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1.
Tomography ; 10(2): 203-214, 2024 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-38393284

RESUMO

BACKGROUND: Constructive interference in steady state (CISS) is a gradient echo magnetic resonance imaging (MRI) pulse sequence that provides excellent contrast between cerebrospinal fluid and adjacent structures but is prone to banding artifacts due to magnetic field inhomogeneities. We aimed to characterize artifacts in the inner ear and eye. METHODS: In 30 patients (60 ears/eyes) undergoing CISS sequence MRI, nine low-signal intensity regions were identified in the inner ear and compared to temporal bone histopathology. The number and angle of bands across the eye were examined. RESULTS: In the cochlea, all ears had regions of low signal corresponding to anatomy (modiolus (all), spiral lamina (n = 59, 98.3%), and interscalar septa (n = 50, 83.3%)). In the labyrinth, the lateral semicircular canal crista (n = 42, 70%) and utricular macula (n = 47, 78.3%) were seen. Areas of low signal in the vestibule seen in all ears may represent the walls of the membranous utricle. Zero to three banding artifacts were seen in both eyes (right: 96.7%, mean 1.5; left: 93.3%, mean 1.3). CONCLUSION: Low signal regions in the inner ear on CISS sequences are common and have consistent patterns; most in the inner ear represent anatomy, appearing blurred due to partial volume averaging. Banding artifacts in the eye are more variable.


Assuntos
Orelha Interna , Humanos , Orelha Interna/anatomia & histologia , Orelha Interna/patologia , Cóclea/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos
2.
Acta Otolaryngol ; 143(11-12): 951-957, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38108643

RESUMO

BACKGROUND: Acute audiovestibular deficits may be a harbinger of vestibular schwannoma (VS). OBJECTIVE: To investigate clinical and laboratory features of 25 consecutive patients with VS presenting with acute audiovestibular deficits. METHODS: A symptomatic combination of acute audiovestibular deficits was investigated. Audiometric and vestibular function tests, and internal auditory canal magnetic resonance imaging (IAC MRI) results were evaluated. RESULTS: Varying combinations of symptoms may develop in VS patients with acute audiovestibular deficits, of whom sudden hearing loss (HL) without acute vertigo or acute facial nerve palsy (FNP) was most common. The most common audiometric configuration was high-tone hearing loss, and no patient showed low-tone hearing loss. IAC MRI demonstrated that the tumor had an intracanalicular portion and attachment to the bony IAC wall in all patients and widened the IAC wall in some patients. CONCLUSION: Different symptomatic combinations of acute audiovestibular deficits may develop in patients with VS. Awareness about the possibility of VS as a cause of sudden HL, acute vertigo, and acute FNP, as well as subsequent IAC MRI scanning is vital to earlier diagnosis of VS in these patients.


Assuntos
Orelha Interna , Paralisia Facial , Perda Auditiva Súbita , Neuroma Acústico , Humanos , Neuroma Acústico/diagnóstico , Neuroma Acústico/diagnóstico por imagem , Orelha Interna/patologia , Vertigem/diagnóstico , Imageamento por Ressonância Magnética/métodos , Perda Auditiva Súbita/etiologia , Perda Auditiva Súbita/complicações , Síndrome , Paralisia Facial/complicações , Ângulo Cerebelopontino/diagnóstico por imagem , Ângulo Cerebelopontino/patologia
3.
Hum Gene Ther ; 34(19-20): 1064-1071, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37642269

RESUMO

The inner ear is a primary lesion in sensorineural hearing loss and has been a target in gene therapy. The efficacy of gene therapy depends on achieving sufficient levels of transduction at a safe vector dose. Vectors derived from various adeno-associated viruses (AAVs) are predominantly used to deliver therapeutic genes to inner ear cells. AAV9 and its variants vector are attractive candidates for clinical applications since they can cross the mesothelial cell layer and transduce inner hair cells (IHCs), although this requires relatively high doses. In this study, we investigated the effects of sucrose on the transduction of a variant of the AAV9 vector for gene transfer in the inner ear. We found that high concentrations of sucrose increased gene transduction in House Ear Institute-Organ of Corti 1 (HEI-OC1) cells in vitro. In addition, we demonstrated that simultaneous administration of sucrose enhanced the transduction of mouse IHCs and spiral ligament cells using an AAV9 variant vector. The procedure did not increase the thresholds in the auditory brainstem response, suggesting that sucrose had no adverse effect on auditory function. This versatile method may be valuable in the development of novel gene therapies for adult-onset sensorineural hearing loss.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Animais , Camundongos , Cóclea/patologia , Orelha Interna/patologia , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/terapia , Perda Auditiva Neurossensorial/patologia , Células Ciliadas Auditivas Internas , Terapia Genética/métodos
4.
Curr Probl Diagn Radiol ; 52(6): 576-585, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37500297

