RESUMO
How tissues acquire complex shapes is a fundamental question in biology and regenerative medicine. Zebrafish semicircular canals form from invaginations in the otic epithelium (buds) that extend and fuse to form the hubs of each canal. We find that conventional actomyosin-driven behaviors are not required. Instead, local secretion of hyaluronan, made by the enzymes uridine 5'-diphosphate dehydrogenase (ugdh) and hyaluronan synthase 3 (has3), drives canal morphogenesis. Charged hyaluronate polymers osmotically swell with water and generate isotropic extracellular pressure to deform the overlying epithelium into buds. The mechanical anisotropy needed to shape buds into tubes is conferred by a polarized distribution of actomyosin and E-cadherin-rich membrane tethers, which we term cytocinches. Most work on tissue morphogenesis ascribes actomyosin contractility as the driving force, while the extracellular matrix shapes tissues through differential stiffness. Our work inverts this expectation. Hyaluronate pressure shaped by anisotropic tissue stiffness may be a widespread mechanism for powering morphological change in organogenesis and tissue engineering.
Assuntos
Espaço Extracelular/química , Ácido Hialurônico/farmacologia , Morfogênese , Especificidade de Órgãos , Pressão , Canais Semicirculares/citologia , Canais Semicirculares/embriologia , Actomiosina/metabolismo , Animais , Anisotropia , Comportamento Animal , Matriz Extracelular/metabolismo , Ácido Hialurônico/biossíntese , Modelos Biológicos , Morfogênese/efeitos dos fármacos , Especificidade de Órgãos/efeitos dos fármacos , Pressão Osmótica , Canais Semicirculares/diagnóstico por imagem , Comportamento Estereotipado , Peixe-Zebra/embriologia , Proteínas de Peixe-Zebra/metabolismoRESUMO
Inner ear morphogenesis requires tightly regulated epigenetic and transcriptional control of gene expression. CHD7, an ATP-dependent chromodomain helicase DNA-binding protein, and SOX2, an SRY-related HMG box pioneer transcription factor, are known to contribute to vestibular and auditory system development, but their genetic interactions in the ear have not been explored. Here, we analyzed inner ear development and the transcriptional regulatory landscapes in mice with variable dosages of Chd7 and/or Sox2. We show that combined haploinsufficiency for Chd7 and Sox2 results in reduced otic cell proliferation, severe malformations of semicircular canals, and shortened cochleae with ectopic hair cells. Examination of mice with conditional, inducible Chd7 loss by Sox2CreER reveals a critical period (~E9.5) of susceptibility in the inner ear to combined Chd7 and Sox2 loss. Data from genome-wide RNA-sequencing and CUT&Tag studies in the otocyst show that CHD7 regulates Sox2 expression and acts early in a gene regulatory network to control expression of key otic patterning genes, including Pax2 and Otx2. CHD7 and SOX2 directly bind independently and cooperatively at transcription start sites and enhancers to regulate otic progenitor cell gene expression. Together, our findings reveal essential roles for Chd7 and Sox2 in early inner ear development and may be applicable for syndromic and other forms of hearing or balance disorders.
Assuntos
Redes Reguladoras de Genes , Vestíbulo do Labirinto , Animais , Camundongos , Cóclea , Regulação da Expressão Gênica no Desenvolvimento , Mamíferos , Canais Semicirculares , Fatores de TranscriçãoRESUMO
Jawed vertebrates have inner ears with three semicircular canals, the presence of which has been used as a key to understanding evolutionary relationships. Ostracoderms, the jawless stem gnathostomes, had only two canals and lacked the lateral canal1-3. Lampreys, which are modern cyclostomes, are generally thought to possess two semicircular canals whereas the hagfishes-which are also cyclostomes-have only a single canal, which used to be regarded as a more primitive trait1,4. However, recent molecular and developmental analyses have strongly supported the monophyly of cyclostomes5-7, which has left the evolutionary trajectory of the vertebrate inner ear unclear8. Here we show the differentiation of the otic vesicle of the lamprey Lethenteron camtschaticum and inshore hagfish Eptatretus burgeri. This is the first time, to our knowledge, that the development of the hagfish inner ear is reported. We found that canal development in the lamprey starts with two depressions-which is reminiscent of the early developmental pattern of the inner ear in modern gnathostomes. These cyclostome otic vesicles show a pattern of expression of regulatory genes, including OTX genes, that is comparable to that of gnathosomes. Although two depressions appear in the lamprey vesicle, they subsequently fuse to form a single canal that is similar to that of hagfishes. Complete separation of the depressions results in anterior and posterior canals in gnathostomes. The single depression of the vesicle in hagfishes thus appears to be a secondarily derived trait. Furthermore, the lateral canal in crown gnathostomes was acquired secondarily-not by de novo acquisition of an OTX expression domain, but by the evolution of a developmental program downstream of the OTX genes.
