Hennebert's Sign Research Articles

Insights from therapeutic strategies in superior canal dehiscence syndrome: is there anything beyond surgical treatment?

Superior semicircular canal dehiscence syndrome (SCDS) is a clinical syndrome that can cause instability, vertigo, fullness, tinnitus, autophony, hearing loss (HL), Tullio phenomenon, or Hennebert's sign. Historically, surgery has been the primary treatment reported in the literature, although some medical treatments may also be proposed. This study aims to comprehensively characterize SCDS in a large series of patients from clinical, auditory, and vestibular perspectives, and explore medical alternatives to conventional surgical treatments by comparing their results and evolution. A retrospective observational study was designed in a tertiary care center. Audiovestibular tests evaluated included pure-tone audiometry (PTA), VEMPs, video head impulse test (vHIT), and CT imaging. Improvement was assessed over a follow-up period of up to 6 months for seven cardinal symptoms to verify the efficacy of the proposed treatments. 71 subjects with SCDS and a mean age of 51.20 ± 12.22 years were included in the study. The most common symptom found in our sample was instability in 31 patients (43.66%), followed by aural fullness or tinnitus in 29 subjects (40.85%). 36 patients (43.66%) received medical treatment, with 28 of them (77.78%) showing symptom reduction. Surgical repair was indicated in five patients, with all showing symptom improvement. Statistically significant improvement (p < 0.05) was observed, particularly with surgical treatment and acetazolamide, in both symptoms and objective tests such as pure-tone audiometry and VEMPs. SCDS shows significant similarities with other otic capsule dehiscences. It is essential to perform VEMPs and CT scans to complete the diagnosis, which is usually accompanied by clearly recognizable clinical criteria. Surgery for SCDS is effective, safe, and without complications. However, in cases where symptoms are mild to moderate, addressing this condition with medical treatment using diuretics such as acetazolamide has shown promising results.

Skull Vibration-Induced Nystagmus in Superior Semicircular Canal Dehiscence: A New Insight into Vestibular Exploration-A Review.

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms' competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice.

Superior Semicircular Canal Dehiscence Syndrome Manifested as Menière’s Disease: A Case Report

The absence of a temporal bone overlying the superior semicircular canal causes superior semicircular canal dehiscence (SSCD). The vestibular symptom of SSCD syndrome (SCDS) is vertigo and audiologic symptoms include autophony, hyperacusis, and ear fullness. A 52-year-old man presented with left-sided unilateral hearing loss, aural fullness, and recurrent spinning-type vertigo. He had positive Hennebert sign and mixed-type hearing loss, with a prominent low-frequency air-bone gap. These symptoms reminded us of SCDS, and computed tomography (CT) revealed SSCD. However, the patient had not experienced vertigo until 1 week prior to the visit. In addition, the audiogram revealed fluctuation of hearing, which was aggravated when the vestibular symptoms manifested. Vertigo might be due to Menière’s disease rather than SCDS and SSCD was incidentally detected on CT. According to reviews, this is no reported case of SCDS manifested as Menière’s disease, so we report this case with a brief review of the literature.

Inner-Ear Disorders Presenting with Air-Bone Gaps: A Review.

Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease's as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio's phenomenon, and Hennebert's sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs.

Symptom: Dizziness with Ear Pressure

Symptom: Dizziness with Ear Pressure

P70-F Vestibular-evoked myogenic potentials for the diagnosis of superior canal dehiscence syndrome

