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1.
Otolaryngol Head Neck Surg ; 162(4): 530-537, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31986971

RESUMO

OBJECTIVE: To ascertain the relationship among vestibular schwannoma (VS) tumor volume, growth, and hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Single tertiary center. SUBJECTS AND METHODS: Adults with observed VS and serviceable hearing at diagnosis were included. The primary outcome was the development of nonserviceable hearing as estimated using the Kaplan-Meier method. Associations of tumor volume with baseline hearing were assessed using Spearman rank correlation coefficients. Associations of volume and growth with the development of nonserviceable hearing over time were assessed using Cox proportional hazards models and summarized with hazard ratios (HRs). RESULTS: Of 230 patients with VS and serviceable hearing at diagnosis, 213 had serial volumetric tumor data for analysis. Larger tumor volume at diagnosis was associated with increased pure-tone average (PTA) (P < .001) and decreased word recognition score (WRS) (P = .014). Estimated rates of maintaining serviceable hearing at 6 and 10 years following diagnosis were 67% and 49%, respectively. Larger initial tumor volume was associated with development of nonserviceable hearing in a univariable setting (HR for 1-cm3 increase: 1.36, P = .040) but not after adjusting for PTA and WRS. Tumor growth was not significantly associated with time to nonserviceable hearing (HR, 1.57; P = .14), although estimated rates of maintaining serviceable hearing during observation were poorer in the group that experienced growth. CONCLUSION: Larger initial VS tumor volume was associated with poorer hearing at baseline. Larger initial tumor volume was also associated with the development of nonserviceable hearing during observation in a univariable setting; however, this association was not statistically significant after adjusting for baseline hearing status.


Assuntos
Perda Auditiva/etiologia , Neuroma Acústico/complicações , Neuroma Acústico/patologia , Carga Tumoral , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
2.
J Neurol Surg B Skull Base ; 80(4): 399-415, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31316886

RESUMO

Introduction Optimal management of vestibular schwannoma (VS) demands involvement of an experienced multidisciplinary team. As the number of training programs in neurotology and skull base neurosurgery continues to rise, ensuring that trainees are capable of evidence-based decision-making and treatment, whether microsurgical or radiosurgical, is of paramount importance. The purpose of this study is to characterize the landscape of neurotologic and neurosurgical fellowship training programs in North America, with special reference to VS management. Methods A 64-item web-based survey assessing VS practice trends was devised by members of the North American Skull Base Society (NASBS) Research Task Force and distributed electronically to NASBS membership via SurveyMonkey as a cross-sectional study. Participation was entirely voluntary and there was no remuneration for survey completion. The survey link was active from November 29 to December 14, 2016. Results Of 719 members of the NASBS who were emailed a survey link, a total of 57 were returned (8%) completed surveys. Of all respondents, 51 (89%) claimed to have formal training in skull base neurosurgery or neurotology. Thirty-three respondents (65%) were skull base neurosurgeons while the remainder were neurotologists ( n = 18; 35%). Institutions with fellowship programs tended to have a higher surgical, radiosurgical, and overall case volume than those with a residency program alone. However, 20% of respondents at institutions with fellowship programs reported evaluating less than 50 new diagnoses of VS per year and 12% reported a surgical case volume of less than 10 cases per year. Conclusion As the number of skull base training programs expands, it is our duty to ensure that trainees gain sufficient experience to enter independent practice with the ability to exercise informed decision-making and safely perform VS surgery and radiosurgery. In the current training climate, implementing multidisciplinary care models, formalized training requirements, and emerging surgical simulators will support the development of minimum proficiencies in VS care.

3.
J Neurol Surg B Skull Base ; 80(3): 316-322, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31143577

RESUMO

Objectives Although vestibular schwannomas (VS) are known to cause cranial nerve deficits, cerebellar symptoms, and hydrocephalus, the role of these symptoms as the key driver of presentation from the patient's perspective has not been described. Our objective was to survey a large, retrospective VS cohort to document the patient-reported principal initial symptom, and self-reported tumor size, and to study trends in VS patient presentation. Methods Patients diagnosed with VS at our tertiary referral center and belonging to the Acoustic Neuroma Association (ANA) answered a questionnaire between 2015 and 2017. Demographic data, self-reported tumor size, and symptomatology were analyzed. Results 1,304 patients completed the questionnaire. Tumors were diagnosed from 1966 to 2017 at a mean 51.8 years (range: 8-86 years); 66% were female, and 1.1% had confirmed neurofibromatosis type 2 (NF2). Tumor size was reported using a 6-point scale: 0 to 1 cm (22.9%), 1 to 2 cm (28.7%), 2 to 3 cm (20.5%), 3 to 4 cm (10%), greater than 4 cm (7.2%), and unknown (10.6%). Hearing loss was the most common symptom that led to diagnosis (51.5%), followed by dizziness (17%), tinnitus (11.2%), and incidental diagnosis (10.2%); a fraction that has increased significantly in the last decade ( p = 0.022). Larger tumors and NF2 were significantly associated with young age ( p < 0.001). Conclusion Our large-scale questionnaire-driven review of 1,304 patients confirms that VS presentations are stereotypical, with most individuals recalling hearing loss, dizziness, or tinnitus as their chief complaint. Many tumors were incidentally diagnosed; an expanding population, attributable to increased access to magnetic resonance imaging (MRI). Large tumors were significantly more prevalent among younger patients at diagnosis, excluding NF2 patients, suggesting a more aggressive tumor biology that remains incompletely understood.