RESUMO

Inner ear malformation (IEM) with associated sensoryneural hearing loss (SNHL) is a major cause of childhood disability. Computed tomography (CT) and magnetic resonance imaging (MRI) imaging play important and often complementary roles in diagnosing underlying structural abnormalities and surgical planning allows for direct visualization of the cochlear nerve and is the preferred imaging modality prior to cochlear implantation. CT is helpful to assess osseous anatomy and when evaluating children with mixed hearing loss or syndromic associations. When reviewing these cases, it is important for the radiologist to be familiar with the key imaging features. In this article, we will present the imaging findings associated with different inner ear malformations associated with congenital sensorineural hearing loss.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Criança , Humanos , Orelha Interna/diagnóstico por imagem , Orelha Interna/anormalidades , Orelha Interna/patologia , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/etiologia , Tomografia Computadorizada por Raios X/métodos , Imageamento por Ressonância Magnética/métodos
5.
Eur Arch Otorhinolaryngol ; 280(12): 5259-5265, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37266754

RESUMO

PURPOSE: Cochlear implantation in patients with vestibular schwannomas is of increasing importance and interest. Two remaining challenges are the assessment of conduction of the cochlear nerve and the possibility of postoperative surveillance with magnetic resonance imaging. The aim of the current study was to assess follow-up imaging and determine the visibility of the internal auditory canal after vestibular schwannoma resection and cochlear implantation as well as in patients with persistent vestibular schwannomas and cochlear implants in place. Visibility of the internal auditory canal, cerebellopontine angle, and labyrinth were evaluated and graded. METHODS: For this retrospective study, 15 MR examinations of 13 patients after translabyrinthine vestibular schwannoma resection and ipsilateral cochlear implantation were included. All patients had been implanted with an MED-EL cochlear implant. Magnetic resonance imaging was carried out on a 1.5T device. All patients were prepped according to the manufacturer's recommendations. RESULTS: All 15 examinations were carried out without any adverse event during imaging, such as pain, magnet dislocation, or malfunction. The internal auditory canal and the cerebellopontine angle were sufficiently visible in all cases to allow for vestibular schwannoma follow-up. CONCLUSION: Magnetic resonance imaging surveillance of the internal auditory canal following vestibular schwannoma resection and cochlear implantation is feasible and safe with modern implants with a 1.5T magnetic resonance imaging device using metal artifact reduction sequences. Necessary follow-up imaging should not be a contraindication for cochlear implantation in patients with vestibular schwannomas.


Assuntos
Implante Coclear , Implantes Cocleares , Orelha Interna , Neuroma Acústico , Humanos , Implante Coclear/métodos , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/cirurgia , Neuroma Acústico/patologia , Estudos Retrospectivos , Orelha Interna/diagnóstico por imagem , Orelha Interna/cirurgia , Orelha Interna/patologia , Imageamento por Ressonância Magnética/métodos
6.
Sci Transl Med ; 15(702): eabq3916, 2023 06 28.
Artigo em Inglês | MEDLINE | ID: mdl-37379370

RESUMO

Inner ear gene therapy has recently effectively restored hearing in neonatal mice, but it is complicated in adulthood by the structural inaccessibility of the cochlea, which is embedded within the temporal bone. Alternative delivery routes may advance auditory research and also prove useful when translated to humans with progressive genetic-mediated hearing loss. Cerebrospinal fluid flow via the glymphatic system is emerging as a new approach for brain-wide drug delivery in rodents as well as humans. The cerebrospinal fluid and the fluid of the inner ear are connected via a bony channel called the cochlear aqueduct, but previous studies have not explored the possibility of delivering gene therapy via the cerebrospinal fluid to restore hearing in adult deaf mice. Here, we showed that the cochlear aqueduct in mice exhibits lymphatic-like characteristics. In vivo time-lapse magnetic resonance imaging, computed tomography, and optical fluorescence microscopy showed that large-particle tracers injected into the cerebrospinal fluid reached the inner ear by dispersive transport via the cochlear aqueduct in adult mice. A single intracisternal injection of adeno-associated virus carrying solute carrier family 17, member 8 (Slc17A8), which encodes vesicular glutamate transporter-3 (VGLUT3), rescued hearing in adult deaf Slc17A8-/- mice by restoring VGLUT3 protein expression in inner hair cells, with minimal ectopic expression in the brain and none in the liver. Our findings demonstrate that cerebrospinal fluid transport comprises an accessible route for gene delivery to the adult inner ear and may represent an important step toward using gene therapy to restore hearing in humans.


