Superior Canal Dehiscence Syndrome Associated With the Superior Petrosal Sinus in Pediatric and Adult Patients

ArticleinOtology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 32(8):1312-9 · October 2011with104 Reads
DOI: 10.1097/MAO.0b013e31822e5b0a · Source: PubMed
To determine whether pediatric and adult patients with superior canal dehiscence (SCD) at the superior petrosal sinus (SPS) develop superior canal dehiscence syndrome (SCDS). Retrospective review. Tertiary care academic medical center. Pediatric and adult patients with SPS-associated SCD were identified from a database of 131 patients with SCD based on high-resolution temporal bone computed tomography. One pediatric patient experienced incapacitating exercise-induced vertigo, and this patient's superior semicircular canal defect was plugged via a transmastoid approach. The 11 remaining patients were managed by observation. Clinical symptoms and signs, audiologic testing, vestibular evoked myogenic potentials, and radiologic data. Twelve patients, aged 15 to 84 years, with SCD caused by the SPS contacting the superior semicircular canal were identified. The most characteristic clinical feature in this population (5/12) was dizziness related to exercise and exertion. Bilateral SCD was observed in 3 patients. Eleven patients did not have severe symptoms and were managed conservatively. One patient, aged 15, required surgical intervention for incapacitating vertigo and experienced relief of symptoms with reversal of diagnostic indicators postoperatively. This is the first reported surgical repair of SCDS in a pediatric patient. This is the first series of patients who have SCDS due to contact of the SPS with the superior semicircular canal. Exercise and exertion-related symptoms are common in patients who have SCD owing to this cause. Transmastoid superior canal plugging is feasible and successful in treating SCDS in the pediatric patient.
  • [Show abstract] [Hide abstract] ABSTRACT: Otitic meningitis in the postantibiotic era is still a serious condition, requiring intensive treatment and prolonged rehabilitation. In view of the significant morbidity and mortality rate, conditions that may increase the likelihood of otitic meningitis developing should be treated promptly. The incidence of meningitis after asymptomatic encephaloceles of the middle cranial fossa varies greatly, and the management differs between elective surgical repair and expectant careful observation. Superior semicircular canal dehiscences (SSCDs) are postulated to have a congenital origin and are associated with a thin or dehiscent tegmen. Several cases of simultaneous SCCD and tegmen defects have been reported, but the findings of otitic meningitis, SCCD, and encephaloceles has, to the best of our knowledge, not been previously explored in the literature. We reviewed a series of 4 patients who all presented with a combination of otitic meningitis, encephaloceles, and SSCD. All the 4 patients we reviewed had meningitis secondary to otitis media with computed tomographic scans confirming the presence of SCCD with ipsilateral tegmen tympani defects and associated cephaloceles. All patients were treated with intravenous antibiotics and underwent surgery that ranged from myringotomy and ventilation tube insertions, mastoidectomy, and burr hole drainage for temporal lobe abscess. They were all associated with intensive care unit admission, significant morbidity, and prolonged hospital stays. There were no mortalities. We propose that in all SSCD patients, a careful computed tomographic examination of the cranial base should be undertaken to exclude other associated tegmen tympani defects. In cases of SSCD requiring surgery, we support the view that elective surgical repair be recommended where asymptomatic ipsilateral encephaloceles are found, to reduce the risk of otitic meningitis.
    Article · Apr 2012
  • [Show abstract] [Hide abstract] ABSTRACT: To identify clinical factors associated with prolonged recovery after superior canal dehiscence surgery. Retrospective review. Tertiary care academic medical center. Thirty-three patients that underwent surgery for SCDS were identified from a database of 140 patients diagnosed with SCD (2000-2010) at the Massachusetts Eye and Ear Infirmary (U.S.A.). The diagnosis of SCDS was based on clinical signs and symptoms, audiometric and vestibular testing and high-resolution temporal bone computed tomography. For the primary repair, the superior canal was plugged in 31 patients through a middle fossa craniotomy approach and in 1 patient through a transmastoid approach. In 1 patient, the SCD was resurfaced through a middle fossa craniotomy approach. Postoperative clinical signs and symptoms and factors that may influence duration of disequilibrium after surgery. Thirty-three patients (15-71 yr; mean, 43 yr) underwent surgery for SCDS on 35 ears (2 bilateral). Mean follow-up was 28.7 months (range, 3 mo to 10 yr); 33 of 33 (100%) patients experienced initial improvement of the chief complaint. Three patients required revision surgery, improving symptoms in 2 patients. Six patients had dizziness lasting more than 4 months postoperatively, and all had bilateral SCD, migraines, and a dehiscence of 3 mm or greater. Surgical plugging of SCD is an effective management option to provide long-term improvement of the chief complaint in SCDS patients. Patients with bilateral SCD, a history of migraines, and larger defects may be at risk of prolonged recovery and should be appropriately counseled.
    Full-text · Article · Jun 2012
  • [Show abstract] [Hide abstract] ABSTRACT: Background Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin. Objective We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults. Materials and methods Thirty-four temporal bone HRCTs of children younger than 2 years and 40 temporal bone HRCTs of patients older than 18 years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information. Results SCDs were detected in 27.3% of children younger than 2 years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P < 0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2 years and 2.5% of adults (P < 0.0001). Conclusion SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully.
    Article · Sep 2012
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