4.
Otol Neurotol ; 40(6): 820-825, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31135667

RESUMO

OBJECTIVE: The treatment paradigm for jugular paraganglioma (JP) has changed considerably over time with the wider adoption of stereotactic radiosurgery (SRS). To the best of the authors' knowledge, there are no published studies that use validated patient-reported outcome measures to ascertain quality of life (QoL) outcomes following SRS for JP when used in single or combined modality treatment regimens. STUDY DESIGN: Cross-sectional survey. SETTING: Tertiary referral center. PATIENTS: Adult patients with JP treated with primary SRS or SRS following primary surgery between 1990 and 2017. INTERVENTIONS(S): Surgery and/or Gamma Knife SRS. MAIN OUTCOME MEASURES: Global and treatment-related QoL and differences in QoL based on treatment approach. RESULTS: Sixty-nine surveys were distributed and a total of 26 completed surveys were received (38% response rate). Among respondents, the median age at SRS was 53 years and 16 of the 26 patients (62%) were female. Median follow-up was 97 months. Nineteen patients (73%) were treated with primary SRS or staged SRS following intentional subtotal resection (STR; hereafter referred to as "staged SRS"), while the remainder (n = 7, 27%) were treated with SRS for recurrent JP. Median physical and mental health QoL PROMIS-10 T-scores regardless of treatment strategy were 39.8 and 38.8, respectively, while median SF36 physical and mental component subscores were similar to national averages and non-tumor controls. When comparing general physical and mental health QoL scores, there was no significant difference between patients treated with primary or staged SRS and those treated with SRS for recurrent JP. However, age-adjusted swallowing function among patients treated with primary or staged SRS was better than in those patients treated with SRS for recurrent JP (p = 0.05). CONCLUSIONS: Patients treated with primary or staged SRS for JP tend to exhibit better swallowing outcomes than those treated with SRS for recurrent JP. However, the majority of overall and disease-specific quality of life measures were not different between groups. Based on the low incidence of new cranial neuropathy following SRS, it is likely that initial surgical morbidity is the primary contributor to this outcome. Disease-specific overall quality of life measures, akin to those already used for other benign skull base tumors, are necessary to better gauge physical and mental health outcomes following treatment for JP. Though limited by small sample size, this represents the first study to gauge QoL outcomes following treatment for JP.

5.
BMC Cancer ; 19(1): 352, 2019 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-30975103

RESUMO

BACKGROUND: High risk human papillomaviruses (HPV) plays important roles in the development of cervical cancer, a number of other anogenital cancer and they are increasingly found in oropharyngeal squamous cell carcinoma (OPSCC), however there has not been comprehensive analysis about the role how these viruses play in the development of OPSCC. METHODS: To characterize the physical status of HPV within OPSCC and to determine the effect this has throughout the host genome, we have performed 30-40X whole genome sequencing (WGS) on the BGI sequencing platform on 34 OPSCCs: 28 of which were HPV positive. We then examined the sequencing data to characterize the HPV copy number and HPV physical status to determine what effect they have on both HPV and human genome structural changes. RESULTS: WGS determined the HPV copy number across the viral genome. HPV copy number ranged from 1 copy to as high as 150 copies in each individual OPSCC. Independent of HPV copy number, most tumors had either a small or a very large deletion in the viral genome. We discovered that these deletions were the result of either HPV integration into the human genome or HPV-HPV sequence junctions. WGS revealed that ~ 70% of these tumors had HPV integrations within the human genome and HPV integration occurred independent of HPV copy number. Individual HPV integrations were found to be highly disruptive resulting in structural variations and copy number changes at or around the integration sites. CONCLUSIONS: WGS reveals that there is a great complexity in both HPV sequences present and the HPV integrations events in HPV positive OPSCCs tumors. Thus HPV may be playing different roles in the development of different OPSCCs and this further challenge the HPV-driven carcinogenesis model first proposed for cervical cancer.


Assuntos
Biomarcadores Tumorais/genética , Carcinoma de Células Escamosas/genética , Neoplasias Orofaríngeas/genética , Papillomaviridae/genética , Infecções por Papillomavirus/genética , Adulto , Idoso , Carcinogênese/genética , Carcinoma de Células Escamosas/patologia , DNA Viral/genética , DNA Viral/isolamento & purificação , Feminino , Dosagem de Genes , Genoma Humano/genética , Genoma Viral/genética , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Orofaríngeas/patologia , Papillomaviridae/isolamento & purificação , Infecções por Papillomavirus/patologia , Integração Viral/genética , Sequenciamento Completo do Genoma
6.
Otol Neurotol ; 40(4): 504-510, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30870367

