Toward Optimizing cVEMP: 2,000-Hz Tone Bursts Improve the Detection of Superior Canal Dehiscence.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) test measures saccular and inferior vestibular nerve function. The cVEMP can be elicited with different frequency stimuli and interpreted using a variety of metrics. Patients with superior semicircular canal dehiscence (SCD) syndrome generally have lower cVEMP thresholds and larger amplitudes, although there is overlap with healthy subjects. The aim of this study was to evaluate which metric and frequency best differentiate healthy ears from SCD ears using cVEMP. METHODS: Twenty-one patients with SCD and 23 age-matched controls were prospectively included and underwent cVEMP testing at 500, 750, 1,000 and 2,000 Hz. Sound level functions were obtained at all frequencies to acquire threshold and to calculate normalized peak-to-peak amplitude (VEMPn) and VEMP inhibition depth (VEMPid). Third window indicator (TWI) metrics were calculated by subtracting the 250-Hz air-bone gap from the ipsilateral cVEMP threshold at each frequency. Ears of SCD patients were divided into three groups based on CT imaging: dehiscent, thin or unaffected. The ears of healthy age-matched control subjects constituted a fourth group. RESULTS: Comparing metrics at all frequencies revealed that 2,000-Hz stimuli were most effective in differentiating SCD from normal ears. ROC analysis indicated that for both 2,000-Hz cVEMP threshold and for 2,000-Hz TWI, 100% specificity could be achieved with a sensitivity of 92.0%. With 2,000-Hz VEMPn and VEMPid at the highest sound level, 100% specificity could be achieved with a sensitivity of 96.0%. CONCLUSION: The best diagnostic accuracy of cVEMP in SCD patients can be achieved with 2,000-Hz tone burst stimuli, regardless of which metric is used.

The Gopen-Yang Superior Semicircular Canal Dehiscence Questionnaire: development and validation of a clinical questionnaire to assess subjective symptoms in patients undergoing surgical repair of superior semicircular canal dehiscence.

OBJECTIVE: To characterise subjective symptoms in patients undergoing surgical repair of superior semicircular canal dehiscence. METHODS: Questionnaires assessing symptom severity and impact on function and quality of life were administered to patients before superior semicircular canal dehiscence surgery, between June 2011 and March 2016. Questionnaire sections included general quality of life, internal amplified sounds, dizziness and tinnitus, with scores of 0-100 points. RESULTS: Twenty-three patients completed the questionnaire before surgery. Section scores (mean±standard deviation) were: 38.2 ± 25.2 for general quality of life, 52.5 ± 23.9 for internal amplified sounds, 35.1 ± 28.8 for dizziness, 33.3 ± 30.7 for tinnitus, and 39.8 ± 22.2 for the composite score. Cronbach's α statistic averaged 0.93 (range, 0.84-0.97) across section scores, and 0.83 for the composite score. CONCLUSION: The Gopen-Yang Superior Semicircular Canal Dehiscence Questionnaire provides a holistic, patient-centred characterisation of superior semicircular canal dehiscence symptoms. Internal consistency analysis validated the questionnaire and provided a quantitative framework for further optimisation in the clinical setting.

Upbeat nystagmus after bilateral superior canal plugging: A peripheral cause of vertical nystagmus.

Vertical nystagmus without a torsional component is generally considered a finding indicative of central nervous system pathology. We report two cases of purely upbeat nystagmus elicited with mastoid vibration after bilateral superior canal plugging, to highlight the vestibular pathophysiology involved in this unusual peripheral cause for upbeat nystagmus. Laryngoscope, 127:1698-1700, 2017.

Vestibular evoked myogenic potentials in patients with ankylosing spondylitis.

