Reversible Canalith Jam of the Horizontal Semicircular Canal Mimicking Cupulolithiasis.

OBJECTIVE: To describe a case of benign paroxysmal positional vertigo (BPPV) resulting in reversible horizontal semicircular canalith jam successfully treated with horizontal canal occlusion. A brief literature review of similar cases was performed. METHODS: Case report and literature review. RESULTS: A 68-year-old female presented with apogeotropic positional nystagmus, attributed to reversible horizontal canalith jam mimicking cupulolithiasis that was refractory to tailored repositioning maneuvers across months. She was unable to work due to the severity of her symptoms. She underwent surgical occlusion of the affected canal with immediate resolution of her symptoms. A literature review revealed similar cases of canalith jam mimicking cupulolithiasis. CONCLUSIONS: Reversible canalith jam, in which particles moving with horizontal head position alternate between obstructing the semicircular canal and resting on the cupula, can mimic signs of cupulolithiasis. This variant of BPPV can be effectively managed with surgical canal occlusion should symptoms fail to resolve after tailored repositioning maneuvers.

Evolution of Cochlear implant mapping and vestibular function in a pediatric case of Labyrinthitis.

BACKGROUND: Vestibular symptoms such as vertigo and imbalance are known to occur in some cochlear implant patients during the immediate postoperative period; however, acute vertigo in implanted children occurring remotely from the postoperative period has not been previously well-described. CASE PRESENTATION: A three-year-old girl with a history of bilateral sequential cochlear implantation presented with acute labyrinthitis associated with sudden onset of vertigo, balance impairment, and decline in right cochlear implant function 2 years after her most recent implant surgery. We describe her audiological and vestibular testing results during both the acute phase and following medical management and recovery. CONCLUSION: Acute labyrinthitis should be considered when sudden onset vertigo and/or imbalance presents in children with cochlear implants outside of the perioperative period. Such symptoms should prompt early assessment of cochlear implant function, so that the device can be reprogrammed accordingly.

Balance disorders in childhood: Main etiologies according to age. Usefulness of the video head impulse test.

UNLABELLED: Balance disorders are common in adult patients but less usual in the pediatric population. When this symptomatology appears in children it is a cause for concern, both for parents and health-care professionals. OBJECTIVES: To explain the balance disorders in children describing a case series and to discuss the main etiologies found according to age. STUDY DESIGN: A retrospective, observational, descriptive, and cross-sectional study was conducted. POPULATION: Patients aged 1-18 years who consulted because of balance disorders at the otolaryngology department of a pediatric tertiary-care hospital between March 2012 and July 2015. RESULTS: Two hundred and six patients were included in the study. Median age was 10 years. The most common diagnoses were vestibular migraine in 21.8% of the children, ataxia in 9.22%, benign paroxysmal vertigo of childhood in 7.77%, and post-traumatic vertigo in 6.31%.Overall, 61 videonystagmographies- of which 46 were normal - and 55 video head impulse tests - which were normal in 45 and showed abnormalities in the vestibulo-ocular reflex gain in 10 - were performed. CONCLUSIONS: In a child with balance disorders, the medical history and neurotological examination are essential. Vestibular migraine is the most commonly found disorder in every age group, and most of the patients have a family history of migraine. Ancillary studies, especially the video head-impulse test, provide important data to confirm the diagnosis.

Nystagmus in patients with unilateral acute otitis media complicated by serous labyrinthitis.

