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Superior semicircular canal dehiscence

A Ernst, I Todt, J Wagner
BACKGROUND: Dehiscence syndromes of the semicircular canals are a relatively new group of neurotological disorders. They have a variety of symptoms with hearing/balance involvement. Younger patients have clinically relevant symptoms in only about one third of cases. In addition to etiology and pathogenesis, the present paper describes diagnostic and therapeutic possibilities using a patient series of the authors. MATERIALS AND METHODS: This nonrandomized prospective study included 52 patients with uni-/bilateral dehiscence syndromes of the superior and/or posterior canal (SCDS/PCDS), diagnosed with high-resolution computed tomography (HR-CT) of the petrous bone...
October 14, 2016: HNO
Neil S Patel, Jacob B Hunter, Brendan P O'Connell, Natalie M Bertrand, George B Wanna, Matthew L Carlson
OBJECTIVE: Patients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time. STUDY DESIGN: Retrospective review at two tertiary care academic referral centers. METHODS: Adults analyzed were diagnosed with SSCD by high-resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms...
October 14, 2016: Laryngoscope
P Bertholon, A Karkas
This chapter will focus on vertigo/dizziness due to inner-ear malformations, labyrinthine fistula, otosclerosis, infectious processes, and autoimmune inner-ear disorders. Inner-ear malformation due to dehiscence of the superior semicircular canal is the most recently described inner-ear malformation. Vertigo/dizziness is typically induced by sound and pressure stimuli and can be associated with auditory symptoms (conductive or mixed hearing loss). Labyrinthine fistula, except after surgery for otosclerosis, in the context of trauma or chronic otitis media with cholesteatoma, still remains a challenging disorder due to multiple uncertainties regarding diagnostic and management strategies...
2016: Handbook of Clinical Neurology
Eric M Sugihara, Seilesh C Babu, Dennis J Kitsko, Michael S Haupert, Prasad J Thottam
OBJECTIVE: To determine the pediatric incidence and association of superior semicircular canal dehiscence (SSCD) with inner ear (IE) anomalies. STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary referral centers. PATIENTS: Children less than 18 years who received a 0.5 mm or less collimated computed tomography study including the temporal bones between 2010 and 2013 for reasons including, but not limited to, hearing loss, trauma, and infection...
October 2016: Otology & Neurotology
Kathryn Y Noonan, Jack Russo, Jun Shen, Heidi Rehm, Sara Halbach, Einar Hopp, Sarah Noon, Jacqueline Hoover, Clifford Eskey, James E Saunders
OBJECTIVE: To investigate the prevalence and relative risk of semicircular canal dehiscence (SCD) in pediatric patients with CDH23 pathogenic variants (Usher syndrome or non-syndromic deafness) compared with age-matched controls. STUDY DESIGN: Retrospective cohort study. SETTING: Multi-institutional study. PATIENTS: Pediatric patients (ages 0-5 years) were compared based on the presence of biallelic pathogenic variants in CDH23 with pediatric controls who underwent computed tomography (CT) temporal bone scan for alternative purposes...
September 14, 2016: Otology & Neurotology
Georgios Mantokoudis, Ali S Saber Tehrani, Aaron L Wong, Yuri Agrawal, Angela Wenzel, John P Carey
OBJECTIVE: To describe vestibulo-ocular function and compensatory mechanisms in the immediate postoperative period after superior canal dehiscence surgery. STUDY DESIGN: Prospective longitudinal study. SETTING: Tertiary medical center. PATIENTS: Five patients who underwent plugging of superior semicircular canal via middle cranial fossa approach. INTERVENTIONS: Bedside quantitative video head impulse testing (vHIT)...
October 2016: Otology & Neurotology
James G Naples, Marc D Eisen
The history of surgery on the vestibular labyrinth is rich but sparsely documented in the literature. The story begins over a century ago with the labyrinthectomy in an era that consisted exclusively of ablative surgery for infection or vertigo. Improved understanding of vestibular physiology and pathology produced an era of selective ablation and hearing preservation that includes semicircular canal occlusion for benign paroxysmal positional vertigo. An era of restoration began with a discovery of superior semicircular canal dehiscence and its repair...
August 23, 2016: Otolaryngology—Head and Neck Surgery
J S Sandhu, J Manickavasagam, D Connolly, A Raghavan, M Fernando, J Ray
High-resolution CT data confirms the presence of superior semicircular canal dehiscence in 3% of specimens. Analysis of inter-reporter measurements of dehiscence shows excellent correlation. Radiological data under-estimates the bone thickness overlying the superior semicircular canal (SSC) and therefore over-estimates the incidence of dehiscence compared to histological data. Radiological findings must be correlated with audiological data in order to diagnose Minor's Syndrome accurately. This article is protected by copyright...
July 6, 2016: Clinical Otolaryngology
Mikail Inal, Veysel Burulday, Nuray Bayar Muluk, Ahmet Kaya, Gökçe Şimşek, Birsen Ünal Daphan
OBJECTIVES: We investigated the semicircular canal (SC) dehiscence using temporal computed tomography (CT) and magnetic resonance (MR) imaging. METHODS: We retrospectively reviewed 114 (228 ears) consecutive MR images and CT scans of the temporal bones for dehiscence of the SCs. In the 1.5 Tesla (T) MR imaging, T1 and T2-weighted images were obtained. Dehiscence of the SCs was defined by absence of high attenuation bone coverage on the CT scans, and absence of low-signal bone margins on the MR images...
