上半规管裂综合征

doi:10.6040/j.issn.1673-3770.1.2020.080

摘要/Abstract

摘要： 上半规管裂(SSCD)综合征是由于颅底上半规管骨质缺损所导致,特别是在弓状隆起处。骨迷路缺损导致在硬脑膜和膜迷路之间形成直接连通,形成可移动的第三窗,导致各种听觉和前庭症状。耳鸣和自听增强是最常见的听力学表现,头晕和不平衡是最常见的前庭表现。听力检查的结果根据疾病的严重程度而有所不同,低频传导性听力下降是常见的表现,骨传导阈值可能为负值。SSCD综合征患者患耳的前庭诱发肌源性电位(VEMP)反应阈值通常降低,也可能具有比正常VEMP更大的振幅。颞骨的高分辨率计算机断层(CT)扫描在确诊SSCD中起着重要作用。通常建议使用Pöschl和Stenver重新格式化的视图。外科治疗适用于前庭症状和听力症状严重影响生活质量的患者,有两种主要的手术径路(颅中窝,乳突)和几种技术(封堵、加帽、重铺表面和组合法)。目前,没有足够的证据确切表明哪种手术方法或技术更好。通过颅中窝径路或经乳突径路的手术修复SSCD对听力和前庭症状的改善非常有效且并发症风险较低。

关键词: 上半规管裂综合征, 临床表现, 诊断治疗

Abstract: Superior semicircular canal dehiscence(SSCD)syndrome occurs as a result of a bony defect of the skull base involving the superior semicircular canal, particularly at the arcuate eminence. The bony labyrinthine defect creates a direct communication between the dura and the labyrinthine membranous structure and acts as a mobile third window which may result in various auditory and vestibular manifestations. Tinnitus and autophony are the most common audiological manifestations. Dizziness and disequilibrium are the most common vestibular manifestations. Audiometric findings vary based on the severity of the disease. Low-frequency conductive hearing loss is a common finding. Bone conduction thresholds may be negative. A patient with SSCD will typically have a lower Vestibular Evoked Myogenic Potentials(VEMP)threshold response in the affected ear and may also have a larger than normal VEMP amplitude. High-resolution computed tomography(CT)scan of temporal bone plays an important role in confirming the diagnosis of SSCD. Pöschl and Stenver reformatted views are often recommended. Surgical treatment is reserved for patients presenting with debilitating vestibular and auditory manifestations that substantially interfere with their quality of life. There are two main surgical approaches(middle fossa, trans-mastoid)and several techniques(plugging, capping, resurfacing and combination). Presently, there is insufficient evidence to clearly determine which surgical approach or technique is superior. Surgical repair of SSCD through either the middle cranial fossa approach or trans-mastoid approach is highly effective for auditory and vestibular symptom improvement and is associated with a low risk of complications.

Key words: Superior semicircular canal dehiscence, Clinical features, Diagnosis and treatment

中图分类号:

R764

Fahad Alkherayf, Hussein Kheshaifati, Abdulhadi Algahtani, Santanu Chakraborty, David Schramm. 上半规管裂综合征[J]. 山东大学耳鼻喉眼学报, 2020, 34(5): 89-96.

