Superior canal dehiscence syndrome: A review.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.

Description of a New Labyrinthine Dehiscence: Horizontal Semicircular Canal Dehiscence at the Tympanic Segment of the Facial Nerve.

Objective: This report is a case series of patients with findings suspicious for a labyrinthine dehiscence syndrome not previously described in the medical literature. We describe the clinical and test findings in 16 patients with CT findings suspicious for dehiscence of the ampullated end of the horizontal semicircular canal at the tympanic segment of the facial nerve. Study Design: Observational case series. Setting: Neurotology vestibular referral center. Patients: To be included in this study the patients were seen at our center in 2019 and had a high-resolution CT scan with a collimation of 0.6 mm. Patients who were identified as having findings suspicious for dehiscence of bone where the facial nerve crosses the ampullated end of the horizontal semicircular canal (HSC-FND) were identified and further analyzed. Interventions: Case series retrospective record review of patient symptoms, physical findings, audiometry, vestibular testing, and CT scans was performed. CT findings of other dehiscent sites were noted. A comparison to surgically treated perilymph fistula (PLF) patients of the same period was performed. Main Outcome Measures: History and physical exam were reviewed for auditory symptoms, vestibular symptoms, and exacerbating factors. and. Audiometry and vestibular testing were reviewed to determine which tests were most likely to be abnormal. CT scans were independently graded according to degree of suspicion for HSC-FND. Finally, patients with HSC-FND as the sole dehiscence identified were compared to those who had HSC-FND plus other dehiscent sites (HSC-FND+O) and to the group of surgically treated PLF patients. Results: Of 18 patients, 16 met inclusion criteria. Nine (56%) of those suspicious for HSC-FND had dehiscences in other parts of the labyrinth. Additional dehiscent sites included: six superior semicircular canal dehiscences (SSCD), two cochlear facial dehiscences and one cochlear carotid dehiscence. The most common auditory symptoms were autophony followed by tinnitus and aural fullness. The most common vestibular symptoms were pulsion sensation (feeling of being pushed to one side) followed by vertigo spells. The most common exacerbating factors for vertigo were straining, and sound. The most commonly abnormal vestibular test was nasal Valsalva testing, which was positive in all but one patient. Anamnesis and examination observations were similar in both groups, but the HSC-FND group were less likely to demonstrate a caloric weakness or an abnormal ECOG compared to the HSC-FND+O group. Of note, cVEMP was more often found to have lower thresholds in the HSC-FND group compared to the HSC-FND+O group. An example case is highlighted. Comparison to the PLF patients revealed statistically significant difference in the presenting symptoms of autophony, fullness and pulsion sensation. When comparing testing, HSC-FND patients were more likely to have an abnormal cVEMP and PLF patients were more likely to have asymmetric hearing. The incidence of bilateral disease was also more common among the HSC-FND patients than the PLF patients. Conclusions: A new labyrinthine dehiscence has been described to occur where the tympanic segment of the facial nerve crosses over the ampullated end of the horizontal semicircular canal. HSC-FND patients can present in a similar manner as HSC-FND+O patients with similar test findings except as mentioned above. The identification of one dehiscence such as SSCD does not preclude the presence of another dehiscence such as HSC-FND. HSC-FND could be the source of persistent symptoms post SSCD surgery as illustrated in the case presented. HSC-FND patients seem to identify themselves compared to PLF patients by a much more likely presenting symptoms of autophony, fullness, pulsion, abnormal cVEMP, bilaterality of disease, and symmetric hearing.

Vestibular Hypersensitivity in Patients with Chronic Noise Exposure.

It has been demonstrated that high-intensity noise exposure adversely affects the human balance function. The Tullio phenomenon (TP) refers to sound-induced imbalance which is resulted from hypersensitivity of vestibular end organs to normal acoustic stimuli. Although different etiologies have been attributed to TP, evidence on the role of excessive noise exposure in the development of this symptom is limited. The present study aims to assess the vestibular functions in patients manifesting TP symptom who were exposed to long-term excessive noise levels. This was an analytic cross-sectional study conducted on 17 males diagnosed with TP with a history of chronic noise-induced hearing loss (TP group) and 17 healthy individuals. All subjects in both groups underwent complete otological, videonystagmography (VNG), and cervical vestibular myogenic potential (cVEMP) assessments. The most common complaint in TP subjects was vertigo and imbalance. During the VNG assessment, we found abnormal positional nystagmus and caloric irrigation (vestibular hyperfunction) results in 4 (23.53%) and 9 (52.94%) patients, respectively. Seven (41.17%) patients indicated cVEMP thresholds which were abnormally lower than the normal values ( ≤ 70 dB HL). However, when both VNG and cVEMP results were considered together, the abnormal rate reached 70.58% (12 of 17 cases). Our findings showed that both the semicircular canal as well as otolith stuctures could be affected in TP patients with a history of chronic noise exposure.