Assuntos
Orelha Interna , Adulto , Animais , Humanos , Camundongos , Orelha Interna/patologia , Cóclea , Audição , Terapia Genética/métodos , Técnicas de Transferência de Genes
7.
J Pathol ; 260(3): 353-364, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37256677

RESUMO

Alport syndrome (AS), a type IV collagen disorder, leads to glomerular disease and, in some patients, hearing loss. AS is treated with inhibitors of the renin-angiotensin system; however, a need exists for novel therapies, especially those addressing both major pathologies. Sparsentan is a single-molecule dual endothelin type-A and angiotensin II type 1 receptor antagonist (DEARA) under clinical development for focal segmental glomerulosclerosis and IgA nephropathy. We report the ability of sparsentan to ameliorate both renal and inner ear pathologies in an autosomal-recessive Alport mouse model. Sparsentan significantly delayed onset of glomerulosclerosis, interstitial fibrosis, proteinuria, and glomerular filtration rate decline. Sparsentan attenuated glomerular basement membrane defects, blunted mesangial filopodial invasion into the glomerular capillaries, increased lifespan more than losartan, and lessened changes in profibrotic/pro-inflammatory gene pathways in both the glomerular and the renal cortical compartments. Notably, treatment with sparsentan, but not losartan, prevented accumulation of extracellular matrix in the strial capillary basement membranes in the inner ear and reduced susceptibility to hearing loss. Improvements in lifespan and in renal and strial pathology were observed even when sparsentan was initiated after development of renal pathologies. These findings suggest that sparsentan may address both renal and hearing pathologies in Alport syndrome patients. © 2023 Travere Therapeutics, Inc and The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.


Assuntos
Orelha Interna , Nefrite Hereditária , Animais , Camundongos , Nefrite Hereditária/metabolismo , Receptores de Angiotensina/metabolismo , Receptores de Angiotensina/uso terapêutico , Membrana Basal Glomerular/metabolismo , Colágeno Tipo IV/genética , Orelha Interna/metabolismo , Orelha Interna/patologia , Endotelinas/metabolismo , Endotelinas/uso terapêutico
8.
Otolaryngol Clin North Am ; 56(3): 611-622, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37019772

RESUMO

The future of the management of both sporadic and neurofibromatosis type 2-asscoiated vestibular schwannomas (VSs) will be shaped by cutting-edge technologic and biomedical advances to enable personalized, precision medicine. This scoping review envisions the future by highlighting the most promising developments published, ongoing, planned, or potential that are relevant for VS, including integrated omics approaches, artificial intelligence algorithms, biomarkers, liquid biopsy of the inner ear, digital medicine, inner ear endomicroscopy, targeted molecular imaging, patient-specific stem cell-derived models, ultra-high dose rate radiotherapy, optical imaging-guided microsurgery, high-throughput development of targeted therapeutics, novel immunotherapeutic strategies, tumor vaccines, and gene therapy.


Assuntos
Orelha Interna , Neurofibromatose 2 , Neuroma Acústico , Humanos , Neuroma Acústico/terapia , Inteligência Artificial , Orelha Interna/patologia , Algoritmos
9.
Laryngoscope ; 133(11): 2856-2867, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37078512