Background Superior canal dehiscence syndrome (SCDS) is a clinical entity characterized by vertigo and oscillopsia in response to sound (Tullio phenomenon), pressure (Hennebert sign) and vestibular stimulation with Valsalva maneuvers, disequilibrium, autophony, bone-conductive hyperacusis and pulsatile tinnitus. Vestibular-evoked myogenic potentials (VEMPs) is a technique used to determine the function of the otolithic organs. The aim of the study is to examine the VEMPs characteristics in patients with SCDS. Material and methods 5 patients with SCDS between 2016 and 2018 were included. VEMPs were recorded from the ipsilateral sternocleidomastoid muscle (cVEMP) and periocular sites (oVEMP). 500 Hz tone burst at 105 dB nHL was used as stimuli at 5 Hz stimulation rate and averaged for 200 repetitions. Bandpass-filtered between 20 Hz and 2 kHz. Statistical analysis included paired sample Mann-Whitney U test in the SPSS program. We compared the latency and amplitude of the affected ear with the healthy ear. Data shown as mean ± SE. Results The mean age of the test group was 61.4 ± 8.08 years with 3 males and 2 females. Comparing the affected ear and healthy ear: oVEMP amplitude (33.2 ± 5.9/8 ± 2.1 μ V, p 0.11); cVEMPamplitude (158.8 ± 41.2/125 ± 29.86 μ V, p > 0.56); cVEMP-p13 (15.2 ± 0.4/15.1 ± 0.7 ms, p > 0.8); In 100% of patients with SCDS, oVEMP amplitudes were significantly higher than normal values (p > 0.01 Mann-Whitney U). No statistically significant differences were obtained in the remaining parameters. Conclusions oVEMP amplitude in patients diagnosed as SCDS are significantly higher than normal values. These techniques have proven to be useful for diagnosis of superior canal dehiscence syndrome.

Incidence of intraoperative hearing loss during middle cranial fossa approach for repair of superior semicircular canal dehiscence

IntroductionSuperior semicircular canal dehiscence is a rare inner ear disorder characterized by an abnormal third opening between the superior semicircular canal and middle fossa. Symptoms include amplification of internal sounds, aural fullness, tinnitus, hearing loss, autophony, sound-induced vertigo (Tullio phenomenon), pressure-induced vertigo (Hennebert sign), disequilibrium, nystagmus, oscillopsia, and headache. While no cure exists for SSCD, surgical treatment has proven to effectively minimize these symptoms. This study reviewed brainstem auditory evoked potentials (BAEPs) that were monitored intraoperatively to better understand hearing loss risks associated with surgical treatment for SSCD. MethodsA retrospective chart review was conducted at the University of California, Los Angeles on adult patients with a confirmed diagnosis of SSCD who had undergone a middle cranial fossa repair from March 2011 to October 2017. A total of 142 cases of SSCD in 118 patients were repaired. ResultsThe majority of patients’ BAEPs remained stable and had no intraoperative hearing changes (n = 135; 95.1%). Seven patients experienced intraoperative changes as determined by a prolongation and reduction of Wave V latency (4.9%). Of these seven cases, five experienced a return to baseline prior to the end of surgery, and had no post-operative changes in hearing (71.4%). Overall, only two of the 142 surgeries (1.4%) resulted in failure to normalize and, as such, these patients experienced permanent changes in hearing. ConclusionThe results of this retrospective review demonstrate a low risk for hearing loss due to SSCD surgery via the middle fossa craniotomy approach.

Round window plugging in the treatment of superior semicircular canal dehiscence.

Objectives were to describe the use of round window plugging for superior semicircular canal dehiscence syndrome and review further recommendations regarding the procedure based on our experience and to compare results with recent literature on round window plugging. Retrospective case series. Fourteen patients underwent round window plugging for superior semicircular canal dehiscence at our institution from 2012 to 2015. All patients underwent the same surgical procedure. Available pre- and postoperative data were reviewed. Fourteen patient charts were reviewed. Symptoms of autophony improved in nine of 14 (64%) patients. Symptoms of pressure-induced vertigo improved in seven of 12 (58%) patients. Hennebert's sign that was positive preoperatively only improved in one of six (17%) patients. A positive preoperative vestibular evoked myogenic potential improved in only one of six (17%) patients. Six of 13 (46%) patients had increased air conduction thresholds postoperatively. Round window plugging has been described as a less-invasive treatment for patients with superior semicircular canal dehiscence. Although the procedure did benefit some of our patients, successful outcomes were not predictable. Improvement in at least one objective finding was seen in only 21% of the patients studied. Hennebert's sign and vestibular evoked myogenic potentials that were positive preoperatively only improved in 17% of patients. At our institution, round window plugging is no longer considered a reasonable treatment option for most patients with superior semicircular canal dehiscence. We recommend that further study on this topic follow a standardized pre- and postoperative assessment. 4. Laryngoscope, 128:1445-1452, 2018.

Clinical Characteristics of Posterior and Lateral Semicircular Canal Dehiscence.