Superior Semicircular Canal Dehiscence : Covering Defects in Understanding from Clinical to Radiologic Evaluation.

Superior semicircular canal dehiscence alters the normal fluid mechanics of the vestibulocochlear system and can be a debilitating condition. This article reviews the current understanding of the bony labyrinthine defect, including symptoms, etiology, surgical approach, as well as preoperative and postoperative imaging pearls.

Bone-Conducted oVEMP Latency Delays Assist in the Differential Diagnosis of Large Air-Conducted oVEMP Amplitudes.

Background: A sensitive test for Superior Semicircular Canal Dehiscence (SCD) is the air-conducted, ocular vestibular evoked myogenic potential (AC oVEMP). However, not all patients with large AC oVEMPs have SCD. This retrospective study sought to identify alternate diagnoses also producing enlarged AC oVEMPs and investigated bone-conducted (BC) oVEMP outcome measures that would help differentiate between these, and cases of SCD. Methods: We reviewed the clinical records and BC oVEMP results of 65 patients (86 ears) presenting with dizziness or balance problems who underwent CT imaging to investigate enlarged 105 dB nHL click AC oVEMP amplitudes. All patients were tested with BC oVEMPs using two different stimuli (1 ms square-wave pulse and 8 ms 125 Hz sine wave). Logistic regression and odds ratios were used to determine the efficacy of BC oVEMP amplitudes and latencies in differentiating between enlarged AC oVEMP amplitudes due to dehiscence from those with an alternate diagnosis. Results: Fifty-three ears (61.6%) with enlarged AC oVEMP amplitudes were identified as having frank dehiscence on imaging; 33 (38.4%) had alternate diagnoses that included thinning of the bone covering (near dehiscence, n = 13), vestibular migraine (n = 12 ears of 10 patients), enlarged vestibular aqueduct syndrome (n = 2) and other causes of recurrent episodic vertigo (n = 6). BC oVEMP amplitudes of dehiscent and non-dehiscent ears were not significantly different (p > 0.05); distributions of both groups overlapped with the range of healthy controls. There were significant differences in BC oVEMP latencies between dehiscent and non-dehiscent ears for both stimuli (p < 0.001). A prolonged n1 125 Hz latency (>11.5 ms) was the best predictor of dehiscence (odd ratio = 27.8; 95% CI:7.0-111.4); abnormal n1 latencies were identified in 79.2% of ears with dehiscence compared with 9.1% of ears without dehiscence. Conclusions: A two-step protocol of click AC oVEMP amplitudes and 125 Hz BC oVEMP latency measures optimizes the specificity of VEMP testing in SCD.

Bilateral Vestibular Atelectasis with Tullio's Phenomenon.

Vestibular dehiscence or atelectasis is a rarity known to cause vertigo. This pathology poses many challenges especially in diagnosis and treatment simply due to the scarcity of reported cases. The etiologic factors, disease pathophysiology and complications remain unclear. We report a young adult female who presented with unsteadiness for 2 years associated with headache. Tullio's phenomenon was observed which led to a finding of bilateral vestibular dehiscence involving the medial walls on high resolution computed tomography.

Two cases of Tullio phenomenon in which oval and round window reinforcement surgery was effective.

AIM: To report the effect of oval and round window reinforcement surgery performed in two patients with the Tullio phenomenon. Case 1: A male with bilateral superior canal dehiscence syndrome. Downbeat nystagmus with leftward horizontal eye movement was recorded in an electronystagmogram using a pure-tone sound of 110dB at 2000 or 4000Hz in the right ear. Case 2: A female who had undergone stapes surgery. Computed tomography revealed an ossicular prosthesis in the vestibule. An audiogram indicated mild to moderate hearing impairment in the right ear. Leftward horizontal nystagmus was recorded in an electronystagmogram using a pure-tone sound of 110dB at 500 or 1000Hz in the right ear. Surgical findings indicated that the prosthesis was inserted deep into the oval window, which was closed with thin connective tissue. OUTCOMES: After oval and round window reinforcement surgery was performed in the right ear, and loud, pure-tone sounds elicited neither nystagmus nor dizziness in either patient.

Diagnostic performance of reformatted isotropic thin-section helical CT images in the detection of superior semicircular canal dehiscence.