参考文献

[1] Minor LB, Solomon D, Zinreich JS, et al. Sound- and/or pressure-induced Vertigo due to bone dehiscence of the superior semicircular canal[J]. Arch Otolaryngol Head Neck Surg, 1998, 124(3): 249. doi:10.1001/archotol.124.3.249.
[2] Bi WL, Brewster R, Poe D, et al. Superior semicircular canal dehiscence syndrome[J]. J Neurosurg, 2017, 127(6): 1268-1276. doi:10.3171/2016.9.jns16503.
[3] Yuen HW, Boeddinghaus R, Eikelboom RH, et al. The relationship between the air-bone gap and the size of superior semicircular canal dehiscence[J]. Otolaryngol Head Neck Surg, 2009, 141(6): 689-694. doi:10.1016/j.otohns.2009.08.029.
[4] Carey JP, Minor LB, Nager GT. Dehiscence or thinning of bone overlying the superior semicircular canal in a temporal bone survey[J]. Arch Otolaryngol Head Neck Surg, 2000, 126(2): 137-147. doi:10.1001/archotol.126.2.137.
[5] Licht AK, Schulmeyer F, Allert M, et al. Vertigo and hearing disturbance as the first sign of a glioblastoma(world health organization grade IV)[J]. Otol Neurotol, 2004, 25(2): 174-177. doi:10.1097/00129492-200403000-00016.
[6] Chi FL, Ren DD, Dai CF. Variety of audiologic manifestations in patients with superior semicircular canal dehiscence[J]. Otol Neurotol, 2010, 31(1): 2-10. doi:10.1097/mao.0b013e3181bc35ce.
[7] Hirvonen TP, Weg N, Zinreich SJ, et al. High-resolution CT findings suggest a developmental abnormality underlying superior canal dehiscence syndrome[J]. Acta Oto- Laryngol, 2003, 123(4): 477-481. doi:10.1080/0036554021000028099.
[8] Brantberg K, Baggersjoback D, Mathiesen T, et al. Posterior canal dehiscence syndrome caused by an apex cholesteatoma[J]. Otol Neurotol, 2006, 27(4): 531-534. doi:10.1097/01.mao.0000201433.50122.62.
[9] Takahashi N, Tsunoda A, Shirakura S, et al. Anatomical feature of the middle cranial Fossa in fetal periods: Possible etiology of superior canal dehiscence syndrome[J]. Acta Oto-Laryngol, 2012, 132(4): 385-390. doi:10.3109/00016489.2011.637234.
[10] Nadgir RN, Ozonoff A, Devaiah AK, et al. Superior semicircular canal dehiscence: congenital or acquired condition?[J]. AJNR Am J Neuroradiol, 2011, 32(5): 947-949. doi:10.3174/ajnr.a2437.
[11] Christopher AS, Paul N, Ravi NS, et al. The correlation between obesity, obstructive sleep apnea, and superior semicircular canal dehiscence: a new explanation for an increasingly common problem[J]. Otol Neurotol, 2015, 36(3):551-554. doi: 10.1097/MAO.0000000000000555.
[12] Brandolini C, Modugno GC, Pirodda A. Dehiscence of the superior semicircular canal: a review of the literature on its possible pathogenic explanations[J]. Eur Arch Otorhinolaryngol, 2014, 271(3): 435-437. doi:10.1007/s00405-013-2497-x.
[13] Yu A, Teich DL, Moonis G, et al. Superior semicircular canal dehiscence in East Asian women with osteoporosis[J]. BMC Ear Nose Throat Disord, 2012, 12: 8. doi:10.1186/1472-6815-12-8.
[14] Crovetto MA, Whyte J, Rodriguez OM, et al. Influence of aging and menopause in the origin of the superior semicircular canal dehiscence[J]. Otol Neurotol, 2012, 33(4): 681-684. doi:10.1097/mao.0b013e31824f9969.
[15] Puwanarajah P, Pretorius P, Bottrill I. Superior semicircular canal dehiscence syndrome: a new aetiology[J]. J Laryngol Otol, 2008, 122(7): 741-744. doi:10.1017/s0022215108001965.
