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.