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]

A woman in her 30s, the daughter of the aforementioned patient, presented with dizziness and headaches in 2011. The daughter’s symptoms started during pregnancy (1 year prior to presentation). [jamanetwork.com]

Battista is an excellent presenter...clear, concise. Highly recommend. by Danielle on June 6, 2017 Dr. Battista is an excellent presenter. [audiologyonline.com]

We present a protocol to establish the diagnosis of a similar condition, especially those presenting atypically. [ejo.eg.net]

• Asymptomatic

  He suspects that all SCDS people are asymptomatic until a second event, such as a pressure altering occurrence, or trauma - this could be direct such as a blow to the head, or barotrauma as happened to me on a flight. [actiononhearingloss.org.uk]

  Dr Gerard Gianoli at the Ear and Balance Institute in Covington, Louisiana, who became my specialist after a failed first surgery in England, suspects that all SCDS people are asymptomatic until a second event. [hearinghealthmatters.org]

  Additionally, SSCD has also been reported to be asymptomatic. In the past, many patients with SSCD had been misdiagnosed as having otosclerosis, patulous eustachian tubes, middle ear perilymphatic fistulas, or Ménière’s disease. [rarediseases.org]

  […] the body, such as self-generated sounds like the heartbeat and eye movement. 9 If an SSCD exists close to the cerebrospinal fluid (CSF) space, the abnormal opening into the inner ear results in the abnormal perception of these sounds. 9, 11, 12 Using asymptomatic [ncbi.nlm.nih.gov]

  […] lateral skull base, more so than patients with spontaneous CSF otorrhea and control groups with different BMIs. " Lim et al (2012) recommended "In cases of SSCD requiring surgery, we support the view that elective surgical repair be recommended where asymptomatic [dizziness-and-balance.com]

• Surgical Procedure

  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]

  In one case, the reconstruction could not be visualised in CT owing to the radiotransparency of certain materials used in the surgical procedure (wax, fascia, cartilage). The full text of this article is available in PDF format. [em-consulte.com]

  Surgical procedure used to treat both cases were superior canal plugging with bone pate, fascia and muscle in the lumen of the canal, and cortical bone graft over the defect, with excellent outcome on the follow-up. [scitechnol.com]

  The round window reinforcement is a procedure with low risk compared to the middle fossa or transmastoid approaches, and it may be offered as a first procedure in patients with mild symptoms. [ncbi.nlm.nih.gov]

  I cannot emphasize enough that one should never have a surgical procedure just because the CT scan shows evidence of superior canal dehiscence or thinning. [hopkinsmedicine.org]

• Weakness

  Complications/Risks Risks include bleeding, infection, hearing loss, spinal fluid leak, dizziness, facial nerve weakness, change in taste, aphasia, stroke, and death. [csurgeries.com]

  "We found that if the temporal bone in one ear was thin, the second ear showed the same thinness, an indication that the weakness is likely to be congenital if not inherited," says Minor, associate professor of otolaryngology-head and neck surgery, biomedical [jhu.edu]

  Caloric responses were normal in amplitude and without significant unilateral weakness or directional preponderance. Results of cerebral MRI and electroencephalography were normal. [doi.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 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]

  […] 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]

  Patients showing this symptom may experience a loss of equilibrium, a feeling of motion sickness or even actual nausea, triggered by normal everyday sounds. Although this is often associated with loud noises, volume is not necessarily a factor. [en.wikipedia.org]

• Osteopenia

  Moreover, estrogen interacts with GH and IGF-1, serving as an integral regulator of bone metabolism in both males and females. 19 Estrogen deficiency, particularly during postmenopausal stages, is correlated with bone resorption. 19 The prevalence of osteopenia [thejns.org]

  Do you find any connection with SCDS and osteoporosis and osteopenia? We have not seen a connection, but this has been suggested by other researchers. I will mention that the vast majority of our patients do not have osteoporosis. [hopkinsmedicine.org]

• Osteoporosis

  Do you find any connection with SCDS and osteoporosis and osteopenia? We have not seen a connection, but this has been suggested by other researchers. I will mention that the vast majority of our patients do not have osteoporosis. [hopkinsmedicine.org]

  […] estrogen interacts with GH and IGF-1, serving as an integral regulator of bone metabolism in both males and females. 19 Estrogen deficiency, particularly during postmenopausal stages, is correlated with bone resorption. 19 The prevalence of osteopenia and osteoporosis [thejns.org]

• Hennebert's Sign

  "Hennebert's sign in superior semicircular canal dehiscence syndrome: A Video Case Report ." The Laryngoscope 122(2): 412-414. [deepblue.lib.umich.edu]

  Hennebert sign, and postural sway induced by external auditory canal pressure. [medlink.com]

  The Hennebert sign of vestibular symptoms due to changes in external auditory canal pressure is frequently found in conjunction with the Tullio phenomenon (as in perilymphatic fistula, syphilis, Ménière disease). [emedicine.medscape.com]

  This has long been recognised as a possible complication of cholesteotoma eroding bone over the horizontal canal accounting for horizontal nystagmus induced by applying alternating pressure to the external ear canal, called Hennebert’s sign. [nzma.org.nz]

  sign in superior semicircular canal dehiscence syndrome: a video case report. ( 22252740 ) Shuman A.G....Heidenreich K.D. 2012 46 Mutation analysis of Netrin 1 and HMX3 genes in patients with superior semicircular canal dehiscence syndrome. ( 22779713 [malacards.org]

