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.

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.

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.

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.

[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.

Inflammatory signals increase Fas ligand expression by inner ear cells.

There is considerable evidence that hearing and vestibular function can be influenced by immune processes. The inner ear has evolved mechanisms, such as the blood-labyrinthine barrier that limit immune responses and autoimmune processes to reduce the potential for damage to cochlear cells. Recently, expression of Fas ligand (FasL) in some non-lymphoid tissue, as in the anterior chamber of the eye, has been hypothesized to play a role in protection of sensitive organs from activated T-cells. We show that under resting conditions, cochlear cells express little or no FasL. However, after exposure to interferon-gamma in vitro, FasL is induced in many neonatal cochlear cells. In addition, we show that FasL is upregulated in adult cochlear cells after induction of a sterile labyrinthitis in vivo. The induction of FasL by inflammation may serve to limit cochlear immune responses, and to protect sensorineural tissue from immune and autoimmune damage.

Clinical diagnoses associated with histologic findings of fibrotic tissue and new bone in the inner ear.

Fibrotic tissue or new bone occurs following inner ear inflammation, fracture, or surgery. The prevalence is unknown and was investigated using the National Temporal Bone, Hearing and Balance Pathology Resource Registry database. A search yielded 264 temporal bones with diagnoses of otosclerosis, tumor, Meniere's disease, meningitis, labyrinthitis, chronic otitis media, autoimmune disease, temporal bone fracture, or sensorineural hearing loss. All autoimmune cases contained some new bone, whereas only 20% to 30% of the labyrinthitis/meningitis cases were reported to contain new bone. Otosclerosis, Meniere's disease, and otitis media had relatively few cases containing new bone. Although new bone may derive from surgical trauma, it is also likely to be a result of the disease process. It seems that all these disease processes may contain a common feature that acts as a stimulus to induce fibrosis or bone growth in the inner ear.

Absent vestibular evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior vestibular nerve involvement?

BACKGROUND: Benign paroxysmal positioning vertigo (BPPV) is generally thought to be caused by canalolithiasis in the posterior semicircular canal, an organ that is innervated by the inferior vestibular nerve. We hypothesized that absent vestibular evoked myogenic potentials (VEMPs) would indicate involvement of the inferior vestibular nerve and that posterior semicircular canal-type BPPV could not develop after vestibular neurolabyrinthitis (VNL) in patients with absent VEMPs. OBJECTIVE: To find out if VEMPs could be helpful in evaluating involvement of the inferior vestibular nerve in acute VNL. DESIGN: We reviewed the VEMP findings in 47 patients (34 men and 13 women) with acute VNL, 10 of whom had then developed posterior semicircular canal-type BPPV. RESULTS: While p13-n23, the first positive-negative peak of the VEMP, was ipsilaterally present on stimulation of the unaffected side in all patients, it was absent on the affected side in 16 patients (34%). The absence or presence of p13-n23 was independent of the results of caloric tests, pure tone audiometry, and auditory brain-stem responses. Typical posterior semicircular canal BPPV developed in 10 of the 47 patients after the acute attack of VNL, always on the same side as the neurolabyrinthitis. The p13-n23 potentials were preserved on stimulation of the affected ear in all 10 patients with BPPV. CONCLUSIONS: These results suggest that if VEMPs are absent from an ear that has suffered acute VNL, then posterior semicircular canal BPPV is unlikely to develop as a consequence of the VNL. The reason for this appears to be that the absence of VEMPs is due to involvement of the inferior vestibular nerve or involvement of the structures that it innervates.

Successful revision of failed cochlear implants in severe labyrinthitis ossificans.

Labyrinthitis ossificans may complicate the insertion of a multichannel cochlear implant in patients deafened after meningitis. Two children who initially underwent partial insertion of a 22-channel cochlear implant because of severe cochlear ossification required revision surgery after several months of unsuccessful device use. At the time of revision, resection of the car canal, tympanic membrane, malleus, and incus provided access to the lateral wall of the cochlea, permitting extensive drilling of the basal turn and a circumodiolar placement of the electrode. Functional integrity of the electronic components of the original device was documented intraoperatively, avoiding the expense of a new receiver-stimulator. Complete insertion of the active electrodes was accomplished in both cases, and electrophysiologic responsiveness to the implant was documented using intraoperative electrically evoked auditory brainstem response recordings. Postoperative performance has been similar to that of cochlear implant patients with nonossified ears. Experience with these two cases suggests that efforts to optimize electrode insertion at the original surgical procedure are appropriate and may help to avoid the disappointment of an unsuccessful cochlear implant.

Characteristics of human gait related variables in association with vestibular system disorders.