The objective of this study was to evaluate the characteristic symptoms of and treatments for lateral semicircular canal dehiscence (LSCD) and posterior semicircular canal dehiscence (PSCD) and its proposed mechanism. A dehiscence acquired in any of the semicircular canals may evoke various auditory symptoms (autophony and inner ear conductive hearing loss) or vestibular symptoms (vertigo, the Tullio phenomenon, and Hennebert sign) by creating a "third mobile window" in the bone that enables aberrant communication between the inner ear and nearby structures. A PubMed search was performed using the keywords lateral, posterior, and semicircular canal dehiscence to identify all relevant cases. Our data suggest that PSCD, although clinically rare, is most likely associated with a high-riding jugular bulb and fibrous dysplasia. Patients may experience auditory manifestations that range from mild conductive to extensive sensorineural hearing loss. LSCD is usually associated with chronic otitis media with cholesteatoma.

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.

Low-Frequency Sound Pressure and Transtympanic Endoscopy of the Middle Ear in Assessment of “Spontaneous” Perilymphatic Fistula

This study was designed to verify an eventual perilymphatic fistula (PLF) in 264 patients with sensorineural hearing loss (SNHL) and/or vertigo. The patients were exposed to a low-frequency sound stimulation (LFS) on posturography to objectively test Tullio's phenomenon and Hennebert's sign. Endoscopes with 5 degree and 25 degree of visual angle and an outer diameter of 1.7 mm were used. The round window niche, with its foldings, oval window with stapes superstructure, a part of the facial recess and the area in the fissula ante fenestram were examined and video recorded. In one patient, we endoscopically verified a fistula in the round window membrane (resulting from a diving accident) that was covered with a fibrinous layer. In 4 cases, there was abnormal mucosal shining in the round window but without PLF. In 7 cases, the tympanic cavity could not be visualized because of the adhesive middle ear process, the abnormal anatomy, or the prominent exostoses of the ear canal prohibited vision. In 34 patients, LFS provoked unsteadiness on posturography without PLF. In 6 cases, a postoperative middle ear infection was recorded. No permanent tympanic membrane perforation occurred. It is unlikely that disease entity of “spontaneous PLF” exists. Tympanoscopy should be regarded as the first choice when a PLF is suspected.

Superior semicircular canal dehiscence in East Asian women with osteoporosis.

BackgroundSuperior semicircular canal dehiscence (SSCD) may cause Tullio phenomenon (sound-induced vertigo) or Hennebert sign (valsalva-induced vertigo) due to the absence of bone overlying the SSC. We document a case series of elderly East Asian women with atypical SSCD symptoms, radiologically confirmed dehiscence and concurrent osteoporosis.MethodsA retrospective record review was performed on patients with dizziness, vertigo, and/or imbalance from a neurology clinic in a community health center serving the East Asian population in Boston. SSCD was confirmed by multi-detector, high-resolution CT of the temporal bone (with Pöschl and Stenvers reformations) and osteoporosis was documented by bone mineral density (BMD) scans.ResultsOf the 496 patients seen in the neurology clinic of a community health center from 2008 to 2010, 76 (17.3%) had symptoms of dizziness, vertigo, and/or imbalance. Five (6.6%) had confirmed SSCD by multi-detector, high-resolution CT of the temporal bone with longitudinal areas of dehiscence along the long axis of SSC, ranging from 0.4 to 3.0 mm, as seen on the Pöschl view. Two of the 5 patients experienced motion-induced vertigo, two fell due to disequilibrium, and one had chronic dizziness. None had a history of head trauma, otologic surgery, or active intracerebral disease. On neurological examination, two patients had inducible vertigo on Dix-Hallpike maneuver and none experienced cerebellar deficit, Tullio phenomenon, or Hennebert sign. All had documented osteoporosis or osteopenia by BMD scans. Three of them had definite osteoporosis, with T-scores < −2.5 in the axial spine, while another had osteopenia with a T-score of −2.3 in the left femur.ConclusionsWe describe an unusual presentation of SSCD without Tullio phenomenon or Hennebert sign in a population of elderly, East Asian women. There may be an association of SSCD and osteoporosis in this population. Further research is needed to determine the incidence and prevalence of this disorder, as well as the relationship of age, race, osteoporosis risk, and the development of SSCD.

