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Cochlear incomplete partition type III

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

The genetic disorder goes by many names 8:

  • X-linked deafness type 2 (DFNX2)
  • deafness type 3 (DFN3)
  • conductive deafness with stapes fixation
  • Nance deafness
  • X-linked mixed conductive and sensorineural hearing loss
  • X-linked progressive mixed deafness with perilymphatic gusher during stapes surgery
  • X-linked stapes gusher 1

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

It is caused by 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.

The key findings of IP-III concern the cochlea, which is normal in size but has internal abnormalities (incomplete partition) 6,7:

Additional findings are seen in X-linked deafness:

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.

The term incomplete partition III was coined in 2006 by L Sennaroglu 6.

Article information

rID: 23401
System: Head & Neck
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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Cases and figures

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