Gait analysis was performed in patients with vestibular system disorders, using foot switches and electromyography. They were divided into three groups: 11 cases with vestibular neuronitis (VN), 10 cases with large acoustic neuroma (LAN) who had central lesion evidenced by neurotological examinations, and 10 cases with olivopontocerebellar atrophy (OPCA). A total of 14 healthy adults served as controls. Results showed that time from heel strike to forefoot strike was the most sensitive variable to indicate gait abnormality but had no specificity. As for the variables of stance, swing, and time to reach the initial peak of the tibialis anterior's activity from forefoot off, occurrence rate of abnormality was high in the OPCA group, followed by the LAN and VN groups. Concerning the double support period which is related with body weight transfer from one leg to the other, the LAN and OPCA groups showed higher occurrence rate of abnormality than the VN group. These differences among the three groups could reflect different effects to the gait control systems caused by lesion.

Adoptive transfer of an autoimmunological labyrinthitis in the guinea pig; animal model for a sympathetic cochleolabyrinthitis.

Sensorineural hearing loss is a common problem in the otolaryngologist's practice, with autoimmune disease of the inner ear being one possible cause. The restoration of auditory function in some patients following immunosuppressive therapy has created a desire to define and understand this disease better. Because of the lack of a well defined detection method to identify this entity clinically, this study was undertaken in order to provide an animal model for autoimmune disease of the inner ear. Previous studies with guinea pigs have demonstrated that sensitized lymphocytes from the systemic circulation migrate to the labyrinth during an immune response in the inner ear. The aim of this study was to prove the capacity of sensitized lymphocytes to transfer autoimmune inner ear disease, and to describe the resulting morphological and physiological changes. Therefore two groups of sensitized lymphocytes partially labelled with a radioactive marker from inbred guinea pigs with an immune response within the inner ear were injected into the bloodstream of naive recipient animals. Most of the labelled cells were observed in the apical turn of the experimental cochlea, while only few cells were detectable in the control cochleas. In addition, the absence of otoacoustic emissions and the loss of outer hair cells observed by electron microscopy were interpreted as a sign of damage caused by the provoked immunopathologic mechanism. The results are discussed as a possible model for a sympathetic cochleolabyrinthitis.

Detection of cochlear dysfunction by the measurement of transiently evoked otoacoustic emissions in guinea pigs with autoimmune-induced labyrinthitis.

For the evaluation of functional inner ear lesions caused by an autoimmune-induced labyrinthitis, transiently evoked otoacoustic emissions (TEOAEs) were detected in guinea pigs before and after transfer of sensitized lymphocytes from animals suffering from a labyrinthitis induced by the foreign protein keyhole limpet hemocyanin (KLH). Initially TEOAEs were detectable from 47 of 62 ears (76%) in healthy guinea pigs. These animals then were used as recipients for sensitized lymphocytes from donors exclusively. Three months after cell transfer the incidence of TEOAEs was reduced to 48% in animals receiving lymphocytes from donors sensitized with KLH intradermally and intracochlearly. In recipients of lymphocytes from donors sensitized only intradermally and in untreated animals no significant alteration of the TEOAE incidence was found. These findings showed strong correlation with those from scanning electron microscopy of the organ of Corti, indicating that the measurement of TEOAE is a useful, time-saving tool for the detection of cochlear dysfunction caused by sensitized lymphocytes in experimental animal. The present findings also show that the migration of sensitized lymphocytes actually leads to functional lesions in the cochlea.

Acceleration registrography of head movement during alternating inclination of the support platform.

The righting reflex, essential for maintaining equilibrium, keeps the head and trunk in a state of balance with regard to gravity. Because patients suffering from vestibular disorders almost always complain of dizziness during everyday movements such as rising and walking, the righting reflex should be evaluated within the context of these dynamic states. A new method of acceleration registrography was designed to enable the testing of this reflex as the subject stands upon a moving platform. Participating in the study were 49 normal control subjects and 80 patients with peripheral vestibular disorders. The test we devised proved to be more successful in detecting labyrinthine righting reflex dysfunction in patients than the tests of righting reflex and deviation evaluations used thus far, such as Romberg's, Mann's and the stepping test, and to more accurately represent the disequilibrium and/or dizziness experienced by patients in everyday movements.

Auditory performance of children with cochlear ossification and partial implant insertion.

The management of the profoundly deaf child with a cochlear implant poses a special challenge, particularly when total ossification of the cochlea is present. In this setting, insertion of an electrode array into a child's cochlea is often difficult. Our experience supports the feasibility of partial insertion of a multichannel implant into the basal turn of an ossified cochlea. Five children with ossified cochleae who had undergone partial implantation of a multichannel electrode were compared with the performance of matched controls who had full insertion of multichannel implants. No dramatic differences were detected during a 6- to 18-month follow-up period on selected test measures. These preliminary results suggest that active electrode number may exert a limited effect on performance with a cochlear implant. Drilling out the basal turn of an ossified cochlea in conjunction with partial insertion of a multichannel implant appears to be an acceptable surgical and rehabilitational alternative for placement of a cochlear implant prosthesis in children with complete cochlear ossification.

Labirintite pós-meringoplastia; apresentaçäo de um caso / Labyrinthitis after a meryngoplasty: relate the one case

Os autores descrevem o caso de um paciente que submetido a uma simples meringoplastia, apresentou um quadro de labirintite serosa no pós-operatório e que apesar do tratamento adequado, evoluiu para uma labirintite cicatricial