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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The Mondini dysplasia describes a cochlea with incomplete partitioning and a reduced number of turns, an enlarged vestibular aqueduct and a dilated vestibule. This article needs additional citations for verification. Velocardiofacial syndrome with facia displassia pinna asymetries. Audiological evaluation, including pure tone audiometry, timpanometry, acoustical reflex, and Computerized Tomography CT of temporal bones.

This article aims at describing and characterizing the middle and inner ear malformations found in two Brazilian boys with the VCFS. He had a delay in language development and, currently, shows a hypernasal voice and compensatory articulation errors.

On the other hand these primary middle and inner ear malformations in VCFS leads to the studies about the role of the genes TBX1, in the morphogenesis of middle and inner ear Born by cesarean he developed respiratory infection, hyperbilirubinemia and hypoglycemia in his 4th day of life, treated for 10 days.

The oral examination shows a dispasia uvula, a short palate with reduced elevation movements.

This finding was only detected after an oblique reformation parallel to the stapes about 30o to 45o. Two boys with clinical signs of VCFS, at the ages of 4. Support Radiopaedia and see fewer ads.

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Articles Cases Courses Quiz. The hearing loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth. He shows clinical signs df VCFS, without cardiac defects.

Mondini dysplasia

Further displasai are necessary to establish whether this is a consistent morphological trait in VCFS. How to cite this article. Journal of Postgraduate Medicine. Synonyms or Alternate Spellings: This deformity was first described in by Mondini after examining the inner ear of a deaf boy.

Mondini dysplasia: recurrent bacterial meningitis in adolescence :

Timpanometry was of type B and the acoustical reflex was absent bilaterally. He has clinical signs of the VCFS. If any doubt it is best to just describe the anatomical abnormalities. Middle and inner ear malformations were foud in two children with VCFS. It’s caused by delection of the long arm of the chromosome 22 22q Our findings, which have already been ddisplasia by others, shows that there can be also middle and inner ear malformations, along with malformations of vestibule and semicircular canal.

To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Accessory auricle Mondini dysplasia. Articles needing additional references from April All articles needing additional references Infobox medical condition new All stub articles. He was born by cesarean, after a week pregnancy.

Edit article Share article View revision history. Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula.


Approved on March 19, Patients have displaasia hearing loss, which is usually bilateral. He showed a submucous cleft palate, which was operated when he was 2.

Mondini dysplasia Mondini malformation Mondini dysplasia Mondini deformity Mondini deformity Mondini’s malformation Mondini anomaly. Retrospective diagnoses of previously missed syndromic disorders among patients with cleft lip, cleft palate, or both. You can also diplasia through stacks with your mouse wheel or the keyboard arrow keys. Int J Pediatr Otorhinolaryngol.

Mondini dysplasia associated to hearing loss and recurrent bacterial meningitis was described in this paper. Additionally, complete or partial absence of the normal interscalar septum is also present, which can now be demonstrated on high-resolution MRI 4. Hearing loss is often progressive and because of the associated widened vestibular aqueduct may progress in a step-wise fashion associated with minor head trauma.

The neuropsychomotor development was normal. 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. Unable to process the form.

Diseases of the ear and mastoid process Congenital disorders of eye, ear, face and neck Disease stubs.