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

Karlberg, Mikael LU ; Annertz, Mårten LU and Magnusson, Måns LU orcid (2006) In Journal of Laryngology and Otology 120(5). p.419-422
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,... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
hearing loss, cochlear diseases, vestibular function tests, conductive
in
Journal of Laryngology and Otology
volume
120
issue
5
pages
419 - 422
publisher
Cambridge University Press
external identifiers
  • wos:000238243300017
  • pmid:16556352
  • scopus:33748751296
  • pmid:16556352
ISSN
1748-5460
DOI
10.1017/S0022215106000934
language
English
LU publication?
yes
id
6ba54da4-2fea-46ef-9b92-66ef745c56df (old id 154453)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16556352&dopt=Abstract
date added to LUP
2016-04-01 11:56:34
date last changed
2020-10-20 03:43:52
@article{6ba54da4-2fea-46ef-9b92-66ef745c56df,
  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.},
  author       = {Karlberg, Mikael and Annertz, Mårten and Magnusson, Måns},
  issn         = {1748-5460},
  language     = {eng},
  number       = {5},
  pages        = {419--422},
  publisher    = {Cambridge University Press},
  series       = {Journal of Laryngology and Otology},
  title        = {Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence.},
  url          = {http://dx.doi.org/10.1017/S0022215106000934},
  doi          = {10.1017/S0022215106000934},
  volume       = {120},
  year         = {2006},
}