[Method of registration of vestibular myogenic evoked potentials in the diagnosis of diseases of the inner ear]. / Metod registratsii vestibulyarnykh miogennykh vyzvannykh potentsialov v diagnostike zabolevanii vnutrennego ukha.

The literature review is devoted to the practical application of the method of recording vestibular evoked myogenic potentials (VEMPs) in the diagnosis of the inner ear diseases: superior semicircular canal dehiscence syndrome, Meniere's disease, benign paroxysmal positional vertigo (BPPV), vestibular neuritis. Registration of VMEP is an electrophysiological research method that allows to assess objectively the functional state of the otolith receptors (sacculus and utriculus) and their pathways, which expands the diagnostic capabilities in diagnosis of the inner ear diseases.

[A case of hearing preservation during surgical treatment of distributed fistula labyrinth in a patient with chronic purulent means otitis and cholesteatoma]. / Sluchai sokhraneniya slukha pri khirurgicheskom lechenii rasprostranennoi fistuly labirinta u bol'noi s khronicheskim gnoinym srednim otitom i kholesteatomoi.

The article provides a brief overview of the epidemiology, diagnosis, clinical manifestations and surgical methods of treatment of labyrinthine fistulas (LF) in patients with chronic suppurative otitis media (CSOM) with cholesteatoma. The efficacy of various LF treatment techniques, their complications, and the principles of prevention of cochleovestibular disorders during surgical treatment are described. The prognostic criteria for the deterioration of bone conduction thresholds during LF surgery, which include a large LF size, the opening of the membranous labyrinth, and the extent of damage to the structures of the inner ear, are presented. A clinical case of surgical treatment of widespread LF in CSOM with cholesteatoma is presented, which proves the possibility of preserving the auditory and vestibular functions while observing the stages of sanitation, manipulations on the LF and obliteration of semicircular canals defects with auto tissues. Combined surgery of such a plan must certainly be accompanied by local instillation of solutions of hormonal preparations in case of a deficiency of perilymphatic fluid in the labyrinth and postoperative antibacterial and hormonal therapy.

[Improvement of cochleovestibular function in chronic non-inflammatory perilymphatic fistulas of the labyrinth windows: cases from practice]. / Vozmozhnosti vosstanovleniya slukha pri khronicheskikh nevospalitel'nykh perilimfaticheskikh fistulakh okon labirinta: sluchai iz praktiki.

After a brief discussion of the problem of idiopathic sensorineural hearing loss and perilymphatic fistulas of the labyrinth windows, audiometric tests are identified that are informative for the preoperative diagnosis of the latter. Taking into account the own results of surgical treatment, attention is drawn to the feasibility of a wider use of exploratory tympanotomy in cases of suspected occurrence of perilymphatic fistulas of the labyrinth windows.

[Peculiarities of differential diagnostics of perilymphatic fistulas of the labyrinth windows of non-inflammatory genesis]. / Osobennosti differentsial'noi diagnostiki perilimfaticheskikh fistul okon labirinta nevospalitel'nogo geneza.

THE PURPOSE OF THE STUDY: To determine the value of the results of various audiological and vestibulometric studies for the differential diagnosis of non-inflammatory perilymphatic fistulas of the labyrinth windows (PFOLW). MATERIAL AND METHODS: The retrospective and prospective analysis of the informative value of the results of audiological and vestibulometric studies was carried out in 124 people with different combinations of cochleovestibular complaints, who had different pathology of the inner and middle ear, with different terms of the disease - from several days to 30 years. To assess the informativeness of the applied testing, the following operational characteristics were determined: general sensitivity (Se), specificity (Sp) and the prognostic value of a positive result (PPV=positive predictive value). A pair-by-pair comparison of the prognostic value of a positive result of vestibulometric and audiometric tests was performed using the Pearson criterion χ2 and the exact Fisher criterion. RESULTS: It is shown that none of the tests used has 100% reliability, but the consistent application of some of them can successfully improve the diagnosis of idiopathic PFOL. When comparing the results of a number of tests, such as the Fukuda walking test and / or the Babinsky-Weil walking test, audiometric tests with head clone and hyperventilation, the test of fluid injection into the external auditory canal and the test of J. Frasser & L. Flood, it was found that the average prognostic value of a positive result was 87.3%, which is statistically significantly higher than the average result (47.9%) for other samples. CONCLUSIONS: Correlations of the results of simple vestibulometric and audiometric tests allow us to recommend them to improve the diagnosis of idiopathic perilymphatic fistulas of the labyrinth windows. The possibilities of modern electrophysiological audiological research methods are subject to further study.