• Benign Paroxysmal Positional Vertigo

  We observed an incidence of 11.2% complications, most commonly postoperative benign paroxysmal positional vertigo. The risk of nonotologic intracranial complications (1.7%) is low. 1. Minor, LB. [journals.sagepub.com]

  SCDS can resemble these more common conditions: Otosclerosis Eustachian Tube Dysfunction Patulous Eustachian Tube BPPV (Benign Paroxysmal Positional Vertigo) Meniere's Disease Vestibular Neuronitis / Neuritis Labyrinthitis Contact Us Today! [masseyeandear.org]

  The Dix-Hallpike maneuver is used to rule out benign paroxysmal positional vertigo. Head-Shake Test - your doctor will shake your head while you are wearing special lenses. [verywellhealth.com]

  Hain (2016) has diagnosed 46 patients compared to several thousand with Benign Paroxysmal Positional Vertigo (BPPV). Thus, SCDS is much less prevalent than BPPV which is known to affect 2% of the population. [hearinghealthmatters.org]

  Corrigan, Clinical Practice Guideline: Benign Paroxysmal Positional Vertigo (Update), Otolaryngology-Head and Neck Surgery, 10.1177/0194599816689667, 156, 3_suppl, (S1-S47), (2017). Eric G. [doi.org]

• Normal Hearing

  –25.81) and 3.31 in CHL (5/61; P = 0.115, 95% confidence interval, 0.75–14.63), compared with normal hearing status. [journals.lww.com]

  The threshold for eliciting vestibular-evoked myogenic potentials from affected ears was (mean +/- SD) 81 +/- 9 dB normal hearing level. The threshold for unaffected ears was 99 +/- 7 dB, and the threshold for control ears was 98 +/- 4 dB. [ncbi.nlm.nih.gov]

  Audiometric examination indicated normal hearing in the right ear; low-frequency hearing loss was detected in the left ear. Air-bone gaps in the left ear were 35, 20, and 15 dB HL at 250, 500, and 1000 Hz, respectively [Figure 1]. [ejo.eg.net]

  E also shows moderate hearing loss in the high frequencies (60 dB at 4 kHz and 65 dB at 8 kHz), whereas F shows normal hearing. AC indicates air conduction; ANSI, American National Standards Institute; BC, bone conduction; PTA, pure-tone average. [jamanetwork.com]

  All in all, I definitely prefer the “too much me” and occasional “feedback” and dizziness to losing hearing in that ear altogether. But there was a time of grieving my gradual loss of normal hearing and permanent change in audio perception. [barbarakessler.com]

• Hearing Problem

  Superior Canal Dehiscence Symptoms Most symptoms of SCDS are either vestibular (balance-related) problems or auditory (hearing) problems. [share.upmc.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]

  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]

  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]

  However, the most common symptoms are vertigo/dizziness elicited by pressure altering activity, Tullio’s phenomenon (sound-induced vertigo), fullness/pressure in the ear and autophony. [rarediseases.org]

• Oscillopsia

  A striking sign of SSCD, a sensation of oscillopsia, is described by patients as the swaying of the eyes or horizon in up and down direction or experiencing vertigo on an upward plane. [neuroequilibrium.in]

  This causes vertigo, oscillopsia, dysequilibrium due to exposure to sound. Ultra high resolution CT scans help in identification of this condition. This dehiscence has been confirmed by surgical exploration of middle cranial fossa. [works.bepress.com]

  • Typically, in affected patients with superior semicircular canal dehiscence syndrome, there is a several-year history of symptoms that may include sound-induced vertigo (Tullio phenomenon), pressure-induced vertigo (Hennebert sign), or oscillopsia [medlink.com]

  There is frequent hearing loss and oscillopsia. Semicircular canal dehiscence represents an abnormal communication between the inner ear and surrounding structure through a lack of an osseous covering over the membranous labyrinth. [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]

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]

Preoperative Workup The diagnosis of SCD is made radiographically, therefore all patients should undergo fine cut temporal bone computed tomography (CT) imaging. [csurgeries.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]

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]

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]

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]

The nurse reads about acute chest syndrome, including risk factors, signs and symptoms, potential complications, laboratory tests, treatment goals and interventions. [health.ebsco.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]

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]

Importance The etiology of superior canal dehiscence (SCD) involving the arcuate eminence is not completely understood, but genetic factors may play a role. [jamanetwork.com]

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]

The etiology is under continuous investigation. [scitechnol.com]

Identifying the etiology is important for purposes of treatment, for if the etiology can be identified, research toward medical management may become feasible. [frontiersin.org]

The etiology of SCD is unknown. Carey et al. [6] suggest that dehiscence arises from a defect in postnatal development. Nadgir et al. [1] study showed that superior SCD is more commonly an acquired rather than congenital or developmental condition. [jscimedcentral.com]

Epidemiology Frequency United States The true incidence of persons with symptomatic SCDS is currently unknown. [emedicine.medscape.com]

[…] canal 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]

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]

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]

Overview The author explains the clinical presentation, pathophysiology, diagnostic work-up, and management of superior semicircular canal dehiscence syndrome. [medlink.com]

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]

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]

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]

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]

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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]