Purpose The purpose of this article is to assess the diagnostic performance of computed tomography (CT) reformatted images for detection of superior semicircular canal (SSC) dehiscence. Material and methods Forty-two patients, with sound- and/or pressure-induced vestibular symptoms, and 42 control participants underwent helical CT examination with a highly collimated beam (0.5 mm). Reformatted images of the vestibular labyrinth were obtained in the standard axial and coronal planes (group A images), and in a plane parallel and perpendicular to the SSC (group B images). Diagnostic performance obtained by evaluating the group A images alone and the group B images alone was analyzed by using the area under the receiver operating characteristic curve (AUC). Results The diagnostic performance of group A images was AUC = 0.929 with an overall accuracy of 92.9%. The diagnostic performance of group B images was AUC = 0.988 with an overall accuracy of 98.8%. The evaluation of group B images alone showed an improved diagnostic performance over the group A images alone. Conclusion Thin-section 0.5-mm collimation CT with reformatted images oriented in the plane parallel and perpendicular to the SSC improves diagnostic accuracy in assessing for SSC dehiscence in comparison to CT images with reconstructions limited to traditional axial and coronal planes.

Superior Semicircular Canal Ampullae Dehiscence As Part of the Spectrum of the Third Window Abnormalities: A Case Study.

A 60-year-old man was referred to the ENT department for intense episodic vertigo triggered by loud sounds. Pure tone audiometry and otoneurological assessment, including videonystagmography using auditory stimulation and cervical vestibular evoked myogenic potential measures, conducted to the hypothesis of a third window syndrome in the left ear. Results from the high-resolution computed tomography of the petrous bone confirmed the hypothesis and revealed the presence of a submillimeter semicircular canal dehiscence, located between the left lateral and superior semicircular canal ampullae on the left side.

Síndrome de dehiscencia del canal semicircular superior: a propósito de un caso / Canal dehiscence syndrome of the superior semicircular: in a case purpose

El síndrome de dehiscencia de canal semicircular superior (DCSS) es una patología descrita en 1998 por Minor y cols, presenta síntomas diversos incluido vértigo inducido por el sonido, hipoacusia y autofonía por la falta de cobertura ósea en dicho canal. El diagnóstico se basa en la clínica y la confirmación se obtiene mediante la tomografía computarizada de peñasco. El tratamiento será expectante o reparación quirúrgica de la continuidad si la clínica es incapacitante. En este artículo se presenta un caso clínico de DCSS con síntomas auditivos y vestibulares al emitir el fonema "mmm".

The superior semicircular dehiscence síndrome is a pathology described en 1998 by Minor et al. Which presents several symptoms incluid sound induced vértigo, hearing loss and autophony due to bone dehiscense of this semicircular canal. The diagnosis was based on clinical and confirmation is given by the temporal bone CT. Treatment is expectant or surgical repair of continuity if the clinic is disabling. In this paper we present a case of DCSS with auditory and vestibular symptoms in issuing the phoneme "mmm".

Acquired auditory neuropathy spectrum disorder after an attack of chikungunya: case study.

Auditory neuropathy spectrum disorder (ANSD) is a retrocochlear disorder in which the cochlear functioning is normal but the transmission in the auditory neural pathway is affected. The present study reports of a 14-year-old teenager with acquired ANSD after an attack of chikungunya. He reported symptoms of difficulty in understanding speech, tinnitus and vertigo when exposed to loud sounds. The audiological characteristics suggested auditory neuropathy spectrum disorder with raising audiogram configuration. The results of tinnitus evaluation showed low-pitched tinnitus and it was persistent causing significant handicap to him based on self report tinnitus handicap questionnaire results. The results of depression, anxiety and stress scale also suggested symptoms of mild depression and anxiety. Chikungunya virus is suspected to be neurotropic in nature which can damage auditory nerve cells and may have caused ANSD. The result also shows presence of tullio's phenomenon and absence of cervical vestibular evoked myogenic potentials suggesting damage to the vestibular neuronal system. The possible pathophysiology of chikungunya virus causing ANSD and vestibular symptoms needs to be explored further in future studies.

Superior canal dehiscence syndrome in children--a case report.

Initially described in 1998, superior semicircular canal dehiscence syndrome (SCDS) has become a well-studied neurootologic entity in adults by now. Unfortunately, experience with children is limited and a diagnostic and therapeutic algorithm is lacking. The article therefore wants to provide an overview of the existing literature on superior semicircular canal dehiscence syndrome in children. Furthermore a diagnostic algorithm for daily clinical life based on a case report from an eleven-year-old girl is presented.

Classification of middle fossa floor dehiscence syndromes.

