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Joseph Junewick, MD FACR
over 5 years ago
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Mondini Malformation

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

9 month old with sensorineural hearing loss.


Case Images


Diagnosis

Mondini Malformation

Findings

MR – Axial high resolution T2 images show preserved basal turn but absence of the apical turns of the cochlea and enlarged vestibule.

Discussion

Cochlear disorders are often all classified as Mondini deformities but more accurately Mondini dysplasia is the result of absence of the distal 1 and 1/2 turns of the cochlea (i.e., the apex). Patients with Mondini deformity often have loss of low-frequency hearing and vertigo. The basilar turn of the cochlea is usually present and there may be deformity of the vestibule, semicircular canals, and/or vestihular aqueduct. Perilymphatic fistulas, caused by absence of or defects in the stapes footplate, may lead to otorrhea, rhinorrhea, or recurrent meningitis. The Mondini defect occurs at approximately 7 weeks of development; most cases have a genetic or syndromic association (DiGeorge, Pendred, Klipple-Feil) but antenatal infections (rubella) and various teratogens (thalidomide and isoretinoic acid) are other potential etiologies.

Reference

Lo WWM. What is a ‘Mondini’ and What Difference Does a Name Make? AJNR (1999); 20:1442-1444.



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