RESUMO

OBJECTIVE: Exclusive endoscopic (EETTA) and expanded (ExpTTA) transcanal transpromontorial approaches have shown promising results for treating internal auditory canal (IAC) lesions. We reviewed the literature to answer the question: "Do EETTA and ExpTTA achieve high rates of complete resection and low rates of complications in treating patients with IAC pathologies?" DATA SOURCES: PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched. REVIEW METHODS: Studies reporting EETTA/ExpTTA for IAC pathologies were included. Indications and techniques were discussed and meta-analyzed rates of outcomes and complications were obtained with random-effect model meta-analyses. RESULTS: We included 16 studies comprising 173 patients, all with non-serviceable hearing. Baseline FN function was mostly House-Brackmann-I (96.5%; 95% CI: 94.9-98.1%). Most lesions were vestibular/cochlear schwannomas (98.3%; 95% CI: 96.7-99.8%) of Koos-I (45.9%; 95% CI: 41.3-50.3%) or II (47.1%; 95% CI: 43-51.1%). EETTA was performed in 101 patients (58.4%; 95% CI: 52.4-64.3%) and ExpTTA in 72 (41.6%; 95% CI: 35.6-47.6%), achieving gross-total resection in all cases. Transient complications occurred in 30 patients (17.3%; 95% CI: 13.9-20.5%), with meta-analyzed rates of 9% (95% CI: 4-15%), comprising FN palsy with spontaneous resolution (10.4%; 95% CI: 7.7-13.1%). Persistent complications occurred in 34 patients (19.6%; 95% CI: 17.1-22.2%), with meta-analyzed rates of 12% (95% CI: 7-19%), comprising persistent FN palsy in 22 patients (12.7%; 95% CI: 10.2-15.2%). Mean follow-up was 16 months (range, 1-69; 95% CI: 14.7-17.4). Post-surgery FN function was stable in 131 patients (75.8%; 95% CI: 72.1-79.5%), worsened in 38 (21.9%; 95% CI: 18.8-25%), and improved in 4 (2.3%; 95% CI: 0.7-3.9%), with meta-analyzed rates of improved/stable response of 84% (95% CI: 76-90%). CONCLUSION: Transpromontorial approaches offer newer routes for IAC surgery, but their restricted indications and unfavorable FN outcomes currently limit their use. Laryngoscope, 133:2856-2867, 2023.


Assuntos
Orelha Interna , Neuroma Acústico , Humanos , Estudos Retrospectivos , Orelha Interna/cirurgia , Orelha Interna/patologia , Neuroma Acústico/cirurgia , Neuroma Acústico/patologia , Endoscopia/métodos , Paralisia
10.
Otolaryngol Head Neck Surg ; 169(3): 633-641, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-36758946

RESUMO

OBJECTIVE: To assess the correlation between linear and volumetric changes in vestibular schwannomas (VS). STUDY DESIGN: Retrospective imaging review was performed on patients diagnosed with sporadic VS from 2000 to 2019 who demonstrated linear growth on observation with serial magnetic resonance imaging (MRI). SETTING: Two large tertiary care centers. METHODS: Changes in diameter on serial MRI scans, measured by 1995 American Academy of Otolaryngology-Head and Neck Surgery guidelines, were compared to changes in volume, calculated by segmentation. RESULTS: Ninety-two patients had VS confined to the internal auditory canal (IAC) with 236 MRIs analyzed, and 108 patients had VS involving the cerebellopontine angle (CPA) with 193 MRIs analyzed. The Spearman rank correlation coefficients between changes in diameter and volume for IAC and CPA tumors were 0.43 (p < .001) and 0.65 (p < .001), respectively. Linear diameter increases of 1 to <2 mm corresponded to a median volume change of 32% (interquartile range [IQR]: 6%-86%) for IAC tumors, compared to 23% (IQR: 13%-40%) for CPA tumors. Linear diameter increases of 2 to <3 mm (ie, the minimum linear diameter change classically considered "true growth") corresponded to a median volume change of 42% (IQR: 23%-100%) and 47% (IQR: 26%-69%) for IAC and CPA tumors, respectively. CONCLUSION: Changes in linear diameter significantly correlated with changes in volume for IAC and CPA tumors, although diameter changes that did not meet the definition of linear growth (<2 mm) had corresponding median volume changes in excess of 20% for both IAC and CPA tumors.


