title: Clinical outcomes of middle fossa craniotomy for superior semicircular canal dehiscence repair.
abstract: OBJECTIVE Superior semicircular canal dehiscence (SSCD) is a rare disorder characterized by the formation of a third opening in the inner ear between the superior semicircular canal and the middle cranial fossa. Aberrant communication through this opening causes a syndrome of hearing loss, pulsatile tinnitus, disequilibrium, and autophony. This study analyzed the clinical outcomes of a single-institution series of patients with SSCD undergoing surgical repair by the same otolaryngologist and neurosurgeon. METHODS All patients who underwent SSCD repair at the University of California, Los Angeles, between March 2011 and November 2014 were included. All patients had their SSCD repaired via middle fossa craniotomy by the same otolaryngologist and neurosurgeon. Outcomes were analyzed with Fisher's exact test. RESULTS A total of 18 patients with a mean age of 56.2 years (range 27-84 years) and an average follow-up of 5.0 months (range 0.2-21.8 months) underwent 21 cases of SSCD repair. Following treatment, all patients (100%) reported resolution in ≥ 1 symptom associated with SSCD. Autophony (p = 0.0005), tinnitus (p = 0.0059), and sound- and/or pressure-induced dizziness (p = 0.0437) showed significant symptomatic resolution. Following treatment, 29% (2/7) of patients developed imbalance, 20% (1/5) of patients developed sound- and/or pressure-induced dizziness, and 18% (2/11) of patients developed aural fullness. Among patients with improved symptoms following surgical repair, none reported recurrence of symptoms at subsequent follow-up visits. CONCLUSIONS SSCD remains an underdiagnosed and undertreated condition. Surgical repair of SSCD using a middle fossa craniotomy is associated with a high rate of symptom resolution. Continued investigation using a larger patient cohort and longer-term follow-up could further demonstrate the effectiveness of using middle fossa craniotomy for SSCD repair.
authors.affiliation: Department of Neurosurgery.
authors.forename: Lawrance K
authors.initials: LK
authors.lastname: Chung
authors.affiliation: Department of Neurosurgery.
authors.forename: Nolan
authors.initials: N
authors.lastname: Ung
authors.affiliation: Department of Neurosurgery.
authors.forename: Marko
authors.initials: M
authors.lastname: Spasic
authors.affiliation: Department of Neurosurgery.
authors.forename: Daniel T
authors.initials: DT
authors.lastname: Nagasawa
authors.affiliation: Department of Neurosurgery.
authors.forename: Panayiotis E
authors.initials: PE
authors.lastname: Pelargos
authors.affiliation: Department of Neurosurgery.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Thill
authors.affiliation: Department of Neurosurgery.
authors.forename: Brittany
authors.initials: B
authors.lastname: Voth
authors.affiliation: Department of Neurosurgery.
authors.forename: Daniel
authors.initials: D
authors.lastname: Hirt
authors.affiliation: Department of Head and Neck Surgery, and.
authors.forename: Quinton
authors.initials: Q
authors.lastname: Gopen
authors.affiliation: Department of Neurosurgery.|Jonsson Comprehensive Cancer Center, University of California, Los Angeles, California.
authors.forename: Isaac
authors.initials: I
authors.lastname: Yang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003399:Craniotomy / Q000379:methods*; D004427:Ear Diseases / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012665:Semicircular Canals / Q000601:surgery*; D016896:Treatment Outcome
keywords: SSCD = superior semicircular canal dehiscence; autophony; skull base; superior semicircular canal dehiscence; tinnitus; vertigo