Ankylosing spondylitis (AS) is a chronic systemic inflammatory disease with unknown etiology. Although sacroiliac joint involvement is the classic sign along with the formed immune mediators, it may result in immune-mediated inner ear disease and may cause damage to the audiovestibular system. Vestibular evoked myogenic potentials (VEMP) is a clinical reflex test used in the diagnosis of vestibular diseases and is performed by recording and evaluating the muscle potentials resulting from the stimulation of the vestibular system with different stimuli. The aim of this study is to evaluate the cervical VEMP test results in AS patients without vestibular symptoms. Thirty-three patients with AS and a control group of 30 healthy volunteers with similar demographic characteristics were evaluated in the study. VEMP wave latency, P13-N23 wave amplitude, and VEMP asymmetry ratio (VAR) values were compared between the groups. The relationship between clinical and laboratory findings of the AS patients and VEMP data were also investigated. Compared with healthy people, this study shows the response rate of patients with ankylosing spondylitis was reduced in the VEMP test, and P13-N23 wave amplitude showed a decrease in AS patients who had VEMP response (p < 0.001). There was no correlation between the clinical and laboratory findings and VEMP findings in patients with ankylosing spondylitis. The data obtained from this study suggest that AS may lead to decreased sensitivity of the vestibular system.

[The analysis of acoustic activity unsynchronized with the stimulus with the use of TEOAE]. / Analiz akusticheskoi aktivnosti, ne sinkhronizirovannoi so stimulom, pri registratsii zaderzhannoi vyzvannoi otoakusticheskoi emissii.

The presence of ambient noise or the troubled child's behavior during the registration of transiently induced optoacoustic emission (TEOAE) can lead to false positive results at the first stage of hearing screening. The aim of this study is to evaluate the effectiveness of the noise reduction method based on weighted averaging of the data in the frequency and time slot, to check TEOAE, and to improve the effectiveness of universal hearing screening. Particular attention is paid to the analysis of the origin of noise recorded during the UAE. PATIENTS AND METHODS: We have developed a method of noise reduction, based on weighted averaging of the data in the frequency and time cells. In this study, 20 people were examined (9 adults and 11 children). TEOAE was performed using the probe of the ILO 288 instrument manufactured by Otodinamics Ltd. that was connected to the E-MU 0204 sound card. The probe microphone was connected through the preamplifier to one input channel of the sound card, and the second channel was used to receive a signal from the ECG and the respiration recorder. Thereafter, the data thus obtained were processed by several methods, including the method of averaging in the frequency and time slot designed to average a set of implementations that contain a noisy signal. The comparison of the results of the use of noise suppression method based on weighted averaging of the data in the frequency and time cells showed that it increases the accuracy of TEOAE. MAIN RESULTS: Our work suggests that the use of the noise suppression method based on weighted averaging of the data in the frequency and time cells, increases the accuracy of TEOAE. Similar results were obtained by the weighted averaging method.

An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies.

The n10 component of the ocular vestibular evoked myogenic potential (oVEMP) to sound and vibration stimuli is a crossed response that has enhanced amplitude and decreased threshold in patients with CT-verified superior semicircular canal dehiscence (SSCD). However, demonstrating enhanced VEMP amplitude and reduced VEMP thresholds requires multiple trials and can be very time consuming and tiring for patients, so a specific indicator of probable SCD that is fast and not tiring would be preferred. Here we report a 1-trial indicator: that the oVEMP n10 in response to a very high frequency stimulus (4000 Hz), either air-conducted sound (ACS) or bone conducted vibration (BCV), is such a fast indicator of probable SCD. In 22 healthy subjects, oVEMP n10 at 4000 Hz was not detectable; however, in all 22 CT-verified SSCD patients tested, oVEMP n10 responses were clearly present to 4000 Hz to either ACS or BCV stimuli.

[Vertigo induced by noise or pressure to the left ear]. / Schwindel durch Lärm oder Druck auf dem linken Ohr.

A 49-year-old male patient presented with recently acquired vertigo induced by noise or pressure to the left ear. With appropriate stimulation, oscillopsia with a rotatory component could be reproduced in videooculography. Cervical vestibular evoked myogenic potentials (VEMP) showed increased amplitudes and a lowered threshold on the left side. CT of the petrous bone showed a bony dehiscence of the left superior semicircular canal. Conservative therapy was initiated as a first step.