[16] Saxby AJ, Gowdy C, Fandiño M, et al. Radiological prevalence of superior and posterior semicircular canal dehiscence in children[J]. Int J Pediatr Otorhinolaryngol, 2015, 79(3): 411-418. doi:10.1016/j.ijporl.2015.01.001.
[17] Baloh RW. Superior semicircular canal dehiscence syndrome: Leaks and squeaks can make You dizzy[J]. Neurology, 2004, 62(5): 684-685. doi:10.1212/01.wnl.0000118644.59800.6a.
[18] Minor L. Superior canal dehiscence syndrome[J]. Am J Otolaryngol, 2000, 21(1): 9-19. doi:10.1016/s0196-0709(00)80105-2.
[19] Minor LB, Cremer PD, Carey JP, et al. Symptoms and signs in superior canal dehiscence syndrome[J]. Ann N Y Acad Sci, 2001, 942: 259-273. doi:10.1111/j.1749-6632.2001.tb03751.x.
[20] Teixido MT, Artz GJ, Kung BC. Clinical experience with symptomatic superior canal dehiscence in a single neurotologic practice[J]. Otolaryngol Head Neck Surg, 2008, 139(3): 405-413. doi:10.1016/j.otohns.2008.06.023.
[21] Jacky Chen CH, Nguyen T, Udawatta M, et al. Clinical assessment of patients with bilateral superior semicircular canal dehiscence[J]. World Neurosurg, 2019, 126: e1549-e1552. doi:10.1016/j.wneu.2019.03.205.
[22] Masaki Y. The prevalence of superior canal dehiscence syndrome as assessed by temporal bone computed tomography imaging[J]. Acta Otolaryngol, 2011, 131(3): 258-262. doi:10.3109/00016489.2010.526145.
[23] Shama SA, Eid M, Mehanna AMA, et al. Dehiscences of the semicircular canals as discrete third window lesions of the inner ear[J]. Egypt J Radiol Nucl Med, 2013, 44(1): 15-21. doi:10.1016/j.ejrnm.2012.12.007.
[24] Suryanarayanan R, Lesser TH. ‘Honeycomb’ tegmen: multiple tegmen defects associated with superior semicircular canal dehiscence[J]. J Laryngol Otol, 2010, 124(5): 560-563. doi:10.1017/s0022215109991411.
[25] Cremer PD, Minor LB, Carey JP, et al. Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal[J]. Neurology, 2000, 55(12): 1833-1841. doi:10.1212/wnl.55.12.1833.
[26] Young AS, McMonagle B, Pohl DV, et al. Superior semicircular canal dehiscence presenting with recurrent positional Vertigo[J]. Neurology, 2019, 93(24): 1070-1072. doi:10.1212/wnl.0000000000008624.
[27] Cawthorne T. Otosclerosis[J]. J Laryngol Otol, 1955, 69(7): 437-456. doi:10.1017/s0022215100050933.
[28] Hennebert, C, A new syndrome in hereditary syphilis of the labyrinth. Press Med Belg Brux, 1911,63:467.
[29] Gianoli GJ, Soileau JS. Superior semicircular canal dehiscence[M] //Dizziness and Vertigo Across the Lifespan. Amsterdam: Elsevier, 2019: 143-151. doi:10.1016/b978-0-323-55136-6.00012-5.
[30] Picavet V, Govaere E, Forton G. Superior semicircular canal dehiscence: prevalence in a population with clinical suspected otosclerosis-type hearing loss[J]. B-ent, 2009, 5(2): 83-88.
[31] Onusko E. Tympanometry[J]. Am Fam Physician, 2004,70(9):1713-1720.
[32] McEvoy TP, Mikulec AA, Armbrecht ES, et al. Quantification of hearing loss associated with superior semi-circular canal dehiscence[J]. Am J Otolaryngol, 2013, 34(4): 345-349. doi:10.1016/j.amjoto.2013.01.009.
[33] Janky KL, Shepard N. Vestibular evoked myogenic potential(VEMP)testing: normative threshold response curves and effects of age[J]. J Am Acad Audiol, 2009, 20(8): 514-522. doi:10.3766/jaaa.20.8.6.
