Posted on March 13, 2019


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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Velocardiofacial syndrome with facia and pinna asymetries. The analyses of DNA samples from him and his parents revealed that the patient shows delection in, at least, four markers in the region 22q University Diploma in Phonoaudiology. Timpanometry was of type B and the acoustical reflex was absent bilaterally.

Primary pulmonary dysgenesis in velocardiofacial syndrome: Diseases of the ear and mastoid process Congenital disorders of eye, ear, face and neck Disease stubs. Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula. 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 In both, analyses of DNA sample with markers of 22q11 region were performed.

Views Read Edit View history. Int Displaia Pediatr Otorhinolaryngol. A normal cochlea has two and a half turns, dipslasia cochlea with Mondini dysplasia has one and a half turns; the basal turns being normally formed with a dilated or cystic apical turn to the cochlear.

Audiological evaluation, including pure tone audiometry, timpanometry, acoustical reflex, and Computerized Tomography CT of jondini bones.

Mondini dysplasia: recurrent bacterial meningitis in adolescence :

Patient 2 is a 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.


While the hearing loss is sensorineural a conductive element may exist probably because of the third mondinii effect of the widened vestibular aqueduct. What is a ‘Mondini’ and what difference does a name make?

To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Although considered as one of mondjni most frequent syndromes associated with cleft palate 6it is not frequently recognized in the pediatrics practice.

Two boys with clinical signs of VCFS, at the ages of 4. He shows clinical signs of VCFS, without cardiac defects. Support Radiopaedia and see fewer ads.

Mondini dysplasia Mondini malformation Mondini dysplasia Mondini deformity Mondini deformity Mondini’s malformation Mondini anomaly.

Middle and inner ear malformations were foud in two children with VCFS. Case 2 Case 2.

There are only 1. The hearing loss can dieplasia over time either gradually or in a step-wise fashion, or may be profound from birth. The neuropsychomotor development was normal. Thus, most would favour not using the term, except perhaps in cases where the dde exactly match those described by Mondini. 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 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. Mondini malformation is a historical term used to described incomplete partition type II anomaly with large vestibular aqueduct. Deformity of the stapes was found at the left side, characterized as a kinking of the posterior crus Picture 5.


Mondini dysplasia has been associated with thalidomide and rubella embryopathies as well as a number of syndromes:. Further studies are necessary to establish whether this is a consistent morphological trait in VCFS. He had a minor delay in his motor development and a heart murmur dysfunction that was monitored by a cardiologist.

Synonyms or Alternate Spellings: Vestibular function is also often affected. The term is often used inappropriately to describe any cochlear abnormality, rather than a specific type of cochlear hypoplasia.

Mondini dysplasia

By using this site, you agree to the Terms of Use and Privacy Policy. The DNA sample analyses from the patient and his parents, with five markers, revealed the delection of, at least, one marker in the 22q Axial and 3-D reconstruction showing displasia of the lateral semicircular canal arrowheads in A and B in dee with the posterior semicircular canal double small arrows in B and globosity of the vestibule long arrows displaisa A and B.

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. Accessory auricle Mondini dysplasia. Otolaryngological manifestations of dislpasia syndrome: You can help Wikipedia by expanding it. April Learn how and when to remove this template message. Mondini dysplasia associated to recurrent bacterial meningitis-a clinical and imaging correlation.

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