RESUMO

BACKGROUND: The Acoustic Neuroma Association (ANA) is a national, nonprofit organization, focused on the education and support of patients with vestibular schwannoma (VS). The aim of the present study is to characterize the profile of ANA survey respondents and compare them with non-ANA patients evaluated at a single tertiary academic referral center to investigate the potential influence of selection bias. METHODS: A prospectively maintained VS quality-of-life (QOL) database, comprised of patients evaluated at the authors' center and members of the ANA, was queried. Demographic variables, patient-reported symptoms and tumor characteristics, as well as patient-reported outcome scores were captured. Health-related QOL was evaluated using the disease-specific Penn Acoustic Neuroma QOL (PANQOL) questionnaire. Multivariable regression models were fitted for PANQOL domain and total scores as well as satisfaction with treatment adjusting for baseline demographics, symptoms, and PANQOL scores. RESULTS: A total of 1,060 patients (802 [76%] ANA respondents) were analyzed. Overall, ANA patients were slightly younger (mean age: 59 vs 60 yr, p = 0.145), more likely to be women (72 vs 55%, p < 0.001), and had a larger tumor size (overall p < 0.001). Furthermore, a significantly higher proportion of ANA patients were more likely to undergo microsurgery (57 vs 21%) or radiation (21 vs 8%) and less likely to be managed with observation (16 vs 65%, overall p < 0.001). A significantly higher proportion of ANA patients reported hearing loss (95 vs 88%, p < 0.001), tinnitus (80 vs 73%, p = 0.034), dizziness (78 vs 64%, p < 0.001), headache (56 vs 45% p = 0.003), and facial paralysis (37 vs 12%, p < 0.001). On multivariable analysis, ANA respondents exhibited significantly lower PANQOL scores for hearing (OR: 0.47, 95% CI: 0.35-0.64, p < 0.001), balance (OR: 0.51, 95% CI: 0.38-0.70, p < 0.001), pain (OR: 0.63, 95% CI: 0.46-0.86, p = 0.004), facial function (OR: 0.58, 95% CI: 0.42-0.80, p = 0.001), energy (OR: 0.44, 95% CI: 0.32-0.59, p < 0.001), anxiety (OR: 0.54, 95% CI: 0.40-0.74, p < 0.001), general (OR: 0.72, 95% CI: 0.53-0.98, p = 0.03), and total QOL (OR: 0.40, 95% CI: 0.30-0.55, p < 0.001). No statistically significant difference was seen with regard to treatment satisfaction.To determine the true clinical relevance of these differences, the two groups were compared using the minimal clinically important difference (MCID) for each domain. MCID is defined as the smallest difference in score in the domain of interest that patients perceive as important, either beneficial or harmful. The domains for hearing, balance, energy, anxiety, and total QOL reached their respective MCID thresholds, indicating that the ANA cohort has QOL scores that are clinically, perceptually worse for these domains compared to the non-ANA group. CONCLUSION: These data help delineate some of the inherent limitations and biases associated with survey studies incorporating data from national patient support organizations. The population profile of ANA survey respondents likely differs significantly from the greater population of patients with VS that may be encountered at a tertiary referral center.


Assuntos
Neuroma Acústico/cirurgia , Seleção de Pacientes , Assistência Centrada no Paciente/estatística & dados numéricos , Viés de Seleção , Adulto , Idoso , Estudos de Coortes , Feminino , Perda Auditiva/cirurgia , Humanos , Masculino , Microcirurgia , Pessoa de Meia-Idade , Análise Multivariada , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Inquéritos e Questionários , Centros de Atenção Terciária/estatística & dados numéricos
7.
Otolaryngol Head Neck Surg ; 160(6): 1081-1086, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30717626

RESUMO

OBJECTIVES: Conflicting research exists surrounding the utility of aspirin to prevent tumor growth in the medical management of vestibular schwannoma (VS). Recent studies demonstrated no association between aspirin and VS growth using linear tumor measurements. Given the heightened sensitivity of volumetric analyses to monitor tumor growth, the current study was conceived with the chief objective of assessing the association between aspirin or other nonsteroidal anti-inflammatory drug (NSAID) use and VS growth using volumetric analyses. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A total of 361 patients totaling 1601 volumetrically analyzed magnetic resonance imaging studies who underwent initial observation since January 1, 2003. RESULTS: In total, 123 (35%) patients took 81 mg aspirin daily, 23 (7%) took 325 mg aspirin daily, and 41 (11%) reported other NSAID use. Among those taking aspirin, 112 (72%) exhibited volumetric tumor growth during observation compared to 33 (80%) among other NSAID users and 137 (67%) among nonaspirin users. Patients taking aspirin or other NSAIDs were significantly older at time of diagnosis (median, 66 vs 56 years; P < .001). Neither aspirin use (hazard ratio [HR], 0.96; P = .73) nor other NSAID use (HR, 1.39; P = .081) was significantly associated with a reduced risk of volumetric tumor growth. These results were similar following age adjustment ( P = .81 and .087, respectively). When separating aspirin users by 81-mg or 325-mg dosing, neither group exhibited a reduced risk of growth ( P = .95 and .73, respectively). CONCLUSION: Despite promising initial results, the preponderance of existing literature suggests that aspirin and other NSAID use does not prevent tumor growth in VS.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Aspirina/uso terapêutico , Neuroma Acústico/patologia , Carga Tumoral , Idoso , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/tratamento farmacológico , Estudos Retrospectivos
8.
Otol Neurotol ; 2019 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-30807524