[34] Murofushi T, Matsuzaki M, Mizuno M. Vestibular evoked myogenic potentials in patients with acoustic neuromas[J]. Arch Otolaryngol Head Neck Surg, 1998, 124(5): 509-512. doi:10.1001/archotol.124.5.509.
[35] Zhou G, Gopen Q, Poe DS. Clinical and diagnostic characterization of canal dehiscence syndrome: a great otologic mimicker[J]. Otol Neurotol, 2007, 28(7): 920-926. doi:10.1097/mao.0b013e31814b25f2.
[36] Brantberg K. Vestibular evoked myogenic potentials(VEMPs): usefulness in clinical neurotology[J]. Semin Neurol, 2009, 29(5): 541-547. doi:10.1055/s-0029-1241042.
[37] Chihara Y, Iwasaki S, Ushio M, et al. Vestibular-evoked extraocular potentials by air-conducted sound: Another clinical test for vestibular function[J]. Clin Neurophysiol, 2007, 118(12): 2745-2751. doi:10.1016/j.clinph.2007.08.005.
[38] Zuniga MG, Janky KL, Nguyen KD, et al. Ocular versus cervical VEMPs in the diagnosis of superior semicircular canal dehiscence syndrome[J]. Otol Neurotol, 2013, 34(1): 121-126. doi:10.1097/mao.0b013e31827136b0.
[39] Manzari, L, Burgess AM, McGarvie LA, et al. An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies[J]. Otolaryngol Head Neck Surg, 2013,149(1):142-145. doi:10.1177/0194599813489494.
[40] Piker EG, Jacobson GP, Burkard RF, et al. Effects of age on the tuning of the cVEMP and oVEMP[J]. Ear Hear,2013,34(6):e65-73. doi: 10.1097/AUD.0b013e31828fc9f2.
[41] Thabet EM, Abdelkhalek A, Zaghloul H. Superior semicircular canal dehiscence syndrome as assessed by oVEMP and temporal bone computed tomography imaging[J]. Eur Arch Otorhinolaryngol, 2012, 269(5): 1545-1549. doi:10.1007/s00405-011-1893-3.
[42] Inal M, Burulday V, Bayar Muluk N, et al. Magnetic resonance imaging and computed tomography for diagnosing semicircular canal dehiscence[J]. J Cranio - Maxillofac Surg, 2016, 44(8): 998-1002. doi:10.1016/j.jcms.2016.06.006.
[43] Ward BK, Carey JP, Minor LB. Superior canal dehiscence syndrome: lessons from the first 20 years[J]. Front Neurol, 2017, 8: 177. doi:10.3389/fneur.2017.00177.
[44] Carey JP, Migliaccio AA, Minor LB. Semicircular canal function before and after surgery for superior canal dehiscence[J]. Otol Neurotol, 2007, 28(3): 356-364. doi:10.1097/01.mao.0000253284.40995.d8.
[45] Goddard JC, Wilkinson EP. Outcomes following semicircular canal plugging[J]. Otolaryngol Head Neck Surg, 2014, 151(3): 478-483. doi:10.1177/0194599814538233.
[46] Xie YJ, Sharon JD, Pross SE, et al. Surgical complications from superior canal dehiscence syndrome repair: two decades of experience[J]. Otolaryngol Head Neck Surg, 2017, 157(2): 273-280. doi:10.1177/0194599817706491.
[47] Gioacchini FM, Alicandri-Ciufelli M, Kaleci S, et al. Outcomes and complications in superior semicircular canal dehiscence surgery: a systematic review[J]. Laryngoscope, 2016, 126(5): 1218-1224. doi:10.1002/lary.25662.
[48] Ziylan F, Kinaci A, Beynon AJ, et al. a comparison of surgical treatments for superior semicircular canal dehiscence[J]. Otol Neurotol, 2017, 38(1): 1-10. doi:10.1097/mao.0000000000001277.
[49] Nguyen T, Lagman C, Sheppard J, et al. Middle cranial Fossa approach for the repair of superior semicircular canal dehiscence is associated with greater symptom resolution compared with transmastoid approach[J]. J Neurol Surg B, 2018, 79(S 01): S1-S188. doi:10.1055/s-0038-1633737.