Assuntos
Orelha Interna , Neuroma Acústico , Humanos , Neuroma Acústico/patologia , Estudos Retrospectivos , Orelha Interna/patologia , Imageamento por Ressonância Magnética/métodos
11.
Biochem Pharmacol ; 210: 115457, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36806583

RESUMO

NOD-like receptor protein 3 (NLRP3) inflammasomes trigger the inflammatory cascades and participate in various inflammatory diseases, including noise-induced hearing loss (NIHL) caused by oxidative stress. Recently, the anti-inflammatory traditional medicine oridonin (Ori) has been reported to provide hearing protection in mice after noise exposure by blocking the NLRP3-never in mitosis gene A-related kinase 7 (NEK7)-inflammasome complex assembly. Using RNA sequencing analysis, we further elucidated that interleukin 1 receptor type 2 (IL1R2) may be another crucial factor regulated by Ori to protect NIHL. We observed that IL1R2 expression was localized in spiral ganglion neurons, inner and outer hair cells, in Ori-treated mouse cochleae. Additionally, we confirmed that ectopic overexpression of IL1R2 in the inner ears of healthy mice using an adeno-associated virus delivery system significantly reduced noise-induced ribbon synapse lesions and hearing loss by blocking the "cytokine storm" in the inner ear. This study provides a novel theoretical foundation for guiding the clinical treatment of NIHL.


Assuntos
Orelha Interna , Perda Auditiva Provocada por Ruído , Otite , Camundongos , Animais , Perda Auditiva Provocada por Ruído/tratamento farmacológico , Perda Auditiva Provocada por Ruído/etiologia , Perda Auditiva Provocada por Ruído/patologia , Proteína 3 que Contém Domínio de Pirina da Família NLR/metabolismo , Orelha Interna/metabolismo , Orelha Interna/patologia , Inflamação/complicações , Anti-Inflamatórios/farmacologia , Otite/complicações , Receptores de Interleucina-1
12.
Acta Otolaryngol ; 143(3): 250-253, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36639139

RESUMO

BACKGROUND: Otosclerosis is a common ear disease that causes fixation of the stapes and conductive hearing impairment. However, the pathogenesis of otosclerosis is still unknown. Otosclerosis could be associated with the unique bony environment found in the otic capsule. Normal bone remodelling is almost completely absent around the inner ear after birth allowing degenerative changes and dead osteocytes to accumulate. High levels of inner ear anti resorptive osteoprotegerin (OPG) is most likely responsible for this capsular configuration. Studies have demonstrated how osteocyte lifespan variation creates occasional clusters of dead osteocytes, so-called cellular voids, at otosclerotic predilection sites in the human otic capsule. These cellular voids have been suggested as possible starting points of otosclerosis. AIM: To describe the cellular viability in otosclerotic lesions and compare it to that of cellular voids. MATERIALS AND METHODS: The study was based on unbiased stereological quantifications in undecalcified human temporal bones with otosclerosis. RESULTS: Osteocyte viability was found to vary within the otosclerotic lesions. Furthermore, the results presented here illustrate that inactive otosclerotic lesions consist of mainly dead interstitial bone, much like cellular voids. CONCLUSIONS AND SIGNIFICANCE: Focal degeneration in the otic capsule may play an important role in the pathogenesis of otosclerosis.


Assuntos
Orelha Interna , Osteócitos , Osteoprotegerina , Otosclerose , Humanos , Remodelação Óssea/genética , Remodelação Óssea/fisiologia , Sobrevivência Celular/genética , Sobrevivência Celular/fisiologia , Orelha Interna/metabolismo , Orelha Interna/patologia , Osteócitos/metabolismo , Osteócitos/patologia , Osteoprotegerina/genética , Osteoprotegerina/metabolismo , Otosclerose/etiologia , Otosclerose/genética , Otosclerose/metabolismo , Otosclerose/patologia , Estribo/metabolismo , Estribo/patologia , Osso Temporal/metabolismo , Osso Temporal/patologia
13.
Laryngoscope ; 133(2): 282-286, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-35819874

RESUMO

Axial sections from preoperative magnetic resonance imaging without contrast, showing a cone-shaped lesion of the internal auditory canal, extending toward the most lateral part of the cerebello-pontine angle. (A) T1-weighted high-resolution isotropic volume excitation (THRIVE) sequence; (B) T1-weighted sequence; (C) Fluid attenuated inversion recovery (FLAIR) sequence. Laryngoscope, 133:282-286, 2023.


