By Marcello Cherchi, MD PhD

For patients

Superior semicircular canal dehiscence (SSCD) can sometimes be fixed by surgery, and there are different surgeries for this. The surgeries do not always work. There is some risk of hearing loss, and some risk that the surgery will not work. Some patients talk with two or more surgeons before deciding whether to undergo a surgery.

For clinicians

Overview

There are several possible surgical approaches to treating superior semicircular canal dehiscence (SSCD). There is little consensus on selection criteria for these procedures, and in some instances, more than one approach may be feasible. There is debate in the literature regarding which techniques pose the least risk to hearing. Sometimes a repair procedure will appear successful initially, but will not be durable. Some patients benefit from consulting with more than one surgeon when considering surgical intervention for SSCD.

Introduction

Superior semicircular canal dehiscence (SSCD) is a “third window phenomenon” in which there is an abnormal connection between the superior semicircular canal and the intracranial space due to dehiscence of the bony roof that usually separates these two spaces.

There are several approaches to surgical repair of SSCD, the main ones of which are:

  • Canal re-roofing, which requires neurosurgical assist via craniotomy to access the floor of the middle cranial fossa (Mozaffari et al. 2021b; Salvinelli et al. 2022; Tugrul et al. 2022).
  • Canal occlusion (colloquially referred to as “plugging”), usually by a transmastoid approach (de Wolf et al. 2021; Gersdorff et al. 2022; Hassannia, Douglas-Jones, Rutka 2019; Hussain, Irving, Baruah 2023; Kawamura et al. 2022; Kontorinis and Gaggini 2021; Lin et al. 2021; Shaul et al. 2023)
  • Round window reinforcement (Ahmed, Rajagopal, Lloyd 2019; Bunne, Andersson, Myhrum 2022; Chemtob et al. 2019; Conway et al. 2021) or oval window reinforcement (Conway et al. 2021).

There is little agreement on the selection criteria for which surgical approach to take in a particular case. In some instances, more than one approach may be feasible.

Adverse effects

One of the main concerns for surgery on the labyrinth is the effect on hearing. Surgeries aimed at treating SSCD attempt to preserve hearing, and there are debates in the literature about which approach poses the least risk to hearing (Conway et al. 2021; Ellsperman et al. 2021; Elms, Canick, Kaylie 2023; Kawamura et al. 2022; Lin et al. 2021; Wolfovitz et al. 2019; Yang, Yang, Gopen 2023).

Another potential “adverse effect” is that the surgery, even if initially successful, will not be durable. Re-roofed canals can re-dehisce; plugged canals can become un-plugged; reinforced round and oval windows can lose reinforcement. There is a modest literature regarding surgical revision of prior attempts at treating SSCD (Mozaffari et al. 2021a).

Other notes

Some patients benefit from consulting with more than one otologic surgeon when considering an invasive procedure for the treatment of SSCD.

References

Ahmed W, Rajagopal R, Lloyd G (2019) Systematic Review of Round Window Operations for the Treatment of Superior Semicircular Canal Dehiscence. J Int Adv Otol 15: 209-214. doi: 10.5152/iao.2019.6550

Bunne M, Andersson H, Myhrum M (2022) Long-Term Outcomes of Round Window Reinforcement for Superior Semicircular Canal Dehiscence Syndrome. Otol Neurotol 43: 709-716. doi: 10.1097/MAO.0000000000003561

Chemtob RA, Noij KS, Qureshi AA, Klokker M, Nakajima HH, Lee DJ (2019) Superior Canal Dehiscence Surgery Outcomes Following Failed Round Window Surgery. Otol Neurotol 40: 535-542. doi: 10.1097/MAO.0000000000002185

Conway RM, Tu NC, Pinther S, Shareef Z, Hong RS, Zappia JJ, Babu SC (2021) Audiologic Outcomes After Oval and Round Window Reinforcement Surgery. Otol Neurotol 42: 1051-1057. doi: 10.1097/MAO.0000000000003115

de Wolf MJF, Dawe N, Jervis S, Kumar R, Dalton CL, Lindley K, Irving R (2021) Transmastoid Occlusion Surgery for Superior Semicircular Canal Dehiscence Syndrome Improves Patient-Reported Quality-of-Life Measures and corrects cVEMP Thresholds and Amplitudes. Otol Neurotol 42: 1534-1543. doi: 10.1097/MAO.0000000000003329

Ellsperman SE, Telian SA, Kileny PR, Welch CM (2021) Auditory Outcomes Following Transmastoid and Middle Cranial Fossa Approaches for Superior Semicircular Canal Dehiscence Repair. Otol Neurotol 42: 1544-1552. doi: 10.1097/MAO.0000000000003323

