Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Vestibular rehabilitation following surgical repair for Superior Canal Dehiscence Syndrome: A complicated case report.

Superior Canal Dehiscence Syndrome (SCDS) causes auditory and vestibular symptoms. Following surgical repair of the dehiscence, patients often experience dizziness and imbalance. This case report describes a postoperative vestibular exercise program, focusing on the principles of central compensation and habituation, and how it was modified for a patient with delayed progress secondary to strabismus and visual vertigo. A 63-year-old male with history of strabismus eye surgery, right hearing loss, aural fullness, and sensitivity to loud sounds was referred for vestibular rehabilitation (VR). He was seen for one preoperative and six postoperative PT visits over eight months. Outcome measures two weeks postoperative were as follows: Dizziness Handicap Inventory (DHI) 38/100; Timed Up & Go (TUG) 9.92 seconds; Dynamic Gait Index (DGI) 16/24; and a 3-line difference in Dynamic Visual Acuity (DVA). Improved outcomes at discharge included: DHI 18/100; TUG 6.87 seconds; DGI 23/24; and 1-line difference in DVA. He was able to return to work and previously enjoyed recreational activities. Postoperative vestibular rehabilitation programs are functionally and symptomatically beneficial following surgical repair for SCDS. Deviations from expected recovery should be addressed to achieve optimal outcomes as demonstrated in this complicated case report.

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.

Home-Based Computer Gaming in Vestibular Rehabilitation of Gaze and Balance Impairment.

Disease or damage of the vestibular sense organs cause a range of distressing symptoms and functional problems that could include loss of balance, gaze instability, disorientation, and dizziness. A novel computer-based rehabilitation system with therapeutic gaming application has been developed. This method allows different gaze and head movement exercises to be coupled to a wide range of inexpensive, commercial computer games. It can be used in standing, and thus graded balance demands using a sponge pad can be incorporated into the program. A case series pre- and postintervention study was conducted of nine adults diagnosed with peripheral vestibular dysfunction who received a 12-week home rehabilitation program. The feasibility and usability of the home computer-based therapeutic program were established. Study findings revealed that using head rotation to interact with computer games, when coupled to demanding balance conditions, resulted in significant improvements in standing balance, dynamic visual acuity, gaze control, and walking performance. Perception of dizziness as measured by the Dizziness Handicap Inventory also decreased significantly. These preliminary findings provide support that a low-cost home game-based exercise program is well suited to train standing balance and gaze control (with active and passive head motion).

[Kinesitherapy in patients with the peripheral vestibular system disorders]. / Kinezyterapia u pacjentów z obwodowym uszkodzeniem narzadu przedsionkowego.

INTRODUCTION: Kinesitherapy is widely accepted management in patients with vertigo and imbalance, but there has been inadequate evidence that one form of therapy is superior to another. THE AIM: of the study was to compare effectiveness of two kinesitherapy protocols in patients with the peripheral vestibular system disorders. MATERIAL AND METHODS: Fifty patients (mean age 46.0±13.1 year) with vertigo and balance instability lasting over 3 months with unilateral vestibular disorder, confirmed in Videnystagmography, were included in the study. Thirty patients underwent supervised and 20 patients home-based exercise programs. All of them were assessed three times at the baseline, after 4 weeks and 3 months, on vertigo intensity and frequency with the Vertigo Syndrome Scale (VSS), Vertigo Visual Analog Scale (VAS) and clinical unsteadiness with tests (Romberg, Amended Motor Club Assesment (AMCA), Eurofit test - standing on one leg. RESULTS: In both groups the clinical tasks and the intensity of vertigo in VAS significantly decreased. The mean value of VSS (part physical and emotional)score significantly decreased only in supervised group at the end of 4 weeks and 3 months (p=ns). Recovery was more dynamic in supervised group than home-based exercises group, in AMCA test (3.9 vs. 1.3 s, p<0.05) in Eurofit tests eye open (14.1 vs. 0.9 s, p<0.05) and eye closed (3.5 vs. 1 s, p<0.05). CONCLUSIONS: In patients with unilateral peripheral vestibular dysfunction supervised and home-based group kinesitherapy is an effective treatment method. In supervised group patients recovery has been faster.

[Design and development of an automatic rotatory chair for canalith repositioning maneuver].

This paper demonstrates a 3D rotatory chair and the development of control rotatory system based on PLC, corresponding software and user interface. The PLC-based three-dimensional rotatory chair has the advantage of maneuverability and stability according to the result of the experiment.