Assuntos
Orelha Interna , Neuroma Acústico , Humanos , Neuroma Acústico/cirurgia , Neuroma Acústico/patologia , Endoscopia/métodos , Orelha Interna/patologia , Osso Petroso , Cerebelo , Imageamento por Ressonância Magnética
14.
Laryngoscope ; 133(4): 914-923, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-35766261

RESUMO

OBJECTIVES: 1. Determine the feasibility and efficiency of local magnetic targeting delivery of gadolinium (Gad) contrast to the inner ear in rodents. 2. Assess any potential ototoxicity of magnetic targeting delivery of Gad in the inner ear. 3. Study the utility of magnetic targeting delivery of Gad to visualize and quantify endolymphatic hydrops (EH) in a transgenic mouse model. STUDY DESIGN: Controlled in vivo animal model study. METHODS: Paramagnetic Gad was locally delivered to the inner ear using the magnetic targeting technique in both rat and mouse models. Efficiency of contrast delivery was assessed using magnetic resonance imaging (MRI). Ototoxicity of Gad was examined with histology of the cochlea and functional audiological tests. The Phex mouse model was used to study EH, hearing loss, and balance dysfunction. Magnetic targeting delivery of Gad contrast was used in the Phex mouse model to visualize the effects of EH using MRI. RESULTS: Magnetic targeting improved the delivery of Gad to the inner ear and the technique was reproducible in both rat and mouse models. The delivery method did not result in microstructural damage or any significant hearing loss in a normal animal. Magnetic targeting of Gad in the Phex mouse model allowed detailed visualization and quantification of EH. CONCLUSION: This study provided the first evidence of the effectiveness and efficiency of the local magnetic targeting delivery of gadolinium contrast to the inner ear and its application to the visualization and quantification of EH. Laryngoscope, 133:914-923, 2023.


Assuntos
Surdez , Orelha Interna , Hidropisia Endolinfática , Ototoxicidade , Camundongos , Ratos , Animais , Gadolínio , Meios de Contraste , Hidropisia Endolinfática/diagnóstico por imagem , Orelha Interna/diagnóstico por imagem , Orelha Interna/patologia , Imageamento por Ressonância Magnética/métodos , Modelos Animais de Doenças , Camundongos Transgênicos
15.
HNO ; 71(2): 92-99, 2023 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-36326852

RESUMO

BACKGROUND: Otosclerosis is an osteodystrophy of the otic capsule and presents with progressive conductive hearing loss. Imaging studies, especially computed tomography (CT) and cone-beam CT, have gained increased relevance in the diagnosis of otosclerosis. OBJECTIVE: This study investigated whether there is a correlation between the extent of otosclerosis in high-resolution or cone-beam CT and hearing loss in pure-tone audiometry. MATERIALS AND METHODS: Based on an existing classification of otosclerotic foci, a classification was established. Preoperative CT scans of patients undergoing stapedotomy between 2015 and 2019 were evaluated and classified by two independent otorhinolaryngologists. The preoperative pure-tone audiograms were analysed and compared to the results of CT. RESULTS: A total of 168 CT studies (i.e., 168 ears) in 156 patients with intraoperatively confirmed otosclerosis were included in our study. A correlation between the extent of the otosclerotic focus or the calculated scores and hearing loss in pure-tone audiometry (air conduction, bone conduction and air-bone-gap) could not be proven. CONCLUSION: Preoperative CT is not obligatory. However, preoperative imaging using CT or cone-beam CT can be helpful to confirm the diagnosis and exclude other middle or inner ear pathologies as well as in planning of the surgical procedure in the overall context of otoscopy and audiometry. A correlation with the degree of hearing impairment could not be demonstrated and remains unclear.


Assuntos
Surdez , Orelha Interna , Perda Auditiva , Otosclerose , Cirurgia do Estribo , Humanos , Otosclerose/diagnóstico por imagem , Otosclerose/cirurgia , Perda Auditiva Condutiva/diagnóstico , Perda Auditiva/cirurgia , Audiometria de Tons Puros , Cirurgia do Estribo/métodos , Orelha Interna/patologia , Tomografia Computadorizada por Raios X , Surdez/cirurgia , Estudos Retrospectivos
16.
Hear Res ; 426: 108633, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36288662