RESUMO

BACKGROUND: The Acoustic Neuroma Association (ANA) is a national, nonprofit organization, focused on the education and support of patients with vestibular schwannoma (VS). The aim of the present study is to characterize the profile of ANA survey respondents and compare them with non-ANA patients evaluated at a single tertiary academic referral center to investigate the potential influence of selection bias. METHODS: A prospectively maintained VS quality-of-life (QOL) database, comprised of patients evaluated at the authors' center and members of the ANA, was queried. Demographic variables, patient-reported symptoms and tumor characteristics, as well as patient-reported outcome scores were captured. Health-related QOL was evaluated using the disease-specific Penn Acoustic Neuroma QOL (PANQOL) questionnaire. Multivariable regression models were fitted for PANQOL domain and total scores as well as satisfaction with treatment adjusting for baseline demographics, symptoms, and PANQOL scores. RESULTS: A total of 1,060 patients (802 [76%] ANA respondents) were analyzed. Overall, ANA patients were slightly younger (mean age: 59 vs 60 yr, p = 0.145), more likely to be women (72 vs 55%, p < 0.001), and had a larger tumor size (overall p < 0.001). Furthermore, a significantly higher proportion of ANA patients were more likely to undergo microsurgery (57 vs 21%) or radiation (21 vs 8%) and less likely to be managed with observation (16 vs 65%, overall p < 0.001). A significantly higher proportion of ANA patients reported hearing loss (95 vs 88%, p < 0.001), tinnitus (80 vs 73%, p = 0.034), dizziness (78 vs 64%, p < 0.001), headache (56 vs 45% p = 0.003), and facial paralysis (37 vs 12%, p < 0.001). On multivariable analysis, ANA respondents exhibited significantly lower PANQOL scores for hearing (OR: 0.47, 95% CI: 0.35-0.64, p < 0.001), balance (OR: 0.51, 95% CI: 0.38-0.70, p < 0.001), pain (OR: 0.63, 95% CI: 0.46-0.86, p = 0.004), facial function (OR: 0.58, 95% CI: 0.42-0.80, p = 0.001), energy (OR: 0.44, 95% CI: 0.32-0.59, p < 0.001), anxiety (OR: 0.54, 95% CI: 0.40-0.74, p < 0.001), general (OR: 0.72, 95% CI: 0.53-0.98, p = 0.03), and total QOL (OR: 0.40, 95% CI: 0.30-0.55, p < 0.001). No statistically significant difference was seen with regard to treatment satisfaction.To determine the true clinical relevance of these differences, the two groups were compared using the minimal clinically important difference (MCID) for each domain. MCID is defined as the smallest difference in score in the domain of interest that patients perceive as important, either beneficial or harmful. The domains for hearing, balance, energy, anxiety, and total QOL reached their respective MCID thresholds, indicating that the ANA cohort has QOL scores that are clinically, perceptually worse for these domains compared to the non-ANA group. CONCLUSION: These data help delineate some of the inherent limitations and biases associated with survey studies incorporating data from national patient support organizations. The population profile of ANA survey respondents likely differs significantly from the greater population of patients with VS that may be encountered at a tertiary referral center.

9.
Neurosurgery ; 85(6): 779-785, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-30395303

RESUMO

BACKGROUND: The diagnosis of vestibular schwannomas (VS) is associated with reduced patient quality of life (QOL). Minimal clinically important difference (MCID) was introduced as the lowest improvement in a patient-reported outcome (PRO) score discerned as significant by the patient. We formerly presented an MCID for the Penn Acoustic Neuroma QOL (PANQOL) battery based on cross-sectional data from 2 tertiary referral centers. OBJECTIVE: To validate the PANQOL MCID values using prospective data. METHODS: A prospective registry capturing QOL was queried, comprising patients treated at the authors' institution and Acoustic Neuroma Association members. Anchor- and distribution-based techniques were utilized to determine the MCID for domain and total scores. We only included anchors with Spearman's correlation coefficient larger than 0.3 in the MCID threshold calculations. Most domains had multiple anchors with which to estimate the MCID. RESULTS: A total of 1254 patients (mean age: 57.4 yr, 65% females) were analyzed. Anchor-based methods produced a span of MCID values (median, 25th-75th percentile) for each PANQOL domain and the total score: hearing (13.1, 13-16 points), balance (14, 14-19 points), pain (21, 20-28 points), face (25, 16-36 points), energy (16, 15-18 points), anxiety (16 [1 estimate]), general (13 [1 estimate]), and total (12.5, 10-15 points). CONCLUSION: Current findings corroborate our formerly shared experience using multi-institutional, cross-sectional information. These MCID thresholds can serve as a pertinent outcome when deciphering the clinical magnitude of VS QOL endpoints in cross-sectional and longitudinal studies.


Assuntos
Diferença Mínima Clinicamente Importante , Neuroma Acústico/diagnóstico , Neuroma Acústico/psicologia , Qualidade de Vida/psicologia , Adulto , Idoso , Estudos de Coortes , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/terapia , Estudos Prospectivos , Sistema de Registros , Resultado do Tratamento
10.
Otol Neurotol ; 39(9): e849-e855, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30199501

RESUMO

OBJECTIVES: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. STUDY DESIGN: Multicenter retrospective review. SETTING: Three tertiary referral centers. PATIENTS: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. INTERVENTION: Surgical repair or observation. MAIN OUTCOME MEASURE: Radiographic findings and clinical symptoms were analyzed. RESULTS: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. CONCLUSIONS: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.