[Vibrotactile neurofeedback training with the Vertiguard®-RT-system. A placebo-controlled double-blinded pilot study on vestibular rehabilitation]. / Vibrotaktiles Neurofeedbacktraining mit dem Vertiguard®-RT-System. Placebokontrollierte Doppelblind-Pilot-Studie zur Rehabilitation von Gleichgewichtsstörungen.

BACKGROUND: The success of neurofeedback training for vestibular rehabilitation depends largely on the type of feedback signal as well as on the specific parameters. Ideal training should be based on the individual balance deficits in patients in everyday situations. The present study is therefore aimed at investigating the therapeutic outcome of a new vibrotactile neurofeedback system for the first time in a pilot study. PATIENTS AND METHODS: A total of 36 patients performed daily vibrotactile neurofeedback training (for 10 days) based on a sway analysis on the first day. The reduction of body sway and vertigo symptom scale (VSS) scores were calculated after the training and compared with those of the placebo group. RESULTS: All five patient groups (characterized by different vestibular disorders) which performed the training with the correct feedback signal showed reduced body sway and VSS score (n=30). This effect was not visible in the placebo group. CONCLUSIONS: Individualized vibrotactile neurofeedback training as presented here with the Vertiguard® system appeared to improve balance during daily activities in all patient groups investigated, but not in controls. Future studies should investigate the efficacy of this new method in a larger sample as well as its long term effects.

Traumatic perilymphatic fistula with pneumolabyrinth: diagnosis and management.

A perilymphatic fistula (PLF) is an abnormal communication between the inner and middle ear resulting in vestibular or cochlear symptoms. We review three pediatric traumatic temporal bone fractures with pneumolabyrinth, confirmed radiologically by the presence of air within the cochlea (pneumocochlea) or vestibule (pneumovestibule). Patients were treated conservatively with complete resolution of vestibulopathy. Hearing outcome was variable and worse in two patients with pneumocochlea. A pneumolabyrinth on radiologic imaging confirms a PLF and obviates the need for exploration to reach a diagnosis. We suggest exploration be reserved for patients with persisting cerebrospinal fluid leakage, progressive sensorineural hearing loss, or vestibular symptomatology.

Vestibular rehabilitation's effect over the quality of life of geriatric patients with labyrinth disease.

UNLABELLED: Dizziness is a symptom that affects the population world over, being more prevalent in the elderly due to the process of functional deterioration of the hearing and vestibular systems with aging. AIM: The objective of this study was to evaluate prospectively the effect of Vestibular Rehabilitation (VR) as treatment for labyrinth disease of vascular and metabolic origin in the quality of life of geriatric patients. METHODS: The study was outlined as clinical-prospective, longitudinal, and observed, with the participation of 40 elder citizens of both genders, divided in 2 groups, dizziness of vascular or metabolic origin. The patients were evaluated and underwent VR - based on Cawthorne and Cooksey's protocol. The statistical analysis from the data was done through the t-Student test, the coefficients of Pearson and Spearman. RESULTS: based on quality of life scales showed that the individuals treated and assessed improved after Vestibular Rehabilitation. CONCLUSION: we concluded that VR, based on the protocols of Cawthorne and Cooksey, could be beneficial to this population.

Efeito da reabilitação vestibular sobre a qualidade de vida de idosos labirintopatas / Vestibular rehabilitation's effect over the quality of life of geriatric patients with labyrinth disease

A tontura é um sintoma que acomete a população mundial, sendo observado maior prevalência em idosos devido ao processo de deterioração funcional dos sistemas auditivo e vestibular com o envelhecimento. OBJETIVO: O objetivo deste trabalho foi avaliar prospectivamente o efeito da Reabilitação Vestibular (RV) como tratamento das labirintopatias de origem vascular e metabólica sobre a qualidade de vida de idosos. MATERIAL E MÉTODO: O estudo foi delineado como um ensaio clínico prospectivo, longitudinal, com a participação de 40 idosos de ambos os gêneros, divididos em 2 grupos, tontura de origem vascular ou metabólica. Os pacientes passaram por avaliações, orientações e a RV, que se baseou no protocolo de Cawthorne e Cooksey. A análise estatística dos dados foi feita através do teste t-Student e dos coeficientes de Pearson e Spearman. RESULTADOS: Pelas escalas de qualidade de vida utilizadas podemos observar que os aspectos avaliados melhoraram após a Reabilitação Vestibular. CONCLUSÃO: Conclui-se que a RV baseada nos protocolos de Cawthorne e Cooksey pode ser utilizada de modo benéfico nesta população.

