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Lens dislocation
Joseph Junewick, MD FACR
over 8 years ago
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Inner Ear Dysplasia

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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History

2 month old male with profound neurosensory hearing loss.


Case Images


Diagnosis

Incomplete cochlear partition

Findings

CT – Axial and coronal images demonstrate incomplete partition of the cochlea with ectasia of the endolymphatics bilaterally.

Discussion

Incomplete partition of the cochlea , also known as Mondini deformity, is a manifestation of an insult at approximately 7 weeks of gestation resulting in loss of the interscalar septum and spiral lamina with confluence of the apical and middle turns of the cochlea. Mondini deformity accounts for 55% of inner ear malformations. Anomalies of the vestibule, semicircular canal and endolymphatic system are often present.

Reference

Swartz JD, Harnsberger HR. Imaging of the temporal bone, 3rd Ed. Thieme (1998).



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