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Research ArticleHead & Neck

The Unwound Cochlea: A Specific Imaging Marker of Branchio-Oto-Renal Syndrome

A. Hsu, N. Desai and M.J. Paldino
American Journal of Neuroradiology December 2018, 39 (12) 2345-2349; DOI: https://doi.org/10.3174/ajnr.A5856
A. Hsu
aFrom the Department of Radiology, Texas Children's Hospital, Houston, Texas.
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N. Desai
aFrom the Department of Radiology, Texas Children's Hospital, Houston, Texas.
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M.J. Paldino
aFrom the Department of Radiology, Texas Children's Hospital, Houston, Texas.
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  • Fig 1.
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    Fig 1.

    Unwound cochlea. A, Axial CT image through the right cochlea of a patient with BOR demonstrates characteristic unwound dysmorphology with anteromedial rotation and displacement of the middle turn away from a tapered basal turn (arrow). B, Axial CT image through a normal right cochlea demonstrates normal apposition of the middle and basal turns (arrowhead).

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    Fig 2.

    Cochlear morphology in patients with branchio-oto-renal syndrome. Axial CT images through the right temporal bone in 4 representative patients (A–D) with unwound cochleae.

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    Fig 3.

    Branchio-oto-renal syndrome without the unwound cochlea. Axial CT image through the right temporal bone demonstrates a truncated basal turn with complete absence of the middle and apical turns of the cochlea.

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    Fig 4.

    Interreader disagreement. Axial CT images through the right temporal bone in the single patient with BOR with interreader discrepancy. This cochlea was considered unwound at consensus re-review.

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    Table 1:

    Summary of inner ear findings in patients with branchio-oto-renal syndrome

    Patients with BORHearing LossUnwound Cochlea (Reader 1)Unwound Cochlea (Reader 2)Other Inner Ear Abnormalities
    1B MixedBilateralBilateralR vestibular aqueduct enlargement; B medialized facial nerve; B funnel IAC
    2B MixedBilateralBilateralB hypoplastic apical turn/modiolus; right hypoplastic posterior semicircular canal; B funnel IAC
    3B ConductiveBilateralBilateralB medialized facial nerve; B funnel IAC
    4B ConductiveBilateralBilateralB vestibular aqueduct enlargement; B funnel IAC
    5B ConductiveBilateralBilateralL vestibular aqueduct enlargement; B hypoplastic lateral semicircular canal; L medialized facial nerve; B funnel IAC
    6B SensorineuralBilateralBilateralB hypoplastic apical turn/modiolus; B cochlear aperture stenosis; B vestibular aqueduct enlargement; B medialized facial nerve; L funnel IAC
    7B SensorineuralNoNoB cochlear hypoplasia with absent middle and apical turns; B medialized facial nerve
    8B MixedBilateralNoB medialized facial nerve
    9B SensorineuralBilateralBilateralB hypoplastic apical turn/modiolus; B vestibular aqueduct enlargement; B medialized facial nerve; B funnel IAC
    • Note:—IAC indicates internal auditory canal; R, right side; L, left side; B, bilateral.

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    Table 2:

    Abnormalities of the cochlea and inner ear in control patients with hearing loss not associated with branchio-oto-renal syndrome

    Cochlear AbnormalityOther Inner Ear Abnormalities
    Incomplete partition defect type 2 (n = 3)Posterior semicircular canal hypoplasia (n = 1)
    Cochlear aperture stenosis (n = 1)Posterior semicircular canal dehiscence (n = 1)
    Labyrinthitis ossificans (n = 1)Vestibular aqueduct enlargement (n = 1)
    Funnel-shaped internal auditory canal (n = 2)
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    Table 3:

    Test characteristics of the unwound cochlear morphology for a clinical diagnosis of branchio-oto-renal syndromea

    ReaderSensitivitySpecificityPPVNPV
    10.89 (0.51–0.99)1.0 (0.91–1.0)1.0 (0.60–1.0)0.98 (0.88–1.0)
    20.78 (0.40–0.96)1.0 (0.91–1.0)1.0 (0.56–1.0)0.96 (0.86–0.99)
    • Note:—NPV indicates negative predictive value; PPV, positive predictive value.

    • ↵a Numbers in parentheses are 95% CIs.

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American Journal of Neuroradiology: 39 (12)
American Journal of Neuroradiology
Vol. 39, Issue 12
1 Dec 2018
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Cite this article
A. Hsu, N. Desai, M.J. Paldino
The Unwound Cochlea: A Specific Imaging Marker of Branchio-Oto-Renal Syndrome
American Journal of Neuroradiology Dec 2018, 39 (12) 2345-2349; DOI: 10.3174/ajnr.A5856

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The Unwound Cochlea: A Specific Imaging Marker of Branchio-Oto-Renal Syndrome
A. Hsu, N. Desai, M.J. Paldino
American Journal of Neuroradiology Dec 2018, 39 (12) 2345-2349; DOI: 10.3174/ajnr.A5856
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  • The Cochlea in Branchio-Oto-Renal Syndrome: An Objective Method for the Diagnosis of Offset Cochlear Turns
  • Re-Examining the Cochlea in Branchio-Oto-Renal Syndrome: Genotype-Phenotype Correlation
  • Characteristic Cochlear Hypoplasia in Patients with Walker-Warburg Syndrome: A Radiologic Study of the Inner Ear in {alpha}-Dystroglycan-Related Muscular Disorders
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