Middle-ear cholesteatoma co-existing with labyrinthine fistula and vestibular schwannoma.

BACKGROUND: Many conditions, among them vestibular schwannoma and middle ear cholesteatoma with lateral semicircular canal destruction, may be associated with asymmetrical sensorineural hearing loss (SNHL) and vertigo. However, the probability that these two distinct disease entities causing the same symptoms occur in a single patient is very low, approximately 1 per 28 billion per 1 year. METHODS: We present the case of a 40-year-old male admitted to our clinic because of chronic middle ear inflammation with concomitant tinnitus vertigo, and deafness in the right ear. The patient was diagnosed with lateral semicircular canal fistula caused by middle-ear cholesteatoma and concomitant vestibular schwannoma. Canal wall-down surgery was carried out to remove the cholesteatoma, followed by gamma knife radiosurgery for the vestibular schwannoma. RESULTS: Vertigo and tinnitus resolved within 3 days after the ear surgery, and gamma knife treatment resulted in the complete involution of the vestibular schwannoma. The patient presented with completely dry middle-ear cavity and no recurrence of the cholesteatoma was observed during a 3-year follow-up. CONCLUSION: As the hereby reported condition is very rare, the results cannot be compared with any similar report published previously. Nevertheless, based on the outcome, the treatment strategy seems to be both reasonable and effective.

Can miR-34a be suitable for monitoring sensorineural hearing loss in patients with mitochondrial disease? A case series.

Purpose: We aimed at evaluating the feasibility of using MicroRNA (miR)-34a and miR-29b to detect inner ear damage in patients with mitochondrial disease (MD) and sensorineural hearing loss (SNHL).Material and Methods: Three patients with MD and SNHL and seven healthy control subjects were included in this case series. MD patients underwent pure tone audiometry (PTA), distortion product otoacoustic emission (DPOAE) and auditory brain response tests to investigate the specific cochlear and retrocochlear functions; control patients underwent PTA. MiR-34a and miR-29b were extracted from blood in all subjects included in the study. The expression of miR-34a and miR-29b in MD patients and healthy controls were statistically compared, then the expression of these two miRs was compared with DPOAE values.Results: In MD patients, miR-34a was significantly up-regulated compared to healthy controls; miR-34a and DPOAEs were negatively correlated. Conversely, miR-29b was up-regulated only in the youngest patient who suffered from the mildest forms of MD and SNHL, and negatively correlated with DPOAEs.Conclusion: In MD patients, miR-34a and miR-29b might be a marker of inner ear damage and early damage, respectively. Additional studies on larger samples are necessary to confirm these preliminary results.

Isolated horizontal canal hypofunction differentiating a canalith jam from an acute peripheral vestibular loss.

OBJECTIVES: To describe a unique case of acute vertigo presenting with spontaneous horizontal nystagmus (SHN) and a clinical picture consistent with right acute peripheral vestibular loss (APVL) in which an isolated hypofunction of a horizontal semicircular canal (HSC) permitted to detect a spontaneous canalith jam and treat the patient accordingly. METHODS: Case report and literature review. RESULTS: A 74-year old woman presented with acute vertigo, left-beating SHN and a clinical picture consistent with right APVL. Nevertheless, vestibular evoked myogenic potentials were normal with symmetrical amplitudes and the video head impulse test (vHIT) revealed an isolated hypofunction of the right HSC. After repeated head shakings, the supine roll test evoked bilaterally a positioning paroxysmal geotropic horizontal nystagmus suggesting benign paroxysmal positional vertigo involving the non-ampullated arm of the right HSC. vHIT and caloric testing confirmed restitution of HSC function after repositioning maneuvers. CONCLUSIONS: In case of acute vertigo with SHN, a complete functional assessment of vestibular receptors and afferents should always be given in order to avoid misdiagnosis. Canalith jam should be considered in case of spontaneous nystagmus and isolated canal hypofunction.

Recovery of ocular and cervical vestibular evoked myogenic potentials after treatment of inner ear diseases.