Dizziness is a symptom that affects the population world over, being more prevalent in the elderly due to the process of functional deterioration of the hearing and vestibular systems with aging. AIM: The objective of this study was to evaluate prospectively the effect of Vestibular Rehabilitation (VR) as treatment for labyrinth disease of vascular and metabolic origin in the quality of life of geriatric patients. METHODS: The study was outlined as clinical-prospective, longitudinal, and observed, with the participation of 40 elder citizens of both genders, divided in 2 groups, dizziness of vascular or metabolic origin. The patients were evaluated and underwent VR - based on Cawthorne and Cooksey's protocol. The statistical analysis from the data was done through the t-Student test, the coefficients of Pearson and Spearman. RESULTS: based on quality of life scales showed that the individuals treated and assessed improved after Vestibular Rehabilitation. CONCLUSION: we concluded that VR, based on the protocols of Cawthorne and Cooksey, could be beneficial to this population

[Protection and regeneration of sensory epithelia of the inner ear]. / Protektion und Regeneration sensorischer Epithelien des Innenohres.

Dysfunctions of the inner ear such as hearing impairment due to noise exposure or presbycusis and vertigo are often caused by loss of hair cells in the sensory epithelium. There is still no specific therapy, just technical aids. Options for protecting and regenerating hair cells are explained here. The inhibition of apoptosis via caspases is presently the main target of research. They are involved in damage caused by aminoglycosides, cisplatin, or noise exposure. Bcl-2, growth factors, and oxidative stress are discussed. In regeneration the transdifferentiation of supporting cells to hair cells is explained. This can be achieved with local gene therapy using math1. Approach and media for the application are discussed, while viral vectors such as the adenovector seem the most promising in research.

Sensory reweighting as a method of balance training for labyrinthine loss.

BACKGROUND AND PURPOSE: Instability is a significant risk factor for falls in individuals with a bilateral labyrinthine deficit. The purpose of this case report is to describe an intervention that we found to improve balance in a patient with bilateral labyrinthine deficit using a training paradigm based on the sensory reweighting hypothesis. CASE DESCRIPTION: The participant was a female and 10 years post-onset of bilateral labyrinthine deficit. The participant was instructed to focus on the motion of her hips and knees while standing on a dynamic platform that was either stationary or matched to the excursion of her center of mass (COM) but in the opposite direction and with gradually increasing amplitude. She was tested for her ability to maintain her balance under conditions of sensory conflict both before the training and on two periods after training. OUTCOMES: Decreases in anteroposterior and mediolateral motion of the COM were observed between the pretest and both posttests with a stationary and a moving platform when in the dark and under conditions of sensory conflict. Using the method of approximate entropy, we found that the complexity of the center of pressure (COP) response increased in both the anteroposterior and medolateral directions from the pretest to both posttests when on the platform matched to the COM motion. SUMMARY: Results indicated that training on a dynamic platform diminished the destabilizing effect of conflicting sensory signals. Additionally, a relationship was observed between decreased COM motion and increased complexity in COP, which represents a more self-organized system. This finding suggests that improved stability may be associated with an increased complexity in the COP trajectory.

Vestibular rehabilitation outcomes in chronic vertiginous patients through computerized dynamic visual acuity and Gaze stabilization test.

OBJECTIVE: To evaluate the efficiency of the rehabilitative protocols in patients with labyrinthine hypofunction, focusing on computerized dynamic visual acuity test (DVAt) and Gaze stabilization test (GST) specifically evaluating the vestibulo-oculomotor reflex (VOR) changes due to vestibular rehabilitation. DESIGN: Consecutive sample study. SETTING: Day hospital in Ears, Nose, and Throat Rehabilitation Unit. SUBJECTS: Thirty-two patients with chronic dizziness with a mean age of 60.74 years. INTERVENTION: Patients performed one cycle of 12 daily rehabilitation sessions (2 h each) consisting of exercises aimed at improving VOR gain. The rehabilitation program included substitutional and/or habitudinal exercises, exercises on a stability platform, and exercises on a moving footpath with rehabilitative software. MAIN MEASURES: Dizziness Handicap Inventory and Activities-specific Balance Confidence Scale. Computerized dynamic posturography, computerized DVAt, and GST. RESULTS: The patients significantly improved in all the tests. CONCLUSION: Vestibular rehabilitation improved the quality of life by reducing the handicap index and improving the ability in everyday tasks. The recovery of the vestibular-ocular reflex and vestibular-spinal reflex efficiency was objectively proven by instrumental testing. The DVAt and the GST allow to objectively quantify the fixation ability at higher frequencies and speeds (main VOR function). Moreover, these new parameters permit to completely evaluate vestibular rehabilitation outcomes, adding new information to the generally used tests that only assess vestibulospinal reflex.

