scholarly journals Assessment of the Effects of Superior Canal Dehiscence Location and Size on Intracochlear Sound Pressures

2014 ◽  
Vol 20 (1) ◽  
pp. 62-71 ◽  
Author(s):  
Marlien E.F. Niesten ◽  
Christof Stieger ◽  
Daniel J. Lee ◽  
Julie P. Merchant ◽  
Wilko Grolman ◽  
...  

Superior canal dehiscence (SCD) is a defect in the bony covering of the superior semicircular canal. Patients with SCD present with a wide range of symptoms, including hearing loss, yet it is unknown whether hearing is affected by parameters such as the location of the SCD. Our previous human cadaveric temporal bone study, utilizing intracochlear pressure measurements, generally showed that an increase in dehiscence size caused a low-frequency monotonic decrease in the cochlear drive across the partition, consistent with increased hearing loss. This previous study was limited to SCD sizes including and smaller than 2 mm long and 0.7 mm wide. However, the effects of larger SCDs (>2 mm long) were not studied, although larger SCDs are seen in many patients. Therefore, to answer the effect of parameters that have not been studied, this present study assessed the effect of SCD location and the effect of large-sized SCDs (>2 mm long) on intracochlear pressures. We used simultaneous measurements of sound pressures in the scala vestibuli and scala tympani at the base of the cochlea to determine the sound pressure difference across the cochlear partition - a measure of the cochlear drive in a temporal bone preparation - allowing for assessment of hearing loss. We measured the cochlear drive before and after SCDs were made at different locations (e.g. closer to the ampulla of the superior semicircular canal or closer to the common crus) and for different dehiscence sizes (including larger than 2 mm long and 0.7 mm wide). Our measurements suggest the following: (1) different SCD locations result in similar cochlear drive and (2) larger SCDs produce larger decreases in cochlear drive at low frequencies. However, the effect of SCD size seems to saturate as the size increases above 2-3 mm long and 0.7 mm wide. Although the monotonic effect was generally consistent across ears, the quantitative amount of change in cochlear drive due to dehiscence size varied across ears. Additionally, the size of the dehiscence above which the effect on hearing saturated varied across ears. These findings show that the location of the SCD does not generally influence the amount of hearing loss and that SCD size can help explain some of the variability of hearing loss in patients. i 2014 S. Karger AG, Basel

2006 ◽  
Vol 120 (5) ◽  
pp. 419-422 ◽  
Author(s):  
M Karlberg ◽  
M Annertz ◽  
M Magnusson

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.


2004 ◽  
Vol 25 (2) ◽  
pp. 121-129 ◽  
Author(s):  
Anthony A. Mikulec ◽  
Michael J. McKenna ◽  
Mitchell J. Ramsey ◽  
John J. Rosowski ◽  
Barbara S. Herrmann ◽  
...  

2020 ◽  
Vol 13 (3) ◽  
pp. e233042
Author(s):  
Diogo Pereira ◽  
Abílio Leonardo ◽  
Delfim Duarte ◽  
Nuno Oliveira

Superior semicircular canal dehiscence is caused by a bone defect on the roof of the superior semicircular canal. The estimated prevalence when unilateral varies between 0.4% and 0.7% and is still unknown when bilateral. Patients may present with audiologic and vestibular symptoms that may vary from asymptomatic to disabling. We report a case of a 72-year-old Caucasian woman presented to otolaryngology department reporting imbalance, bilateral pulsatile tinnitus, hypoacusis while being very sensitive to certain sounds. Physical examination was unremarkable, except for the Rinne test that was negative in both sides. The patient underwent an audiometry revealing a mild bilateral conductive hearing loss. A temporal bone CT scan was performed which evidenced bilateral superior semicircular canal dehiscence. Cervical vestibular evoked myogenic potentials and electrocochleography confirmed diagnosis. Although rare, superior semicircular canal dehiscence shall be considered in conductive hearing loss with vestibular symptoms.


2018 ◽  
Vol 2018 ◽  
pp. 1-10
Author(s):  
Bu-Sheng Tong ◽  
Zi-Yu He ◽  
Chen-Ru Ding ◽  
Juan-Mei Yang ◽  
Jing Wang ◽  
...  

Defective acoustic transmission in the cochlea is closely related with various auditory and vestibular symptoms. Among them, semicircular canal dehiscence (SCD) with a defective semicircular bone is typical. Currently, the pathogenesis of SCD is usually explained by the third window hypothesis; however, this hypothesis fails to explain the variability in the symptoms and signs experienced by superior SCD (SSCD) patients. We evaluated the mechanism of hearing loss in a guinea pig model of bony dehiscence with various sizes and locations along the superior semicircular canal. Auditory brainstem responses (ABRs) and laser Doppler velocimetry were used to measure hearing loss and vibration changes before and after fenestration, as well as after restorative patching. ABR thresholds at low frequencies (e.g., 1000 Hz) increased after fenestration and decreased back to the normal range after we repaired the defect. Energy leakage from the surgically introduced third window was detected in the range of 300–1500 Hz, accompanied by increased vibration at the umbo, stapes head, and the dehiscence site, while decreased vibration was observed at the round window membrane in the same frequency range. After the patching procedure, the deviant vibrations were recovered. The degree of postfenestration energy leakage was proportional to the size of fenestration and the proximity of the fenestration site to the oval window. These results suggest that the bony fenestration of the superior semicircular canal mimics the hearing loss pattern of patients with SSCD. The decrease in perilymph wave impedance likely accounts for the auditory changes.


2017 ◽  
Vol 12 (4) ◽  
pp. 185-191 ◽  
Author(s):  
Marrigje A. de Jong ◽  
David J. Carpenter ◽  
David M. Kaylie ◽  
Erin G. Piker ◽  
Dennis O. Frank-Ito

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