Bilateral superior semicircular canal dehiscence in a child with sensorineural hearing loss and without vestibular symptoms

2011 ◽  
Vol 75 (6) ◽  
pp. 877-879 ◽  
Author(s):  
Alyssa A. Kanaan ◽  
Roy A. Raad ◽  
Roula G. Hourani ◽  
Georges M. Zaytoun
Author(s):  
F Alkherayf ◽  
C Agbi ◽  
D Schramm

Background: Superior semicircular canal dehiscence (SSCD) is a recently described rare condition. SSCD symptoms include vertigo, oscillopsia, autophony, sound hypersensitivity , and conductive hearing loss. Patients with sever symptoms may require surgical treatment. Tranmastoid and middle fossa (MCF) approaches are common approaches. Methods: We are presenting our experience at the Ottawa Hospital over the last three years. Also we describe our multidisciplinary surgical approach and modalities to localize the SSCD intraoperatively. Demographic data, presenting symptoms, co-morbidities, radiologic imaging, and surgery length were recorded. All patients had hearing and vestibular tests before and after their surgeries. Results: 14 surgeries were performed in 11 patients (three patients had bilateral SSCD). Most patients were males (82%). Age range was 32-68 years. Surgeries were done by a team of a neurosurgeon and a neuro-otologist. Localization of SSCD was done using stereotactic guidance. Five layers’ reconstruction was performed in all patients. All patients had significant improvement in symptoms without sensorineural hearing loss. None of the patients developed post-operative hematoma, infection, seizures, CSF leakage or facial palsy. LOS was 1-2 days. Conclusions: MCF with multi layers reconstruction should be considered as a safe and effective approach in severely symptomatic patients. We demonstrated that this approach has minimal risks especially in regards to sensorineural hearing loss.


2020 ◽  
Vol 13 (3) ◽  
pp. e233042
Author(s):  
Diogo Pereira ◽  
Abílio Leonardo ◽  
Delfim Duarte ◽  
Nuno Oliveira

Superior semicircular canal dehiscence is caused by a bone defect on the roof of the superior semicircular canal. The estimated prevalence when unilateral varies between 0.4% and 0.7% and is still unknown when bilateral. Patients may present with audiologic and vestibular symptoms that may vary from asymptomatic to disabling. We report a case of a 72-year-old Caucasian woman presented to otolaryngology department reporting imbalance, bilateral pulsatile tinnitus, hypoacusis while being very sensitive to certain sounds. Physical examination was unremarkable, except for the Rinne test that was negative in both sides. The patient underwent an audiometry revealing a mild bilateral conductive hearing loss. A temporal bone CT scan was performed which evidenced bilateral superior semicircular canal dehiscence. Cervical vestibular evoked myogenic potentials and electrocochleography confirmed diagnosis. Although rare, superior semicircular canal dehiscence shall be considered in conductive hearing loss with vestibular symptoms.


2004 ◽  
Vol 25 (2) ◽  
pp. 121-129 ◽  
Author(s):  
Anthony A. Mikulec ◽  
Michael J. McKenna ◽  
Mitchell J. Ramsey ◽  
John J. Rosowski ◽  
Barbara S. Herrmann ◽  
...  

2004 ◽  
Vol 100 (1) ◽  
pp. 123-124 ◽  
Author(s):  
Mitchell J. Ramsey ◽  
Michael J. McKenna ◽  
Fred G. Barker

✓ The authors present the case of a man who had superior semicircular canal dehiscence syndrome in addition to chronic otitis media. This case is atypical because the patient coincidentally had middle ear and mastoid disease, which previously had been treated surgically. The prior ear surgery delayed the diagnosis of superior semicircular canal dehiscence syndrome and increased the complexity of the repair of the superior semicircular canal dehiscence. Superior semicircular canal dehiscence syndrome is a recently recognized syndrome resulting in acute or chronic vestibular symptoms. The diagnosis is made using history, vestibular examination, and computerized tomography studies. Neurosurgeons should be aware that patients with superior semicircular canal dehiscence syndrome who experience disabling chronic or acute vestibular symptoms can be treated using a joint neurosurgical—otological procedure through the middle cranial fossa.


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