A newborn with three cochlear turns: Case report and literature review

Laryngoscope. 2016 Feb;126(2):469-71. doi: 10.1002/lary.25560. Epub 2015 Aug 26.


Objectives/Hypothesis: The 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.

Keywords: Three cochlear turns; cochlea; cochlear malformations; conductive hearing loss; conductive hearing loss (CHL); hearing loss; hyperplasia congenital; hyperplastic; sensorineural hearing loss; sensorineural hearing loss (SNHL).

Publication types

  • Case Reports
  • Review

MeSH terms

  • Audiometry
  • Cochlea / abnormalities*
  • Cochlea / surgery
  • Hearing Loss, Sensorineural / congenital*
  • Hearing Loss, Sensorineural / diagnosis
  • Hearing Loss, Sensorineural / surgery
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Middle Ear Ventilation / methods*
  • Tomography, X-Ray Computed