Assuntos
Feiticeiras (Peixe)/anatomia & histologia , Lampreias/anatomia & histologia , Organogênese , Filogenia , Canais Semicirculares/anatomia & histologia , Canais Semicirculares/embriologia , Vertebrados/anatomia & histologia , Vertebrados/embriologia , Animais , Regulação da Expressão Gênica no Desenvolvimento , Feiticeiras (Peixe)/embriologia , Feiticeiras (Peixe)/genética , Lampreias/embriologia , Lampreias/genética , Camundongos/anatomia & histologia , Camundongos/embriologia , Organogênese/genética , Tubarões/anatomia & histologia , Tubarões/embriologia , Vertebrados/genética , Peixe-Zebra/anatomia & histologia , Peixe-Zebra/embriologiaRESUMO
Noninvasive electrical stimulation of the vestibular system in humans has become an increasingly popular tool with a broad range of research and clinical applications. However, common assumptions regarding the neural mechanisms that underlie the activation of central vestibular pathways through such stimulation, known as galvanic vestibular stimulation (GVS), have not been directly tested. Here, we show that GVS is encoded by VIIIth nerve vestibular afferents with nonlinear dynamics that differ markedly from those predicted by current models. GVS produced asymmetric activation of both semicircular canal and otolith afferents to the onset versus offset and cathode versus anode of applied current, that in turn produced asymmetric eye movement responses in three awake-behaving male monkeys. Additionally, using computational methods, we demonstrate that the experimentally observed nonlinear neural response dynamics lead to an unexpected directional bias in the net population response when the information from both vestibular nerves is centrally integrated. Together our findings reveal the neural basis by which GVS activates the vestibular system, establish that neural response dynamics differ markedly from current predictions, and advance our mechanistic understanding of how asymmetric activation of the peripheral vestibular system alters vestibular function. We suggest that such nonlinear encoding is a general feature of neural processing that will be common across different noninvasive electrical stimulation approaches.SIGNIFICANCE STATEMENT Here, we show that the application of noninvasive electrical currents to the vestibular system (GVS) induces more complex responses than commonly assumed. We recorded vestibular afferent activity in macaque monkeys exposed to GVS using a setup analogous to human studies. GVS evoked notable asymmetries in irregular afferent responses to cathodal versus anodal currents. We developed a nonlinear model explaining these GVS-evoked afferent responses. Our model predicts that GVS induces directional biases in centrally integrated head motion signals and establishes electrical stimuli that recreate physiologically plausible sensations of motion. Altogether, our findings provide new insights into how GVS activates the vestibular system, which will be vital to advancing new clinical and biomedical applications.
Assuntos
Movimentos Oculares , Vestíbulo do Labirinto , Animais , Masculino , Humanos , Vestíbulo do Labirinto/fisiologia , Canais Semicirculares/fisiologia , Primatas , Sensação , Estimulação Elétrica/métodosRESUMO
BACKGROUND: Bilateral vestibular hypofunction is associated with chronic disequilibrium, postural instability, and unsteady gait owing to failure of vestibular reflexes that stabilize the eyes, head, and body. A vestibular implant may be effective in alleviating symptoms. METHODS: Persons who had had ototoxic (7 participants) or idiopathic (1 participant) bilateral vestibular hypofunction for 2 to 23 years underwent unilateral implantation of a prosthesis that electrically stimulates the three semicircular canal branches of the vestibular nerve. Clinical outcomes included the score on the Bruininks-Oseretsky Test of Motor Proficiency balance subtest (range, 0 to 36, with higher scores indicating better balance), time to failure on the modified Romberg test (range, 0 to 30 seconds), score on the Dynamic Gait Index (range, 0 to 24, with higher scores indicating better gait performance), time needed to complete the Timed Up and Go test, gait speed, pure-tone auditory detection thresholds, speech discrimination scores, and quality of life. We compared participants' results at baseline (before implantation) with those at 6 months (8 participants) and at 1 year (6 participants) with the device set in its usual treatment mode (varying stimulus pulse rate and amplitude to represent rotational head motion) and in a placebo mode (holding pulse rate and amplitude constant). RESULTS: The median scores at baseline and at 6 months on the Bruininks-Oseretsky test were 17.5 and 21.0, respectively (median within-participant difference, 5.5 points; 95% confidence interval [CI], 0 to 10.0); the median times on the modified Romberg test were 3.6 seconds and 8.3 seconds (difference, 5.1; 95% CI, 1.5 to 27.6); the median scores on the Dynamic Gait Index were 12.5 and 22.5 (difference, 10.5 points; 95% CI, 1.5 to 12.0); the median times on the Timed Up and Go test were 11.0 seconds and 8.7 seconds (difference, 2.3; 95% CI, -1.7 to 5.0); and the median speeds on the gait-speed test were 1.03 m per second and 1.10 m per second (difference, 0.13; 95% CI, -0.25 to 0.30). Placebo-mode testing confirmed that improvements were due to treatment-mode stimulation. Among the 6 participants who were also assessed at 1 year, the median within-participant changes from baseline to 1 year were generally consistent with results at 6 months. Implantation caused ipsilateral hearing loss, with the air-conducted pure-tone average detection threshold at 6 months increasing by 3 to 16 dB in 5 participants and by 74 to 104 dB in 3 participants. Changes in participant-reported disability and quality of life paralleled changes in posture and gait. CONCLUSIONS: Six months and 1 year after unilateral implantation of a vestibular prosthesis for bilateral vestibular hypofunction, measures of posture, gait, and quality of life were generally in the direction of improvement from baseline, but hearing was reduced in the ear with the implant in all but 1 participant. (Funded by the National Institutes of Health and others; ClinicalTrials.gov number, NCT02725463.).
