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1.
Br J Cancer ; 130(10): 1659-1669, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38480935

RESUMO

BACKGROUND: Vestibular schwannomas (VSs) remain a challenge due to their anatomical location and propensity to growth. Macrophages are present in VS but their roles in VS pathogenesis remains unknown. OBJECTIVES: The objective was to assess phenotypic and functional profile of macrophages in VS with single-cell RNA sequencing (scRNAseq). METHODS: scRNAseq was carried out in three VS samples to examine characteristics of macrophages in the tumour. RT-qPCR was carried out on 10 VS samples for CD14, CD68 and CD163 and a panel of macrophage-associated molecules. RESULTS: scRNAseq revealed macrophages to be a major constituent of VS microenvironment with three distinct subclusters based on gene expression. The subclusters were also defined by expression of CD163, CD68 and IL-1ß. AREG and PLAUR were expressed in the CD68+CD163+IL-1ß+ subcluster, PLCG2 and NCKAP5 were expressed in CD68+CD163+IL-1ß- subcluster and AUTS2 and SPP1 were expressed in the CD68+CD163-IL-1ß+ subcluster. RT-qPCR showed expression of several macrophage markers in VS of which CD14, ALOX15, Interleukin-1ß, INHBA and Colony Stimulating Factor-1R were found to have a high correlation with tumour volume. CONCLUSIONS: Macrophages form an important component of VS stroma. scRNAseq reveals three distinct subsets of macrophages in the VS tissue which may have differing roles in the pathogenesis of VS.


Assuntos
Macrófagos , Neuroma Acústico , Análise de Sequência de RNA , Análise de Célula Única , Humanos , Neuroma Acústico/genética , Neuroma Acústico/patologia , Neuroma Acústico/metabolismo , Análise de Célula Única/métodos , Macrófagos/metabolismo , Macrófagos/patologia , Microambiente Tumoral/genética , Feminino , Masculino , Pessoa de Meia-Idade , Antígenos CD/genética , Antígenos de Diferenciação Mielomonocítica/genética , Antígenos de Diferenciação Mielomonocítica/metabolismo , Interleucina-1beta/genética , Interleucina-1beta/metabolismo
2.
Clin Otolaryngol ; 49(1): 41-61, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37885344

RESUMO

OBJECTIVES: To assess outcomes associated with photobiomodulation therapy (PBMT) for hearing loss in human and animal studies. DESIGN: Systematic review and narrative synthesis in accordance with PRISMA guidelines. SETTING: Data bases searched: MEDLINE, EMBASE, CENTRAL, ClinicalTrials.gov and Web of Science. No limits were placed on language or year of publication. Review conducted in accordance with the PRISMA 2020 statement. PARTICIPANTS: All human and animal subjects treated with PBMT for hearing loss. MAIN OUTCOME MEASURES: Pre- and post-PBMT audio metric outcomes. RESULTS: Searches identified 122 abstracts and 49 full text articles. Of these, 17 studies met the inclusion criteria, reporting outcomes in 327 animals (11 studies), 30 humans (1 study), and 40 animal specimens (5 studies). PBMT parameters included 6 different wavelengths: 908 nm (1 study), 810 nm (1 study), 532 & 635 nm (1 study), 830 nm (3 studies), 808 nm (11 studies). The duration ranged from 4 to 60 minutes in a session, and the follow-up ranged from 5-28 days. Outcomes improved significantly when wavelengths within the range of 800-830 nm were used, and with greater duration of PBMT exposure. Included studies predominantly consisted of non-randomized controlled trials (10 studies). CONCLUSIONS: Hearing outcomes following PBMT appear to be superior to no PBMT for subjects with hearing loss, although higher level evidence is required to verify this. PBMT enables concentrated, focused delivery of light therapy to the inner ear through a non-invasive manner with minimal side effects. As a result of heterogeneity in reporting PBMT parameters and outcomes across the included studies, direct comparison is challenging.


Assuntos
Perda Auditiva , Terapia com Luz de Baixa Intensidade , Animais , Humanos , Audição , Perda Auditiva/radioterapia
3.
Otol Neurotol ; 44(10): e755-e765, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-37733967

RESUMO

BACKGROUND AND AIM: Vestibular schwannomas (VSs), despite being histologically benign, cause significant morbidity because of their challenging intracranial location and the propensity for growth. The role of the stroma and particularly fibroblasts, in the progression of VS, is not completely understood. This study examines the profile of fibroblasts in VS. METHODS: Seventeen patients undergoing surgical excision of VS were recruited into the study. Reverse transcription with quantitative polymerase chain reaction (RT-qPCR) was performed on VS tissue samples and fibroblast-associated molecules examined. Immunofluorescence and immunohistochemistry in VS tissue were used to study the expression of fibroblast markers CD90 and podoplanin in situ. Fibroblast cultures were established from VS, and RT-qPCR analysis was performed on a panel of fibroblast markers on VS and control tissue fibroblasts. RESULTS: Several fibroblast-associated molecules including members of galectin family and matrix metalloproteinases were found to be expressed in VS tissue on RT-qPCR analysis. In situ, expression of CD90 and podoplanin was observed in VS tissue both on immunohistochemistry and immunofluorescence. RT-qPCR analysis of fibroblasts from VS and control vestibular neuroepithelium (NE) showed a higher expression of several molecules of the galectin and matrix metalloproteinases family on VS fibroblasts compared with NE fibroblasts. CONCLUSION: This work examines fibroblasts from VS and shows qualitative differences from NE fibroblasts on RT-qPCR. Further understanding of the fibroblast function in the progression of VS will potentially unveil new targets to manage VS growth.


Assuntos
Neuroma Acústico , Humanos , Neuroma Acústico/patologia , Fibroblastos/metabolismo , Metaloproteinases da Matriz/metabolismo , Galectinas/metabolismo
4.
J Neurol Surg B Skull Base ; 84(5): 433-443, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37671296

RESUMO

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 2, we present a codified operative workflow for the translabyrinthine approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Seventeen consultant skull base surgeons (nine neurosurgeons and eight ENT [ear, nose, and throat]) with median of 13.9 years of experience (interquartile range: 18.1 years) of independent practice participated. There was a 100% response rate across both the Delphi rounds. The translabyrinthine approach had the following five phases and 57 unique steps: Phase 1, approach and exposure; Phase 2, mastoidectomy; Phase 3, internal auditory canal and dural opening; Phase 4, tumor debulking and excision; and Phase 5, closure. Conclusion We present Part 2 of a national, multicenter, consensus-derived, codified operative workflow for the translabyrinthine approach to vestibular schwannomas. The five phases contain the operative, steps, instruments, technique errors, and event errors. The codified translabyrinthine approach presented in this manuscript can serve as foundational research for future work, such as the application of artificial intelligence to vestibular schwannoma resection and comparative surgical research.

5.
J Neurol Surg B Skull Base ; 84(5): 423-432, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37671298

RESUMO

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 1, we present a codified operative workflow for the retrosigmoid approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus, was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Eighteen consultant skull base surgeons (10 neurosurgeons and 8 ENT [ear, nose, and throat]) with median 17.9 years of experience (interquartile range: 17.5 years) of independent practice participated. There was a 100% response rate across both Delphi's rounds. The operative workflow for the retrosigmoid approach contained three phases and 40 unique steps as follows: phase 1, approach and exposure; phase 2, tumor debulking and excision; phase 3, closure. For the retrosigmoid approach, technique, and event error for each operative step was also described. Conclusion We present Part 1 of a national, multicenter, consensus-derived, codified operative workflow for the retrosigmoid approach to vestibular schwannomas that encompasses phases, steps, instruments, technique errors, and event errors. The codified retrosigmoid approach presented in this manuscript can serve as foundational research for future work, such as operative workflow analysis or neurosurgical simulation and education.

6.
Eur Arch Otorhinolaryngol ; 280(2): 661-669, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35834014

RESUMO

PURPOSE: Studies have assessed the trauma and change in hearing function from the use of otological drills on the ossicular chain, but not the effects of partial laser ablation of the incus. A study of the effectiveness of a novel middle-ear microphone for a cochlear implant, which required an incus recess for the microphone balltip, provided an opportunity to compare methods and inform a feasibility study of the microphone with patients. METHODS: We used laser Doppler vibrometry with an insert earphone and probe microphone in 23 ears from 14 fresh-frozen cadavers to measure the equivalent noise level at the tympanic membrane that would have led to the same stapes velocity as the creation of the incus recess. RESULTS: Drilling on the incus with a diamond burr created peak noise levels equivalent to 125.1-155.0 dB SPL at the tympanic membrane, whilst using the laser generated equivalent noise levels barely above the baseline level. The change in middle ear transfer function following drilling showed greater variability at high frequencies, but the change was not statistically significant in the three frequency bands tested. CONCLUSIONS: Whilst drilling resulted in substantially higher equivalent noise, we considered that the recess created by laser ablation was more likely to lead to movement of the microphone balltip, and therefore decrease performance or result in malfunction over time. For patients with greatly reduced residual hearing, the greater consistency from drilling the incus recess may outweigh the potential benefits of hearing preservation with laser ablation.


Assuntos
Terapia a Laser , Prótese Ossicular , Humanos , Bigorna/cirurgia , Orelha Média/cirurgia , Ossículos da Orelha , Estribo
7.
Otol Neurotol ; 43(3): e374-e381, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-35061638

RESUMO

OBJECTIVE: Compare hearing outcome for vestibular schwannoma patients following stereotactic radiosurgery (SRS) or conservative management. STUDY DESIGN: Retrospective review. SETTING: University Hospital. PATIENTS: Patients with small- or medium-sized sporadic vestibular schwannoma (intracanalicular or with CPA component <2 cm) who were managed conservatively or underwent SRS with available clinical, radiological, and audiometric data from the time of presentation (or just before radiotherapy for the SRS group) and most recent follow-up; with the two sets of data to be compared being at least 3 years apart (minimum follow-up period). INTERVENTIONS: SRS or observation. MAIN OUTCOME MEASURE: Pure-tone averages, speech discrimination scores, and corresponding hearing classifications. RESULTS: Two hundred forty-seven patients met our inclusion criteria; 140 were managed conservatively with a mean follow-up period of 5.9 ±â€Š1.6 years and 107 underwent SRS with a mean follow-up period of 7.1 ±â€Š1.9 years. There was significant deterioration of hearing measures for both groups; with the SRS group displaying consistently worse measures. SRS patients showed worse mean pure-tone averages and speech discrimination scores decline rates by 2.72 dB/yr and 2.98 %/yr, respectively, when compared with conservatively managed patients. Stratifying patients according to Tokyo's hearing classification revealed that 68.75% of conservatively managed patients who had baseline serviceable hearing preserved their hearing throughout the studied period compared with only 15.38% of the SRS patients. CONCLUSION: Based on our data we conclude that patients with small- and medium-sized tumors will have a better hearing outcome if managed via an initial conservative approach with radiotherapy reserved for those demonstrating disease progression.


Assuntos
Neuroma Acústico , Radiocirurgia , Audição , Testes Auditivos , Humanos , Neuroma Acústico/complicações , Neuroma Acústico/radioterapia , Radiocirurgia/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
8.
J Int Adv Otol ; 18(4): 308-314, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35072629

RESUMO

BACKGROUND: Congenital cholesteatomas account for just up to 5% of all cholesteatomas and most commonly arise in the petrous apex and middle ear. Congenital cholesteatomas arising in the mastoid are rare and typically present late. METHODS: In this study, we report a case series of 3 cases managed in our department between 2006 and 2021 and present a summary of the current literature. RESULTS: Congenital cholesteatomas arising in the mastoid is a rare finding and even among reported cases, not all are clearly mastoid in origin. Their location allows for considerable growth before symptoms develop. Pain and localized swelling in the temporal area are the most common presenting symptoms which can lead to diagnostic challenges. Our cases show that although surgery is often appropriate, conservative manage- ment may be suitable in certain situations. CONCLUSION: Congenital cholesteatoma of mastoid origin is rare and can present a diagnostic challenge. Greater awareness is important to facilitate early detection. A high index of suspicion is needed in those presenting with retro-auricular pain and swelling in the context of a normal ontological examination.


Assuntos
Doenças Ósseas , Colesteatoma da Orelha Média , Colesteatoma , Colesteatoma/congênito , Colesteatoma/diagnóstico , Colesteatoma/cirurgia , Colesteatoma da Orelha Média/diagnóstico , Colesteatoma da Orelha Média/cirurgia , Orelha Média , Dor de Orelha/etiologia , Humanos , Processo Mastoide/diagnóstico por imagem , Processo Mastoide/cirurgia
9.
Hear Res ; 412: 108371, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34689069

RESUMO

Cochlear Implant provides an electronic substitute for hearing to severely or profoundly deaf patients. However, postoperative hearing outcomes significantly depend on the proper placement of electrode array (EA) into scala tympani (ST) during cochlear implant surgery. Due to limited intra-operative methods to access array placement, the objective of the current study was to evaluate the relationship between EA complex impedance and different insertion trajectories in a plastic ST model. A prototype system was designed to measure bipolar complex impedance (magnitude and phase) and its resistive and reactive components of electrodes. A 3-DoF actuation system was used as an insertion feeder. 137 insertions were performed from 3 different directions at a speed of 0.08 mm/s. Complex impedance data of 8 electrode pairs were sequentially recorded in each experiment. Machine learning algorithms were employed to classify both the full and partial insertion lengths. Support Vector Machine (SVM) gave the highest 97.1% accuracy for full insertion. When a real-time prediction was tested, Shallow Neural Network (SNN) model performed better than other algorithms using partial insertion data. The highest accuracy was found at 86.1% when 4 time samples and 2 apical electrode pairs were used. Direction prediction using partial data has the potential of online control of the insertion feeder for better EA placement. Accessing the position of the electrode array during the insertion has the potential to optimize its intraoperative placement that will result in improved hearing outcomes.


Assuntos
Implante Coclear , Implantes Cocleares , Procedimentos Cirúrgicos Robóticos , Cóclea/cirurgia , Implante Coclear/métodos , Impedância Elétrica , Eletrodos Implantados , Humanos , Aprendizado de Máquina , Rampa do Tímpano/cirurgia
10.
Otol Neurotol ; 42(10): 1534-1543, 2021 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-34420021

RESUMO

OBJECTIVE: To determine the pre- and postoperative clinical, audiological, vestibular, and patient-reported measures in patients undergoing transmastoid occlusion surgery for superior canal dehiscence syndrome (SCDS). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral centre, UK. PATIENTS: All primary transmastoid occlusion surgeries for SCDS were included (tertiary centre, single-surgeon), January 2008 to July 2019. INTERVENTIONS: Transmastoid superior canal occlusion surgery for SCDS. MAIN OUTCOME MEASURES: We collated audiological (pure tone audiogram), vestibular (cervical vestibular evoked myogenic potentials [cVEMPs]), and patient-reported outcome measures (Dizziness Handicap Inventory and subjective symptom grading). RESULTS: Fifty-two patients (55 ears) met the inclusion criteria. Thirty-one (56%) were female. Mean age was 47 years (range 29-63) and mean follow-up of 11.2 months. Six patients had bilateral disease, four of whom underwent sequential, bilateral surgery.Autophony was the most frequent presenting symptom, improving in 92%.Significant improvements were self-reported in patients' autophony (p < 0.0001), pressure- and noise-induced dizziness (p < 0.0001 and p < 0.0001), aural fullness (p = 0.0159), pulsatile tinnitus (p < 0.0001), perceived hearing loss (p = 0.0058), and imbalance (p = 0.0303).Overall Dizziness Handicap Inventory scores reduced from 45.9 to 27.4 (p < 0.0001), and across all subgroups of functional (p = 0.0003), emotional (p < 0.0001), and physical handicap (p = 0.0005).A 6.4-dB HL improvement in the air-bone gap (500-1000 Hz) occurred (95% confidence intervals 3.3-9.4 dB HL, p < 0.0001). There were no dead ears. cVEMP thresholds, when recordable, normalized in all except two ears. CONCLUSIONS: Transmastoid occlusion is effective at improving patient-reported outcomes and normalizing cVEMP thresholds, though some symptoms, notably disequilibrium, persist to a variable degree.


Assuntos
Deiscência do Canal Semicircular , Potenciais Evocados Miogênicos Vestibulares , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Estudos Retrospectivos , Canais Semicirculares/cirurgia , Potenciais Evocados Miogênicos Vestibulares/fisiologia
11.
Head Neck ; 43(11): 3448-3458, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34418219

RESUMO

BACKGROUND: This study aims to develop and validate a new classification system that better predicts combined risk of neurological and neurovascular complications following CBT surgery, crucial for treatment decision-making. METHODS: Multinational retrospective cohort study with 199 consecutive cases. A cohort of 132 CBT cases was used to develop the new classification. To undertake external validation, assessment was made between the actual complication rate and predicted risk by the model on an independent cohort (n = 67). RESULTS: Univariate analyses showed statistically significant associations between developing a complication and the following factors: craniocaudal dimension, volume, Shamblin classification, and Mehanna types. In the multivariate prognostic model, only Mehanna type remained as a significant risk predictor. The risk of developing complications increases with increasing Mehanna type. CONCLUSIONS: We have developed and then validated a new classification and risk stratification system for CBTs, which demonstrated better prognostic power for the risk of developing neurovascular complications after surgery.


Assuntos
Tumor do Corpo Carotídeo , Estudos de Coortes , Humanos , Prognóstico , Estudos Retrospectivos , Medição de Risco , Resultado do Tratamento
12.
Otol Neurotol ; 42(1): 116-120, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33201079

RESUMO

OBJECTIVE: Menière's disease can be a debilitating condition but in most cases the symptoms are controlled by lifestyle changes and medical management. However, some patients remain symptomatic despite medical treatment and have the option of more invasive surgical treatments. Surgical intervention for Menière's includes a range of interventions from grommet insertion, intratympanic steroids/Gentamicin, endolymphatic sac decompression, labyrinthectomy, and vestibular neurectomy. A recently described technique involves the occlusion of all three semi-circular canals as an alternative in intractable Menière's disease. STUDY DESIGN: This is a case series of three patients who underwent triple canal occlusion for the treatment of intractable Menière's disease. SETTING: Patients were selected from those who were referred to Queen Elizabeth Hospital in Birmingham, a tertiary referral center. PATIENTS: Patients who were severely symptomatic despite medical treatment who were considering ablative therapy were offered the option of triple canal occlusion as an alternative. INTERVENTION: We report a series of Menière's patients treated by triple canal occlusion, describe the rationale behind this intervention, the surgical technique, and preliminary results. MAIN OUTCOME MEASURE: Each patient was followed up for a minimum of 2 years following the procedure. The main outcomes measures were the class of vertigo control and hearing threshold levels according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines. RESULTS: Of the three patients, two were men and one was woman, the age range was 45 years to 61 years old. Two patients with unilateral disease achieved class A control whereas one patient with bilateral disease achieved class B control. Two patients who underwent the procedure had little or no effect to their hearing on the treated side however one patient suffered a 30 dB hearing loss on the operative side. CONCLUSIONS: Based on our limited experience and the early reports in the literature we consider that there are potential patient benefits for triple canal occlusion for intractable Menière's disease as an alternative to vestibular neurectomy due to the reduced morbidity and long-term efficacy.


Assuntos
Saco Endolinfático , Doença de Meniere , Procedimentos Cirúrgicos Otológicos , Feminino , Gentamicinas/uso terapêutico , Humanos , Masculino , Doença de Meniere/cirurgia , Pessoa de Meia-Idade , Resultado do Tratamento , Vertigem/cirurgia
13.
Otol Neurotol ; 41(7): e829-e835, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32558760

RESUMO

OBJECTIVE: To investigate the disturbance induced in the cochlea during cochleostomy using conventional drill and a hand guided robotic drill. STUDY DESIGN: The study is based on experimental measurements using the Laser Doppler Vibrometer during the drilling processes converted to Sound Pressure Levels (SPL) for comparison. SETTING: The study is based on experimental results of three sets of cochleostomies on human cadaver heads. MAIN OUTCOME MEASURE(S): Robotic drilling, in comparison to the conventional drilling method, creates a consistently lower level of disturbance in cochlea across the hearing frequency range. RESULTS: Robotic drilling, in comparison to the conventional drilling method, creates a consistently lower level of disturbance in cochlea across the hearing frequency range. CONCLUSIONS: It is reasonable to conclude that robotic drilling has a lower possibility of creating acoustic trauma in cochlea that endangers the residual hearing of patients.


Assuntos
Implante Coclear , Procedimentos Cirúrgicos Robóticos , Robótica , Cóclea/cirurgia , Implante Coclear/efeitos adversos , Mãos , Humanos , Procedimentos Cirúrgicos Robóticos/efeitos adversos
14.
Clin Neurophysiol ; 129(11): 2350-2360, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30248625

RESUMO

OBJECTIVES: We determined if eye movements evoked by Electrical Vestibular Stimulation (EVS) can be used to detect vestibular dysfunction in patients with unilateral vestibular schwannoma (VS). METHODS: Ocular torsion responses to monaural sinusoidal EVS currents (±2 mA, 2 Hz) were measured in 25 patients with tumours ranging in size from Koos grade 1-3. For comparative purposes we also measured postural sway response to EVS, and additionally assessed vestibular function with the lateral Head Impulse Test (HIT). Patient responses were compared to age-matched healthy control subjects. RESULTS: Patients exhibited smaller ocular responses to ipsilesional versus contralesional EVS, and showed a larger asymmetry ratio (AR) than control subjects (19.4 vs. 3.3%, p < 0.05). EVS-evoked sway responses were also smaller in ipsilesional ear, but exhibited slightly more variability than the eye movement response, along with marginally lower discriminatory power (patients vs. controls: AR = 16.6 vs 2.6%, p < 0.05). The HIT test exhibited no significant difference between groups. CONCLUSIONS: These results demonstrate significant deficits in the ocular torsion response to EVS in VS patients. SIGNIFICANCE: The fast, convenient and non-invasive nature of the test are well suited to clinical use.


Assuntos
Neuroma Acústico/fisiopatologia , Potenciais Evocados Miogênicos Vestibulares , Idoso , Movimentos Oculares , Feminino , Movimentos da Cabeça , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/diagnóstico , Postura
15.
Contemp Clin Trials Commun ; 10: 137-140, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30023447

RESUMO

BACKGROUND: The Vibrant Soundbridge middle ear implant and the Bonebridge bone conducting hearing device are hearing implants that use radio frequency transmission to send information from the sound processor to the internal transducer. This reduces the risk of skin problems and infection but requires a more involved surgical procedure than competitor skin penetrating devices. It is not known whether more complex surgery will lead to additional complications. There is little information available on the reliability of these systems and adverse medical or surgical events. The primary research question is to determine the reliability and complication rate for the Vibrant Soundbridge and Bonebridge. The secondary research question explores changes in quality of life following implantation of the devices. The tertiary research question looks at effectiveness via changes in auditory performance. METHOD: The study was designed based on a combination of a literature search, two clinician focus groups and expert review.A multi-centre longitudinal observational study was designed. There are three study groups, two will have been implanted prior to the start of the study and one group, the prospective group, will be implanted after initiation of the study. Outcomes are surgical questionnaires, measures of quality of life, user satisfaction and speech perception tests in quiet and in noise. CONCLUSION: This is the first multi-centre study to look at these interventions and includes follow up over time to understand effectiveness, reliability, quality of life and complications.

16.
BMJ Case Rep ; 20182018 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-29754138

RESUMO

Temporal bone osteoma is an unusual pathology which can occur by birth or can be acquired and mostly involves the tympanomastoid segment of the temporal bone. Osteomas arising from the otic capsule are extremely rare, and there has been only one other report of a lateral semicircular canal osteoma in the literature. We report a similar case of an acquired lateral canal osteoma which presented as a chronic postaural fistula in an ear previously operated for paediatric cholesteatoma.


Assuntos
Neoplasias Ósseas/diagnóstico , Colesteatoma da Orelha Média/cirurgia , Fístula/cirurgia , Processo Mastoide/patologia , Osteoma/diagnóstico , Procedimentos Cirúrgicos Otológicos , Canais Semicirculares/patologia , Adulto , Neoplasias Ósseas/cirurgia , Feminino , Humanos , Osteoma/cirurgia , Canais Semicirculares/cirurgia , Resultado do Tratamento
17.
J Med Genet ; 55(6): 384-394, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29386252

RESUMO

BACKGROUND: Germline pathogenic variants in SDHB/SDHC/SDHD are the most frequent causes of inherited phaeochromocytomas/paragangliomas. Insufficient information regarding penetrance and phenotypic variability hinders optimum management of mutation carriers. We estimate penetrance for symptomatic tumours and elucidate genotype-phenotype correlations in a large cohort of SDHB/SDHC/SDHD mutation carriers. METHODS: A retrospective survey of 1832 individuals referred for genetic testing due to a personal or family history of phaeochromocytoma/paraganglioma. 876 patients (401 previously reported) had a germline mutation in SDHB/SDHC/SDHD (n=673/43/160). Tumour risks were correlated with in silico structural prediction analyses. RESULTS: Tumour risks analysis provided novel penetrance estimates and genotype-phenotype correlations. In addition to tumour type susceptibility differences for individual genes, we confirmed that the SDHD:p.Pro81Leu mutation has a distinct phenotype and identified increased age-related tumour risks with highly destabilising SDHB missense mutations. By Kaplan-Meier analysis, the penetrance (cumulative risk of clinically apparent tumours) in SDHB and (paternally inherited) SDHD mutation-positive non-probands (n=371/67 with detailed clinical information) by age 60 years was 21.8% (95% CI 15.2% to 27.9%) and 43.2% (95% CI 25.4% to 56.7%), respectively. Risk of malignant disease at age 60 years in non-proband SDHB mutation carriers was 4.2%(95% CI 1.1% to 7.2%). With retrospective cohort analysis to adjust for ascertainment, cumulative tumour risks for SDHB mutation carriers at ages 60 years and 80 years were 23.9% (95% CI 20.9% to 27.4%) and 30.6% (95% CI 26.8% to 34.7%). CONCLUSIONS: Overall risks of clinically apparent tumours for SDHB mutation carriers are substantially lower than initially estimated and will improve counselling of affected families. Specific genotype-tumour risk associations provides a basis for novel investigative strategies into succinate dehydrogenase-related mechanisms of tumourigenesis and the development of personalised management for SDHB/SDHC/SDHD mutation carriers.


Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Proteínas de Membrana/genética , Paraganglioma/genética , Feocromocitoma/genética , Succinato Desidrogenase/genética , Neoplasias das Glândulas Suprarrenais/patologia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Estudos de Associação Genética , Genótipo , Mutação em Linhagem Germinativa/genética , Heterozigoto , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto/genética , Paraganglioma/patologia , Feocromocitoma/patologia , Fatores de Risco , Caracteres Sexuais
18.
Cochlear Implants Int ; 19(1): 54-60, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29032725

RESUMO

Percutaneous pedestals have been integral to the development of cochlear implants since 1969. By enabling direct electrical access to implanted electrodes or other devices, they allow optimization of control of stimulation strategies. Similarly, technology not validated for implantable use can be safely tested. These advantages have facilitated the development of cochlear implants and also resulted in their inclusion in trials investigating electronic implants developed for other organs. Surgery is straightforward, but post-operative care, in particular, skin-care is crucial to ensure complications are minimized. This review discusses the history of percutaneous pedestal use in cochlear implants and other electronic devices. Surgical technique, aftercare, and complications of surgery are discussed along with possibilities for future development.


Assuntos
Implante Coclear/instrumentação , Implantes Cocleares , Estimulação Elétrica/instrumentação , Assistência ao Convalescente , Implante Coclear/métodos , Estimulação Elétrica/métodos , Humanos
19.
Robot Surg ; 5: 13-18, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30697569

RESUMO

BACKGROUND: An arm supported robotic drill has been recently demonstrated for preparing cochleostomies in a pilot research clinical trial. In this paper, a hand-guided robotic drill is presented and tested on human cadaver trials. METHODS: The innovative smart tactile approach can automatically detect drilling mediums and decided when to stop drilling to prevent penetrating the endosteum. The smart sensing scheme has been implemented in a concept of a hand guided robotic drill. RESULTS: Experiments were carried out on two adult cadaveric human bodies for verifying the drilling process and successfully finished cochleostomy on three cochlea. The advantage over a system supported by a mechanical arm includes the flexibility in adjusting the trajectory to initiate cutting without slipping. Using the same concept as a conventional drilling device, the user will also be benefit from the lower setup time and cost, and lower training overhead. CONCLUSION: The hand-guided robotic drill was recently developed for testing on human cadavers. The robotic drill successfully prepared cochleostomies in all three cases.

20.
Otol Neurotol ; 38(5): 672-677, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28333779

RESUMO

OBJECTIVE: Analyze the presentation and evolution of chronic suppurative otitis media (CSOM) in children with cochlear implants (CI) and explore the merit of early intervention. STUDY DESIGN: Retrospective patient review. SETTING: Tertiary referral hospital and cochlear implant programme. PATIENTS: Children with a CI who developed CSOM. INTERVENTION: Tympanoplasty. MAIN OUTCOME MEASURES: Disease control, recurrence of cholesteatoma, cochlear implant preservation. RESULTS: Eight children fit our inclusion criteria with a mean follow up of 8 years. Onset of CSOM symptoms was observed on an average of 5 years after implantation (range, 2-13 yr) and led to surgical intervention in an average of 15.6 months following symptom onset. Cholesteatoma was found in four of the eight patients. Of these, one patient underwent a subtotal petrosectomy and explantation with reimplantation at the same stage but the reimplant failed to function and was explanted subsequently. One patient was initially managed by a canal wall up mastoidectomy and explantation but went on to require subtotal petrosectomy and labyrinthectomy for recurrent disease. One patient underwent a subtotal petrosectomy with explantation and is awaiting a reimplantation. The fourth patient had limited disease around the electrodes that was excised without compromising the implant. In the group of patients with CSOM without cholesteatoma, one underwent an explantation due to recurrent ear infections and a subsequent cartilage tympanoplasty for a retracted eardrum. The remaining three patients underwent successful excision of retraction pockets and repair of eardrums using cartilage with the implant in situ. A mean follow up of 2 years after the implant preservation surgeries shows good functioning of the CI. CONCLUSION: Early recognition of CSOM is paramount in patients with CI as delay in treatment can result in the infection spreading via the cochleostomy resulting in loss of the cochlea. Recurrent ear infections in an implanted ear should prompt early examination to exclude the presence of middle ear disease, which may require anesthesia in a young child.


Assuntos
Implantes Cocleares , Otite Média Supurativa/complicações , Adolescente , Criança , Pré-Escolar , Colesteatoma da Orelha Média/etiologia , Doença Crônica , Remoção de Dispositivo , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Centros de Atenção Terciária , Resultado do Tratamento
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