RESUMO

CHARGE syndrome is a multiple anomaly developmental disorder characterized by a variety of sensory deficits, including sensorineural hearing loss of unknown etiology. Most cases of CHARGE are caused by heterozygous pathogenic variants in CHD7, the gene encoding Chromodomain DNA-binding Protein 7 (CHD7), a chromatin remodeler important for the development of neurons and glial cells. Previous studies in the Chd7Gt/+ mouse model of CHARGE syndrome showed substantial neuron loss in the early stages of the developing inner ear that are compensated for by mid-gestation. In this study, we sought to determine if early developmental delays caused by Chd7 haploinsufficiency affect neurons, glial cells, and inner hair cell innervation in the mature cochlea. Analysis of auditory brainstem response recordings in Chd7Gt/+ adult animals showed elevated thresholds at 4 kHz and 16 kHz, but no differences in ABR Wave I peak latency or amplitude compared to wild type controls. Proportions of neurons in the Chd7Gt/+ adult spiral ganglion and densities of nerve projections from the spiral ganglion to the organ of Corti were not significantly different from wild type controls. Inner hair cell synapse formation also appeared unaffected in mature Chd7Gt/+ cochleae. However, histological analysis of adult Chd7Gt/+ cochleae revealed diminished satellite glial cells and hypermyelinated Type I spiral ganglion axons. We characterized the expression of CHD7 in developing inner ear glia and found CHD7 to be expressed during a tight window of inner ear development at the Schwann cell precursor stage at E9.5. While cochlear neurons appear to differentiate normally in the setting of Chd7 haploinsufficiency, our results suggest an important role for CHD7 in glial cells in the inner ear. This study highlights the dynamic nature of CHD7 activity during inner ear development in mice and contributes to understanding CHARGE syndrome pathology.


Assuntos
Síndrome CHARGE , Orelha Interna , Camundongos , Animais , Gânglio Espiral da Cóclea/patologia , Síndrome CHARGE/genética , Síndrome CHARGE/patologia , Cromatina , Orelha Interna/patologia , Neuroglia , Proteínas de Ligação a DNA/genética
17.
Mol Genet Metab ; 137(3): 223-229, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36183456

RESUMO

Damages to the ear are very diverse and can depend on the type of inherited metabolic diseases (IMD). Indeed, IMDs can affect all parts of the auditory system, from the outer ear to the central auditory process. We have identified 219 IMDs associated with various types of ear involvement which we classified into five groups according to the lesion site of the auditory system: congenital external ear abnormalities, acquired external ear abnormalities, middle ear involvement, inner ear or retrocochlear involvement, and unspecified hearing loss. This represents the ninth issue in a series of educational summaries providing a comprehensive and updated list of metabolic differential diagnoses according to system involvement.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Perda Auditiva , Doenças Metabólicas , Humanos , Orelha Interna/patologia , Perda Auditiva/genética , Doenças Metabólicas/genética , Doenças Metabólicas/patologia , Perda Auditiva Neurossensorial/patologia
18.
Otol Neurotol ; 43(10): e1168-e1173, 2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-36136609

RESUMO

OBJECTIVE: To describe the natural history of primary inner ear schwannomas over a long follow-up period. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Patients with primary inner ear schwannomas with serial audiometric and radiologic follow-up. MAIN OUTCOME MEASURES: Patterns of hearing loss, rate of hearing decline, presence of vestibular symptoms, and rate of tumor growth. RESULTS: A total of 12 patients with 13 tumors were identified. The mean duration of follow-up was 7 years. Forty-six percent of tumors were intracochlear, 15% were intravestibular, 23% were transmodiolar, and 15% were intravestibular-cochlear. Hearing loss was the most common presenting symptom, occurring in all patients. Among patients with serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery Class A or B) at the time of presentation, the average time to decline to a nonserviceable hearing level was 57.3 months (range, 21-117 mo). Hearing loss was sudden in 31% of patients, progressive in 61% and fluctuating in 8%. No patients had intractable vertigo; however, two required vestibular physiotherapy. On initial magnetic resonance imaging, the mean largest tumor dimension was 3.1 mm (standard deviation, 1.2 mm), and the mean largest dimension on most recent magnetic resonance imaging was 4.4 mm (standard deviation, 1.1 mm). Two tumors exhibited no growth over a follow-up of 11.3 and 2.8 years, respectively. Overall, the mean growth was 0.25 mm per year followed. Two patients underwent cochlear implantation with simultaneous tumor resection and had favorable outcomes. CONCLUSION: Long-term follow-up suggests a conservative approach, with possible hearing rehabilitation at the time of deterioration, is a safe management strategy for primary inner ear schwannomas.


