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 syndrome of congenital shortened velum and dual innervation of the soft palate. Further displzsia are necessary to establish whether this is a consistent morphological trait in VCFS. Thank you for updating your details.

Middle and Inner Ear Malformations in Two Cases of Velocardiofacial Syndrome

Mondini congenital malformation and severe unilateral neurosensory hypoacusis; recurrent bacterial meningitis; computerized tomography; cerebrospinal fluid fistula. Approved on March 19, About Blog Go ad-free. He shows clinical signs of VCFS, without cardiac defects. The Mondini dysplasia describes a cochlea with incomplete partitioning and a displasiw number of turns, an enlarged vestibular aqueduct and a dilated vestibule.

See classification of congenital cochlear anomalies.

Mondini dysplasia

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. Patients have sensorineural hearing loss, which is usually bilateral.


Displqsia the hearing loss is sensorineural a conductive element may exist probably because of the third window effect of the widened vestibular aqueduct. Accessory auricle Mondini dysplasia. By using this site, you agree to the Terms of Use and Privacy Policy. Case 3 Case 3. TBX1 is required for inner ear morphogenesis. How to cite this article. Case 2 Case 2.

The hearing loss can deteriorate over time either gradually or in a step-wise fashion, or may be profound from birth. Mkndini 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.

There are only 1. Views Read Edit View history. Born by cesarean he developed respiratory infection, hyperbilirubinemia and hypoglycemia in his 4th dipslasia of life, treated for 10 days. Hearing loss associated with Mondini dysplasia may first become manifest in childhood or early adult life. Support Radiopaedia and see fewer ads.

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


Additionally, complete or partial absence of the normal interscalar septum is also present, which can now be demonstrated on high-resolution MRI 4. University Diploma in Phonoaudiology. Thus, most would favour not using the term, except perhaps in cases where the findings exactly match those described by Mondini.

April Learn how and when to remove this template message. Journal of Postgraduate Medicine.

This deformity was first described in by Mondini after examining the inner ear of a deaf boy. This finding was only detected after an oblique reformation parallel to the stapes about 30o to 45o.

This page was last edited on 24 Aprilat Hearing loss is often progressive and because of the associated widened vestibular mondinni may progress in a step-wise fashion associated with minor head trauma.

Some children may pass newborn hearing screen to lose hearing in infancy but others present with a hearing loss at birth.

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