OBJECT: Middle fossa floor dehiscence (MFFD) can present as multiple syndromes depending on dehiscence location, tissue herniation, and dural integrity. The authors propose a classification system for MFFD with the potential to guide clinical decision making. METHODS: A retrospective analysis of the electronic medical records (years 1995-2012) of patients who had undergone temporal craniotomy for the surgical repair of an MFFD syndrome at a single institution was undertaken. Reviewed data included demographic, operative, presentation, and outcome details. Middle fossa floor dehiscence was classified as follows: Class A, bony dehiscence without herniation of the brain and/or meninges; Class B, herniation of the brain and/or meninges through the middle fossa floor without CSF leakage; Class C, dehiscence with CSF leakage without meningitis; or Class D, dehiscence with meningitis. RESULTS: Fifty-one patients, 22 males and 29 females, were included in the analysis. The mean age was 48.7 ± 15.5 years, mean body mass index was 32.65 ± 6.86 kg/m(2), and mean symptom duration was 33 ± 42 months. Seven patients underwent repeat surgery for symptomatic recurrence; therefore, there were 58 surgical encounters. Repair included bony reconstruction with hydroxyapatite with or without resection of encephaloceles and/or repair of a dural defect. According to the MFFD classification system described, 15, 8, 27, and 8 cases were categorized as Class A, B, C, and D, respectively. The prevalence of hearing loss was 87%, 63%, and 70% in Classes A, B, and C, respectively. Vestibular symptoms were more prevalent in Class A. Seven patients reported persistent symptoms at the last follow-up. Transient complications were similar in each classification (13%-25%), and a single permanent complication related to anesthesia was observed. There were no mortalities or severe neurological morbidities in the series. CONCLUSIONS: Middle fossa floor dehiscence has a spectrum of clinical presentations. A classification system may help to clarify the diagnosis and guide therapy. Surgery, the mainstay of treatment, is safe and well tolerated.

On the biological plausibility of Wind Turbine Syndrome.

An emerging environmental health issue relates to potential ill-effects of wind turbine noise. There have been numerous suggestions that the low-frequency acoustic components in wind turbine signals can cause symptoms associated with vestibular system disorders, namely vertigo, nausea, and nystagmus. This constellation of symptoms has been labeled as Wind Turbine Syndrome, and has been identified in case studies of individuals living close to wind farms. This review discusses whether it is biologically plausible for the turbine noise to stimulate the vestibular parts of the inner ear and, by extension, cause Wind Turbine Syndrome. We consider the sound levels that can activate the semicircular canals or otolith end organs in normal subjects, as well as in those with preexisting conditions known to lower vestibular threshold to sound stimulation.

Tullio Phenomenon Following Spontaneous Intralabyrinthine Gross Hemorrhage / 대한평형의학회지

Tullio phenomenon is a pattern of sound induced unsteadiness, imbalance or vertigo, associated with disturbances of oculomotor and postural control. As a possible cause of sudden sensorineural hearing loss, intralabyrinthine gross hemorrhage has been reported in subjects with bleeding tendency. We report a case of spontaneous intralabyrinthine hemorrhage followed by presentation of Tullio phenomenon. A 35-year-old man presented with sudden left side hearing loss and vertigo. Audiometry results indicated left total deafness and magnetic resonance images revealed left intralabyrinthine hemorrhage. At 1 month after hearing loss, sound and pressure-induced vertigo and disequilibrium newly developed. Follow-up images indicated signs of fibrosis in the left labyrinth and nystagmography results showed induction of nystagmus according to the stapedial reflex. This case suggests possibility of Tullio phenomenon in sudden sensorineural hearing loss patients.

Characteristics and management of superior semicircular canal dehiscence.

Objectives To review the characteristic symptoms of superior semicircular canal dehiscence, testing and imaging of the disease, and the current treatment and surgical options. 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. Potential etiologies noted for canal dehiscence include possible developmental abnormalities, congenital defects, chronic otitis media with cholesteatoma, fibrous dysplasia, and high-riding jugular bulb. Computed tomography (CT), vestibular evoked myogenic potentials, Valsalva maneuvers, and certain auditory testing may prove useful in the detection and evaluation of dehiscence syndrome. Multislice temporal bone CT examinations are normally performed with fine-cut (0.5- to 0.6-mm) collimation reformatted to the plane of the superior canal such that images are parallel and orthogonal to the plane. For the successful alleviation of auditory and vestibular symptoms, a bony dehiscence can be surgically resurfaced, plugged, or capped through a middle fossa craniotomy or the transmastoid approach. SSCD should only be surgically treated in patients who exhibit clinical manifestations.

Multiple dehiscences of bony labyrinthine capsule. A rare case report and review of the literature.

Multiple semicircular canal dehiscences are clinical entities characterised by vestibular and cochlear symptoms induced by enhanced sensitivity of labyrinthine receptors due to a multiple bone defect of the otic capsule. The case is presented of a 38-year-old male with bilateral posterior semicircular canal dehiscence associated with unilateral (right) superior semicircular canal dehiscence. The man suffered from vestibular (recurrent Tullio Phenomenon or sound-induced vertigo) and cochlear symptoms (persistent aural fullness associated with mixed hearing loss and disabling tinnitus).