[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.

The role of the stria vascularis in neglected otologic disease.

The stria vascularis (SV) has been shown to play a critical role in the pathogenesis of many diseases associated with sensorineural hearing loss (SNHL), including age-related hearing loss (ARHL), noise-induced hearing loss (NIHL), hereditary hearing loss (HHL), and drug-induced hearing loss (DIHL), among others. There are a number of other disorders of hearing loss that may be relatively neglected due to being underrecognized, poorly understood, lacking robust diagnostic criteria or effective treatments. A few examples of these diseases include autoimmune inner ear disease (AIED) and/or autoinflammatory inner ear disease (AID), Meniere's disease (MD), sudden sensorineural hearing loss (SSNHL), and cytomegalovirus (CMV)-related hearing loss (CRHL). Although these diseases may often differ in etiology, there have been recent studies that support the involvement of the SV in the pathogenesis of many of these disorders. We strive to highlight a few prominent examples of these frequently neglected otologic diseases and illustrate the relevance of understanding SV composition, structure and function with regards to these disease processes. In this study, we review the physiology of the SV, lay out the importance of these neglected otologic diseases, highlight the current literature regarding the role of the SV in these disorders, and discuss the current strategies, both approved and investigational, for management of these disorders.

[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.

Extracellular Biomarkers of Inner Ear Disease and Their Potential for Point-of-Care Diagnostics.

Rapid diagnostic testing has become a mainstay of patient care, using easily obtained samples such as blood or urine to facilitate sample analysis at the point-of-care. These tests rely on the detection of disease or organ-specific biomarkers that have been well characterized for a particular disorder. Currently, there is no rapid diagnostic test for hearing loss, which is one of the most prevalent sensory disorders in the world. In this review, potential biomarkers for inner ear-related disorders, their detection, and quantification in bodily fluids are described. The authors discuss lesion-specific changes in cell-free deoxyribonucleic acids (DNAs), micro-ribonucleic acids (microRNAs), proteins, and metabolites, in addition to recent biosensor advances that may facilitate rapid and precise detection of these molecules. Ultimately, these biomarkers may be used to provide accurate diagnostics regarding the site of damage in the inner ear, providing practical information for individualized therapy and assessment of treatment efficacy in the future.

[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.

[Traumatic perilymphatic fistula. Report of a pediatric case]. / Fístula perilinfática de causa traumática. Reporte de un caso pediátrico.

Traumatic perilymphatic fistula is an unusual pathology. Generally caused by pencils, swabs, hair buckles, and matches. Among the most frequent symptoms, patients can present hearing loss and vertigo. Diagnosis requires a complete physical examination that includes otomicroscopy, audiometry and computed tomography of both boulders. Treatment depends on the patient's symptoms. In general, it is conservative at first, but may require surgery. We present a clinical case of a 6-year-old boy with perilymphatic fistula secondary to left ear trauma due to swab, which required surgical treatment.

La fístula perilinfática de causa traumática es una patología poco habitual. En general, es causada por lápices, hisopos, hebillas de pelo y fósforos. Dentro de los síntomas más frecuentes, los pacientes pueden presentar hipoacusia y vértigo. Su diagnóstico requiere un examen físico completo que incluya otomicroscopía, audiometría y tomografía computada de ambos peñascos. El tratamiento depende de la sintomatología del paciente. En general, en un principio, es conservador, pero puede llegar a requerir cirugía. Se presenta un caso clínico de un niño de 6 años con fístula perilinfática secundaria a un traumatismo del oído izquierdo por un hisopo, que requirió tratamiento quirúrgico.

Gray-Scale Inversion on High Resolution Computed Tomography of the Temporal Bone: An Observational Study.

OBJECTIVES: This is a qualitative study to explore the utility of gray-scale inversion or the "invert" function of high-resolution computed tomography (HRCT) scans in the diagnosis of temporal bone anatomy and pathology. METHODS: This is a case series describing an innovative application of an existing image processing tool to visualize temporal bone anatomy and pathology. Illustrative patients at a tertiary referral center with otologic symptoms and findings leading to HRCT scans of the temporal bone were included. Diagnostic HRCT scans were evaluated utilizing the gray-scale inversion function (invert function). RESULTS: Nine illustrative cases which demonstrate conditions such as persistent stapedial artery, membranous stapes footplate, total ossicular prosthesis migration into the vestibule, third window syndrome such as superior semicircular canal dehiscence (SSCD) and cochlea-facial nerve dehiscence, otosclerosis, and ossicular chain discontinuity are included. The enhanced visualization was confirmed surgically in 3 cases, and 1 had physiological confirmation using cervical vestibular evoked myogenic potentials (cVEMP). CONCLUSIONS: Gray-scale inversion can be used to improve visualization of temporal bone anatomy and pathologic changes when diagnoses are in doubt. The invert function is a useful adjunct in the armamentarium of both radiologists and otologists when evaluating HRCT of the temporal bone.