[50] Mehta R, Klumpp ML, Spear SA, et al. Subjective and objective findings in patients with true dehiscence versus thin bone over the superior semicircular canal[J]. Otol Neurotol, 2015, 36(2): 289-294. doi:10.1097/mao.0000000000000654.
[51] Teixido M, Seymour PE, Kung B, et al. Transmastoid middle Fossa craniotomy repair of superior semicircular canal dehiscence using a soft tissue graft[J]. Otol Neurotol, 2011, 32(5): 877-881. doi:10.1097/mao.0b013e3182170e39.
[52] Mikulec AA, Poe DS, McKenna MJ. Operative management of superior semicircular canal dehiscence[J]. Laryngoscope, 2005, 115(3): 501-507. doi:10.1097/01.mlg.0000157844.48036.e7.
[53] Ossen ME, Stokroos R, Kingma H, et al. Heterogeneity in reported outcome measures after surgery in superior canal dehiscence syndrome: a systematic literature review[J]. Front Neurol, 2017, 8: 347. doi:10.3389/fneur.2017.00347.
[54] Kwok, P, Gleich O, Spruss T, et al., Different Materials for Plugging a Dehiscent Superior Semicircular Canal: A Comparative Histologic Study Using a Gerbil Model. Otol Neurotol, 2019, 40(5):e532-e541. doi:10.1097/MAO.0000000000002205.
[55] Kim TH, Nam BH, Park CI. Histologic changes of lateral semicircular canal after transection and occlusion with various materials in chinchillas[J]. Korean J Otolaryngol - Head Neck Surg, 2002, 45(4): 318-321.
[56] Nikkar-Esfahani A, Whelan D, Banerjee A. Occlusion of the round window: a novel way to treat hyperacusis symptoms in superior semicircular canal dehiscence syndrome[J]. J Laryngol Otol, 2013, 127(7): 705-707. doi:10.1017/s0022215113001096.
[57] Silverstein H, Kartush JM, Parnes LS, et al. Round window reinforcement for superior semicircular canal dehiscence: a retrospective multi-center case series[J]. Am J Otolaryngol, 2014, 35(3): 286-293. doi:10.1016/j.amjoto.2014.02.016.
[58] Ahmed W, Rajagopal R, Lloyd G. Systematic review of round window operations for the treatment of superior semicircular canal dehiscence[J]. J Int Adv Otol, 2019, 15(2): 209-214. doi:10.5152/iao.2019.6550.
[59] Trieu V, Pelargos PE, Spasic M, et al. Minimally invasive middle Fossa keyhole craniectomy for repair of superior semicircular canal dehiscence[J]. Oper Neurosurg(Hagerstown), 2017, 13(3): 317-323. doi:10.1093/ons/opw046.
[60] Cheng YS, Kozin ED, Lee DJ. Endoscopic-assisted repair of superior canal dehiscence[J]. Otolaryngol Clin North Am, 2016, 49(5): 1189-1204. doi:10.1016/j.otc.2016.05.010.
[61] Crane BT, Carey JP, McMenomey S, et al. Meningioma causing superior canal dehiscence syndrome[J]. Otol Neurotol, 2010, 31(6): 1009-1010. doi:10.1097/mao.0b013e3181a32d85.
[62] Remenschneider AK, Owoc M, Kozin ED, et al. Health utility improves after surgery for superior canal dehiscence syndrome[J]. Otol Neurotol, 2015, 36(10): 1695-1701. doi:10.1097/mao.0000000000000886.
[63] Zhang LS, Creighton FX Jr, Ward BK, et al. a cohort study of hearing outcomes between middle Fossa craniotomy and transmastoid approach for surgical repair of superior semicircular canal dehiscence syndrome[J]. Otol Neurotol, 2018, 39(10): e1160-e1167. doi:10.1097/mao.0000000000002040.

多维度评价

Viewed

Full text

Abstract

Cited

Shared

Discussed