Assuntos
Vestibulopatia Bilateral/cirurgia , Marcha/fisiologia , Perda Auditiva/etiologia , Neuroestimuladores Implantáveis , Equilíbrio Postural/fisiologia , Qualidade de Vida , Vestíbulo do Labirinto/cirurgia , Idoso , Vestibulopatia Bilateral/induzido quimicamente , Vestibulopatia Bilateral/complicações , Tontura/etiologia , Feminino , Transtornos Neurológicos da Marcha/etiologia , Humanos , Neuroestimuladores Implantáveis/efeitos adversos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Estudos Prospectivos , Canais Semicirculares/inervação , Nervo Vestibular/efeitos dos fármacosRESUMO
The semicircular canals of the inner ear are involved in balance and velocity control. Being crucial to ensure efficient mobility, their morphology exhibits an evolutionary conservatism attributed to stabilizing selection. Release of selection in slow-moving animals has been argued to lead to morphological divergence and increased inter-individual variation. In its natural habitat, the house mouse Mus musculus moves in a tridimensional space where efficient balance is required. In contrast, laboratory mice in standard cages are severely restricted in their ability to move, which possibly reduces selection on the inner ear morphology. This effect was tested by comparing four groups of mice: several populations of wild mice trapped in commensal habitats in France; their second-generation laboratory offspring, to assess plastic effects related to breeding conditions; a standard laboratory strain (Swiss) that evolved for many generations in a regime of mobility reduction; and hybrids between wild offspring and Swiss mice. The morphology of the semicircular canals was quantified using a set of 3D landmarks and semi-landmarks analyzed using geometric morphometric protocols. Levels of inter-population, inter-individual (disparity) and intra-individual (asymmetry) variation were compared. All wild mice shared a similar inner ear morphology, in contrast to the important divergence of the Swiss strain. The release of selection in the laboratory strain obviously allowed for an important and rapid drift in the otherwise conserved structure. Shared traits between the inner ear of the lab strain and domestic pigs suggested a common response to mobility reduction in captivity. The lab-bred offspring of wild mice also differed from their wild relatives, suggesting plastic response related to maternal locomotory behavior, since inner ear morphology matures before birth in mammals. The signature observed in lab-bred wild mice and the lab strain was however not congruent, suggesting that plasticity did not participate to the divergence of the laboratory strain. However, contrary to the expectation, wild mice displayed slightly higher levels of inter-individual variation than laboratory mice, possibly due to the higher levels of genetic variance within and among wild populations compared to the lab strain. Differences in fluctuating asymmetry levels were detected, with the laboratory strain occasionally displaying higher asymmetry scores than its wild relatives. This suggests that there may indeed be a release of selection and/or a decrease in developmental stability in the laboratory strain.