August 2016: Journal of Cranio-maxillo-facial Surgery
Neil S Patel, Jacob B Hunter, Brendan P O'Connell, George B Wanna, Matthew L Carlson
OBJECTIVE: The middle fossa floor lacks reliable surface landmarks. In cases of superior semicircular canal dehiscence (SSCD), multiple skull base defects may be present, further confounding the location of the labyrinth. Misidentification of the SSCD during surgery may lead to treatment failure or sensorineural hearing loss. Anecdotally, the authors have observed the distance from the lateral edge of the craniotomy to the SSCD to be consistently 1 inch. Herein, we present radiologic evidence of this practical and clinically useful relationship...
September 2016: Otology & Neurotology
Harry R F Powell, Sherif S Khalil, Shakeel R Saeed
OBJECTIVE: To present the management strategy and outcomes for our series of superior semicircular canal dehiscence syndrome (SSCDS) patients. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Tertiary referral center. PATIENTS: Thirty-seven consecutive patients referred from June 2011 to January 2015. Diagnosis of SSCDS based on presence of classical symptoms, computerized tomography, and concordant reduction in cervical vestibular evoked myogenic potentials...
August 2016: Otology & Neurotology
Habib G Rizk, Jonathan L Hatch, Shawn M Stevens, Paul R Lambert, Ted A Meyer
OBJECTIVES: (1) To quantitatively assess the lateral skull base thickness in patients with superior semicircular canal dehiscense (SSCD) using a standardized and validated radiographic measure and to compare it with that of a population with spontaneous cerebrospinal fluid otorrhea (CSFO). (2) To analyze demographic and clinical factors associated with skull base thickness in the SSCD group. STUDY DESIGN: Case series with chart review. SETTING: Tertiary neurotologic referral center...
October 2016: Otolaryngology—Head and Neck Surgery
Naoharu Kitajima, Akemi Sugita-Kitajima, Seiji Kitajima
OBJECTIVE: To determine the relationship between Eustachian tube function and inner ear function in patulous Eustachian tube (pET). METHODS: We encountered a patient with pET accompanied by dizziness that was induced by nasal respiration. Eye movements were recorded using video-oculography, and Eustachian tube function was assessed using a Eustachian tube function analyzer. Horizontal and vertical components of pupil position were assessed to test fixation, positional, and positioning nystagmus...
December 2016: Auris, Nasus, Larynx
Inge Wegner, Mostafa M A S Eldaebes, Thomas G Landry, Robert B Adamson, Wilko Grolman, Manohar L Bance
HYPOTHESIS: Round window reinforcement leads to conductive hearing loss. BACKGROUND: The round window is stiffened surgically as therapy for various conditions, including perilymphatic fistula and superior semicircular canal dehiscence. Round window reinforcement reduces symptoms in these patients. However, it also reduces fluid displacement in the cochlea and might therefore increase conductive hearing loss. METHODS: Perichondrium was applied to the round window membrane in nine fresh-frozen, nonpathologic temporal bones...
June 2016: Otology & Neurotology
Bryan J Liming, Benjamin Westbrook, Hans Bakken, James V Crawford
OBJECTIVE: Demonstrate that an endoscopic keyhole approach to the middle cranial fossa is technically feasible for repair of semicircular canal dehiscence. STUDY DESIGN: Cadaveric technical feasibility/methods development study. SETTING: Tertiary care military medical center. PATIENTS: Three fresh cadaveric heads (six sides). INTERVENTION(S): Endoscopic minimally invasive approach to the middle cranial fossa using the Medtronic Fusion Guidance system...
June 2016: Otology & Neurotology
X A Zhang, Y Li, C F Dai
OBJECTIVE: The objective of this study is to investigate the clinical manifestations of the superior semicircular canal dehiscence syndrome(SSCDS) and the treatment strategies. METHODS: Data from 20 cases diagnosed with SSCDS from September 2004 to December 2014 were retrospectively analyzed in this study. The clinical presentations including symptoms, signs, audiological and vestibular function examination, and their imaging characteristics and treatment strategies were reviewed...
February 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
Lawrance K Chung, Nolan Ung, Marko Spasic, Daniel T Nagasawa, Panayiotis E Pelargos, Kimberly Thill, Brittany Voth, Daniel Hirt, Quinton Gopen, Isaac Yang
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...
February 12, 2016: Journal of Neurosurgery
Richard Pullicino, Reuben Grech
No abstract text is available yet for this article.
2015: BMJ Case Reports
Yew Song Cheng, Elliott D Kozin, Aaron K Remenschneider, Hideko Heidi Nakajima, Daniel J Lee
HYPOTHESIS: Superior canal dehiscence (SCD) repair using surgical bone wax may result in variable outcomes if large wax volumes are applied. BACKGROUND: SCD is a disorder characterized by a pathologic defect in the bony labyrinth of the superior semicircular canal (SSC), resulting in vestibular and/or auditory symptoms. Repair of SCD using bone wax can provide symptomatic relief, but surgical outcomes are variable. These observations may be associated with the inconsistency in the position and extension of intralabyrinthine bone wax during surgical repair...
January 2016: Otology & Neurotology
P Ashley Wackym, Carey D Balaban, Heather T Mackay, Scott J Wood, Christopher J Lundell, Dale M Carter, David A Siker
OBJECTIVE: Patients with peripheral vestibular dysfunction because of gravitational receptor asymmetries display signs of cognitive dysfunction and are assumed to have neurobehavioral sequelae. This was tested with pre- and postoperatively quantitative measurements in three cohort groups with superior semicircular canal dehiscence syndrome (SSCDS) symptoms with: 1) superior canal dehiscence (SCD) repaired via a middle cranial fossa craniotomy and canal plugging only; 2) otic capsule defects not visualized with imaging (no-iOCD) repaired with round window reinforcement (RWR) only; or 3) both SCD plugging and subsequent development of no-iOCD followed by RWR...
January 2016: Otology & Neurotology
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