Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Feb;41(2):e172-e181.
doi: 10.1097/MAO.0000000000002487.

A Case Series of Patients With Concurrent Otosclerosis and Superior Semicircular Canal Dehiscence

Nicholas A Dewyer  1 , Alicia M Quesnel  2   3 , Felipe Santos  2   3
Affiliations
Case Reports

A Case Series of Patients With Concurrent Otosclerosis and Superior Semicircular Canal Dehiscence

Nicholas A Dewyer et al. Otol Neurotol. 2020 Feb.

Abstract

Objective: To describe the presentations and treatment results for patients with concurrent otosclerosis and superior semicircular canal dehiscence (SCD).

Study design: Retrospective case series and literature review.

Setting: Tertiary academic medical center.

Patients: Patients with concurrent diagnoses of otosclerosis (radiographically and/or surgically confirmed) and SCD (radiographically confirmed) in the same ear.

Intervention(s): Review of medical records.

Main outcome measure(s): Clinical presentations, outcomes following stapedotomy.

Results: Eight patients with 10 affected ears were identified. All patients presented with slowly progressive conductive hearing loss, normal otoscopy, absent acoustic reflexes, and without other symptoms of SCD syndrome. Seven patients were treated with stapedotomy and 1 with hearing aids. Of those treated with stapedotomy, a persistent conductive hearing loss was the most common hearing result. One patient had near-complete closure of their air bone gap. None had a profound sensorineural hearing loss. Four patients had unmasking of SCD symptoms.

Conclusions: The clinical and audiometric presentations of patients with concurrent otosclerosis and SCD are often indistinguishable from those of patients with only otosclerosis. Computed tomography of the temporal bone is the only way to identify concurrent SCD. Stapedotomy in these patients typically results in a persistent conductive hearing loss, though 14 to 33% of patients experience near-complete closure of their air bone gap. SCD symptoms are unmasked in 57 to 63% of patients who undergo stapedotomy. Further work is needed to delineate the utility of routine preoperative computed tomography scan in otosclerosis patients, and to identify prognostic factors for patients with concurrent otosclerosis and SCD who wish to undergo stapedotomy.

PubMed Disclaimer

References

    1. Halmagyi GM, Aw ST, McGarvie LA, et al. Superior semicircular canal dehiscence simulating otosclerosis. J Laryngol Otol 2003; 117:553–557.
    1. Merchant SN, Rosowski JJ, McKenna MJ. Superior semicircular canal dehiscence mimicking otosclerotic hearing loss. Adv Otorhinolaryngol 2007; 65:137–145.
    1. Mikulec AA, McKenna MJ, Ramsey MJ, et al. Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo. Otol Neurotol 2004; 25:121–129.
    1. Teszler CB, Daval M, Altabaa K, Williams MT, Ayache D. Computed tomography-based workup of conductive hearing loss with normal middle ear: Don’t forget superior semicircular canal dehiscence. Int Tinnitus J 2008; 14:53–56.
    1. Whetstone J, Nguyen A, Nguyen-Huynh A, Hamilton BE. Surgical and clinical confirmation of temporal bone CT findings in patients with otosclerosis with failed stapes surgery. AJNR Am J Neuroradiol 2014; 35:1195–1201.

Publication types

MeSH terms

LinkOut - more resources