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CT of the ear in Pendred syndrome

Moshe Goldfeld1, Benjamin Glaser, Elias Nassir

  • 1Departments of Radiology and Internal Medicine, Western Galilee Hospital, Nahariya 22100, Israel. goldme1@yahoo.com

Radiology
|March 11, 2005
PubMed

Insights

Inner ear malformations are consistently found in Pendred syndrome, with modiolus deficiency and enlarged vestibules being the most common structural anomalies identified via thin-section CT.

Area of Science:

  • Radiology
  • Genetics
  • Otolaryngology

Background:

  • Pendred syndrome is a genetic disorder often associated with hearing loss.
  • Inner ear structural anomalies are suspected in Pendred syndrome but require detailed imaging for confirmation.

Purpose of the Study:

  • To prospectively identify and characterize inner ear structural anomalies using thin-section computed tomography (CT) in a family with Pendred syndrome.
  • To correlate imaging findings with the known genetic mutation in the PDS gene.

Main Methods:

  • Thin-section CT scans of the inner ear were performed on 12 patients with Pendred syndrome.
  • Evaluated structures included the cochlea (interscalar septum), vestibule, vestibular aqueduct, and modiolus.
  • Two radiologists assessed all CT scans in consensus.

Main Results:

  • All 12 patients exhibited bilateral inner ear malformations.
  • Modiolus deficiency and enlarged vestibules were present in all patients.
  • Absence of the interscalar septum was noted in 75% of ears, and enlarged vestibular aqueducts in 80% of ears.

Conclusions:

  • Inner ear malformation is a universal finding in this cohort with Pendred syndrome.
  • Modiolus deficiency and vestibular enlargement are the most consistent CT-identified anomalies in Pendred syndrome.
  • Thin-section CT is effective in visualizing these structural abnormalities.
Abstract

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