Assuntos
Cavidades Cranianas/diagnóstico por imagem , Perda Auditiva/classificação , Doenças do Labirinto/classificação , Canais Semicirculares/diagnóstico por imagem , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Cavidades Cranianas/cirurgia , Feminino , Perda Auditiva/diagnóstico por imagem , Perda Auditiva/cirurgia , Humanos , Doenças do Labirinto/diagnóstico por imagem , Doenças do Labirinto/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Canais Semicirculares/cirurgia , Adulto Jovem
11.
Otol Neurotol ; 39(10): e1129-e1136, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30239440

RESUMO

OBJECTIVE: To evaluate quality-of-life (QOL) in patients with sporadic vestibular schwannoma (VS) with particular focus on those recently diagnosed. STUDY DESIGN: Cross-sectional survey, Penn Acoustic Neuroma Quality of Life (PANQOL) instrument. SETTING: Acoustic Neuroma Association and a single tertiary referral center. PATIENTS: Patients with sporadic VS. MAIN OUTCOME MEASURES: Domain-specific and total PANQOL scores comparing treatment modalities after adjusting for baseline covariates of interest. RESULTS: Among all 1,288 respondents there were 229 (18%) who were recently diagnosed and had not yet selected a treatment modality, 303 (24%) who were observed, 185 (14%) who underwent radiosurgery alone, 507 (39%) who underwent microsurgery alone, and 64 (5%) who underwent radiosurgery and microsurgery. After adjusting for covariates of interest, total PANQOL scores were highest for the observation cohort (65; 95% CI 62-68), lowest for patients treated with multimodality therapy (56; 51-61) and those recently diagnosed (58; 55-62), and intermediate for those who received microsurgery alone (60; 58-62) and radiosurgery alone (61; 57-64) (global comparison, p = 0.001). When comparing groups that received single-modality therapy, there were no statistically significant differences in total PANQOL scores at short (0-5 yr), intermediate (6-10 yr), or long-term (≥11 yr) follow-up after adjusting for baseline covariates (all, p > 0.05). CONCLUSION: A new diagnosis of VS frequently imparts significant anxiety on the patient and leads to a temporary reduction in QOL. Notably, anxiety domain QOL scores among patients with newly diagnosed VS are poorer than patients who have observed their tumor for 6 months or longer. When feasible, an initial period of observation may afford patients valuable time to reconcile with the new diagnosis, to learn more about treatment options, and to discover whether there is tumor growth. In turn, this valuable information can be used to inform sound decision-making regarding tumor management.


Assuntos
Neuroma Acústico/psicologia , Qualidade de Vida/psicologia , Adulto , Idoso , Ansiedade/epidemiologia , Ansiedade/etiologia , Estudos de Coortes , Aconselhamento , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/cirurgia
12.
Otol Neurotol ; 39(8): e704-e711, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30036205

RESUMO

OBJECTIVE: To characterize the risk of progression to nonserviceable hearing in patients with sporadic vestibular schwannomas (VS) who elect initial observation. STUDY DESIGN: Retrospective case series. SETTING: Two tertiary care centers. PATIENTS: VS patients with serviceable hearing who underwent at least two audiograms and two MRI studies before intervention or loss to follow-up. MAIN OUTCOME MEASURE(S): Serviceable hearing, defined as the pure tone average ≤ 50 dB HL and word recognition score ≥ 50%. RESULTS: Four-hundred sixty-six patients (median age of 57 yr and median tumor diameter of 7.3 mm) had serviceable hearing at presentation and were followed for a median of 2.3 years (IQR 1.0 - 4.0). Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years following diagnosis were 94% (91-96; 357), 77% (73-82; 172), 66% (60-73; 81), 56% (49-65; 31), and 44% (33-59; 10), respectively. Each 10-dB increase in pure-tone averages at diagnosis was associated with a 2-fold increased likelihood of developing nonserviceable hearing (hazard ratio 2.07; p < 0.001). Each 10% decrease in word recognition score was associated with a 1.5-fold increased likelihood of developing nonserviceable hearing (hazard ratio 1.48; p < 0.001). CONCLUSIONS: Among patients with sporadic VS, good baseline word recognition score and low pure-tone average are jointly associated with maintenance of serviceable hearing. These data may be used to guide patient counseling and optimize management.


Assuntos
Audição/fisiologia , Neuroma Acústico/fisiopatologia , Idoso , Tratamento Conservador , Progressão da Doença , Feminino , Testes Auditivos , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/terapia , Estudos Retrospectivos , Resultado do Tratamento
13.
J Neurol Surg B Skull Base ; 79(3): 289-296, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29765827

RESUMO

Background Very few studies have examined vestibular schwannoma (VS) management trends across centers and between providers. The objective of this study is to examine current practice trends, variance in treatment philosophies, and nuanced or controversial aspects of VS care across North America. Methods This is a cross-sectional survey of North American Skull Base Society (NASBS) members who report regular involvement in VS care. Results A total of 57 completed surveys were returned. Most respondents claimed to have over 20 years of experience and the majority reported working in an academic practice with an affiliated otolaryngology and/or neurosurgery residency program. Sixty-three percent of respondents claimed to evaluate VS patients in clinic with both an otolaryngologist and neurosurgeon involved. Eighty-six percent of respondents claimed to operate on VS with both an otolaryngologist and neurosurgeon involved, while only 18% of neurosurgeons and 9% of otolaryngologists performed surgery alone. There was a wide range in the number of cases evaluated at each center annually. Similarly, there was wide variation in the number of patients treated with microsurgery and radiation at each center. Additional details regarding management preferences for microsurgery, stereotactic radiosurgery, stereotactic radiotherapy, and conservative observation are presented. Conclusion VS management practices vary between providers and centers. Overall, most centers employ a multidisciplinary approach to management with collaboration between otolaryngology and neurosurgery. Overall, survey responses concur with previous studies suggesting a shift toward conservatism in management.