Assuntos
Orelha Interna , Perda Auditiva , Neurilemoma , Neuroma Acústico , Humanos , Neuroma Acústico/complicações , Neuroma Acústico/cirurgia , Neuroma Acústico/patologia , Seguimentos , Estudos Retrospectivos , Orelha Interna/patologia , Neurilemoma/patologia , Perda Auditiva/etiologia , Perda Auditiva/cirurgia , Perda Auditiva/patologia , Resultado do Tratamento
19.
J Int Adv Otol ; 18(5): 388-391, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36063094

RESUMO

BACKGROUND: A comparative study of 1.5T and 3.0T magnetic resonance imaging inner ear gadolinium enhancement was carried out to further explore the practicality and universality of 1.5T magnetic resonance imaging in the diagnosis of inner ear labyrinthine hydrops positive imaging. METHODS: This dual case-control study was conducted on 25 patients with Meniere's disease (experimental group), diagnosed by People's Hospital of Ordos Dongsheng District between April 2017 and April 2019 and 51 patients with Meniere's disease (control group), diagnosed by People's Hospital Affiliated to Fujian University of Traditional Chinese Medicine between March 2010 and February 2011 and published on Chinese Medical Journal in 2011. Both groups were injected with gadolinium diluent into bilateral tympanic chambers through the tympanic membrane, and 3 dimensional-Fluid Attenuated Inversion Recovery (FLAIR) magnetic resonance imaging scanning of the inner ear was performed 24 hours later. The results of the 2 groups were observed, calculated, and statistically processed. RESULTS: The positive rate of membranous labyrinthine hydrops was 96% (24/25) in the experimental group and 96.1% (49/51) in the control group. The results are very close. CONCLUSION: In clinical diagnoses of Meniere's disease, 1.5T magnetic resonance imaging and 3.0T magnetic resonance imaging have the same value and significance.


Assuntos
Orelha Interna , Hidropisia Endolinfática , Doença de Meniere , Estudos de Casos e Controles , Meios de Contraste , Orelha Interna/diagnóstico por imagem , Orelha Interna/patologia , Edema , Hidropisia Endolinfática/diagnóstico por imagem , Gadolínio , Gadolínio DTPA , Humanos , Imageamento por Ressonância Magnética/métodos , Doença de Meniere/diagnóstico por imagem , Doença de Meniere/patologia
20.
Otol Neurotol ; 43(9): e957-e962, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-36075107

RESUMO

INTRODUCTION: Internal auditory canal (IAC) diverticula, also known as IAC cavitary lesions or anterior cupping of the IAC, observed in otopathologic specimens and high-resolution computed tomography (CT) scans of the temporal bone are thought to be related to otosclerosis. Herein, we examined the usefulness of CT scans in identifying diverticula and determined whether IAC diverticula are associated with otosclerosis on otopathology. METHODS: One hundred five consecutive specimens were identified from the National Temporal Bone Hearing and Balance Pathology Resource Registry. Inclusion criteria included the availability of histologic slides and postmortem specimen CT scans. Exclusion criteria included cases with severe postmortem changes or lesions causing bony destruction of the IAC. RESULTS: Ninety-seven specimens met criteria for study. Of these, 42% of the specimens were from male patients, and the average age of death was 77 years (SD = 18 yr). IAC diverticula were found in 48 specimens, of which 46% were identified in the CT scans. The mean area of the IAC diverticula was 0.34 mm 2 . The sensitivity and specificity of detecting IAC diverticula based on CT were 77% and 63%, respectively. Overall, 27% of specimens had otosclerosis. Histologic IAC diverticula were more common in specimens with otosclerosis than those without (37.5% versus 16%; p = 0.019). Cases with otosclerosis had a greater mean histologic diverticula area compared with nonotosclerosis cases (0.69 mm 2 versus 0.14 mm 2 ; p = 0.001). CONCLUSION: IAC diverticula are commonly found in otopathologic specimens with varied etiologies, but larger diverticula are more likely to be associated with otosclerosis. The sensitivity and specificity of CT scans to detect IAC diverticula are limited.


Assuntos
Divertículo , Orelha Interna , Otosclerose , Idoso , Divertículo/complicações , Divertículo/diagnóstico por imagem , Orelha Interna/patologia , Humanos , Masculino , Otosclerose/complicações , Otosclerose/diagnóstico por imagem , Osso Petroso/patologia , Osso Temporal/patologia , Tomografia Computadorizada por Raios X/métodos
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