Enhanced otolithic function in semicircular canal dehiscence.

The enhanced sound- and vibration-induced vestibular evoked myogenic potentials (VEMPs) and their lower threshold in patients with a thinning of the bony wall of the superior semicircular canal (superior canal dehiscence, SCD) have been interpreted as being due to the dehiscence allowing sound and vibration to activate, unusually, the receptors of the dehiscent semicircular canal. We report a patient with bilateral SCD, as verified by high resolution CT scans, who had bilaterally decreased superior semicircular canal function, as shown by rotational tests of canal function. This patient also showed enhanced VEMPs and reduced thresholds. We conclude that in this patient the enhanced VEMP responses are thus probably due to enhanced otolithic stimulation by sound and vibration after dehiscence.

Use of the loud sound stimulation test in diagnosis of semicircular canal dehiscence syndrome.

Semicircular canal dehiscence (SCD) syndrome is rare, and its diagnosis is a significant challenge in clinical practice. Our aim was to explore application of the loud sound stimulation test for diagnosing SCD syndrome. Eight cases of superior semicircular canal dehiscence (SSCD), among them two patients had bilateral dehiscences and one case of lateral semicircular canal dehiscence (LSCD). A total of 11 dehiscences were studied retrospectively. Loud sounds (pure tones, 100 dB, 110 dB nHL) at frequencies of 500, 1,000, and 2,000 Hz were used to stimulate both ears for 5 s. A temporal bone computed tomography (CT) scan with semicircular canal reconstruction was performed in all patients. Vertigo was present in seven of nine cases following loud sound stimulation. In addition, the patient with LSCD demonstrated horizontal eye movement following loud sound stimulation, whereas six patients with SSCD showed rotational eye movement. Among them, two patients with bilateral superior canal dehiscence showed a positive response to the loud sound stimulation in only one ear. The diagnoses of all patients were confirmed with a high-resolution temporal bone CT with corresponding multi-planar reconstruction of the affected semicircular canals with various size dehiscences. We conclude that the characteristic eye movement following loud sound stimulation is valuable for diagnosing SCD syndrome. In addition, the loud sound stimulation test has unique advantages, especially for confirming the affected ear and the corresponding semicircular canal.

Benign paroxysmal positional vertigo secondary to inner ear disease.

OBJECTIVES: To contrast clinical characteristics of secondary benign paroxysmal positional vertigo (s-BPPV) with idiopathic BPPV (i-BPPV). STUDY DESIGN: Case series with chart review. SETTING: University hospital. SUBJECTS AND METHODS: A total of 718 patients whose medical records were reviewed had BPPV. Sixty-nine patients had existing inner ear diseases and thus were considered to have s-BPPV. We reviewed demographics, concurrent causative disorders, involved area, and response to particle repositioning maneuvers for these s-BPPV patients in comparison with i-BPPV subjects. RESULTS: Female subjects with i-BPPV outnumbered male subjects by a ratio of 1.9:1, but there was no significant sex difference for s-BPPV patients. The diseases associated with s-BPPV were idiopathic sudden sensory hearing loss (ISSHL, 50.7%), Ménière's disease (MD, 28.9%) and unilateral vestibulopathy such as acute vestibular neuronitis and herpes zoster oticus (20.2%). The posterior canal was most commonly involved in both i-BPPV and s-BPPV. The horizontal canal was the second most common, followed by multi-canal involvement. However, MD-associated BPPV most commonly involved the lateral canal. The mean durations of treatment for i-BPPV and s-BPPV were 2.28 and 4.87 days, respectively. The mean duration of treatment was 6.28 days for ISSHL with BPPV, 5.07 days for BPPV with unilateral vestibulopathy, and 2.28 days for BPPV with MD. CONCLUSION: The mean duration of treatment for BPPV with ISSHL or unilateral vestibulopathy was longer than for other groups. The different pathophysiologies of s-BPPV associated with different inner ear diseases may explain its diverse clinical features and courses.