Conclusion The patients with serous labyrinthitis caused by acute otitis media (AOM) exhibited various patterns of nystagmus in which direction-fixed irritative-type nystagmus was the most common pattern. Differential effects on inner ear function by toxic or inflammatory mediators may be responsible for the various manifestation of nystagmus. Objective This study aimed to investigate nystagmus patterns in patients with serous labyrinthitis, and discuss possible mechanisms. Methods From October 2011 to March 2014, 13 consecutive patients with serous labyrinthitis were included. Eye movements of the patients were serially examined using video-nystagmography, and patterns of nystagmus were investigated. Results The most commonly observed pattern was direction-fixed nystagmus (nine of 13 patients). Of these, eight showed irritative-type, and one showed paretic-type. Direction of nystagmus, although the intensity gradually decreased, was not changed during the course of treatment. One patient showed direction-changing spontaneous nystagmus, which changed into paretic-type direction-fixed nystagmus 1 day after myringotomy. Three patients exhibited persistent direction-changing positional nystagmus in a supine head-roll test. Of them, two showed apogeotropic and one showed geotropic type. In all 13 patients, vertigo and hearing loss were improved after the treatment.

[The role of computer and magnetic resonance imaging in patients with deafness]. / Rol' komp'yuternoi i magnitno-rezonansnoi tomografii u patsientov s glukhotoi.

The objective of the present study was to evaluate the potential of CT and MRI for diagnostics of congenital and acquired pathology of the inner ear in the deaf patients. Two groups of the patients were examined. The first group consisted of 75 patients with congenital or acquired deafness etiology. The second group was comprised of 75 patients with deafness associated with acute bacterial meningitis suffered in the preceding period. All the patients were examined by CT and MRI of temporal bones. The results of the study provided a basis for the development of indications for the application of CT and MRI to examine the patients presenting with hearing loss and deafness. CONCLUSION: CT and MRI make it possible to identify individual features of the temporal bone structure significant for the surgical treatment. MRI appears to have an advantage over CT for diagnostics of early obliteration of the cochlea. Both CT and MRI are the optional methods for the examination of the patients with deafness developing after meningitis.

Head-shaking aids in the diagnosis of acute audiovestibular loss due to anterior inferior cerebellar artery infarction.

OBJECTIVE: To determine the patterns and diagnostic value of head-shaking nystagmus (HSN) in patients with acute audiovestibular loss. METHOD: Eighteen patients underwent evaluation of spontaneous nystagmus, gaze-evoked nystagmus, HSN, head impulse test, ocular tilt reaction, subjective visual vertical, bithermal caloric tests, and pure-tone audiogram. The findings were compared with those of 21 patients with labyrinthitis. RESULTS: Fifteen patients (83%) exhibited HSN, and the horizontal HSN usually beat contralesionally (10/14, 71%). However, 9 (50%) patients also showed patterns of central HSN that included perverted HSN (n=7), HSN in the opposite direction of spontaneous nystagmus (n=4), and HSN beating towards unilateral canal paresis or abnormal head impulse testing (n=3). Overall, central HSN, gaze-evoked nystagmus, and normal head impulse testing were specific for anterior inferior cerebellar artery (AICA) infarction. Moreover, central HSN was the only sign that indicated stroke in 1 of our patients with isolated audiovestibular syndrome. Lesion subtraction analyses revealed that damage to the flocculus was relatively frequent in patients with perverted HSN. CONCLUSIONS: In AICA infarction, HSN was common with both peripheral and central patterns. Careful evaluation of HSN may provide clues for AICA infarction in patients with acute audiovestibular loss.

Does unilateral utricular dysfunction cause horizontal spontaneous nystagmus?