The Pathogenesis of secondary forms of Autoimmune Inner Ear Disease (AIED): advancing beyond the audiogram data.

INTRODUCTION: Autoimmune Inner Ear Disease (AIED) can be of a primary or secondary type. To date, a clear pathogenesis of the disease is still not available. Focusing on the secondary forms of AIED, the aim of this review is to (i) assess and describe the hearing involvement in patients affected by autoimmune diseases, (ii) describe the possible association between clinical features (among serological/laboratory data and disease activity/duration) and hearing impairment, (iii) show evidence connecting the AIED types with various etiopathogenetic mechanisms. AREAS COVERED: A PRISMA-compliant systematic review was performed. Medline, Cochrane, Embase, and Cinahl were searched from 1 January 2015 through to 5 August 2020. Overall, 16 studies (involving 1043 participants) were included in the review. The data in the literature suggested that bilateral mild-to-moderate sensorineural hearing loss is a commonly reported clinical symptom of AIED. EXPERT OPINION: Patients with systemic autoimmune disorders present a cochlear injury which might be associated with the humoral and/or cellular immune response against the inner ear. To date, AIED pathogenesis remains an open issue, due to the rarity of these clinical entities and due to the difficulties in investigating the inner ear immunology, considering the inner ear inaccessibility for tissue sampling.

Meningiomas of the Internal Auditory Canal.

OBJECTIVE: Identify and define specific preoperative and postoperative characteristics of intracanalicular meningiomas (ICMs) in order to improve their diagnosis and management, and to differentiate them from intrameatal vestibular schwannomas (IMVSs). METHODS: Preoperative symptomatology, magnetic resonance imaging (MRI), and postoperative outcomes of 28 ICMs were analyzed. The results were compared to the literature and IMVSs treated by our group. RESULTS: Anacusis and progressive hearing loss were more frequent in the present population than the cases reviewed (P = .0064 and P = .0001, respectively). Hearing loss affected more than 90% of the patients, with anacusis in 32.1% of the cases. Facial palsy affected 17.9% of the patients. In comparison to IMVSs, preoperative anacusis was more associated to meningiomas (P = .0037), and the facial nerve was more compromised in ICMs than IMVSs, both preoperatively (P = .0011) and at follow-up (P < .0001). According to a re-evaluation of preoperative MRIs and comparison with IMVSs, linear tumor borders, and linear morphology along the internal auditory canal wall, but not the presence of a dural tail, were significantly more present in ICMs (P = .0035, P = .0004, P = .1963, respectively). These characteristics could have led to a correct preoperative diagnosis in 61% of our cases. CONCLUSION: Contrariwise to IMVSs, the frequent preoperative anacusis and facial palsy demonstrate the more aggressive nature of ICMs, which also carry a higher risk of postoperative facial palsy and difficulty to preserve hearing. An attentive evaluation of imaging should ease diagnosis, and asymptomatic or stable ICMs should be enrolled in a wait-and-scan protocol. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E413-E419, 2021.

Internal Auditory Canal Diverticula in Children: A Congenital Variant.

OBJECTIVES/HYPOTHESIS: Internal auditory diverticula in adults have been found to exist independent of otosclerosis, and in the presence of otosclerosis. We sought to determine the prevalence of internal auditory canal (IAC) diverticula in a pediatric cohort, to assess whether IAC diverticula are a risk factor for hearing loss, and the co-occurrence of otic capsule hypoattenuation. STUDY DESIGN: Retrospective review. METHODS: A single-site retrospective review of high-resolution temporal bones computed tomography (CT) scans including the presence and size of diverticula and hypoattenuation of the otic capsule. Demographic, imaging, and audiometric data were collected and descriptively analyzed. Bivariate analysis of collected variables was conducted. Comparisons between sides in unilateral cases were also performed. RESULTS: 16/600 (2.7%; 95% CI [2.0%, 3.4%]) were found to have IAC diverticula. Six were bilateral. Thirty-one patients (5.2%) were found to have hypoattenuation of the otic capsule. There were no coincident cases of IAC diverticulum and hypoattenuation of the otic capsule. There was no association between the presence of IAC diverticula and age (P = .13). In six patients with unilateral diverticula, pure tone average (P = .42), and word recognition (P = .27) scores were not significantly different when compared to the normal, contralateral side. CONCLUSIONS: The prevalence of IAC diverticula in children is lower than the prevalence in adults. IAC diverticula in children likely represent congenital variants of temporal bone anatomy. Similar to adult populations, there is evidence that IAC diverticula in children are likely not an independent risk factor for hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1683-E1687, 2021.