Sound-induced vertigo due to bone dehiscence of the lateral semicircular canal.

Dehiscence of the lateral semicircular canal (LSCD) has been reported much but mainly in association with cholesteatoma and canal wall down mastoidectomy, while idiopathic LSCD was rarely reported. Bassim reported one case with lateral semicircular canal dehiscence, but presented no vestibular or auditory symptoms. The patient in this study complained significant sound-induced vertigo and autophony in his right ear. The axis of nystagmus was orthogonal to the lateral semicircular canal, and no torsional or vertical motions were observed, so pathology of the lateral semicircular canal was preferentially considered. Benign paroxysmal positional vertigo was excluded since vertigo attacks had no relation to the change of head position. The dehiscence of the right lateral semicircular canal was then confirmed through the high-resolution temporal bone computer tomography scan and the reconstructed images. The cause of the LSCD is poorly understood, since no history of head trauma, otological infection or surgery was documented.

Testing vestibular-evoked myogenic potentials with 90-dB clicks is effective in the diagnosis of superior canal dehiscence syndrome.

Vestibular-evoked myogenic potentials (VEMP) in response to 90-dB-nHL clicks were studied in 20 patients (22 ears) with superior canal dehiscence syndrome. Their amplitude was compared to the VEMP from the 'unaffected' ears of 113 patients using the same stimulus level. The 113 control subjects were those from a previous study on 1,000 patients who had had large VEMP amplitudes in response to 500-Hz 129-dB-SPL tone bursts, and, because of this, had been tested with 90-dB-nHL clicks (which are a much weaker sound stimulus than our routine 500-Hz tone burst). It was found that 90-dB-nHL clicks clearly distinguished patients with vestibular hypersensitivity to sounds. In patients, the VEMP amplitude was usually larger than the simultaneously recorded background electromyographic activity (i.e. 'corrected' amplitude >1), whereas this was not the case for the controls. Consequently, it is suggested that 90-dB-nHL clicks can be used to screen for vestibular hypersensitivity to sounds. This finding has clinical implications for patients with suspected Tullio phenomenon because the definitive VEMP test for this (i.e. estimation of VEMP threshold) is not only time-consuming, but there is also difficulty related to the low signal-to-noise ratio close to the threshold.

Assessment of changes in cochlear function with pneumolabyrinth after middle ear trauma.

OBJECTIVE: To describe a case of pneumolabyrinth secondary to tympanic membrane/ossicular trauma and the subsequent recovery of sensorineural hearing loss managed with conservative measures. PATIENTS: A 15-year-old boy presented to an outside hospital with signs and symptoms of acute hearing loss, vertigo, and tinnitus after penetrating injury to his right tympanic membrane. In addition, computed tomography demonstrated air density within the vestibule. INTERVENTIONS: The patient was managed conservatively with bed rest, avoidance of straining, corticosteroids, and antibiotics. MAIN OUTCOME MEASURES: Computed tomography, audiologic testing. RESULTS: Patient recovered near-normal hearing subjectively. There was closure of the air-bone gap (

Vestibular evoked myogenic potentials.

PURPOSE OF REVIEW: Vestibular evoked myogenic potential testing is the only clinically feasible way to measure function of the saccule. Interest in this test has exploded in recent years because of its potential utility in diagnosing third-window disorders and in diagnosing and monitoring Ménière's disease. RECENT FINDINGS: Recent literature on vestibular evoked myogenic potential covers a wide range of topics. Review articles, method articles and many case reports and uncontrolled case series are seeking new applications for vestibular evoked myogenic potential in diagnosis and monitoring of neurotologic disease, and in shedding light on inner ear diseases by mapping anatomic sites of involvement. The most informative work is still in the areas of superior semicircular canal dehiscence and in Ménière's disease. Also, many aspects of vestibular evoked myogenic potential and its use have not yet been adequately studied or described. SUMMARY: Vestibular evoked myogenic potential is a new test of saccular and inferior vestibular nerve integrity. It holds great promise for diagnosing and monitoring Ménière's disease and some other neurotologic disorders. It is still an evolving field, however. The methods, equipment, and applications for vestibular evoked myogenic potential testing are not yet standardized. It is not yet time for this test to be widely applied, but that time is not far off.