Assuntos
Evolução Biológica , Canais Semicirculares , Animais , Camundongos , Canais Semicirculares/anatomia & histologia , Mamíferos , FrançaRESUMO
Vestibular nuclei and cerebellar function comprise vestibular neural networks that control vestibular-related responses. However, the vestibular-related responses to simultaneous stimulation of these regions are unclear. This study aimed to examine whether the combination of noisy galvanic vestibular stimulation (nGVS) and cerebellar transcranial direct current stimulation (ctDCS) using a complex transcranial electrical stimulation device alters vestibular-dominant standing stability and vestibulo-ocular reflex (VOR) function. The center of foot pressure (COP) sway and VOR of participants (28 healthy, young adults) were assessed under four conditions of transcranial electrical stimulation using nGVS and ctDCS. The COP was calculated with the participant standing on a soft-foam surface with eyes closed using a force plate to evaluate body sway. VOR measurements were collected via passive head movements and fixation on a target projected onto the front wall using a video head impulse test (vHIT). VOR gain was calculated in six directions using a semicircular canal structure based on the ratio of eye movement to head movement. The nGVS + ctDCS and nGVS + sham ctDCS conditions decreased COP sway compared to the sham nGVS + ctDCS and sham nGVS + sham ctDCS conditions. No significant differences were observed in the main effect of stimulation or the interaction of stimulation and direction on the vHIT parameters. The results of this study suggest that postural stability may be independently affected by nGVS. Our findings contribute to the basic neurological foundation for the clinical application of neurorehabilitation using transcranial electrical stimulation of the vestibular system.
Assuntos
Estimulação Transcraniana por Corrente Contínua , Vestíbulo do Labirinto , Adulto Jovem , Humanos , Equilíbrio Postural/fisiologia , Vestíbulo do Labirinto/fisiologia , Canais Semicirculares/fisiologia , Movimentos Oculares , Reflexo Vestíbulo-Ocular/fisiologia , Estimulação ElétricaRESUMO
INTRODUCTION: Surgical treatment of Ménière's disease (MD) and deafness aims to treat vertigo and hearing disabilities. Current treatment options like labyrinthectomy and cochlear implantation (CI) have shown acceptable results but are destructive. Less destructive procedures, like the occlusion of the lateral semicircular canal and endolymphatic sac surgery, have been shown to be successful in vertigo control. The combination of both procedures with CI has not been investigated; therefore the objective of this study was to investigate the outcome of this combination in patients with single-sided MD and moderately severe to complete sensorineural hearing loss. METHODS: In this retrospective study, 10 patients with single-sided MD and moderately severe to complete sensorineural hearing loss were included. In all of them, a single-staged surgery, which consisted of CI, endolymphatic sac surgery, and occlusion of the lateral semicircular canal, was performed. The surgery was performed after a failed conservative therapy trial. The clinical outcome was evaluated by the Dizziness Handicap Inventory (DHI) and audiological tests. These were assessed preoperatively, 3 and 6 months after surgery. An MRI with a hydrops sequence was performed to support the clinical diagnosis. RESULTS: After the combined surgery, the mean DHI testing improved significantly from 71 to 30. Mean audiological monosyllabic speech testing outcome with the cochlea implant was 65% at 65 dB. The residual hearing of 2 patients could be preserved after the surgical procedure. CONCLUSION: The combination of occlusion of the lateral semicircular canal, endolymphatic sac surgery, and CI is an efficient low traumatic treatment for patients with a single-sided MD and moderately severe to complete sensorineural hearing loss.
Assuntos
Implante Coclear , Saco Endolinfático , Perda Auditiva Neurossensorial , Doença de Meniere , Canais Semicirculares , Humanos , Doença de Meniere/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Feminino , Canais Semicirculares/cirurgia , Saco Endolinfático/cirurgia , Adulto , Idoso , Perda Auditiva Neurossensorial/cirurgia , Resultado do Tratamento , Surdez/cirurgiaRESUMO
OBJECTIVES: Canalith repositioning procedures to treat benign paroxysmal positional vertigo are often applied following standardized criteria, without considering the possible anatomical singularities of the membranous labyrinth for each individual. As a result, certain patients may become refractory to the treatment due to significant deviations from the ideal membranous labyrinth, that was considered when the maneuvers were designed. This study aims to understand the dynamics of the endolymphatic fluid and otoconia, within the membranous labyrinth geometry, which may contribute to the ineffectiveness of the Epley maneuver. Simultaneously, the study seeks to explore methods to avoid or reduce treatment failure. DESIGN: We conducted a study on the Epley maneuver using numerical simulations based on a three-dimensional medical image reconstruction of the human left membranous labyrinth. A high-quality micro-computed tomography of a human temporal bone specimen was utilized for the image reconstruction, and a mathematical model for the endolymphatic fluid was developed and coupled with a spherical particle model representing otoconia inside the fluid. This allowed us to measure the position and time of each particle throughout all the steps of the maneuver, using equations that describe the physics behind benign paroxysmal positional vertigo. RESULTS: Numerical simulations of the standard Epley maneuver applied to this membranous labyrinth model yielded unsatisfactory results, as otoconia do not reach the frontside of the utricle, which in this study is used as the measure of success. The resting times between subsequent steps indicated that longer intervals are required for smaller otoconia. Using different angles of rotation can prevent otoconia from entering the superior semicircular canal or the posterior ampulla. Steps 3, 4, and 5 exhibited a heightened susceptibility to failure, as otoconia could be accidentally displaced into these regions. CONCLUSIONS: We demonstrate that modifying the Epley maneuver based on the numerical results obtained in the membranous labyrinth of the human specimen under study can have a significant effect on the success or failure of the treatment. The use of numerical simulations appears to be a useful tool for future canalith repositioning procedures that aim to personalize the treatment by modifying the rotation planes currently defined as the standard criteria.
