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Superior Semicircular Canal Dehiscence

SCCD


Presentation

  • Battista is an excellent presenter...clear, concise. Highly recommend. by Danielle on June 6, 2017 Dr. Battista is an excellent presenter.[audiologyonline.com]
  • We hereby present a rare cause of disequilibrium and SSCDS, diagnosed by clinical presentations, audiological tests and imaging. [ FULL TEXT ] [ PDF ]*[amhsjournal.org]
  • Case presentation A 45-year-old Caucasian patient presented with a 5-year isolated history of noise induced vertigo and a history of head trauma 12 years ago.[jmedicalcasereports.biomedcentral.com]
  • Vestibular manifestations were present in 60 and exclusively auditory manifestations without vestibular symptoms or signs were noted in 5 patients.[ncbi.nlm.nih.gov]
  • Like perilymphatic fistula, SSCD presents with vertigo in association with strain and the Valsalva maneuver. There is frequent hearing loss and oscillopsia.[oxfordmedicine.com]
Down Syndrome
  • A single patient with Down syndrome and SSCD was identified.[thejns.org]
  • Lange, Vestibular Pathology Presenting as Behavioral Problems in a Child With Down Syndrome: A Case Report, Journal of Developmental & Behavioral Pediatrics, 10.1097/DBP.0b013e3181976a6c, 30, 1, (66-68), (2009).[doi.org]
Rigor
  • Based on our comprehensive review of the literature and our experience in this age group, we recommend a multidisciplinary approach, which includes obtaining a detailed neurodevelopmental and otologic history and a rigorous assessment of auditory and[thejns.org]
Cerebral Palsy
  • Pediatr Radiol 42 : 1456 – 1464 2012 Search Google Scholar Export Citation 12 Henderson RC Lark RK Gurka MJ Worley G Fung EB Conaway M : Bone density and metabolism in children and adolescents with moderate to severe cerebral palsy .[thejns.org]
Nausea
  • The headaches were becoming severe, the nausea persistent, my vision was often blurred or my eyes seemed to jump around, and it was extremely uncomfortable to raise my voice, sing, or even eat crunchy foods.[hearinghealthmatters.org]
  • "The headaches were becoming severe, the nausea persistent, my vision was often blurred, and it was extremely uncomfortable to raise my voice, sing or eat crunchy foods.[eurordis.org]
  • The headaches were becoming severe, the nausea persistent, my vision was often blurred, and it was extremely uncomfortable to raise my voice, sing or even eat crunchy foods. All the sounds reverberated inside my head.[actiononhearingloss.org.uk]
  • […] sounds more loudly, like creaking joints, pulse, and breathing) Low-frequency conductive hearing loss Increased sensitivity to bone-conducted sounds Feeling of fullness in the ear Other symptoms of superior canal dehiscence Headache and/or migraines Nausea[share.upmc.com]
  • . - The Tullio Phenomenon: This occurrence may be defined as a sound-induced loss of hearing triggered by normal sounds; this feeling presents with the loss of equilibrium, motion sickness, or even actually nausea.[abc.go.com]
Vertigo
  • Case Report A 51-year old female patient presented with intermittent vertigo and left-side aural fullness for one month after traffic accident, especially left sided head trauma. Loud sound evoked sensation of vertigo and disequilibrium.[ejao.org]
  • Historical note and terminology The Tullio phenomenon is sound-induced vertigo, nystagmus, or both.[medlink.com]
  • Case presentation A 45-year-old Caucasian patient presented with a 5-year isolated history of noise induced vertigo and a history of head trauma 12 years ago.[jmedicalcasereports.biomedcentral.com]
  • The result was postoperative reduction in vertigo and closure of the air-bone gap on the audiogram (Table 1). The stapedius reflexes were present, with no vertigo. The postoperative CT verified reconstruction of the bone ( Figure 3 b). Figure 3.[em-consulte.com]
  • Superior semicircular canal dehiscence (SSCD) is a recently identified entity associated with vertigo. Like perilymphatic fistula, SSCD presents with vertigo in association with strain and the Valsalva maneuver.[oxfordmedicine.com]
Episodic Vertigo
  • Results and Conclusions Symptoms of superior semicircular canal dehiscence (SSCD) include autophony, inner ear conductive hearing loss, Hennebert sign, and sound-induced episodic vertigo and disequilibrium (Tullio phenomenon), among others.[doi.org]
  • Thomas Lempert and Michael von Brevern, Episodic vertigo, Current Opinion in Neurology, 10.1097/00019052-200502000-00003, 18, 1, (5-9), (2005). M.[doi.org]
Pendular Nystagmus
  • . • Patients with bilateral superior semicircular canal dehiscence may have vertical oscillopsia and impaired vision during locomotion, disequilibrium, spontaneous pulse-synchronous vertical pendular nystagmus, and Valsalva-induced upbeat jerk nystagmus[medlink.com]
  • "Pulse-synchronous torsional pendular nystagmus in unilateral superior canal dehiscence." Neurology 70 (14): 1217-8. Halmagyi GM and others. The click-evoked vestibulo-ocular reflex in superior semicircular canal dehiscence.[dizziness-and-balance.com]
Language Delays
  • However, he did have speech and language delay related to his hearing loss.[thejns.org]

