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J Laryngol Otol. 2006 May;120(5):419-22. Epub 2006 Mar 24.

Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence.

Author information

1
Department of Otorhinolaryngology - Head & Neck Surgery, Lund University Hospital, Sweden.

Abstract

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.

PMID:
16556352
DOI:
10.1017/S0022215106000934
[Indexed for MEDLINE]

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