Skip to main content
Erschienen in: HNO 1/2018

14.12.2017 | Original articles

Superior canal dehiscence syndrome

Diagnosis with vestibular evoked myogenic potentials and fremitus nystagmus

verfasst von: Prof. Dr. med. R. Gürkov, C. Jerin, W. Flatz, R. Maxwell

Erschienen in: HNO | Sonderheft 1/2018

Einloggen, um Zugang zu erhalten

Abstract

Background

Superior canal dehiscence syndrome (SCDS) is a relatively rare neurotological disorder that is characterized by a heterogeneous clinical picture. Recently, vestibular evoked myogenic potential (VEMP) measurement was established for the diagnosis of SCDS. In the present study, a case series of patients with SCDS were analyzed, with a focus on VEMP.

Methods

Four patients with SCDS were prospectively examined with ocular VEMP (oVEMP) and cervical VEMP (cVEMP). The clinical features and the standard audiovestibular test battery results are summarized and analyzed. The diagnostic accuracy of VEMP testing is evaluated.

Results

The increased oVEMP amplitudes had a specificity of 100% in this patient population. All patients had normal caloric function and head impulse testing. The Tullio sign was observed in two patients. Three patients had autophony. The air-bone gap was not greater than 10 dB in any of the patients. Two patients had marked fremitus nystagmus. All patients had a bony dehiscence of the superior semicircular canal on computed tomography imaging.