Assuntos
Vertigem Posicional Paroxística Benigna , Humanos , Vertigem Posicional Paroxística Benigna/fisiopatologia , Vertigem Posicional Paroxística Benigna/diagnóstico por imagem , Microtomografia por Raio-X , Simulação por Computador , Osso Temporal/diagnóstico por imagem , Membrana dos Otólitos/fisiologia , Imageamento Tridimensional , Endolinfa/fisiologia , Orelha Interna/diagnóstico por imagem , Canais Semicirculares/diagnóstico por imagem , Canais Semicirculares/fisiologia , Posicionamento do Paciente/métodosRESUMO
BACKGROUND: Superior Semicircular Canal Dehiscence (SSCD) is a dehiscence of the otic capsule which normally lies over the superior semicircular canal. This database constitutes the largest series of SSCD patients to date. OBJECTIVE: To determine what preoperative factors, if any, contribute to postoperative outcomes and evaluate symptom resolution in a large SSCD patient cohort. METHODS: A single-institution, retrospective chart review collected patient demographics, intraoperative findings, and pre-and postoperative symptoms. Fisher's exact t-test was performed for unpaired categorical variables, with a significance level of p < 0.05. RESULTS: 350 SSCD repairs were performed. The median age was 52 years (range: 17-86 years, ± 6.4 years), and the median follow-up duration was 4.6 months (range: 0.03-59.5 months, ± 6.8 months). Preoperative hearing loss was significantly associated with female sex (p = 0.0028). The most reported preoperative symptoms were tinnitus (77.4%), dizziness (74.0%), autophony (66.3%), amplification (63.7%), and disequilibrium (62.6%). Between patients who received unilateral versus bilateral SSCD repair, the greatest postoperative symptomatic resolution was seen in autophony (74.9%, p < 0.001), amplification (77.3%, p = 0.00027), hyperacusis (77.4%, p = 0.023), hearing (62.9%, p = 0.0063), and dizziness (54.6%, p < 0.001) for patients with unilateral SSCD repair. CONCLUSION: Surgical repair via the middle cranial fossa approach can significantly resolve auditory, vestibular, and neurological symptoms of patients with SSCD. Although this is one of the largest single-institution SSCD studies to date, future multi-institutional, prospective studies would be beneficial to validate these results.
Assuntos
Deiscência do Canal Semicircular , Humanos , Pessoa de Meia-Idade , Feminino , Masculino , Adulto , Idoso , Idoso de 80 Anos ou mais , Adolescente , Adulto Jovem , Estudos Retrospectivos , Deiscência do Canal Semicircular/cirurgia , Resultado do Tratamento , Canais Semicirculares/cirurgia , Complicações Pós-Operatórias/etiologia , Zumbido/etiologia , Zumbido/cirurgiaRESUMO
BACKGROUND: About 12.5 % of patients diagnosed with benign paroxysmal positional vertigo (BPPV), experience persistent BPPV where it is unknown why some BPPV cases are very refractory (vrBPPV) to treatment. OBJECTIVES: The primary objective was to investigate if patients with vrBPPV could be successfully treated with a mechanical rotation chair (MRC) adjusted to the exact vertical semicircular canal (SCC) angles of the individual patient. Secondary endpoint was to determine if inner ear anomalies were predominant in these patients. METHODS: This prospective clinical trial included 20 patients (main group) who underwent computed tomography (CT) with measurements of the bony island and the width between the SCC walls of the posterior leg of the lateral SCCs. The inter-SCC angles, the angles between the sagittal plane and the vertical SCCs were compared to the presumed mean standard angles of the SCCs. Of these, 14 patients (subgroup) underwent individualized treatment with the Rotundum® repositioning chair according to their measured SCC angles. RESULTS: All measured SCC angles differed significantly (p < 0.05) from the presumed mean standard SCC angles, except the angle between the sagittal plane and the left posterior SCC (p-SCC). Three out of 14 patients experienced subjective and objective remission after treatment with this MRC. Six out of 14 patients experienced either subjective remission or objective remission. CONCLUSIONS: Patients with vrBPPV have vertical SCC angles that differ significantly from the presumed mean standard SCC angles. Individualized treatment with this MRC successfully treated 21.4 % of the patients with vrBPPV and provided subjective relief for 42.9 %.