The presence of spontaneous nystagmus in darkness with a strong horizontal component has been taken to indicate that there is asymmetrical function of the horizontal semicircular canals. If this horizontal spontaneous nystagmus can be suppressed by vision, then it is regarded as due to peripheral horizontal canal dysfunction. However, we report evidence from one patient (61-year-old male), who visited the MSA ENT Clinic, Cassino (FR) Italy, reporting acute, severe vertigo, postural unsteadiness, nausea and vomiting associated with right sudden hearing loss. The patient received instrumental audiovestibular testing to obtain objective measurements of his inner-ear receptors. At the time of the attack, the patient showed spontaneous nystagmus, mainly with horizontal and vertical components (3D infrared video-oculography). Video head-impulse tests of dynamic horizontal canal function showed that the functional status of both horizontal canals was within the normal range. Cervical VEMPs to 500 Hz bone-conducted vibration at Fz showed normal results; ocular VEMPs to the same stimulus showed a reduced n10 amplitude beneath the left eye, corresponding to the right ear. For this reason, the patient was diagnosed as having right unilateral selective utricular macula lesion due to labyrinthitis. There is considerable evidence of convergence of neural input from the otoliths onto horizontal canal neurons in the vestibular nuclei. The firing of such neurons could reflect either asymmetrical horizontal canal function or asymmetrical utricular function. The problem with this patient was not due to asymmetrical horizontal canal function, but only to asymmetrical utricular function, demonstrated by the results of the oVEMP test.

[Bilateral deafness due to labyrinthitis in a patient with Crohn's disease]. / Beidseitige Ertaubung durch Labyrinthitis bei M. Crohn.

Labyrinthitis with vestibulocochlear dysfunction in chronic inflammatory bowl disease is a rare but well described manifestation or complication. The diagnostic and therapeutic possibilities and limitations of this inflammatory otopathy are presented and discussed in the context of a case report. A bilateral loss of vestibular function and sensorineural hearing loss occurred in a 26-year-old male patient with previously diagnosed Crohn's disease. He was treated with immune suppressive therapy during the onset and development of cochleovestibular symptoms. Diagnostic tests included pure tone audiograms, speech audiometry, caloric tests, VEMPs, and MRI. Therapy included azathioprine, corticosteroids (systemic and intratympanic), monoclonal antibodies, and cochlear implants. Despite immunosuppressive therapy including monoclonal antibodies, the patient progressively lost his hearing. Finally, bilateral cochlear implantation was carried out with good results. The treatment of patients with systemic inflammatory or autoimmune disease affecting the cochlear-vestibular organ is challenging. An interdisciplinary approach is needed. In cases of bilateral deafness, cochlear implantation is a satisfactory treatment and should not be delayed unnecessarily.

Effect of Ginkgo biloba extract on endotoxin-induced labyrinthitis.

OBJECTIVE: There are no reports on the therapeutic effect of Ginkgo biloba extract (GBE) on otitis media-induced labyrinthitis. The present study examined whether GBE can protect against cochlear damage induced by intratympanic instillation of lipopolysaccharide (LPS)-induced labyrinthitis. MATERIALS AND METHODS: Experiments were performed in 20 healthy young male guinea pigs. The control group (n=10) received an intratympanic instillation of LPS (20 µl, 3mg/ml). The experimental group (n=10) received intratympanic instillation of LPS immediately after instillation of GBE (10mg/kg) and then experimental groups received GBE (100mg/kg) by intraperitoneal injection every day for 3 days. Instillation of LPS or LPS immediately after GBE was done in the right ear; the untreated left ear was considered normal. Physiological and morphological changes were evaluated. RESULTS: Statistical analysis of treatment of GBE revealed significantly less hearing loss than LPS group (p<0.05). The ratio of the value of cochlear blood flow (CBF) compared to untreated left side was significantly higher in the GBE treated group than in the LPS-treated group (p<0.05). This result indicated the recovery of CBF in GBE treated group compared to LPS treated group. In the LPS group, scanning electron microscopy revealed hair cell damage with edema. Missing stereocilia in the third layer of the outer hair cell was revealed. However, both the inner hair cells and the outer hair cells had normal appearance in the GBE group. LPS group showed that cochlear Evans blue extravasation was increased strongly in the stria vascularis, spiral limbus, and in the spiral ligament compared with the GBE treated group. CONCLUSION: GBE significantly minimizes cochlear damage against LPS-induced otitis media with labyrinthitis in a guinea pig model. GBE has potential as an adjunctive therapy to antibiotics in the treatment of acute otitis media with complicated labyrinthitis.