Workup

  • Preoperative Workup The diagnosis of SCD is made radiographically, therefore all patients should undergo fine cut temporal bone computed tomography (CT) imaging.[csurgeries.com]
  • Treatment for SSCD can only be determined after a comprehensive medical examination of an otologist and a full audiological workup. A specialized CT scan of the ear is needed to accurately diagnose the condition.[californiaearinstitute.com]
  • Computed tomography-based workup of conductive hearing loss with normal middle ear: don’t forget superior semicircular canal dehiscence Int Tinnitus J 2008 ; 14 : 53-56 [6] Branstetter B.F., Harrigal C., Escott E.J., Hirsch B.E.[em-consulte.com]
  • It is very important during the workup of SCD to look for other diseases that can cause similar symptoms. The most common one fitting that bill is migraine, which can also co-occur with SCD.[hopkinsmedicine.org]

Treatment

  • The minor complications are hematoma, cellulitis, tinnitus. 5, 6) Medical treatment is also considered for treatment, even though no medical treatment has been proven fully effective for SSCD.[ejao.org]
  • What Are the Treatment Options for Superior Semicircular Canal Dehiscence? For people with only mild symptoms of SSCD, treatment may be as simple as learning techniques to avoid triggers and manage dizziness.[dariuskohanmd.com]
  • In the cases with bilateral dehiscence, if symptoms continue after surgical treatment, it is proposed the same treatment for the other ear. This is the situation of the fourth case that is pending of surgical treatment in the left ear.[medcraveonline.com]
  • Treatment is symptomatic, however is severe cases occlusion of the involved canal, resurfacing techniques, and transcanal occlusion of the round window have been described as helpful.[oxfordmedicine.com]
  • This article will review our strategy and discuss the advantages and disadvantages of the different surgical treatments used for patients with severe symptoms from superior canal dehiscence.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis Surgical procedures are performed through a middle cranial fossa approach. Plugging or resurfacing the superior semicircular canal has been documented to resolve or improve the symptoms 4 .[radiopaedia.org]
  • Prognosis is generally good, but depends on the treatment employed. Complications from surgery can cause incomplete resolution of hearing loss or balance disturbances and, in some patients, symptoms increase in severity after surgery.[health.ebsco.com]
  • What is the long-term prognosis if I choose NOT to have MF surgery? Sadly, your story is not uncommon![hopkinsmedicine.org]

Etiology

  • The etiology is under continuous investigation.[scitechnol.com]
  • Anatomical feature of the middle cranial fossa in fetal periods: Possible etiology of superior canal dehiscence syndrome . Acta Oto-Laryngologica, Vol. 132, Issue. 4, p. 385. CrossRef Google Scholar McCall, Andrew A. McKenna, Michael J.[cambridge.org]
  • Recent findings Canal dehiscence involving the superior, lateral, and posterior semicircular canal can have different etiologies, including developmental abnormality, congenital defect, chronic otitis media with cholesteatoma, and high-riding jugular[doi.org]
  • Indeed, the term “conductive hearing loss” is itself a bit of a misnomer, and implies an etiology based on inadequate conduction to the inner ear.[canadianaudiologist.ca]
  • Good advice on what to add to test battery (supra threshold bone conduction and acoustic reflexes on all air bone gaps) by kai on May 9, 2017 Very clear interpretation of the etiology and anatomy. by Ghalia on May 8, 2017 It was straightford and easy[audiologyonline.com]

Epidemiology

  • Epidemiology Frequency United States The true incidence of persons with symptomatic SCDS is currently unknown.[emedicine.medscape.com]
  • […] dehiscence (SSCD) is a defect of the skull base that is gaining increasing recognition among neurosurgeons. 1 , 2 , 5 It is described as a pathological aperture commonly at the level of the arcuate eminence of the petrous temporal bone. 2 The exact epidemiology[thejns.org]
Sex distribution
Age distribution

Pathophysiology

  • This video case report demonstrates Hennebert's sign associated with SSCD syndrome and discusses its pathophysiological basis. ISSN: 0023-852X, 1531-4995 PMID: 22252740[deepblue.lib.umich.edu]
  • Overview The author explains the clinical presentation, pathophysiology, diagnostic work-up, and management of superior semicircular canal dehiscence syndrome.[medlink.com]
  • Pathophysiology: Superior Semicircular Canal Dehiscence Syndrome is characterized by thinning or loss of bone that covers the SSC and which separates it from of the cranial cavity.[medcraveonline.com]
  • Concerning the pathophysiology of this condition, we hypothesized that the increased volume of inner ear liquid can modify the micromechanical function of the cochlea and the labyrinthine hydrodynamics.[ncbi.nlm.nih.gov]
  • Pathophysiology The cochleovestibular system has 2 functional windows. The oval window, which houses the footplate of the stapes, allows sound to enter the inner ear (vestibule) and to be carried via hydroacoustic waves through the perilymph.[emedicine.medscape.com]

Prevention

  • Resurfacing of the dehiscent canal also prevents chronic stimulation from the pulsating temporal lobe. Transmastoid Approach The surgical technique begins with a post auricular incision, as it usually performed for a mastoidectomy.[ncbi.nlm.nih.gov]
  • All of this led to this medical conclusion – and I quote – “you may or may not have a problem in your inner ear, but either way, I cannot prevent your fate.” He actually repeated that line (in case my hearing WAS affected?)[barbarakessler.com]
  • If you are affected by vertigo, dizziness or other balance issues, schedule an appointment with your physician or our office to determine the underlying cause, discuss treatment options, and, in some cases, prevent future problems such as hearing loss[centerforbetterhearing.com]
  • Caulk Co.) to prevent release of fluid from the cochlea, and to prevent air leaking into the cochlea.[karger.com]
  • Find a Doctor Request an Appointment Refer a Patient Apply for a Job Make a Gift Get a Second Opinion Pay My Bill Health Health Home Conditions and Diseases Treatments, Tests and Therapies Wellness and Prevention Caregiving I Want to...[hopkinsmedicine.org]

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