14.
J Neurol Surg B Skull Base ; 79(3): 297-301, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29765828

RESUMO

Introduction Perioperative care of vestibular schwannoma (VS) patients is extremely variable across surgeons and institutions making practice patterns difficult to standardize. No data currently exist detailing this practice variability. Methods The North American Skull Base Society membership was electronically surveyed regarding perioperative care of surgically operated VS patients. Results There were 87 respondents to the survey. Surgical positioning, surgical approach utilized, and perioperative medical adjuncts are quite variable. However, of those performing retrosigmoid approaches, 49% perform this in the supine position, while 33% use a park-bench position with only 2% using the sitting position. In those performing translabyrinthine approaches, 86% perform this in supine position. Although the use of neuromonitoring appears to be standard of care (98%), other than the seventh nerve, there is substantial variability between respondents regarding monitoring of additional cranial nerves. Postoperative antibiotics are used by 65%, postoperative steroids 81%, and postoperative chemical deep vein thrombosis prophylaxis in 68% of survey respondents. Conclusion Although the perioperative adjuncts to VS surgery are variable, there does appear to be a trend in common practice. Therefore, making surgeons aware of these trends may lead to standardized practice or alternatively trials of these variances to instruct which truly improve patient outcomes.

15.
Otolaryngol Head Neck Surg ; 159(3): 535-542, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29685084

RESUMO

Objective (1) Assess 3-dimensional volumetric growth of untreated sporadic vestibular schwannomas (VSs) in a large cohort of patients treated with conservative observation. (2) Compare volumetric and conventional linear diameter measurements for detecting tumor growth. Study Design Case series with chart review. Setting Tertiary skull base referral center. Subjects and Methods Patients with sporadic VS who elected initial conservative treatment with at least 2 serial magnetic resonance imaging (MRI) scans were included. Tumor volume was determined with 3-dimensional segmentation of MRI sequences. The volumetric threshold for tumor growth was an increase ≥20% from baseline tumor volume. Tumor size based on linear diameter was assessed with the 1995 American Academy of Otolaryngology-Head and Neck Surgery Foundation guidelines for VS outcome reporting, with growth defined as an increase ≥2 mm. Results A total of 361 patients were included with a median radiologic follow-up of 4.1 years (interquartile range [IQR], 2.5-6.8). At diagnosis, 232 VSs (64%) were purely intracanalicular, and 129 (36%) extended into the cerebellopontine angle. The median baseline tumor volume was 0.161 cm3 (IQR, 0.054-0.418). Overall, 69% of tumors demonstrated volumetric growth at a median of 1.1 years (IQR, 0.6-2.1) after initial MRI. In contrast, based on linear measurement assessment, 48% of tumors demonstrated growth at a median of 1.8 years (IQR, 0.8-3.1) from first MRI scan. Disequilibrium, facial hypoesthesia, aural fullness, initial tumor size, and nonincidental diagnosis were associated with tumor growth. Conclusion Three-dimensional volumetric assessment of VS provides a more sensitive measure of tumor growth when compared with linear diameter assessment. Through volumetric analysis, the current study revealed that a significant proportion of VSs demonstrate growth during observation.


Assuntos
Imageamento Tridimensional , Imagem por Ressonância Magnética/métodos , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/fisiopatologia , Carga Tumoral/fisiologia , Conduta Expectante/métodos , Centros Médicos Acadêmicos , Adulto , Idoso , Estudos de Coortes , Bases de Dados Factuais , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Neuroma Acústico/mortalidade , Neuroma Acústico/terapia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento
16.
World Neurosurg ; 114: e1245-e1252, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29625305

RESUMO

OBJECTIVES: To ascertain primary motivation and long-term satisfaction with treatment selection in patients with vestibular schwannoma. METHODS: A multicenter, cross-sectional survey was performed. Patients with small- to medium-sized sporadic vestibular schwannoma who underwent stereotactic radiosurgery (SRS; n = 247), microsurgery (n = 144), or observation (n = 148) between 1998 and 2008 were surveyed regarding their motivation behind treatment selection and hindsight satisfaction with their choice of management. RESULTS: "Physician recommendation" was the most commonly stated reason for modality selection in all 3 groups. The second and third most common reasons for selecting SRS included "less invasive option than surgery" in 80 patients (32%) and "less recovery time than surgery" in 16 patients (6%). The second and third most common reasons for selecting observation included "to avoid side-effects of treatment" in 25 patients (17%) and "symptoms not severe enough to warrant intervention" in 22 patients (15%). The second and third most common reasons for selecting microsurgery included "do not want tumor in head" in 35 patients (24%) and "most definitive treatment" in 15 patients (10%). Overall, 232 patients (96%) treated with SRS, 141 observed patients (97%), and 121 patients (85%) who underwent microsurgical treatment were satisfied with their original decision (P < 0.001). CONCLUSIONS: Motivation behind treatment selection varies between individuals. Those who select observation and SRS commonly reference less invasiveness and lower risk, whereas those who select microsurgery are commonly motivated by having their tumor physically removed and the more definitive nature of treatment. Posttreatment satisfaction is highest in patients who undergo SRS and observation, although all 3 groups report high levels of satisfaction.