The role of the osteoimmune axis in the inflammation of the inner auditory ear and with regard to the putative anticarcinogenetic principle: part 2.

Having defined a putative anticarcinogenic principle under physiological conditions of the auditory inner ear in the previous part of this study, part 2 is the continuation to evaluate this hypothesis under pathological conditions, i.e. with regard to the inflamed auditory inner ear. Inflammation of the auditory inner ear shows three characteristic phases. It starts with an acute phase, progresses to a fibrotic one and ends with ossification as the last phase. At the end of the fibrotic phase the inflammatory cells disappear, probably due to apoptosis, the ganglion neurons die and the fibrous matrix ossifies. No proliferating cells can be found in this area any more. Thus this kind of inflammation does not result in a restoration of the former state, but always in the destruction of the hearing organ and corresponding neurons. The osteoimmune axis is involved in this characteristic type of inflammation. Important factors hereby are M-CSF, RANKL/RANK and osteoprotegerin. In the inner auditory ear they inhibit the resorption of the fibrotic matrix, induce apoptosis of inflammatory cells and calcification. This inflammatory osteoimmune axis plays an important role at the cancer site as well. The relation of each factor to the other ones is however fundamentally different from that in the inner ear. Consequently inflammatory processes induced by a tumour foster its progression and may induce bone metastasis. Instead of a dominating and enclosing osteoblastic activity like in the inner ear, matrix resolving and often osteoclastic properties are developed allowing the spread of cancer cells. These processes allows us to set out the anticarcinogenic hypothesis more precisely and define its putative mechanistic rationale for the inflamed auditory inner ear.

Blockade of interleukin-6 signaling suppressed cochlear inflammatory response and improved hearing impairment in noise-damaged mice cochlea.

Hearing impairment can be the cause of serious socio-economic disadvantages. Recent studies have shown inflammatory responses in the inner ear co-occur with various damaging conditions including noise-induced hearing loss. We reported pro-inflammatory cytokine interleukin-6 (IL-6) was induced in the cochlea 6h after noise exposure, but the pathophysiological implications of this are still obscure. To address this issue, we investigated the effects of IL-6 inhibition using the anti-IL-6 receptor antibody (MR16-1). Noise-exposed mice were treated with MR16-1 and evaluated. Improved hearing at 4kHz as measured by auditory brainstem response (ABR) was noted in noise-exposed mice treated with MR16-1. Histological analysis revealed the decrease in spiral ganglion neurons was ameliorated in the MR16-1-treated group, while no significant change was observed in the organ of Corti. Immunohistochemistry for Iba1 and CD45 demonstrated a remarkable reduction of activated cochlear macrophages in spiral ganglions compared to the control group when treated with MR16-1. Thus, MR16-1 had protective effects both functionally and pathologically for the noise-damaged cochlea primarily due to suppression of neuronal loss and presumably through alleviation of inflammatory responses. Anti-inflammatory cytokine therapy including IL-6 blockade would be a feasible novel therapeutic strategy for acute sensory neural hearing loss.

[Innovations in pathogenetic therapy for peripheral affection of the cranial nerves in otorhinolaryngology].

A total of 150 patients with peripheral affection of the facialnerve, 100 patients with acute neurosensory hypoacusis, 50 patients with affection of the reciprocal nerve were treated by the method using reciprocal interaction between M- and H-cholinergic mechanisms. Treatment outcomes showed that anticholinesterase therapy raises the efficacy of rehabilitation in patients with peripheral affection of the cranial nerves.

Inner ear and facial nerve complications of acute otitis media, including vertigo.

Acute otitis media is one of the most common diagnoses made in children in the United States. Intracranial and extracranial (intratemporal) complications have greatly decreased in the antibiotic era, but still remain a challenge when they arise. This article addresses two intratemporal complications with significant associated morbidity: facial nerve paralysis/paresis, and labyrinthitis. Epidemiology, pathology, clinical diagnosis, and treatment options are discussed, focusing on an evidence-based approach to diagnosis and management. In addition, the future of treatment and current questions regarding otitis media are briefly discussed.