Hennebert's sign in superior semicircular canal dehiscence syndrome: A Video Case Report

Superior semicircular canal dehiscence (SSCD) syndrome has been called the great otologic mimicker because its presentation overlaps with otosclerosis, Meniere's disease, perilymphatic fistula, and patulous eustachian tube. A valuable examination finding that can help distinguish SSCD syndrome from other pathologic conditions is the presence of Hennebert's sign, in which pressure changes in the external auditory canal evoke stereotyped eye movements that align in the plane of the dehiscent semicircular canal. This video case report demonstrates Hennebert's sign associated with SSCD syndrome and discusses its pathophysiological basis.

Superior semicircular canal dehiscence syndrome: report of three cases

Superior semicircular canal dehiscence syndrome is a newly defined and rare disease. The disease shows itself with vertigo and nystagmus induced by loud noise and pressure. Vertigo and nystagmus induced by loud noise is named as Tullio's phenomenon and nystagmus induced by pressure is named as Hennebert's sign. The definitive diagnosis is made by the demonstration of bone defect in superior semicircular channel with high-resolution computed tomography.

Nueva causa de vértigo estructural: la dehiscencia del canal semicircular superior

The medical history of vertigo must be updated to accommodate current knowledge. In 1998 a new cause of vertigo associated with a structural anomaly was reported: superior semicircular canal dehiscence. This condition causes vestibular and auditory disorders, which are frequently associated, and a well-directed medical history allows a suspected diagnosis to be reached: the subject may suffer from vertigo triggered by loud sounds (Tullio's phenomenon) and by changes in pressure within the ear or in the intracranial space, when Valsalva's manoeuvres are performed or on pressing on the tragus (Hennebert's sign). It is not uncommon for subjects to suffer from a chronic imbalance that is exacerbated by the aforementioned precipitating factors. One frequent auditory symptom of superior semicircular canal dehiscence is autophony in the dehiscent ear, associated with hypoacusis of its transmission. This article outlines the questions that must be included in the medical history of vertigo in order to determine whether these dehiscences are present or not. The diagnostic procedures that are best suited to confirming it are also addressed. Superior semicircular canal dehiscence can be resolved satisfactorily by surgery.

Variety of Audiologic Manifestations in Patients With Superior Semicircular Canal Dehiscence

To present diverse symptoms, signs, and findings on diagnostic tests of 11 patients with superior semicircular canal dehiscence (SSCD) syndrome and surgical treatments for dehiscence of 3 patients. Retrospective case review. Tertiary neurotologic and audiologic center. Patients with SSCD documented by history, physical examination, vestibular function testing, and high-resolution computed tomographic scans. Two patients underwent surgical procedures through middle fossa approach, and 1 patient underwent transmastoid repair. The rest underwent conservative treatments. Eleven patients were identified as SSCD. The variety of clinical manifestations, including 1) hearing presentations: mix hearing loss (4 of 11), conductive hearing loss (3 of 11), profound sensorineural hearing loss (2 of 11), and normal hearing (2 of 11); 2) vestibular manifestations: chronic disequilibrium (8 of 11), Tullio phenomenon (7 of 11), Hennebert sign (8 of 11), tinnitus (3 of 11), and autophony (3 of 11); and 3) accompanying disorders: encephalomeningocele (1 of 11). Abnormal nystagmus was identified in 3 patients. Dehiscence of bone overlying superior semicircular canal was confirmed by high-resolution computed tomographic scan in all cases. Three patients underwent operative management. (2 through the middle fossa approach and 1 through a transmastoid repair). Superior semicircular canal dehiscence demonstrates diverse and complex clinical features. Tegmental or petrosal bone rarefaction or mild dehiscences can be a harbinger or an aggressive cause of developing SSCD. No correlation was observed between the size of dehiscence and the severity of vestibular symptoms or the degree of hearing loss. Surgical repair of dehiscence can relieve symptoms with low morbidity.