Conclusion

The subjective and clinical features in this case series of SCDS patients were heterogeneous. However, objective oVEMP testing had the highest diagnostic value. Furthermore, we describe a new diagnostic clinical sign: fremitus nystagmus.
Anhänge
Nur mit Berechtigung zugänglich
Literatur
1.
Zurück zum Zitat Minor LB et al (1998) Sound- and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg 124(3):249–258CrossRefPubMed Minor LB et al (1998) Sound- and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg 124(3):249–258CrossRefPubMed
3.
Zurück zum Zitat Janky KL et al (2013) Air-conducted oVEMPs provide the best separation between intact and superior canal dehiscent labyrinths. Otol Neurotol 34(1):127–134CrossRefPubMedPubMedCentral Janky KL et al (2013) Air-conducted oVEMPs provide the best separation between intact and superior canal dehiscent labyrinths. Otol Neurotol 34(1):127–134CrossRefPubMedPubMedCentral
4.
Zurück zum Zitat Jongkees LB, Maas JP, Philipszoon AJ (1962) Clinical nystagmography. A detailed study of electro-nystagmography in 341 patients with vertigo. Pract Otorhinolaryngol 24:65–93 Jongkees LB, Maas JP, Philipszoon AJ (1962) Clinical nystagmography. A detailed study of electro-nystagmography in 341 patients with vertigo. Pract Otorhinolaryngol 24:65–93
6.
Zurück zum Zitat Rahne T, Weiser C, Plontke S (2014) Neurofeedback-controlled comparison of the head elevation versus head rotation and head-hand methods in eliciting cervical vestibular evoked myogenic potentials. Audiol Neurootol 19(5):327–335CrossRefPubMed Rahne T, Weiser C, Plontke S (2014) Neurofeedback-controlled comparison of the head elevation versus head rotation and head-hand methods in eliciting cervical vestibular evoked myogenic potentials. Audiol Neurootol 19(5):327–335CrossRefPubMed
7.
Zurück zum Zitat Jerin C et al (2015) Enhancing the reproducibility of ocular vestibular evoked myogenic potentials by use of a visual target originating from a head-mounted laser. Eur Arch Otorhinolaryngol 272(10):2737–2740CrossRefPubMed Jerin C et al (2015) Enhancing the reproducibility of ocular vestibular evoked myogenic potentials by use of a visual target originating from a head-mounted laser. Eur Arch Otorhinolaryngol 272(10):2737–2740CrossRefPubMed
8.
Zurück zum Zitat Maxwell R, Jerin C, Gurkov R (2017) Utilisation of multi-frequency VEMPs improves diagnostic accuracy for Meniere’s disease. Eur Arch Otorhinolaryngol 274(1):85–93CrossRefPubMed Maxwell R, Jerin C, Gurkov R (2017) Utilisation of multi-frequency VEMPs improves diagnostic accuracy for Meniere’s disease. Eur Arch Otorhinolaryngol 274(1):85–93CrossRefPubMed
9.
Zurück zum Zitat Manzari L et al (2012) Ocular and cervical vestibular evoked myogenic potentials to 500 Hz fz bone-conducted vibration in superior semicircular canal dehiscence. Ear Hear 33(4):508–520CrossRefPubMed Manzari L et al (2012) Ocular and cervical vestibular evoked myogenic potentials to 500 Hz fz bone-conducted vibration in superior semicircular canal dehiscence. Ear Hear 33(4):508–520CrossRefPubMed
10.
Zurück zum Zitat Jerin C et al (2014) Ocular vestibular evoked myogenic potential frequency tuning in certain Meniere’s disease. Hear Res 310:54–59CrossRefPubMed Jerin C et al (2014) Ocular vestibular evoked myogenic potential frequency tuning in certain Meniere’s disease. Hear Res 310:54–59CrossRefPubMed
11.
Zurück zum Zitat Volgger V et al (2016) Hydropic inner ear disease of the vestibular type. HNO 64(9):676–679CrossRefPubMed Volgger V et al (2016) Hydropic inner ear disease of the vestibular type. HNO 64(9):676–679CrossRefPubMed
12.
Zurück zum Zitat Manzari L et al (2013) An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies. Otolaryngol Head Neck Surg 149(1):142–145CrossRefPubMed Manzari L et al (2013) An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies. Otolaryngol Head Neck Surg 149(1):142–145CrossRefPubMed
13.
Zurück zum Zitat Luers JC, Huttenbrink KB (2013) Acoustic and vestibular effects of superior semicircular canal dehiscence. HNO 61(9):743–749CrossRefPubMed Luers JC, Huttenbrink KB (2013) Acoustic and vestibular effects of superior semicircular canal dehiscence. HNO 61(9):743–749CrossRefPubMed
14.
Zurück zum Zitat Cremer PD et al (2000) Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal. Neurology 55(12):1833–1841CrossRefPubMed Cremer PD et al (2000) Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal. Neurology 55(12):1833–1841CrossRefPubMed
15.
Zurück zum Zitat Aw ST et al (2011) Three-dimensional vibration-induced vestibulo-ocular reflex identifies vertical semicircular canal dehiscence. J Assoc Res Otolaryngol 12(5):549–558CrossRefPubMedPubMedCentral Aw ST et al (2011) Three-dimensional vibration-induced vestibulo-ocular reflex identifies vertical semicircular canal dehiscence. J Assoc Res Otolaryngol 12(5):549–558CrossRefPubMedPubMedCentral
16.
Zurück zum Zitat Grieser BJ, Kleiser L, Obrist D (2016) Identifying mechanisms behind the Tullio Phenomenon: a computational study based on first principles. J Assoc Res Otolaryngol 17(2):103–118CrossRefPubMedPubMedCentral Grieser BJ, Kleiser L, Obrist D (2016) Identifying mechanisms behind the Tullio Phenomenon: a computational study based on first principles. J Assoc Res Otolaryngol 17(2):103–118CrossRefPubMedPubMedCentral
17.
Zurück zum Zitat Curthoys IS (2017) The new vestibular stimuli: sound and vibration-anatomical, physiological and clinical evidence. Exp Brain Res 235(4):957–972CrossRefPubMed Curthoys IS (2017) The new vestibular stimuli: sound and vibration-anatomical, physiological and clinical evidence. Exp Brain Res 235(4):957–972CrossRefPubMed
18.
Zurück zum Zitat Rohrmeier C, Hilber H, Strutz J (2010) Superior semicircular canal dehiscence syndrome: a case of Tullio phenomenon. HNO 58(10):1057–1060CrossRefPubMed Rohrmeier C, Hilber H, Strutz J (2010) Superior semicircular canal dehiscence syndrome: a case of Tullio phenomenon. HNO 58(10):1057–1060CrossRefPubMed
19.
Zurück zum Zitat Schilder AG et al (2015) Eustachian tube dysfunction: consensus statement on definition, types, clinical presentation and diagnosis. Clin Otolaryngol 40(5):407–411CrossRefPubMedPubMedCentral Schilder AG et al (2015) Eustachian tube dysfunction: consensus statement on definition, types, clinical presentation and diagnosis. Clin Otolaryngol 40(5):407–411CrossRefPubMedPubMedCentral
20.
Zurück zum Zitat Kaski D et al (2012) The Tullio phenomenon: a neurologically neglected presentation. J Neurol 259(1):4–21CrossRefPubMed Kaski D et al (2012) The Tullio phenomenon: a neurologically neglected presentation. J Neurol 259(1):4–21CrossRefPubMed
21.
Zurück zum Zitat Westhofen M (2013) Indications for operative therapy of vestibular vertigo and the associated success rates. HNO 61(9):752–761CrossRefPubMed Westhofen M (2013) Indications for operative therapy of vestibular vertigo and the associated success rates. HNO 61(9):752–761CrossRefPubMed
22.
Zurück zum Zitat Vlastarakos PV et al (2009) Efficacy assessment and complications of surgical management for superior semicircular canal dehiscence: a meta-analysis of published interventional studies. Eur Arch Otorhinolaryngol 266(2):177–186CrossRefPubMed Vlastarakos PV et al (2009) Efficacy assessment and complications of surgical management for superior semicircular canal dehiscence: a meta-analysis of published interventional studies. Eur Arch Otorhinolaryngol 266(2):177–186CrossRefPubMed
23.
Zurück zum Zitat Ziylan F et al (2017) A comparison of surgical treatments for superior semicircular canal dehiscence: a systematic review. Otol Neurotol 38(1):1–10CrossRefPubMed Ziylan F et al (2017) A comparison of surgical treatments for superior semicircular canal dehiscence: a systematic review. Otol Neurotol 38(1):1–10CrossRefPubMed
24.
Zurück zum Zitat Silverstein H et al (2014) Round window reinforcement for superior semicircular canal dehiscence: a retrospective multi-center case series. Am J Otolaryngol 35(3):286–293CrossRefPubMed Silverstein H et al (2014) Round window reinforcement for superior semicircular canal dehiscence: a retrospective multi-center case series. Am J Otolaryngol 35(3):286–293CrossRefPubMed
Metadaten
Titel
Superior canal dehiscence syndrome
Diagnosis with vestibular evoked myogenic potentials and fremitus nystagmus
verfasst von
Prof. Dr. med. R. Gürkov
C. Jerin
W. Flatz
R. Maxwell
Publikationsdatum
14.12.2017
Verlag
Springer Medizin
Erschienen in
HNO / Ausgabe Sonderheft 1/2018
Print ISSN: 0017-6192
Elektronische ISSN: 1433-0458
DOI
https://doi.org/10.1007/s00106-017-0441-x

Weitere Artikel der Sonderheft 1/2018

HNO 1/2018 Zur Ausgabe

Update HNO

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert – ganz bequem per eMail.