[Vestibular evoked myogenic potentials]. / Vestibulär evozierte myogene Potenziale.

The recording of vestibular evoked myogenic potentials is a relatively new neuro-otologic method which gives specific information about the function of the sacculus and the inferior vestibular nerve on each side separately. The main indications for this method are vestibular schwannoma and fistula of the labyrinth, involvement of the sacculus or the inferior vestibular nerve in Menière's disease and vestibular neuritis, vertigo of unknown etiology, and forensic questions.

Superior semicircular canal dehiscence: a series of 13 cases.

Increasingly more detailed imaging techniques have recently highlighted the frequent occurrence of bony labyrinthine dehiscence. Among them, superior canal dehiscence (SCD) has been described in a number of cases presenting different features. Here, we report a series of 13 cases, in which the detection of vestibular evoked myogenic potentials (VEMPs) in response to stimuli of abnormally low intensity as compared to normal responses led us to suspect the presence of a 'third window effect'. An accurate HRCT investigation allowed the diagnosis of SCD. Anamnestic and symptomatologic differences seem difficult to explain, although in our opinion a dural rupture could be at the basis of the onset of pathologic manifestations after many years of silence of a probably malformative condition.

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.

Embolic inner ear decompression illness: correlation with a right-to-left shunt.

OBJECTIVES/HYPOTHESIS: Inner ear decompression illness is thought to be a rare phenomenon in recreational divers, isolated signs and symptoms of inner ear dysfunction usually being attributed to inner ear barotrauma. STUDY DESIGN: We present 11 cases of inner ear dysfunction in nine divers with inner ear decompression illness. RESULTS: All nine divers had significant right-to-left shunt as diagnosed by transcranial Doppler sonography. CONCLUSIONS: The authors thought that mechanism of causation in these cases may have been intravascular bubble emboli and that inner ear decompression illness may be more common among recreational divers than currently recognized. Failure to treat inner ear decompression illness with recompression therapy can result in permanent disability. Because the differential diagnosis between inner ear barotrauma and inner ear decompression illness can be impossible, the authors suggested that divers who present with inner ear symptoms following a dive should have recompression immediately after having undergone bilateral paracentesis.

The click-evoked vestibulo-ocular reflex in superior semicircular canal dehiscence.

The authors studied eye movement responses to loud (110dB) clicks in 4 patients with Tullio effect due to superior semicircular canal dehiscence and in 9 normal subjects, by averaging the electro-oculogram. All 4 patients had small (0.1-0.3 deg) but easily reproducible vertical vestibulo-ocular reflex eye movement responses to the clicks. Normal subjects had responses that were at least 10 times smaller. The click-evoked vestibulo-ocular reflex test is a simple, robust way to screen dizzy patients for symptomatic superior semicircular dehiscence.

[The influence of the middle ear status on the measurement of evoked otoacoustic emissions (DPOAE)]. / Wplyw stanu ucha srodkowego na wywolane emisje otoakustyczne (DPOAE).

The aim of this study was to evaluate the influence of the middle ear status on detection and amplitude of otoacoustic emissions-DPOAE. DPOAE were detected in all healthy ears and their amplitude was between 11.4 and 22.4 dB SPL. Responses at frequencies of 0.5 and 0.75 kHz were not detected in the group of children with Eustachian tube dysfunction in 60% and 27% of ears respectively. The absence of DPOAE responses in the group of children with B type tympanometry was found in 3/4 of the investigated ears at frequencies of 0.5 and 0.75 kHz and in over 50% of ears at frequencies of 1.0 and 8.0 kHz. No influence of cochlear reserve on detection and amplitude of DPOAE was found in the studied group. Conductive hearing loss associated with a presence of negative pressure and effusion in the middle ear inhibits DPOAE detection and causes decrease of otoacoustic emission amplitude.