Superior Semicircular Canal Dehiscence Presenting as Conductive Hearing Loss Without Vertigo

Mikulec, Anthony A.; McKenna, Michael J.; Ramsey, Mitchell J.; Rosowski, John J.; Herrmann, Barbara S.; Rauch, Steven D.; Curtin, Hugh D.; Merchant, Saumil N.

doi:10.1097/00129492-200403000-00007

Cited by 286 publications

(329 citation statements)

References 14 publications

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“…We previously reported low-frequency increases in CP response to BC stimuli in chinchilla after the introduction of a SCD with a mean gain of 8.1 dB near 500 Hz . The increased sensitivity to BC in chinchilla is consistent with clinical observations of super-sensitive (<0 dB) BC thresholds in many patients with SCD syndrome (Minor et al, 2003;Mikulec et al, 2004) as well as those found in sandrat (Sohmer et al, 2004). At 500 Hz, the present study showed an 8.7 dB decrease in the chinchillas response to AC stimuli.…”

Section: Comparison To Clinical Studiessupporting

confidence: 92%

“…These studies demonstrated that sensitivity to BC stimuli increases after the introduction of a dehiscence and that the increase is greatest at frequencies below 2 kHz . The increase in sensitivity to BC sound in animal models is consistent with the increased bone-conduction sensitivity (decreased thresholds) in some SCD patients (Minor et al, 2003;Mikulec et al, 2004) (Fig. 1).…”

Section: Introductionsupporting

confidence: 79%

“…Two studies have reported air-bone gaps in SCD patients: Minor et al reported on 4 patients who presented with air-bone gaps of 24 ± 7 dB for frequencies between 250 and 4000 Hz (Minor et al, 2003), and Mikulec et al reported a mean air-bone gap of 37 dB at 500 Hz in 10 ears (Mikulec et al, 2004) from patients that presented with auditory symptoms.…”

Section: Comparison To Clinical Studiesmentioning

confidence: 99%

“…In addition to vestibular symptoms (Minor et al, 1998;Cremer et al, 2000;Minor, 2000;Brantberg et al, 2000), patients with the syndrome frequently have a super-sensitivity to bone-conducted auditory stimuli, and a decreased sensitivity to air-conducted sound (Minor et al, 2003;Mikulec et al, 2004). The increased air-conduction thresholds (20-60 dB) and the decreased bone-conduction thresholds (5-10 dB) together lead to a conductive hearing loss (e.g., air-bone gap) that is largest in the low frequencies (Mikulec et al, 2004). The audiogram from a right-ear SCD patient in Fig.…”

Section: Introductionmentioning

confidence: 99%

“…Some patients with SCD syndrome present with decreased sensitivity to air-conducted sound (Minor et al, 2003;Mikulec et al, 2004). The air-bone gap (calculated as the difference between the bone-conducted and air-conducted thresholds) observed clinically in patients with SCD syndrome has been qualitatively explained by the 'third-window hypothesis' (Minor et al, 1998;Rosowski et al, 2004) that proposes that some of the volume velocity produced by the stapes in response to air-conducted (AC) stimuli is shunted away from the cochlea, through the dehiscent canal.…”

Section: Introductionmentioning

confidence: 99%

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The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla

Songer

Rosowski

2005

Hearing Research

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A superior semicircular canal dehiscence (SCD) is a break or hole in the bony wall of the superior semicircular canal. Patients with SCD syndrome present with a variety of symptoms: some with vestibular symptoms, others with auditory symptoms (including low-frequency conductive hearing loss) and yet others with both. We are interested in whether or not mechanically altering the superior canal by introducing a dehiscence is sufficient to cause the low-frequency conductive hearing loss associated with SCD syndrome. We evaluated the effect of a surgically introduced dehiscence on auditory responses to air-conducted (AC) stimuli in 11 chinchilla ears. Cochlear potential (CP) was recorded at the round-window before and after a dehiscence was introduced. In each ear, a decrease in CP in response to low frequency (<2 kHz) sound stimuli was observed after the introduction of the dehiscence. The dehiscence was then patched with cyanoacrylate glue leading to a reversal of the dehiscence-induced changes in CP. The reversible decrease in auditory sensitivity observed in chinchilla is consistent with the elevated AC thresholds observed in patients with SCD. According to the 'third-window' hypothesis the SCD shunts sound-induced stapes velocity away from the cochlea, resulting in decreased auditory sensitivity to AC sounds. The data collected in this study are consistent with predictions of this hypothesis.

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Section: Comparison To Clinical Studiessupporting

confidence: 92%

Section: Introductionsupporting

confidence: 79%

Section: Comparison To Clinical Studiesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla

Songer

Rosowski

2005

Hearing Research

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Audiological Profile of Children and Young Adults With Syndromic and Complex Craniosynostosis

Jong

Toll²,

Gier³

et al. 2011

Arch Otolaryngol Head Neck Surg

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Most patients with syndromic and complex craniosynostosis have recurrent otitis media with effusion, causing episodes of conductive hearing loss throughout their lives. Sensorineural hearing loss can occur in all 4 syndromes studied but is the primary cause of hearing loss in children and young adults with Muenke syndrome. For patients with these syndromes, we recommend routine visits to the general practitioner or otolaryngologist, depending on national standards of care, to screen for otitis media with effusion throughout life. We also advise early screening for sensorineural hearing loss among children and young adults with these syndromes.

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A Clinical and Histopathologic Study of Jugular Bulb Abnormalities

Friedmann

Eubig²,

Winata³

et al. 2012

Arch Otolaryngol Head Neck Surg

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Jugular bulb abnormalities can erode into the vestibular aqueduct, facial nerve, and the posterior semicircular canal. While symptoms may include pulsatile tinnitus, vertigo, or conductive hearing loss, in contrast to earlier reports, half of the patients were asymptomatic. Dehiscence of vestibular aqueduct rarely leads to clinical or histologic hydrops. The VEMP testing was useful in confirming the presence of inner ear dehiscence due to JBAs. Because the natural history of JBAs is unknown, these patients should be followed closely to evaluate for progression of the JBA or development of symptoms.

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Superior Semicircular Canal Dehiscence Presenting as Conductive Hearing Loss Without Vertigo

Cited by 286 publications

References 14 publications

The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla

The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla

Audiological Profile of Children and Young Adults With Syndromic and Complex Craniosynostosis

A Clinical and Histopathologic Study of Jugular Bulb Abnormalities

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