Bilateral Meningoencephalocele Repair Complicated by Superior Semicircular Canal Dehiscence: Case Report

To describe an unusual case of bilateral meningoencephaloceles with concurrent bilateral superior semicircular canal dehiscene (SSCD) and to discuss the clinical presentation, diagnosis, and treatment of SSCD. A 34-year-old man presented with unsteadiness and bilateral conductive hearing loss. He was diagnosed with bilateral meningoencephaloceles and underwent staged middle fossa approaches for repair. Following the second (right-sided) surgery, he developed sensorineural hearing loss and severe dizziness, indicating labyrinthine insult in the operated ear. He was then referred to our institution for further management. On our evaluation, the patient was continuing to experience disequilibrium and sensitivity to loud sounds. Examination revealed a positive Hennebert's sign and nystagmus consistent with symptomatic SSCD in the left ear. Computed tomography scanning with reformatting into Poeschel and Stenvers views identified bilateral SSCD. Plugging of the left SSCD was performed via a middle cranial fossa approach and resulted in improvement of the conductive hearing loss and after a period of compensation, resolution of the vestibular symptoms. This case illustrates that tegmental defects may result in simultaneous meningoencepaholcele and SSCD that may complicate their repair. The importance of having a high index of suspicion and evaluation with high resolution CT scanning with appropriate reformatting is emphasized. When present and symptomatic, SSCD can be successfully managed by plugging the canal.

Pulse‐synchronous eye oscillations revealing bone superior canal dehiscence

Superior canal dehiscence syndrome is a newly recognized syndrome characterized by vertigo and nystagmus induced by sound (Tullio phenomenon) or changes of middle ear (Hennebert sign) or intracranial pressure. We report on a patient with bilateral superior canal dehiscence syndrome who presented with unusual manifestations including pulse-synchronous vertical pendular nystagmus and Valsalva-induced, up and counterclockwise-beating jerk nystagmus. These unusual symptoms may be a clue to a better understanding of the pathophysiology of superior canal dehiscence syndrome. Abnormal communication between the inner ears and the intracranial space may explain the vertical pendular and pulse-synchronous nystagmus, modulated by increased intracranial pressure.

Can low frequency sound stimulation during posturography help diagnosing possible perilymphatic fistula in patients with sensorineural hearing loss and/or vertigo?

Perilymphatic fistula (PLF) is often difficult to diagnose because of the similar symptomatology, such as vertigo, tinnitus and hearing loss, which is found in several inner ear diseases. We attempted to correlate a positive result of low frequency sound (LFS) stimulation tests in posturography with the presence or absence of a PLF confirmed by transtympanic endoscopy in 209 patients with various inner ear diseases (Meniere's disease ( n=128), vestibulopathy ( n=41), cochleopathy ( n=28) and sudden deafness ( n=12). LFS provoked unsteadiness in posturography without PLF in 24 patients with Meniere's disease, in 5 patients with vestibulopathy, in 3 patients with cochleopathy and in 2 patients with sudden deafness. In one patient, tympanoscopy revealed fistula in the round window membrane that was covered with a fibrinous layer. In four cases there was abnormal light reflex in the round window but without PLF. In eight cases, Hennebert's sign was present with nystagmus, without PLF. We conclude that pathological responses to the LFS test in posturography can also be encountered in other inner ear diseases without PLF.

メニエール病における偽ろう孔症状について 外耳道圧負荷平衡機能検査による検討

It is well known that pressure changes applied to the external auditory canal produce eye movements in some cases of Meniere, s disease (Hennebert's sign or pseudofistula sign). Theire influence on posture was studied by measuring the change in lateral body sway when a quantitatively regulated pressure load was applied to the external ear canal (PLT). A total of 82 subjects were sutudied by this method. Of these 47 suffered from Meniere's disease and 15 from sud-den profound sensorineural hearing loss; 20 normal subjects were served as controls. Our test results showed that 26 (55%) of the 47 patients with Meniere's disease showed a difference in PLT thres-hold between the right and left ears and only 3 (20%) of the 15 patients suffering from sudden profound hearing loss and 1 (5%) of the normal subjects exibited such a differece. In the patients with Meniere's disease, a statistically significant correlation was found between the PLT and low tone hearing level and also between the PLT and the flluctuation of hearing in the low tone range. The presence of a positive PLT provides usefull information not only about the site of the lesion but also about the stage of Meniere's disease.