Cochlear incomplete partition type III

Cochlear incomplete partition type III (IP-III), also termed X-linked deafness, is a rare type of genetic cochlear anomaly associated with mixed conductive and sensorineural hearing loss

It is caused a mutation in the POU3F4 gene located on the X chromosome.

Male patients present with profound hearing loss (can be progressive) that is predominantly sensorineural +/- conductive. Female carriers have normal hearing or only mild hearing loss. Vestibular function is impaired in affected males, however normal in females.

It is the most common cause of X-linked deafness.

Imaging findings organised by structures include:

Because of the abnormal communication between the subarachnoid and perilymphatic spaces there is a high risk of gushing during stapes manipulation, and so recognition of the X-linked deformity is essential, precluding stapes surgery.

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Article information

rID: 23401
System: Head & Neck
Section: Pathology
Synonyms or Alternate Spellings:
  • X-linked ear deformity
  • Incomplete partition type III
  • DFNX2
  • Deafness X-linked 2
  • X-linked mixed hearing loss
  • XLMHL
  • Deafness 3, conductive, with stapes fixation
  • DFN3
  • Deafness, mixed, with perilymph gusher
  • X-linked deafness with stapes gusher
  • Nance deafness
  • IP-III

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