Assuntos
Tomada de Decisões , Motivação , Neuroma Acústico/psicologia , Neuroma Acústico/terapia , Satisfação do Paciente , Relações Médico-Paciente , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Microcirurgia/estatística & dados numéricos , Pessoa de Meia-Idade , Neuroma Acústico/epidemiologia , Radiocirurgia/estatística & dados numéricos , Fatores de Tempo , Resultado do Tratamento
17.
Otol Neurotol ; 39(1): e12-e19, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29210952

RESUMO

OBJECTIVE: To examine practice variance of cochlear implant candidacy assessment and off-label indications across centers in the United States. METHODS: Cross-sectional survey of the American Neurotology Society (ANS). RESULTS: A total of 81 surveys were returned from ANS members who report regular involvement in cochlear implant care. Overall there was a broad distribution in age and clinical experience, with most respondents reporting ACGME accreditation in neurotology and employment at an academic center. The annual volume of cochlear implant surgeries varied considerably across centers.Seventy-eight percent of respondents performed cochlear implantation for at least one of the following indications within the last 2 years: profound hearing loss in children less than 12 months of age (35, 43%), children with asymmetrical hearing loss where at least one ear was better than performance cutoff for age (25, 31%), adults with asymmetrical hearing where at least one ear was better than the performance cutoff for adult criteria (49, 61%), single-sided deafness (37, 46%), and ipsilateral vestibular schwannoma (28, 35%). Centers with a higher annual implant volume more frequently performed off-label implantation in all queried populations (all, p≤0.001), and performed surgery on infants with congenital deafness at a younger age (p = 0.013), compared with centers with lower surgical volume.When surveyed regarding speech perception testing practices for adult candidacy assessment, 75 (100%) respondents who answered this question reported routine use of AzBio sentences, 42 (56%) CNC word scores, and 26 (35%) HINT testing; only 7 (9%) reported using BKB-SIN testing and 6 (8%) reported using CUNY scores. Fifty-one (68%) reported routine use of speech-in-noise testing to determine adult cochlear implant candidacy, 21 (28%) reported selective use only when patient scores were borderline in quiet, and 3 (4%) reported that their center does not currently use testing in noise for candidacy determination. Nineteen (26%) solely used +10 dB signal-to-noise ratio (SNR), 12 (16%) solely used +5 dB SNR, and 41 (55%) used both +10 and +5 dB SNR. Overall, 19% (N = 14) only perform unilateral implantation in the Medicare population, while 81% (N = 58) consider bilateral implantation. CONCLUSION: Significant variation in cochlear implant candidacy assessment and off-label implantation exists across centers and providers in the United States resulting in healthcare inequities. The high percentage of surgeons performing implantations for off-label or nontraditional indications reflects the overly restrictive and dated status of current implant guidelines. With greater adoption of more difficult speech perception testing in noise, careful clinical judgment is needed to maintain a favorable risk-benefit balance for prospective implant candidates.


Assuntos
Implante Coclear , Perda Auditiva/cirurgia , Neuro-Otologia/estatística & dados numéricos , Seleção de Pacientes , Padrões de Prática Médica/estatística & dados numéricos , Adulto , Idoso , Criança , Implante Coclear/métodos , Implante Coclear/estatística & dados numéricos , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Inquéritos e Questionários , Estados Unidos
18.
Otol Neurotol ; 38(6): e128-e133, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28538468

RESUMO

OBJECTIVE: Currently, there is a paucity of literature evaluating hearing preservation outcomes in children following cochlear implantation. The objective of the current study is to report pediatric hearing preservation results following cochlear implantation with conventional full-length electrodes. STUDY DESIGN: Retrospective review (2000-2016). SETTING: Tertiary referral center. PATIENTS: All pediatric patients with a ≤ 75 dB preoperative low-frequency pure tone average (LFPTA; 250-500 Hz average), who underwent cochlear implantation with a conventional length electrode. INTERVENTION(S): Cochlear implantation. MAIN OUTCOME MEASURE(S): Complete, partial, minimal, or no hearing preservation following cochlear implantation (Skarzynski et al., 2013); maintenance of functional low frequency hearing (≤85 dB LFPTA). RESULTS: A total of 43 ears, in 35 pediatric patients, met inclusion criteria. The mean age at time of implantation was 8.6 years (range, 1.4-17.8 yr), 20 (57.1%) patients were female, and 25 (58.1%) cases were left-sided.The mean preoperative ipsilateral low frequency PTA and conventional four-frequency PTA (500, 1000, 2000, 3000 Hz average) were 54.2 dB (range, 15-75 dB) and 82.2 dB (range, 25-102.5 dB), respectively. The mean low frequency PTA and conventional four-frequency PTA shifts comparing the pre- and first postoperative audiogram were Δ25.2 dB (range, -5 to 92.5 dB) and Δ18.3 dB (range, -8.8 to 100 dB), respectively. Overall, 17 (39.5%) ears demonstrated complete hearing preservation, 19 (44.2%) ears partial hearing preservation, 0 minimal hearing preservation, and 7 (16.3%) exhibited no measurable acoustic hearing after surgery. In total, 28 (65.1%) ears maintained functional low-frequency hearing (i.e., ≤85 dB LFPTA) based on the initial postoperative audiogram. There was no statistically significant difference in the initial low frequency PTA shift comparing lateral wall and perimodiolar electrodes (Δ22.2 versus Δ28.1 respectively; p = 0.44), cochleostomy and round window insertions (Δ25.2 vs. Δ24.7 respectively; p = 0.95), or statistically significant association between age at implantation and low frequency PTA shift (r = 0.174; p = 0.26).In total, 22 ears in 19 patients had serial audiometric data available for review. Over a mean duration of 43.8 months (range, 2.6-108.3 mo) following surgery, the mean low frequency PTA and conventional four-frequency PTA shift comparing the initial postoperative and most recent postoperative audiogram was Δ9.7 dB (range, -27.5 to 57.5 dB) and Δ8.1 dB (range, -18.8 to 31.9 dB), respectively. CONCLUSIONS: Varying levels of hearing preservation with conventional length electrodes can be achieved in most pediatric subjects. In the current study, 82% of patients maintained detectable hearing thresholds and 65% maintained functional low-frequency acoustic hearing. These data may be used to guide preoperative counseling in pediatric patients with residual acoustic hearing. Additionally, the favorable rates of hearing preservation achieved in children provide further evidence for the expansion of pediatric cochlear implant candidacy to include patients with greater degrees of residual hearing.