[Advances in diagnosis and therapies of immune-mediated inner ear diseases].

Immune-Mediated Inner Ear Diseases（IMIED） is one of the curable sensorineural hearing loss caused by abnormal immune response, which may be primary inner ear diseases or associated with systemic autoimmune diseases. Since the complex differential diagnosis and no reliable diagnostic tests, the early identification of IMIED was extremely tricky. The treatment still mainly depends on glucocorticoid, but as researchs move along, directing at different pathogenesis, more and more therapies have been proposed. This article reviews the diagnosis and different treatment methods of IMIED.

Clinical Characteristics of Patients with Cochlear Fistulas Caused by Chronic Otitis Media with Cholesteatoma.

OBJECTIVES: To analyze the clinical characteristics of cochlear fistulas (CFs) and propose a new fistula classification system with regard to the cochlea. MATERIALS AND METHODS: A retrospective chart review was conducted between January 2008 and December 2015 to identify patients who had undergone surgery for cholesteatoma with an associated CF. The following data were collected: preoperative symptoms, findings of temporal bone computed tomography (TBCT), fistula stage, cholesteatoma classification, surgical technique, and pre- and postoperative pure-tone audiometry. RESULTS: We analyzed a total of 159 patients, out of which 9 (5.7%) were diagnosed with a CF. The average duration of the chronic otitis media was 19.8 years. Cholesteatomas that induced CF rarely existed in the nonaggressive state; recurrent otorrhea was observed in all but one of our subjects. All the patients with CF had a distinct origin of cholesteatoma that developed from the retraction of posterior pars tensa; further, 88.9% cholesteatomas extended to and filled the sinus tympani. Preoperative audiometry revealed total hearing loss in 4 (44.4%) patients. Further, five patients with residual hearing before surgery had stage I fistulas, and the bone conduction thresholds remained stable after surgery. CONCLUSION: Cochlear fistulas were often detected in patients with (1) a history of chronic otitis media (exceeding 10 years), (2) frequently recurring otorrhea, and (3) pars tensa cholesteatomas that extended to the posterior mesotympanum and filled the sinus tympani. Such patients can suffer from potentially severe and irreparable sensorineural hearing loss.

Application of Cervical Vestibular-Evoked Myogenic Potentials in Adults with Moderate to Profound Sensorineural Hearing Loss: A Preliminary Study

Abstract Introduction The cochlea and the vestibular receptors are closely related in terms of anatomy and phylogeny. Patients with moderate to profound sensorineural hearing loss (MPSHL) should have their vestibular organ functions tested. Objective To evaluate the incidence of vestibular abnormalities in patients with MPSHL and to study the correlation between the etiology of hearing loss (HL) and a possible damage to the labyrinth. Methods A case-control retrospective study was performed. In the case group, 20 adults with MPSHL of known etiology were included. The control group was composed of 15 adults with normal hearing. The case group was divided into 4 subgroups based on the etiology (bacterial meningitis, virus, vascular disease, congenital). Cervical vestibular-evoked myogenic potentials (cVEMPs) were used to rate the saccular function and lower vestibular nerve. Results The study was performed in 70 ears, and it highlighted the presence of early biphasic P1-N1 complex in 29 (71.5%) out of 40 ears in the study group, and in all of the 30 ears in the control group (p = 0.001). Regarding the presence or absence of cVEMPs among the four subgroups of patients with MPSHL, the data were statistically significant (p < 0.001). The comparison between the latencies and amplitude of P1-N1 in case and control groups from other studies and in the four subgroups of cases in the present study did not detect statistically significant differences. Conclusion The present study demonstrates that patients with MPSHL have a high incidence of damage to the labyrinthine organs, and it increases the current knowledge about the etiopathogenesis of sensorineural HL, which is often of unknown nature.

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.

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.