Skip to main content Accessibility help

Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence

  • M Karlberg (a1), M Annertz (a2) and M Magnusson (a1)


In 2003, it was reported that superior semicircular canal dehiscence can mimic otosclerosis because of low-frequency bone conduction hearing gain and dissipation of air-conducted acoustic energy through the dehiscence. We report the case of a 17-year-old girl with left-sided combined hearing loss thought to be due to otosclerosis. Bone conduction thresholds were −10 dB at 250 and 500 Hz and she had a 40 dB air–bone gap at 250 Hz. When a tuning fork was placed at her ankle she heard it in her left ear. Acoustic reflexes and vestibular evoked myogenic potentials could be elicited bilaterally. Imaging of the temporal bones showed no otosclerosis, superior semicircular canal dehiscence or large vestibular aqueduct, but a left-sided, Mondini-like dysplasia of the cochlea with a modiolar deficiency could be seen. Mondini-like cochlear dysplasia should be added to the causes of inner-ear conductive hearing loss.


Corresponding author

Address for correspondence: Dr Mikael Karlberg, Department of Otorhinolaryngology – Head & Neck Surgery, Lund University Hospital, SE-221 85 Lund, Sweden. Fax:  + 46 46 2110968 E-mail:



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed