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1.
J Laryngol Otol ; 134(4): 366-368, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32106896

RESUMO

BACKGROUND: Individuals with superior semi-circular canal syndrome often describe vestibular symptoms elicited by loud sounds, as well as other pressure-induced symptoms. They also often report other symptoms, including autophony, hyperacusis, cognitive dysfunction, spatial disorientation, anxiety and migraine headaches. Symptoms occur due to the presence of a 'third window' created by the dehiscence of the superior semi-circular canal. This case report describes a minimally invasive technique to provide soft reinforcement of the round window. CASE REPORT: Our patient underwent a permeatal procedure whereby the tympanic membrane was raised to allow inspection of the middle ear. The round window niche was identified and the round window membrane was reinforced with fat. The mucosa of the bony meatus leading to the round window was then disrupted before the application of a double layer of perichondrium to allow further reinforcement. CONCLUSION: The case provides support for the use of 'soft reinforcement' as a simple and effective technique to treat the symptoms of superior canal dehiscence syndrome.


Assuntos
Orelha Interna/cirurgia , Janela da Cóclea/cirurgia , Canais Semicirculares/cirurgia , Orelha Interna/patologia , Orelha Interna/fisiopatologia , Orelha Média/cirurgia , Feminino , Humanos , Hiperacusia/diagnóstico , Hiperacusia/etiologia , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Canais Semicirculares/patologia , Síndrome , Osso Temporal/diagnóstico por imagem , Zumbido/diagnóstico , Zumbido/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Potenciais Evocados Miogênicos Vestibulares/fisiologia
2.
J Comp Pathol ; 172: 93-106, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31690422

RESUMO

Hearing represents the major sense in harbour porpoises (Phocoena phocoena) and impairment of hearing has a great impact on the survival of these animals. In this communication, some anatomical and histological aspects of the tympanoperiotic complex of harbour porpoises are presented. In addition, the ears of 21 incidentally bycaught or stranded freshly dead harbour porpoises of different age groups and sex were investigated histologically. At the entrance to the middle ear cavity, mucosa-associated lymphoid tissue was present that was often hyperplastic in juvenile (9/10) and adult individuals (7/8). Solitary lymphoid follicles were additionally found in the corpus cavernosum and adjacent to the stapedius muscle in single porpoises. The nematode Stenurus minor represented the most common pathogen observed in the middle ear cavity of juvenile and adult harbour porpoises and the parasite was associated with chronic inflammation with metaplastic and hyperplastic epithelial changes. An unusual bone formation at the attachment of the corpus cavernosum to the perioticum was a common finding, even in young individuals. Whether this represents a normal structure or a metaplastic change remains undetermined. Acute haemorrhages in the cochlea and/or the tympanic cavity occurred in all animals and were most likely agonal changes. Single porpoises suffered from purulent otitis media, mycotic otitis media with osteolysis or chronically fractured tympanic bones, likely causing impairment of hearing that may have contributed to by-catch. There was no evidence that stranding in five porpoises was associated with the aural changes. Histological examination of the ears in harbour porpoises is a valuable part of the assessment of their health status. Damage to hearing structures may explain starvation due to impaired ability to catch prey or unusual behaviour such as stranding or entanglement in nets.


Assuntos
Orelha Interna , Otite/veterinária , Phocoena , Animais , Orelha Interna/anatomia & histologia , Orelha Interna/parasitologia , Orelha Interna/patologia , Masculino , Infecções por Nematoides/patologia , Infecções por Nematoides/veterinária , Otite/parasitologia , Otite/patologia
3.
Laryngoscope ; 129(11): 2574-2579, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31633822

RESUMO

OBJECTIVE: To investigate the relations of monoallelic (M1), biallelic (M2), or the absence of mutations (M0) in SLC26A4 to inner ear morphology and hearing levels in individuals with Pendred syndrome (PS) or nonsyndromic enlarged vestibular aqueduct (NSEVA) associated with hearing loss. METHODS: In a cohort of 139 PS/NSEVA individuals, 115 persons from 95 unrelated families had full genetic sequencing of SLC26A4, and 113 had retrievable images for re-assessment of inner ear morphology. The association between the number of mutant alleles in SLC26A4, inner ear morphology (including endolymphatic sac size and protein content on magnetic resonance imaging), and hearing level (pure tone average) was explored. RESULTS: Biallelic SLC26A4 mutations (M2) occurred in three-quarters of the cohort and was invariably associated with poor hearing; in 87%, it was associated with incomplete partition type II of the cochlea as well as enlarged endolymphatic sac and vestibular aqueduct. M1 or M0 individuals exhibited a greater variability in inner ear morphology. Endolymphatic sac size and presence of "high-protein" sac contents were significantly higher in M2 individuals compared to M1 and M0 individuals. CONCLUSION: The number of SLC26A4 mutations is associated with severity and variability of inner ear morphology and hearing level in individuals with PS or NSEVA. M2 individuals have poorer hearing and present largely incomplete partition type II of the cochleas with enlarged endolymphatic sacs, whereas individuals with M1 and no detectable SLC26A4 mutations have less severe hearing loss and more diverse inner ear morphology. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2574-2579, 2019.


Assuntos
Bócio Nodular/genética , Perda Auditiva Neurossensorial/genética , Transportadores de Sulfato/genética , Aqueduto Vestibular/anormalidades , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Alelos , Criança , Cóclea/patologia , Orelha Interna/patologia , Saco Endolinfático/patologia , Feminino , Bócio Nodular/patologia , Audição/genética , Perda Auditiva Neurossensorial/patologia , Testes Auditivos , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Estudos Retrospectivos , Aqueduto Vestibular/patologia , Adulto Jovem
4.
World Neurosurg ; 132: e223-e227, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31493598

RESUMO

OBJECTIVE: This study focused on the changes in the internal acoustic canal (IAC) caused by vestibular schwannomas (VSs) and their prognostic significance for postoperative hearing outcome. METHODS: A total of 125 consecutive cases of VS were included. We used a neuronavigation software to perform the following measurements on both the tumor side and healthy side: volume of the IAC (VIAC), maximal diameter of the IAC (DIAC), and length of the IAC (LIAC). A statistical analysis was realized using Spearman correlation to test the correlation of the morphometric measure of the IAC and postoperative hearing. Multivariate analysis was performed to test the impact of measurements of the IAC and preoperative hearing on postoperative hearing. RESULTS: The mean VIAC on the tumor side and on the healthy side was 0.271 and 0.169 cm3, respectively. The mean DIAC was 9.438 mm on the tumor side and 7.034 mm contralateral. The correlations tests showed significant correlations of both postoperative hearing deficit and degree of hearing loss with 1) VIAC on the tumor side, 2) difference between VIAC on the tumor side and healthy side, 3) DIAC on the tumor side, and 4) difference between the DIAC on the tumor side and healthy side. The multivariate analysis showed significant impact of the DIAC (P = 0.01) and preoperative hearing status (P = 0.02) on postoperative hearing. CONCLUSIONS: Enlargement of the VIAC and DIAC are negative prognostic factors for hearing preservation. Reasons may be long-standing compression of the auditory nerve and an increased vulnerability of the inner ear structures during the drilling of the IAC.


Assuntos
Orelha Interna/patologia , Perda Auditiva/etiologia , Neuroma Acústico/patologia , Neuroma Acústico/cirurgia , Adulto , Idoso , Orelha Interna/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neuronavegação , Prognóstico , Adulto Jovem
5.
Eur Arch Otorhinolaryngol ; 276(12): 3275-3280, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31486934

RESUMO

PURPOSE: To determine the clinical significance of vascular loops (VL) in the internal auditory meatus (IAM) and cerebellopontine angle (CPA). METHODS: We carried out a retrospective case series in a tertiary referral centre. Out of 6978 patients undergoing magnetic resonance imaging (MRI) of the IAM for unilateral cochleovestibular symptoms we identified the ones with VLs and reviewed their medical notes. We performed a statistical correlation between the laterality of the VL in the IAM/ CPA as graded according to the Chavda classification (type 1 in the CPA, type 2 extending in the IAM, type 3 extending to the distal IAM end), the laterality of symptoms and the patient's age. RESULTS: A total of 77 VL were identified in 64 patients (0.9%); 39 patients had the VL on the same side of the main symptom, while 25 patients had the VL on the contralateral side. There were 37 Type 1 loops, 29 Type 2 loops and 11 Type 3 loops. The comparison between the grading of the VL and the laterality of symptoms did not reach the level of significance (p = 0.321). There was also no association between the presence of the loop and the patients' age (p = 0.5). All patients were reassured and discharged without any representation in three years follow-up. CONCLUSIONS: We did not identify any significant correlation between the laterality of VLs and the laterality of symptoms, irrespective of the grading of the loop or the patients' age. Such VLs should be considered an incidental rather than causal findings.


Assuntos
Ângulo Cerebelopontino/diagnóstico por imagem , Orelha Interna/diagnóstico por imagem , Perda Auditiva/diagnóstico , Imagem por Ressonância Magnética/métodos , Zumbido/diagnóstico , Adulto , Idoso , Ângulo Cerebelopontino/patologia , Orelha Interna/patologia , Feminino , Lateralidade Funcional , Humanos , Achados Incidentais , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
7.
Genes (Basel) ; 10(7)2019 07 12.
Artigo em Inglês | MEDLINE | ID: mdl-31336982

RESUMO

Labyrinthine aplasia, microtia, and microdontia (LAMM) is an autosomal recessive condition causing profound congenital deafness, complete absence of inner ear structures (usually Michel's aplasia), microtia (usually type 1) and microdontia. To date, several families have been described with this condition and a number of mutations has been reported. We report on eight further cases of LAMM syndrome including three novel mutations, c. 173T>C p.L58P; c. 284G>A p.(Arg95Gln) and c.325_327delinsA p.(Glu109Thrfs*18). Congenital deafness was the primary presenting feature in all affected individuals and consanguinity in all but two families. We compare the features in our patients to those previously reported in LAMM, and describe a milder, asymmetrical phenotype associated with FGF3 mutations.


Assuntos
Microtia Congênita/genética , Microtia Congênita/patologia , Orelha Interna/anormalidades , Fator 3 de Crescimento de Fibroblastos/genética , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/patologia , Anormalidades Dentárias/genética , Anormalidades Dentárias/patologia , Adulto , Pré-Escolar , Consanguinidade , Análise Mutacional de DNA , Surdez/congênito , Orelha Interna/patologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Mutação , Fenótipo
8.
Stem Cell Reports ; 13(1): 147-162, 2019 07 09.
Artigo em Inglês | MEDLINE | ID: mdl-31204303

RESUMO

Mutations in the gene encoding the type II transmembrane protease 3 (TMPRSS3) cause human hearing loss, although the underlying mechanisms that result in TMPRSS3-related hearing loss are still unclear. We combined the use of stem cell-derived inner ear organoids with single-cell RNA sequencing to investigate the role of TMPRSS3. Defective Tmprss3 leads to hair cell apoptosis without altering the development of hair cells and the formation of the mechanotransduction apparatus. Prior to degeneration, Tmprss3-KO hair cells demonstrate reduced numbers of BK channels and lower expressions of genes encoding calcium ion-binding proteins, suggesting a disruption in intracellular homeostasis. A proteolytically active TMPRSS3 was detected on cell membranes in addition to ER of cells in inner ear organoids. Our in vitro model recapitulated salient features of genetically associated inner ear abnormalities and will serve as a powerful tool for studying inner ear disorders.


Assuntos
Orelha Interna/patologia , Células Ciliadas Auditivas Internas/patologia , Proteínas de Membrana/genética , Organoides/citologia , Serina Proteases/genética , Animais , Apoptose/genética , Membrana Celular/metabolismo , Códon sem Sentido , Técnicas de Inativação de Genes , Homeostase/genética , Humanos , Canais de Potássio Ativados por Cálcio de Condutância Alta/metabolismo , Mecanotransdução Celular , Proteínas de Membrana/metabolismo , Camundongos , Análise de Sequência de RNA , Serina Proteases/metabolismo , Análise de Célula Única
9.
Ann Otol Rhinol Laryngol ; 128(6_suppl): 61S-68S, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31092027

RESUMO

OBJECTIVES: The aims of this study were to clarify the clinical value of the bony cochlear nerve canal (BCNC) and internal auditory canal (IAC) in children with bilateral sensorineural hearing loss (b-SNHL) and to reveal the correlation between these parameters and outcomes after cochlear implantation (CI). METHODS: Ninety-four ears with b-SNHL that received CI and 100 ears with normal hearing were enrolled. Parameters of IAC and pre- and post-CI categories of auditory performance scores were analyzed. RESULTS: The width of the BCNC and the width, height, and length of the IAC were shorter in the b-SNHL group. BCNC and IAC width were associated with b-SNHL. The calculated cutoff values for BCNC and IAC width were 2.055 mm in the BCNC and 4.245 mm in the IAC, setting the sensitivity to 90%. Patients with narrow BCNCs and IACs had significantly worse post-CI auditory performance. CONCLUSIONS: BCNC and IAC widths were narrower in children with b-SNHL than in normal-hearing children. Narrow BCNC and IAC width had a negative impact on post-CI outcomes. The proposed cutoff values for BCNC and IAC width were meaningful when predicting the auditory outcome after CI, especially considering both.


Assuntos
Implante Coclear , Orelha Interna/patologia , Perda Auditiva Neurossensorial/terapia , Fatores Etários , Limiar Auditivo , Estudos de Casos e Controles , Criança , Pré-Escolar , Nervo Coclear , Feminino , Perda Auditiva Neurossensorial/patologia , Humanos , Masculino , Valor Preditivo dos Testes , Resultado do Tratamento
10.
Eur Arch Otorhinolaryngol ; 276(5): 1335-1340, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30887165

RESUMO

PURPOSE: To clarify the anatomical distribution of otosclerotic loci in otosclerosis. METHODS: Ninety-five patients with surgically confirmed uni- or bilateral otosclerosis were enrolled into the study. Hypodense areas observed in the otic capsule by high-resolution computed tomography (HRCT) were defined as otosclerotic loci. The location and number of lesions were examined, and the probability of lesion overlap and correlation with age/hearing parameters (air and bone conduction threshold, air-bone gaps) were tested. RESULTS: Otosclerotic loci were confirmed by HRCT in 77 out of 115 operated ears. The three commonly affected sites were the anterior part of the oval window (ant-OW), anterior part of the internal auditory canal (ant-IAC), and pericochlear area (PCochA), with lesions detected in 96.1%, 46.8%, and 26.0% of ears, respectively. Only the ant-OW area was affected in 48.1% of the ears; the ant-IAC in 3.9%; and PCochA in none with significant differences (p < 0.01). The ant-OW lesions preferentially overlapped with ant-IAC (44.6%) than PCochA lesions (27.0%) (p < 0.05). Among double sites diseases, triple sites diseases occurred more commonly in the ant-OW + PCochA group (80%) than ant-OW + ant-IAC group (48.5%) (p < 0.05). There was no correlation between a number of lesions and age/hearing parameters. CONCLUSIONS: Based on the probability of lesion overlap, otosclerotic lesions may initiate at ant-OW followed by ant-IAC and later PCochA. Although the number of lesions showed no immediate correlation with hearing level or age, anatomical stage of the disease estimated by the location and the number of otosclerotic loci could be useful in predicting the future hearing status.


Assuntos
Orelha Interna , Orelha Média , Otosclerose , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Condução Óssea , Cóclea/diagnóstico por imagem , Cóclea/patologia , Correlação de Dados , Orelha Interna/diagnóstico por imagem , Orelha Interna/patologia , Orelha Média/diagnóstico por imagem , Orelha Média/patologia , Feminino , Perda Auditiva Condutiva/diagnóstico , Perda Auditiva Condutiva/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Otosclerose/patologia , Otosclerose/fisiopatologia , Gravidade do Paciente
11.
Sci Transl Med ; 11(482)2019 03 06.
Artigo em Inglês | MEDLINE | ID: mdl-30842313

RESUMO

Permanent hearing loss affects more than 5% of the world's population, yet there are no nondevice therapies that can protect or restore hearing. Delivery of therapeutics to the cochlea and vestibular system of the inner ear is complicated by their inaccessible location. Drug delivery to the inner ear via the vasculature is an attractive noninvasive strategy, yet the blood-labyrinth barrier at the luminal surface of inner ear capillaries restricts entry of most blood-borne compounds into inner ear tissues. Here, we compare the blood-labyrinth barrier to the blood-brain barrier, discuss invasive intratympanic and intracochlear drug delivery methods, and evaluate noninvasive strategies for drug delivery to the inner ear.


Assuntos
Sistemas de Liberação de Medicamentos , Orelha Interna/patologia , Preparações Farmacêuticas/administração & dosagem , Animais , Cóclea/irrigação sanguínea , Perda Auditiva/patologia , Humanos , Permeabilidade
12.
Biosci Rep ; 39(3)2019 03 29.
Artigo em Inglês | MEDLINE | ID: mdl-30842343

RESUMO

To investigate the possible association of pathogenic mutations of SLC26A4 and computerized tomography (CT) phenotypes of inner ear, and explore the feasibility of using the method of gene sequence analysis. A total of 155 patients with bilateral hearing loss carrying SLC26A4 gene mutations were further subjected to high-resolution temporal bone CT and thyroid B ultrasound tests. The potential relationship between the pathogenic mutations of gene and the CT phenotypes were analyzed. As a result, 65 patients (41.9%, 65/155) carried SLC26A4 gene mutations, and 27 cases were detected with pathogenic mutations of SLC26A4 where IVS7-2A>G (55.6%, 15/27) was the most common pathogenic mutation. Amongst them, 19 patients carrying bi-allelic SLC26A4 mutations were all confirmed to have inner ear malformation by CT scan including four cases of enlarged vestibular aqueduct (EVA) and 15 cases of Mondini dysplasia (MD). However, there was only one in eight cases of single allele pathogenic mutation who was confirmed to have EVA by CT scan. Further, only one patient with EVA was confirmed to be slightly higher of total T3 than normal by thyroid ultrasound scan and thyroid hormone assays. These findings suggested that CT detection and SLC26A4 gene detection are efficient methods to diagnose EVA, which can complement each other. Also, the bi-allelic pathogenic mutations of SLC26A4 are more likely to induce inner ear malformation than single allele pathogenic mutation.


Assuntos
Orelha Interna/metabolismo , Perda Auditiva Neurossensorial/genética , Mutação , Transportadores de Sulfato/genética , Osso Temporal/metabolismo , Aqueduto Vestibular/anormalidades , Adolescente , Alelos , Criança , Análise Mutacional de DNA , Orelha Interna/diagnóstico por imagem , Orelha Interna/patologia , Feminino , Genótipo , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Masculino , Fenótipo , Osso Temporal/diagnóstico por imagem , Osso Temporal/patologia , Tomografia Computadorizada por Raios X/métodos , Tri-Iodotironina/metabolismo , Adulto Jovem
13.
Clin Otolaryngol ; 44(3): 336-342, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30729710

RESUMO

OBJECTIVE: The aim of this study was to investigate the clinical significance of 4-hour delayed-enhanced 3.0 Tesla (3T) 3D-fluid-attenuated inversion recovery (FLAIR) MR imaging in sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Case series with comparisons. SETTING: Tertiary referral centre. PARTICIPANTS: Eighty-seven idiopathic SSNHL patients were enrolled between January 2015 and December 2016 and received high dose steroid therapy and intratympanic steroid injections as salvage treatment. INTERVENTION: Pre-contrast, 10-minute and 4-hour delayed-enhanced 3D-FLAIR MR images were obtained using double-dose IV gadolinium. MAIN OUTCOME MEASURES: The results of treatment were evaluated according to Siegel's criteria 3 months after the start of treatment. Where possible lesion-side laterality of the inner ear was identified based on the MR images, the associations between MR findings and other clinical parameters were analysed, and the relationships between hearing recovery and MR image findings were assessed. RESULTS: Lesion-side laterality was identified on MRI in 52 (59.7%), 18 (20.1%) and 8 (9.2%) patients, based on 4-hour delayed, 10-minute delayed, and pre-contrast images, respectively. The hearing recovery rate was significantly lower in the patients with lesion-side laterality on 4-hour delayed images (P < 0.001). In a multivariate analysis, lesion-side laterality on 4-hour delayed images was associated with poor prognosis (OR = 5.6) after adjusting other prognostic factors including initial hearing level, lesion-side laterality on 10-min delayed images and presence of vertigo. In addition, as the extent of enhancement in the inner ear increased the probability of hearing recover decreased (P = 0.001). CONCLUSIONS: Contrast enhancement of inner ear structures can be seen on 4-hour delayed-enhanced 3T 3D-FLAIR MR images in idiopathic SSNHL. Asymmetric lesion-side enhancement of the inner ear may be associated with a poor prognosis.


Assuntos
Orelha Interna/patologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Súbita/diagnóstico , Audição/fisiologia , Imageamento Tridimensional/métodos , Imagem por Ressonância Magnética/métodos , Feminino , Seguimentos , Perda Auditiva Neurossensorial/fisiopatologia , Perda Auditiva Súbita/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Fatores de Tempo
14.
PLoS One ; 14(2): e0212855, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30807592

RESUMO

This study proposes a practical model for a new approach to the posterior fossa in common domestic pigs. Several surgical procedures can be simulated in the nonliving pig model, including soft tissue dissection, drilling of temporal bone, dural incision, access to the cerebellopontine angle, exposure of cranial nerves and drilling of the internal auditory canal. The pig model perfectly simulates standard otological and neurosurgical procedures, and we highlight the feasibility of our approach for further experiments in a living pig model with the possibility of reproducing the model for research on cranial nerves in pigs to study their electrophysiological behavior.


Assuntos
Fossa Craniana Posterior/cirurgia , Nervos Cranianos/cirurgia , Orelha Interna/cirurgia , Procedimentos Neurocirúrgicos , Animais , Cadáver , Fossa Craniana Posterior/patologia , Nervos Cranianos/patologia , Modelos Animais de Doenças , Dissecação , Orelha Interna/patologia , Humanos , Suínos
15.
Otol Neurotol ; 40(3): e186-e190, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30741893

RESUMO

OBJECTIVE: To investigate the effects of cavitating lesions involving the internal auditory canal (IAC) in subjects with cochlear otosclerosis with regard to poststapedotomy hearing outcome. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: A retrospective chart review of 134 subjects with otosclerosis treated from January 2011 to June 2017 at Seoul National University Bundang Hospital was conducted. Sixteen subjects (23 ears) with temporal bone computed tomography (TBCT)-confirmed cochlear otosclerosis who underwent stapedotomy were included in the study. MAIN OUTCOME MEASURES: Pure tone audiometry (PTA) (i.e., air and bone conduction; AC and BC, respectively) thresholds and air-bone gap (ABG), measured at 6 months postoperatively were compared between cochlear otosclerosis with and without IAC involvement (IAC group and non-IAC group, respectively). RESULTS: A total of 14 of 23 ears showed involvement of the IAC. There were no significant differences in age, side of otosclerosis, or preoperative hearing threshold between the two groups. The mean postoperative AC and BC thresholds and ABG of the IAC group were significantly poorer (45.7 dB, 33.8 dB, and 11.8 dB, respectively) than those of the nonIAC group (24.1 dB, 20.0 dB, and 4.1 dB, respectively). CONCLUSIONS: Cochlear otosclerosis with cavitating lesions involving the IAC showed significantly poorer postoperative audiological outcomes than those without any cavitating lesion. Cavitation extending to the IAC may act as a third window providing a route for sound energy shunting, and thus precluding successful hearing outcome in some subjects with cavitating otosclerosis after stapedotomy.


Assuntos
Orelha Interna/patologia , Otosclerose/patologia , Otosclerose/cirurgia , Cirurgia do Estribo , Resultado do Tratamento , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Seul , Cirurgia do Estribo/métodos
16.
Ann Otol Rhinol Laryngol ; 128(4): 286-292, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30556402

RESUMO

OBJECTIVES:: The purpose of this study was to investigate the correlation between grades of endolymphatic hydrops (ELH) and clinical characteristics and determine the detailed clinical characteristics of Ménière's disease (MD) patients with evidence of hydrops based on magnetic resonance imaging (MRI). METHODS:: One hundred ninety-eight MD patients (396 ears) with MRI evidence of hydrops were included. ELH grades were evaluated using the Nakashima grading standard. Correlations between the extent of ELH and clinical features were evaluated. Detailed clinical characteristics were analyzed to assess the clinical diagnostic criteria. RESULTS:: Of 198 patients, ELH was observed in 100% of cases on the clinically affected side and 8.6% of cases on the asymptomatic side. In addition, 98.5% of ELH was classified as moderate or significant grade. Low-frequency hearing loss was significantly correlated with the extent of both vestibular and cochlear hydrops, whereas the vertigo attack frequency showed no significant correlation with ELH grades. The disease duration of MD with bilateral ELH was longer than that with unilateral ELH. The clinical characteristics were variant and did not completely fit the proposed diagnostic criteria. CONCLUSIONS:: MRI findings have relevance to the clinical severity, to a certain extent, but not vestibular symptoms. The proposed diagnostic criteria based on clinical characteristics may be partially effective; analysis of the detailed clinical characteristics of MD was meaningful. Diagnosis of MD based on both MRI and clinical symptoms could facilitate an early diagnosis.


Assuntos
Orelha Interna/diagnóstico por imagem , Hidropisia Endolinfática , Perda Auditiva , Imagem por Ressonância Magnética/métodos , Doença de Meniere , Adulto , Doenças Assintomáticas , China , Correlação de Dados , Orelha Interna/patologia , Orelha Interna/fisiopatologia , Diagnóstico Precoce , Hidropisia Endolinfática/complicações , Hidropisia Endolinfática/diagnóstico por imagem , Hidropisia Endolinfática/fisiopatologia , Feminino , Perda Auditiva/diagnóstico , Perda Auditiva/etiologia , Humanos , Masculino , Doença de Meniere/diagnóstico , Doença de Meniere/etiologia , Doença de Meniere/fisiopatologia , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Avaliação de Sintomas/métodos , Vertigem/diagnóstico , Vertigem/etiologia
17.
Eur Arch Otorhinolaryngol ; 276(1): 27-40, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30306317

RESUMO

INTRODUCTION: Hydropic ear disease, initially described by and named after Prosper Menière, is one of the most frequent vertigo disorders and one of the most frequent inner ear disorders. It is the syndrome of endolymphatic hydrops which until 2007 could be diagnostically confirmed only by post-mortem histology. In the past, various attempts to formulate clinical diagnostic criteria have been undertaken but were hampered by the inability to ascertain the diagnosis in living patients. With the milestone achievement of endolymphatic hydrops imaging, today the pathology can be ascertained. In this study, we have performed a detailed analysis of the clinical features of hydropic ear disease for the first time by examining a large cohort of patients with morphologically confirmed endolymphatic hydrops using a detailed physician-administered neurotologic face-to-face interview. RESULTS: During a hydropic vertigo attack, the patients report nausea, vomiting, sweating, urge to defecate, urge to urinate, phosphenes, headache, photophobia, phonophobia and even transient loss of consciousness. A third of the patients does not experience auditory symptoms during the vertigo attacks. Vertigo attacks last less than 20 min in more than one-fourth of the patients. Audiometric hearing loss has its greatest diagnostic value at the frequencies of 1 kHz and below. Cochleovestibular symptom onset simultaneity is associated with a high frequency of drop-attacks. Migraine and autoimmune disorders are not associated with hydropic ear disease. CONCLUSION: This study marks the beginning of the clinical characterization of hydropic ear disease. The findings have important implications for the future formulation of clinical diagnostic criteria.


Assuntos
Audiometria/métodos , Orelha Interna/patologia , Hidropisia Endolinfática/diagnóstico , Imagem por Ressonância Magnética/métodos , Hidropisia Endolinfática/complicações , Hidropisia Endolinfática/fisiopatologia , Feminino , Seguimentos , Perda Auditiva/etiologia , Perda Auditiva/fisiopatologia , Humanos , Hiperacusia , Masculino , Pessoa de Meia-Idade
18.
Acta Neuropathol ; 137(2): 343-357, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30390121

RESUMO

Meniere's disease (MD), a syndromal inner ear disease, is commonly associated with a pathological accumulation of endolymphatic fluid in the inner ear, termed "idiopathic" endolymphatic hydrops (iEH). Although numerous precipitating/exacerbating factors have been proposed for MD, its etiology remains elusive. Here, using immunohistochemistry and in situ protein-protein interaction detection assays, we demonstrate mineralocorticoid-controlled sodium transport mechanisms in the epithelium of the extraosseous portion of the endolymphatic sac (eES) in the murine and human inner ears. Histological analysis of the eES in an extensive series of human temporal bones consistently revealed pathological changes in the eES in cases with iEH and a clinical history of MD, but no such changes were found in cases with "secondary" EH due to other otological diseases or in healthy controls. Notably, two etiologically different pathologies-degeneration and developmental hypoplasia-that selectively affect the eES in MD were distinguished. Clinical records from MD cases with degenerative and hypoplastic eES pathology revealed distinct intergroup differences in clinical disease presentation. Overall, we have identified for the first time two inner ear pathologies that are consistently present in MD and can be directly linked to the pathogenesis of EH, and which potentially affect the phenotypical presentation of MD.


Assuntos
Orelha Interna/patologia , Transporte de Íons/fisiologia , Doença de Meniere/metabolismo , Doença de Meniere/patologia , Sódio/metabolismo , Animais , Orelha Interna/metabolismo , Hidropisia Endolinfática/metabolismo , Hidropisia Endolinfática/patologia , Saco Endolinfático/metabolismo , Saco Endolinfático/patologia , Humanos , Masculino , Camundongos , Osso Temporal/metabolismo , Osso Temporal/patologia
20.
Eur J Pharm Sci ; 126: 33-48, 2019 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-29933075

RESUMO

This paper investigates the safety of a novel 'magnetic injection' method of delivering therapy to the cochlea, in a rodent model. In this method of administration, a magnetic field is employed to actively transport drug-eluting superparamagnetic iron-oxide core nanoparticles into the cochlea, where they then release their drug payload (we delivered the steroid prednisolone). Our study design and selection of control groups was based on published regulatory guidance for safety studies that involve local drug delivery. We tested for both single and multiple delivery doses to the cochlea, and found that magnetic delivery did not harm hearing. There was no statistical difference in hearing between magnetically treated ears versus ears that received intra-tympanic steroid (a mimic of a standard-of-care for sudden sensorineural hearing loss), both 2 and 30 days after treatment. Since our treatment is local to the ear, the levels of steroid and iron circulating systemically after our treatment were low, below mass-spectrometry detection limits for the steroid and no different from normal for iron. No adverse findings were observed in ear tissue histopathology or in animal gross behavior. At 2 and 30 days after treatment, inflammatory changes examined in the ear were limited to the middle ear, were very mild in severity, and by day 90 there was ongoing and almost complete reversibility of these changes. There were no ear tissue scarring or hemorrhage trends associated with magnetic delivery. In summary, after conducting a pre-clinical safety study, no adverse safety issues were observed.


Assuntos
Cóclea , Nanopartículas de Magnetita/química , Prednisolona/toxicidade , Animais , Comportamento Animal/efeitos dos fármacos , Sistemas de Liberação de Medicamentos , Liberação Controlada de Fármacos , Orelha Interna/efeitos dos fármacos , Orelha Interna/patologia , Humanos , Inflamação/induzido quimicamente , Inflamação/patologia , Injeções , Masculino , Prednisolona/administração & dosagem , Ratos Long-Evans
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