Computed tomography and/or magnetic resonance imaging before pediatric cochlear implantation? Developing an investigative strategy.

OBJECTIVE: To investigate and compare the usefulness of preoperative magnetic resonance (MR) imaging and high-resolution temporal bone computed tomography (HRCT) in pediatric cochlear implant candidates. STUDY DESIGN: Prospective. SETTING: Tertiary referral center. PATIENTS: A cohort of 92 pediatric patients with profound hearing. Inclusion criteria were MR, computed tomography, and cochlear implantation. INTERVENTION. DIAGNOSTIC: All patients had preoperative imaging of the petrous temporal bone (HRCT, T2-weighted fast spin echo, axial 3D Fast Imaging Employing Steady-state Acquisition [FIESTA] MR) and brain (Fast Fluid-attenuated Inversion-recovery [FLAIR] MR). MAIN OUTCOME MEASURE(S): Overall prevalence of inner ear dysplasias in this population and comparison of detection rates between HRCT, T2 Fast Spin Echo (FSE), and FIESTA MR sequences. RESULTS: Radiological abnormalities were observed in 32 and 59% of MR and HRCT temporal bone, respectively. Synchronous intracranial findings were noted in 40% on brain MR. Common vestibulocochlear nerve was observed in 3% ears and directed side of implantation. Consistent discrepancies noted on HRCT were inability to diagnose early obliterative labyrinthitis and presence of the cochlear nerve in the internal auditory canal. With respect to MR, enlarged vestibular aqueducts and narrow cochlear nerve canals were consistently under identified. CONCLUSION: Dual-modality imaging with HRCT and MR of petrous bone and MR brain in the precochlear implant pediatric population detects abnormalities related to deafness, which would not otherwise be found using either modality alone. There is overlap between the imaging modalities in the type of abnormalities detected, and we present a case for selective use of HRCT within a diagnostic algorithm, using the patient risk factors we have identified.

Does acute dysfunction of the saccular afferents affect the subjective visual horizontal in patients with vestibular neurolabyrinthitis?

CONCLUSIONS: The results of a series of studies including the present study suggest that acute dysfunction of the utricular afferents accompanied by acute dysfunction of the saccular afferents might require more time for the compensation of the otolith-ocular system than acute utricular dysfunction that was not accompanied by acute saccular dysfunction. Perhaps the inputs from the saccule also have some contribution to the subjective visual horizontal (SVH). OBJECTIVE: To clarify if acute dysfunction of the saccular afferents affects the SVH. PATIENTS AND METHODS: Twenty-six patients with vestibular neurolabyrinthitis (20 men and 6 women, 23-67 years of age) were enrolled in this study. They had undergone measurement of SVH at the early stage (within 1 month after the attack) and 3 months after the attack. For the measurement of SVH, we used a device that has a red bar of light-emitting diodes with a head fixing frame. They also underwent vestibular evoked myogenic potentials (VEMPs) testing. For the recording of VEMPs, 95 dBnHL clicks were presented. RESULTS: Patients with vestibular neurolabyrinthitis showed deviation of SVH toward the affected side-down at the early stage after the attack, irrespective of VEMP results. However, 3 months after the attack SVH was significantly more deviated toward the affected side-down in patients who showed absent VEMPs than those with VEMPs present (p<0.01 Mann-Whitney U test).

Diagnostic value of vestibulo-ocular reflex parameters in the detection and characterization of labyrinthine lesions.