Assuntos
Vertigem Posicional Paroxística Benigna , Posicionamento do Paciente , Canais Semicirculares , Tomografia Computadorizada por Raios X , Humanos , Vertigem Posicional Paroxística Benigna/terapia , Masculino , Feminino , Estudos Prospectivos , Pessoa de Meia-Idade , Idoso , Posicionamento do Paciente/métodos , Resultado do Tratamento , Canais Semicirculares/fisiopatologia , Canais Semicirculares/diagnóstico por imagem , Rotação , AdultoRESUMO
BACKGROUND: The Dix-Hallpike (DH) test is a gold standard for diagnosing benign paroxysmal positional vertigo (BPPV). However, lateral semicircular canal BPPV is not rare. We have been performing the new roll test that begins from the sitting position and contains a head-hanging position, in order not to overlook lateral canal BPPV. We noticed that transient vertical/torsional nystagmus sometimes occurs during the new roll test. OBJECTIVE: To clarify the value of the new roll test in diagnosing posterior canal BPPV and elucidate the position that elicits nystagmus. MATERIALS AND METHODS: The subjects were 100 consecutive patients (79 were female, 21 were male) with posterior canal BPPV. We classified the patients into four types based on a position that induced nystagmus. RESULTS: The patient's position that elicited nystagmus varied. The supine type accounted for 24 %, the lateral type accounted for 62 %, the head-hanging type accounted for 9 %, and the DH type accounted for 5 %. CONCLUSION: The new roll test is valuable for diagnosing posterior canalolithiasis cases. Most patients reveal vertical/torsional nystagmus in the supine or lateral position. Therefore, performing the new roll test first is efficient at the initial visit.
Assuntos
Vertigem Posicional Paroxística Benigna , Canais Semicirculares , Humanos , Vertigem Posicional Paroxística Benigna/diagnóstico , Vertigem Posicional Paroxística Benigna/fisiopatologia , Masculino , Feminino , Canais Semicirculares/fisiopatologia , Pessoa de Meia-Idade , Idoso , Adulto , Idoso de 80 Anos ou mais , Nistagmo Patológico/diagnóstico , Nistagmo Patológico/fisiopatologia , Posicionamento do Paciente/métodos , Testes de Função Vestibular/métodos , Postura/fisiologiaRESUMO
OBJECTIVES: Tegmen and superior semicircular canal defects have been well studied, yet the factors contributing to their onset and progression are widely debated. The clinical utility of intraoperative intracranial pressure measurements has yet to be tested. This report aims to use intraoperative opening pressure and concurrent superior semicircular canal dehiscence (SSCD) to analyze factors influencing disease course and clinical outcomes in patients with tegmen dehiscence. METHODS: A retrospective analysis of 61 patients who underwent tegmen defect repair was performed. Multiple variables of interest including body mass index (BMI), presence of SSCD, presence of dural venous sinus stenosis, opening pressure, and acetazolamide therapy use were recorded. The cohort was divided into those with or without concurrent SSCD and those presenting with or without cerebrospinal fluid (CSF) leak for analysis. RESULTS: A linear relationship between opening pressure and BMI (p = 0.009) was noted; however, intraoperative opening pressure was not associated with disease outcome. Concurrent SSCD was present in 25 % of patients, while 62 % presented with CSF leak. The concurrent SSCD group exhibited higher opening pressure, higher likelihood of having dural sinus stenosis, and higher likelihood of being discharged on acetazolamide. The CSF leak group had higher likelihood of obstructive sleep apnea and persistent symptoms. CONCLUSIONS: In patients undergoing tegmen defect repair, concurrent SSCD suggests increased disease severity. The presence of preoperative CSF leak predicts persistent symptoms following repair. BMI is linearly correlated with intracranial pressure in these patients.
Assuntos
Vazamento de Líquido Cefalorraquidiano , Deiscência do Canal Semicircular , Canais Semicirculares , Humanos , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Vazamento de Líquido Cefalorraquidiano/etiologia , Vazamento de Líquido Cefalorraquidiano/cirurgia , Canais Semicirculares/cirurgia , Deiscência do Canal Semicircular/cirurgia , Deiscência do Canal Semicircular/complicações , Resultado do Tratamento , Adulto , Índice de Massa Corporal , Idoso , Pressão Intracraniana , Complicações Pós-Operatórias/etiologia , AcetazolamidaRESUMO
PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.