Assuntos
Implante Coclear/métodos , Implantes Cocleares , Perda Auditiva Neurossensorial/reabilitação , Adolescente , Audiometria de Tons Puros , Limiar Auditivo , Criança , Pré-Escolar , Feminino , Audição , Humanos , Lactente , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Centros de Atenção Terciária , Resultado do Tratamento
19.
Genes Chromosomes Cancer ; 56(1): 59-74, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27636103

RESUMO

Common fragile sites (CFS) are chromosome regions that are prone to form gaps or breaks in response to DNA replication stress. They are often found as hotspots for sister chromatid exchanges, deletions, and amplifications in different cancers. Many of the CFS regions are found to span genes whose genomic sequence is greater than 1 Mb, some of which have been demonstrated to function as important tumor suppressors. CFS regions are also hotspots for human papillomavirus (HPV) integrations in cervical cancer. We used mate-pair sequencing to examine HPV integration events and chromosomal structural variations in 34 oropharyngeal squamous cell carcinoma (OPSCC). We used endpoint PCR and Sanger sequencing to validate each HPV integration event and found HPV integrations preferentially occurred within CFS regions similar to what is observed in cervical cancer. We also found that many of the chromosomal alterations detected also occurred at or near the cytogenetic location of CFSs. Several large genes were also found to be recurrent targets of rearrangements, independent of HPV integrations, including CSMD1 (2.1Mb), LRP1B (1.9Mb), and LARGE1 (0.7Mb). Sanger sequencing revealed that the nucleotide sequences near to identified junction sites contained repetitive and AT-rich sequences that were shown to have the potential to form stem-loop DNA secondary structures that might stall DNA replication fork progression during replication stress. This could then cause increased instability in these regions which could lead to cancer development in human cells. Our findings suggest that CFSs and some specific large genes appear to play important roles in OPSCC. © 2016 Wiley Periodicals, Inc.


Assuntos
Biomarcadores Tumorais/genética , Carcinoma de Células Escamosas/genética , Sítios Frágeis do Cromossomo/genética , Rearranjo Gênico , Neoplasias Orofaríngeas/genética , Papillomaviridae/genética , Infecções por Papillomavirus/genética , Integração Viral/genética , Pareamento de Bases , Sequência de Bases , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/virologia , Aberrações Cromossômicas , Humanos , Proteínas de Membrana/genética , Neoplasias Orofaríngeas/patologia , Neoplasias Orofaríngeas/virologia , Infecções por Papillomavirus/patologia , Infecções por Papillomavirus/virologia , Prognóstico , Receptores de LDL/genética , Homologia de Sequência do Ácido Nucleico
20.
Laryngoscope ; 127(6): 1420-1426, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27515152

RESUMO

OBJECTIVES: The frequency of intratumoral hemorrhage (ITH) in vestibular schwannoma (VS) remains undefined. METHODS: Retrospective case series of all patients diagnosed with hemorrhagic VS between 2003 and 2015 at a single tertiary academic skull base center. RESULTS: Five patients with ITH were evaluated, representing 0.4% of all newly diagnosed VS evaluated at the authors' center during this time. The median age at time of diagnosis was 66 years (range 39-83), four of five cases occurred in men, and all had sporadic unilateral tumors. The frequency of ITH among patients receiving anticoagulation was 5.6% (2 of 36), compared to only 0.2% (3 of 1356) in non-anticoagulated patients (P = 0.006), representing a 25-fold increase. At time of hemorrhage, all patients had acute onset of headache, disequilibrium, and progression of hearing loss; three reported trigeminal symptoms, and two exhibited acute moderate facial paresis. The median tumor size at diagnosis of hemorrhage was 3.1 cm (range 2.4-4.2 cm), and three patients had radiological evidence of hydrocephalus. All patients underwent microsurgical resection. There were no perioperative deaths. At a median follow-up of 25 months (3-70 months), no patient has experienced tumor recurrence. CONCLUSION: Tumor-associated hemorrhage in VS occurs in 0.4% of cases and commonly presents with acute neurological change. The risk of clinically significant hemorrhage is greater in patients receiving anticoagulation compared to the general VS population. Prompt microsurgical resection should be pursued when possible since tumor removal may improve neurological symptoms, relieve brainstem compression, and reduce the risk of repeat hemorrhage. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1420-1426, 2017.


Assuntos
Hemorragia/etiologia , Neuroma Acústico/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Paralisia Facial/etiologia , Paralisia Facial/cirurgia , Feminino , Seguimentos , Cefaleia/etiologia , Cefaleia/cirurgia , Perda Auditiva/etiologia , Perda Auditiva/cirurgia , Hemorragia/cirurgia , Humanos , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Masculino , Microcirurgia/métodos , Pessoa de Meia-Idade , Estudos Retrospectivos
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