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Severe Juvenile Hypothyroidism
Joseph Junewick, MD FACR
over 5 years ago
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Case History

2 month old male with profound neurosensory hearing loss.

Case Detail

Anatomy: Neck-Face
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: CT
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Case Images

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Reveal Findings

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

Reveal 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.

Reveal Diagnosis

Incomplete cochlear partition

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