OBJECTIVE: To evaluate the power of various parameters of the vestibulo-ocular reflex (VOR) in detecting unilateral peripheral vestibular dysfunction and in characterizing certain inner ear pathologies. STUDY DESIGN: Prospective study of consecutive ambulatory patients presenting with acute onset of peripheral vertigo and spontaneous nystagmus. SETTING: Tertiary referral center. PATIENTS: Seventy-four patients (40 females, 34 males) and 22 normal subjects (11 females, 11 males) were included in the study. Patients were classified in three main diagnoses: vestibular neuritis: 40; viral labyrinthitis: 22; Meniere's disease: 12. METHODS: The VOR function was evaluated by standard caloric and impulse rotary tests (velocity step). A mathematical model of vestibular function was used to characterize the VOR response to rotational stimulation. The diagnostic value of the different VOR parameters was assessed by uni- and multivariable logistic regression. RESULTS: In univariable analysis, caloric asymmetry emerged as the most powerful VOR parameter in identifying unilateral vestibular deficit, with a boundary limit set at 20%. In multivariable analysis, the combination of caloric asymmetry and rotational time constant asymmetry significantly improved the discriminatory power over caloric alone (p<0.0001) and produced a detection score with a correct classification of 92.4%. In discriminating labyrinthine diseases, different combinations of the VOR parameters were obtained for each diagnosis (p<0.003) supporting that the VOR characteristics differ between the three inner ear disorders. However, the clinical usefulness of these characteristics in separating the pathologies was limited. CONCLUSION: We propose a powerful logistic model combining the indices of caloric and time constant asymmetries to detect a peripheral vestibular loss, with an accuracy of 92.4%. Based on vestibular data only, the discrimination between the different inner ear diseases is statistically possible, which supports different pathophysiologic changes in labyrinthine pathologies.

Fine structure histopathology of labyrinthitis ossificans in the gerbil model.

Labyrinthitis ossificans (LO) is the pathological deposition of new bone within the lumen of the cochlea and labyrinth. This process occurs most commonly as a result of infection or inflammation affecting the otic capsule. Trauma and vascular compromise can also lead to neo-ossification within the otic capsule. The mechanism that regulates this process remains unestablished. This study details the end-stage histopathology in high-resolution plastic thin sections. Twenty Mongolian gerbils were infected by intrathecal injection of Streptococcus pneumoniae type 3 followed by subcutaneous penicillin G procaine (8 days) and were painlessly sacrificed 3 months later. The cochleas were serially divided and sectioned for light and electron microscopy. Sixteen of 20 animals (27 of 40 cochleas) demonstrated LO. Cochlear damage was most extensive in the vestibule and basal turn and decreased toward the apex, which often appeared normal. The histopathologic findings consisted of 1) new bone, calcospherites, osteoid, and fibrosis without dense connective tissue or osteoblasts extending from the endosteal wall into the lumen of the vestibule and scala tympani; 2) areas of dense connective tissue and osteoid enclosed by epithelial cells conjoined with the organ of Corti, stria vascularis, spiral ligament, and vestibular (Reissner's) membrane; and 3) partial to complete loss of the organ of Corti, spiral ligament cell bodies, stria vascularis, and spiral ganglion cells. Osteoblastic activity was not demonstrated in end-stage ossification in LO in the gerbil model. Neo-ossification appears to occur by calcospherite deposition along collagen-like fibrils within osteoid. The destruction of the organ of Corti, spiral ganglion cells, stria vascularis, and cells of Reissner's membrane and the spiral ligament occurs even in the absence of ossification of the cochlear duct.

[Correction of vestibular disorders in patients with otitis media purulenta chronica complicated by labyrinthitis].

A vestibulogic examination was made in 66 patients with otitis media purulenta chronica complicated with labyrinthitis. The patients were divided into two groups: group 1 received surgical treatment in an acute period of labyrinthitis, group 2 was treated conservatively with delayed operation. The patients were examined at admission, 2 weeks, 1, 3, 6 and 12 months after the treatment. It was found that compensation of the vestibular function in group 1 took place 6 months after temporal bone surgery, in group 2-9 months after delayed operation, i.e. 1 years after hospitalization.