in the meantime Views Read Edit View history. From the radiological point displqsia view, oblique reformations with zoom parallel to the stapes are very helpful in detecting mild deformities or incomplete crus.


To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome.

Congenital malformations and deformations of ears Q16—Q17 He shows clinical signs of VCFS, without cardiac defects. The term is often used inappropriately to describe any cochlear abnormality, rather than a specific type of cochlear hypoplasia.

The CT of temporal bones shows signs of bilateral inflammatory otomastoidopathy, a common cavity between the vestibule and the lateral semicircular canal on the right side and an assimetry of the lateral semicircular canal on the left Picture 1.

Retrieved from ” https: 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. Hearing loss and otitis media in velo-cardio-facial syndrome. TBX1 is required for inner ear morphogenesis.

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. Unable to process the form.

Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the latter with the use of reformations based on multislice acquisitions on of the computerized tomography of the temporal bones. Oblique CT reformation with evidence of deformity of the posterior crus of the left stapes arrow.

Middle and inner ear malformations in velocardiofacial syndrome [letter]. Synonyms or Alternate Spellings: April Learn how and when to remove this template message. Braz J Med Biol Res. He had a minor delay in his motor development and a heart murmur dysfunction that was monitored by a cardiologist.