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Article

The Cochlea in Branchio-Oto-Renal Syndrome: An Objective Method for the Diagnosis of Offset Cochlear Turns.

AJNR. American journal of neuroradiology - 1 Nov 2022

Juliano A F, D'Arco F, Pao J, Picariello S, Clement E, Moonis G, Robson C D

Abstract excerpt

BACKGROUND AND PURPOSE: An "unwound" or "offset" cochlea has been described as a characteristic imaging feature in patients with branchio-oto-renal syndrome, and recently recognized to be associated in particular to those with EYA1 gene mutations. Determination of this feature has traditionally relied on subjective visual assessment. Our aim was to establish an objective assessment method for cochlear offset (the...

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PMID: 36175083
DOI: 10.3174/ajnr.A7653
PMCID: PMC9731253

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