Purpose: This study aimed to assess the clinical value of ocular vestibular evoked myogenic potential (oVEMP) and cervical vestibular evoked myogenic potential (cVEMP) for monitoring the rehabilitation of vestibular function in patients treated for peripheral vertigo. Materials and methods: Fifteen patients who had been diagnosed with peripheral vertigo and showed no VEMP response on the affected side but exhibited symptom alleviation and VEMP responses after therapies were retrospectively enrolled. We analyzed the restoration and parameters of their VEMP response. Results: After treatment, six patients with sudden sensorineural hearing loss showed VEMP recovery, including two with both oVEMP and cVEMP recovery, three with oVEMP recovery only, and one with cVEMP recovery only. Two patients with Meniere's disease (MD) showed cVEMP recovery, while the other three MD patients showed oVEMP recovery. Three patients with herpes zoster oticus exhibited cVEMP recovery. One patient with vestibular neuritis exhibited cVEMP recovery. Among the patients with cVEMP and/or oVEMP restoration, most patients presented normal VEMP parameters; however, some patients showed abnormal VEMP parameters after treatment. Conclusion: Combined oVEMP and cVEMP are objective tools for assessing vestibular otolithic end organ function during dynamic functional recovery from vestibular diseases.

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.

MRI screening of the internal auditory canal: Is gadolinium necessary to detect intralabyrinthine schwannomas?

OBJECTIVE: Non-contrast MRI of the internal auditory canal (IAC) using high-resolution T2WI (T2 weighted image) has been proposed as the primary screening study in patients with sudden or asymmetric sensorineural hearing loss (ASNHL). However, there are concerns that non-contrast MRI may not detect labyrinthine pathology, specifically intralabyrinthine schwannomas (ILSs). The purpose of this study was to determine if non-contrast high-resolution T2WI alone are adequate to exclude these uncommon intralabyrinthine tumors. METHODS: 31 patients with ILSs and 36 patients without inner ear pathology that had dedicated MRI of the IAC performed with both non-contrast T2WI and post-contrast T1WI (T1 weighted image) were identified. Three board-certified neuroradiologists reviewed only the T2WI from these 67 cases. When an ILS was identified, its location and size were recorded. Sensitivity, specificity, and accuracy were calculated using the post-contrast T1WI as the "gold standard." A consensus review of cases with discordant results was conducted. RESULTS: The sensitivity, specificity, and accuracy were 1.0, 1.0, and 1.0 for Observer 1; 0.84, 1.0, and 0.96 for Observer 2; 0.90, 1.0, and 0.98 for Observer 3. The 5 ILSs with discordant results were correctly identified upon consensus review. The median size of the ILSs was 4.4mm (±2.9mm) and most (18/31) were intracochlear in location. CONCLUSION: Non-contrast high-resolution T2WI alone can detect ILSs with 84-100% sensitivity, suggesting that gadolinium may be unnecessary to exclude ILSs on screening MRI. These findings have implications for reducing cost, time, and adverse events associated with gadolinium administration in patients presenting with sudden or ASNHL. LEVEL OF EVIDENCE: 4.

Superior canal dehiscence syndrome : Diagnosis with vestibular evoked myogenic potentials and fremitus nystagmus.

BACKGROUND: Superior canal dehiscence syndrome (SCDS) is a relatively rare neurotological disorder that is characterized by a heterogeneous clinical picture. Recently, vestibular evoked myogenic potential (VEMP) measurement was established for the diagnosis of SCDS. In the present study, a case series of patients with SCDS were analyzed, with a focus on VEMP. METHODS: Four patients with SCDS were prospectively examined with ocular VEMP (oVEMP) and cervical VEMP (cVEMP). The clinical features and the standard audiovestibular test battery results are summarized and analyzed. The diagnostic accuracy of VEMP testing is evaluated. RESULTS: The increased oVEMP amplitudes had a specificity of 100% in this patient population. All patients had normal caloric function and head impulse testing. The Tullio sign was observed in two patients. Three patients had autophony. The air-bone gap was not greater than 10 dB in any of the patients. Two patients had marked fremitus nystagmus. All patients had a bony dehiscence of the superior semicircular canal on computed tomography imaging. CONCLUSION: The subjective and clinical features in this case series of SCDS patients were heterogeneous. However, objective oVEMP testing had the highest diagnostic value. Furthermore, we describe a new diagnostic clinical sign: fremitus nystagmus.