Assuntos
Perda Auditiva Neurossensorial , Doenças do Labirinto , Deiscência do Canal Semicircular , Aqueduto Vestibular , Humanos , Doenças do Labirinto/diagnóstico por imagem , Doenças do Labirinto/patologia , Perda Auditiva Neurossensorial/patologia , Aqueduto Vestibular/patologia , Cóclea/diagnóstico por imagem , Cóclea/patologia , Canais Semicirculares/diagnóstico por imagem , Canais Semicirculares/patologiaRESUMO
PURPOSE: Determine whether adult cochlear implant users with radiographic superior semicircular canal dehiscence experience clinically significant differences in audiological outcomes when compared to cochlear implant users with normal temporal bone anatomy. MATERIALS AND METHODS: Retrospective, single institution review. Adult, post-lingual deaf patients implanted between 2010 and 2020. Inclusion criteria included age 18 years or older, available preoperative computed tomography imaging, and preoperative and postoperative AzBio audiological data for at least 6 months of cochlear implant use. Preoperative and postoperative AzBio Sentence Test scores were compared between patients with normal temporal bone anatomy and those with radiographic superior semicircular canal dehiscence or near dehiscence. RESULTS: 110 patients met inclusion criteria. Mean AzBio score for normal temporal bone anatomy group improved from 35.2 % (SD 28.2) preoperatively to 70.3 % (SD 25.7) postoperatively, an improvement of 35.1 % (SD 28.6). Mean AzBio score for near dehiscent temporal bone anatomy group improved from 26.6 % (SD 28.9) preoperatively to 64.5 % (SD 30.6) postoperatively, an improvement of 37.9 % (SD 27.9). Mean AzBio score for dehiscent temporal bone anatomy group improved from 26.3 % (SD 20.4) preoperatively to 65.1 % (SD 27.6) postoperatively, an improvement of 38.7 % (SD 26.9). Utilizing the one-way analysis of variance test, there was no significant difference in audiologic outcomes between the three groups. CONCLUSIONS: Patients with complete or near complete radiographic superior canal dehiscence at the time of cochlear implantation achieve similar improvements in speech perception scores compared to normal anatomy adult cochlear implant users.
Assuntos
Implante Coclear , Implantes Cocleares , Deiscência do Canal Semicircular , Osso Temporal , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Implante Coclear/métodos , Adulto , Idoso , Deiscência do Canal Semicircular/cirurgia , Resultado do Tratamento , Osso Temporal/diagnóstico por imagem , Osso Temporal/cirurgia , Canais Semicirculares/cirurgia , Canais Semicirculares/diagnóstico por imagem , Surdez/cirurgia , Surdez/diagnóstico por imagemRESUMO
PURPOSE: Assess otolith and canal involvement in patients with Benign Paroxysmal Positional Vertigo (BPPV) during the acute phase. METHODS: Ninety patients with BPPV in the acute phase underwent a vestibular assessment that included an assessment with videonistagmography, video Head Impulse Test (vHIT) to evaluate horizontal and vertical semicircular canals, and ocular vestibular evoked myogenic potentials (oVEMPs) for the otolithic function. RESULTS: Ninety patients had an involvement of the posterior canal, fifty-five out of ninety patients presented a BPPV of the right ear. No asymmetry of the otolithic functions was found for the utricular macula. Furthermore, no reduction of the Vestibular Ocular Reflex gain was found for the examined canal functions. CONCLUSIONS: The lack of asymmetry suggests that during the acute phase of BPPV, the otolithic function is balanced between the affected and unaffected ears. Moreover, the preserved VOR gain for the examined canal functions suggests that the VOR responses for the examined channels were intact.
Assuntos
Vertigem Posicional Paroxística Benigna , Potenciais Evocados Miogênicos Vestibulares , Humanos , Vertigem Posicional Paroxística Benigna/diagnóstico , Membrana dos Otólitos , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Canais Semicirculares , Teste do Impulso da CabeçaRESUMO
OBJECTIVE: This study investigates the importance of bone density, surface area, and diameter of anatomical structures of the superior semicircular canal (SSC), lateral semicircular canal (LSC), posterior semicircular canal (PSC), utricle, and saccule in patients diagnosed with superior semicircular canal dehiscence (SSCD). MATERIALS AND METHODS: The bone density, surface area, and diameter of SSC, LSC, PSC, utricle, and saccule were measured and compared between the SSCD group and control group. Fifteen ears in the SSCD group and 60 ears in the control group were evaluated. Additionally, within the SSCD group, the dehiscent and healthy sides were evaluated independently. RESULTS: SSC's bone density was significantly lower in the SSCD group compared to the control group (p = 0.008). No significant differences were found in surface area and diameter between the groups (p > 0.05). While most of the anatomical structures showed no significant difference in bone density between dehiscent and healthy ears (p > 0.05), SSC bone density was significantly lower in affected ears (p = 0.000) in SSCD group. CONCLUSION: Based on the data obtained in this study, bone density and anatomical structure may be useful in patients diagnosed with SSCD.