[Obliterated cochlea in Cogan's syndrome -- implications for cochlear implant surgery]. / Obliteration der Cochlea beim Cogan-Syndrom -- Bedeutung für die Cochlear-Implant-Chirurgie.

BACKGROUND: Acute, often bilateral deafness in Cogan's syndrome or other autoimmune diseases is caused by autoimmune mediated inflammatory attack on the membranous labyrinth. Auditory rehabilitation in case of bilateral deafness can be achieved by cochlear implant surgery. METHODS: A retrospective analysis of all patients suffering from Cogan's syndrome that had received a cochlear implant, was carried out. RESULTS: 6 of 295 adult patients (2.6 %) that had received a cochlear implant, had become deaf due to Cogan's syndrome. Partial obliteration or ossifikation was encountered in all cases and influenced surgical procedure. In one case a fibrous obliteration of the scala tympani was found 8 weeks after acute onset of complete deafness. CONCLUSIONS: The course of obliteration is unknown. With regard to our results a fibrous obliteration may occur as early as 8 weeks after complete deafness. This has to be considered in counseling of patients. Only early cochlear implant surgery facilitates best possible rehabilitation results.

[Benign paroxysmal positional vertigo]. / Benigner paroxysmaler Lagerungsschwindel.

Benign paroxysmal positional vertigo is the most common vestibular disorder, accounting for about 20% of referrals in specialized dizziness clinics. Nowadays, canalolithiasis of the posterior semicircular canal has been widely accepted as the biological basis for typical benign paroxysmal positional vertigo as it is compatible with all clinical features of the disorder. Better understanding of its pathophysiological concepts has led to specific therapeutic strategies, which aim to clear the affected semicircular canal from mobile particles. After a single maneuver both Epley's and Semont's procedures lead to complete recovery in about 60% of patients and in nearly 100% when performed repeatedly. These positioning maneuvers have made benign paroxysmal positional vertigo the most successfully treatable cause of vertigo.

Using immersive technology for postural research and rehabilitation.

Posture has traditionally been examined by isolating individual control pathways to determine their specific contributions. However, if these pathways are responsive to functional contexts, then their responses may differ when the system is receiving simultaneous inputs from multiple pathways. Thus, we may never fully understand how the central nervous system (CNS) organizes behaviors in the real world from studies conducted in the minimized environment of the laboratory. The consequence of this is that when findings from the laboratory are applied to therapeutic intervention, the intervention may not be appropriate for all circumstances and will not fully meet the needs of the patient. We have united an immersive dynamic virtual environment with motion of a posture platform to record the biomechanical and physiological responses to combined visual, vestibular, and proprioceptive inputs. The virtual environment possesses content, contrast, and texture so that we can examine postural responses as they might occur in a complex, real-world environment. In this paper we specifically describe the factors guiding our choices of virtual technology and present data from young adults, elderly adults, and an individual with bilateral labyrinthine loss to demonstrate how multimodal inputs influence their postural response organization. Significant implications for future experimental and rehabilitation protocols are also discussed.

Exercícios optovestibulares na reabilitação vestibular / Optovestibular exercises in vestibular rehabilitation

Sete pacientes com queixa de vertigem não postural foram submetidos à avaliação otoneurológica (avaliação otorrinolaringológica, audiométrica e vestibular) e encaminhados para um programa de reabilitação vestibular no Setor de Equilibriometria da Disciplina de Otoneurologia da UNIFESP - EPM. O programa de reabilitação vestibular constou de orientação quanto ao tratamento etiológico, dieta alimentar e exercícios optovestibulares. Foi utilizado um tambor optocinético de Bárány para a realização dos exercícios optovestibulares por 8 sessões de 1 hora cada, 2 vezes por semana, durante 30 dias. O tambor foi colocado a um metro de distância dos olhos do paciente, nas posições vertical, horizontal, inclinado para direita e para esquerda, de forma a propiciar a estimulação dos nistagmos horizontais, verticais e oblíquos, respectivamente, tanto no sentido horário quanto no anti-horário. Após a reavaliação dos pacientes, foi possível concluir que este programa de reabilitação foi efetivo como tratamento em casos de vertigens não posturais, uma vez que houve melhora sintomatológica em 71 por cento dos casos. Cabe ressaltar alguns fatores relevantes com relação ao sucesso do programa: idade, motivação, estado psíquico favorável do paciente, tratamento etiológico e dieta associados aos exercícios optovestibulares.