[Superior canal dehiscence syndrome : Diagnosis with vestibular evoked myogenic potentials and fremitus nystagmus. German version]. / Dehiszenzsyndrom des oberen Bogengangs : Diagnostik mit vestibulär evozierten myogenen Potenzialen und Fremitusnystagmus.

BACKGROUND: Superior canal dehiscence syndrome (SCDS) is a relatively rare neurotological disorder that is characterized by a heterogeneous clinical picture. Recently, vestibular evoked myogenic potential (VEMP) measurementwas established for the diagnosis of SCDS. In the present study, a case series of patients with SCDS were analyzed, with a focus on VEMP. METHODS: Four patients with SCDS were prospectively examined with ocular VEMP (oVEMP) and cervical VEMP (cVEMP). The clinical features and the standard audiovestibular test battery results are summarized and analyzed. The diagnostic accuracy of VEMP testing is evaluated. RESULTS: The increased oVEMP amplitudes had a specificity of 100% in this patient population. All patients had normal caloric function and head impulse testing. The Tullio sign was observed in two patients. Three patients had autophony. The airbone gap was not greater than 10 dB in any of the patients. Two patients had marked fremitus nystagmus. All patients had a bony dehiscence of the superior semicircular canal on computed tomography imaging. CONCLUSION: The subjective and clinical features in this case series of SCDS patients were heterogeneous. However, objective oVEMP testing had the highest diagnostic value. Furthermore, we describe a new diagnostic clinical sign: fremitus nystagmus.

[The peri-lymphatic labyrinthine fistula in a child]. / Perilimfaticheskaia fistula labirinta u rebenka.

The objective of the present work was to describe the successful surgical treatment of the peri-lymphatic round window fistula of the labyrinth in the 9 year old child. The authors briefly mention the difficulties of diagnostics of the peri-lymphatic labyrinthine fistulas in the children and the signs suggesting their presence with special reference to the optimal strategies for their treatment.

Intralabyrinthine schwannomas : Surgical management and hearing rehabilitation with cochlear implants.

Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a "wait-and-test-and-scan" strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine-transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors' opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a "wait-and-test-and-scan" strategy.

Posterior semicircular canal dehiscence secondary to jugular enlargement.

Semicircular canal dehiscence is an important entity often presenting with symptoms of noise or pressure induced vertigo, autophony, aural fullness and conductive hearing loss. Due to its varied presentation and mimic of other otologic conditions its consideration is of increased importance. Within we report a case of right sided posterior semicircular canal dehiscence secondary to an enlarged and high jugular bulb. The results of this observation indicate a need for patients with auditory symptoms and high riding jugular bulbs to be evaluated for possible posterior semicircular canal dehiscence.

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.

Temporal High-Resolution Computed Tomography and Magnetic Resonance Imaging of Congenital Inner Ear Anomalies in Children.

Imaging plays an important role in determining indications of cochlear implantation and choosing candidates for the procedure in children. Temporal high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) can display precisely the complex anatomic structure of inner ear. Although HRCT permits detailed imaging of bony structures, MRI gives valuable information about membranous labyrinth, internal acoustic canal, and vestibulocochlear nerve. Magnetic resonance imaging examination of the brain should be performed at the same time to evaluate any coexistent brain parenchymal abnormality. These imaging modalities are complementary methods in evaluating congenital inner ear anomalies. The aim of this pictorial essay is to reviewing temporal HRCT and MRI findings of congenital inner ear anomalies.

Anterior Inferior cerebellar artery infarction misdiagnosed as inner ear disease.

Anterior Inferior cerebellar artery infarction misdiagnosed as inner ear disease. OBJECTIVE: The clinical >resentation of anterior inferior cerebellar artery (AICA) infarction may mimic that of inner ear disease. lethodology: This report presents two patients with cerebellar artery infarction initially misdiagnosed with inner ear lisease. ase Report: Both the patients presented with sudden hearing loss and vertigo. The patient in case 1 was initially liagnosed with idiopathic sudden sensorineural hearing loss. The patient in case 2 presented with 17 days of vertigo and iearing loss. Both were correctly diagnosed with AICA infarction after performing magnetic resonance imaging. esults and Conclusions: We differentiated AICA from inner ear disease based on the variability in degree and frequency ange of hearing loss, the duration of vertigo, and the manifestation of nystagmus. Because cases of AICA infarction and nner ear disease may present with si'milar symptoms, a detailed examination including clinical course assessments, aboratory findings, and neurological imaging is essential for appropriate diagnosis and treatment.