Hildrew, Douglas M.; Ananth, Ashwin; Rodriguez, Kimsey H.
Objectives/HypothesisThe human cochlea is most commonly considered to have two and a half turns. Although the causes of cochlear hypoplasia are well described, cochlear hyperplasia is a rarer entity that is poorly understood. We describe rare anatomic cochlear malformations identified in a 4-month-old male originally referred for evaluation after a failed newborn hearing screening. The full diagnostic evaluation, imaging findings, treatment, and follow-up are described in detail. Cochleae with three turns are an uncommon malformation that is not included in current classifications schemes and may represent a distinct type of anomaly not caused by developmental arrest. Laryngoscope, 126:469-471, 2016