Assuntos
Densidade Óssea , Deiscência do Canal Semicircular , Canais Semicirculares , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Canais Semicirculares/patologia , Canais Semicirculares/diagnóstico por imagem , Canais Semicirculares/anatomia & histologia , Adulto , Deiscência do Canal Semicircular/patologia , Deiscência do Canal Semicircular/diagnóstico por imagem , Idoso , Estudos de Casos e Controles , Tomografia Computadorizada por Raios X , Osso Temporal/diagnóstico por imagem , Osso Temporal/anatomia & histologia , Osso Temporal/patologia , Sáculo e Utrículo/patologia , Sáculo e Utrículo/diagnóstico por imagemRESUMO
PURPOSE: To analyse the degree of pneumatisation of the temporal bone when there is an association between dehiscence of the superior semicircular canal and dehiscence of the tegmen tympani. MATERIALS AND METHODS: We analysed a retrospective CT study of 124 selected cases. A single inclusion criterion was applied: the presence of a dehiscence of the tegmen tympani. On the other hand, the degree of temporal pneumatisation was assessed by axial and coronal planes, and has been divided into the following grades O, I, II and III, according to the status and relationship of the mastoid, the bony labyrinth, the petrous segment of the carotid canal and sigmoid sinus. RESULTS: Of the 124 cases studied, 35 (28.2%) presented both dehiscences. In 26 of the 35 (47.3%), grade II pneumatisation, 4 (14,8%), grade I, and 5 (11,9%) grade III was observed, with a statistically significant relationship (p < 0.001). On the other hand, we did not find a significant relationship when relating both dehiscences in any age or sex group. However, when relating the degree of pneumatisation to sex, among those with grade III pneumatisation, the proportion of men (52.4%) was significantly higher than that of women (47.6%) (p = 0.017). CONCLUSION: We have detected a statistically significant relationship between the coexistence of grade II pneumatisation and the presence of both dehiscences in the temporal bone.
Assuntos
Orelha Média , Osso Temporal , Masculino , Humanos , Feminino , Estudos Retrospectivos , Osso Temporal/diagnóstico por imagem , Canais Semicirculares/diagnóstico por imagem , Processo MastoideRESUMO
OBJECTIVE: To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases. METHODS: In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans. RESULTS: We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome. CONCLUSION: Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen.
Assuntos
Processo Mastoide , Deiscência do Canal Semicircular , Humanos , Processo Mastoide/diagnóstico por imagem , Processo Mastoide/cirurgia , Estudos de Coortes , Deiscência do Canal Semicircular/complicações , Seguimentos , Estudos Retrospectivos , Vertigem/etiologia , Canais Semicirculares/diagnóstico por imagem , Canais Semicirculares/cirurgiaRESUMO
OBJECTIVES: The aim of our study to contribute to the field of morphometrics by including measurements of the SAC and SAF and their distances from surrounding structures, particularly for surgeons involved in retrosigmoid approach for internal acoustic meatus tumor surgery and cerebellopontine angle surgery. Although there is limited information in the literature regarding the role of the subarcuate fossa (SAF) and subarcuate canal (SAC), it has been suggested that the SAC may be a potential pathway for infection from the middle ear to the posterior cranial fossa, and cerebellar abscesses may have this origin. METHODS: For the images of our study, computerized tomography images of 118 individuals (59 females and 59 males) between the ages of 18-65 who applied to Bayindir Health Group. RESULTS: The width of the cranial opening of the subarcuate canal was determined as 44 ± 0.54 mm, width of the labyrinth opening of the subarcuate canal was determined as 60 ± 0.42 mm, Length of the subarcuate canal was determined as 8.79 ± 2.31 mm, width of the subarcuate canal was determined as 5.54 ± 1.75 mm, and depth of subarcuate fossa was determined as 1.67 ± 0.69 mm. The distance of the cranial opening of the subarcuate canal to the superior semicircular canal (SSC-SAC/C) was measured as 5.33 ± 1.81 mm, The distance of the labyrinth opening of the subarcuate canal to the superior semicircular canal (SSC-SAC/L) was measured as 3.90 ± .98 mm, length of the petrous part of the temporal bone medial to the anterior semicircular canal measured from the apex to the SSCD (PLM) was measured as 33.56 ± 0.42 mm. No statistically significant differences were found between the right and left sides. CONCLUSIONS: The morphometric measurements obtained in this study can provide useful information for neurosurgeons, neurotologist and otolaryngologists involved in retrosigmoid approach for internal acoustic meatus tumor surgery and cerebellopontine angle surgery, and for patients undergoing cochlear implant planning with a retrofacial approach.