Elms HL, Canick JE, Kaylie DM (2023) What Approach Minimizes Hearing Loss in Superior Semicircular Canal Dehiscence Repair? Laryngoscope 133: 1784-1785. doi: 10.1002/lary.30705

Gersdorff G, Blaivie C, de Foer B, Deggouj N, Wyckmans F, Somers T (2022) Evaluation of the transmastoid plugging approach for superior semicircular canal dehiscences: a retrospective series of 30 ears. Eur Arch Otorhinolaryngol 279: 4861-4869. doi: 10.1007/s00405-022-07316-8

Hassannia F, Douglas-Jones P, Rutka JA (2019) Gauging the effectiveness of canal occlusion surgery: how I do it. J Laryngol Otol 133: 1012-1016. doi: 10.1017/S0022215119002032

Hussain MH, Irving R, Baruah P (2023) A report on quality-of-life outcomes following transmastoid plugging of superior semicircular canal dehiscence in a newly established service in a UK hospital. J Laryngol Otol 137: 51-56. doi: 10.1017/S0022215121004643

Kawamura Y, Yamauchi D, Kobayashi T, Ikeda R, Kawase T, Katori Y (2022) Hearing Outcomes of Transmastoid Plugging for Superior Canal Dehiscence Syndrome by Underwater Endoscopic Surgery: With Special Reference to Transient Bone Conduction Increase in Early Postoperative Period. Otol Neurotol 43: 368-375. doi: 10.1097/MAO.0000000000003461

Kontorinis G, Gaggini M (2021) Transmastoid Superior Semicircular Canal Plugging: A Prospective Analysis of Surgical Outcomes. Otol Neurotol 42: 1216-1222. doi: 10.1097/MAO.0000000000003191

Lin KF, Bojrab DI, 2nd, Fritz CG, Vandieren A, Babu SC (2021) Hearing Outcomes After Surgical Manipulation of the Membranous Labyrinth During Superior Semicircular Canal Dehiscence Plugging or Posterior Semicircular Canal Occlusion. Otol Neurotol 42: 806-814. doi: 10.1097/MAO.0000000000003100

Mozaffari K, Ghodrati F, Pradhan A, Ng E, Ding K, Rana S, Duong C, Anderson RN, Enomoto A, Sheppard JP, Sun MZ, Phillips HW, Yang I, Gopen Q (2021a) Superior Semicircular Canal Dehiscence Revision Surgery Outcomes: A Single Institution’s Experience. World Neurosurg 156: e408-e414. doi: 10.1016/j.wneu.2021.09.083

Mozaffari K, Willis SL, Unterberger A, Duong C, Hong M, De Jong R, Mekonnen M, Johanis M, Miao T, Yang I, Gopen Q (2021b) Superior Semicircular Canal Dehiscence Outcomes in a Consecutive Series of 229 Surgical Repairs With Middle Cranial Fossa Craniotomy. World Neurosurg 156: e229-e234. doi: 10.1016/j.wneu.2021.09.038

Salvinelli F, Bonifacio F, Beccaria C, Greco F, Frari V, Iafrati F, Trivelli M (2022) Minimal invasive resurfacing: an innovative technique for the superior semicircular canal dehiscence. A case series. J Surg Case Rep 2022: rjac241. doi: 10.1093/jscr/rjac241

Shaul C, Weder S, Dragovic A, Gerard JM, Briggs RJS (2023) Trans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up. Eur Arch Otorhinolaryngol. doi: 10.1007/s00405-023-08079-6

Tugrul S, Yenigun A, Kulaksiz Y, Dogan R, Aksoy F, Ozturan O (2022) Superior semicircular canal dehiscence repair with small middle fossa craniotomy using an oto-microscopic and co-endoscopic assisted approach. J Laryngol Otol 136: 559-561. doi: 10.1017/S0022215121004308

Wolfovitz A, Grobman AB, Babcock TA, Angeli SI (2019) The pattern of hearing outcome following surgery of the semicircular canals. Laryngoscope Investig Otolaryngol 4: 132-137. doi: 10.1002/lio2.239

Yang HH, Yang I, Gopen QS (2023) Audiometric Outcomes After the Middle Cranial Fossa Repair of Superior Semicircular Canal Dehiscence. Otol Neurotol 44: 593-599. doi: 10.1097/MAO.0000000000003905

Page first published on July 30, 2023. Page last updated on September 22, 2023

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