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1.
Otol Neurotol ; 41(4): e501-e506, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32176143

RESUMO

OBJECTIVE: Closely paralleling previous radiologic studies, recent population-based prevalence data suggest sporadic vestibular schwannoma (VS) affects over one in 2,000 adults and up to one in 500 in those aged 70 years or older. Attributable to increased utilization of magnetic resonance imaging and screening protocols for asymmetrical sensorineural hearing loss, the increasing detection rate of sporadic VS fundamentally changes the perception of VS as a whole. The primary objective of the current study was to contextualize modern epidemiological trends in neurofibromatosis type 2 (NF2) in light of these recent advancements in the understanding of sporadic VS. STUDY DESIGN: Population-based study. SETTING: Olmsted County, Minnesota. Population size on January 1, 2017: 159,689 people. PATIENTS: All patients with NF2 diagnosed between Jan 1, 1966 and Dec 31, 2016, identified using the Rochester Epidemiology Project. MAIN OUTCOME MEASURES: Incidence, prevalence. RESULTS: Six incident cases were identified over the past 50 years. From 1966 to 2016, the age- and sex-adjusted incidence rate was 0.10 per 100,000 person-years. The incidence rate remained at 0.12 over the most recent decade. Five cases met disease prevalence criteria, and the age- and sex-adjusted prevalence of NF2 on Jan 1, 2017 was 3.1 per 100,000 persons. All prevalent cases were women, and the resultant prevalence among women only was 6.0 per 100,000 persons. CONCLUSIONS: The modern age- and sex-adjusted incidence rate and prevalence of NF2 is 0.10 per 100,000 person-years and 3.1 per 100,000 persons, respectively. In contrast to trends in incidence rates of sporadic VS over the last half-century, this study demonstrates that the incidence of NF2 has remained relatively stable since 1966. This divergence in epidemiological trends is likely attributed to the nature of NF2, with early clinical manifestations resulting in diagnosis regardless of modern advances, whereas the increased incidence of sporadic VS is heavily influenced by improved detection in a greater population of patients with minimally symptomatic or asymptomatic tumors.

2.
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.

3.
Laryngoscope ; 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31841234

RESUMO

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 2019.

4.
Otol Neurotol ; 40(10): 1363-1372, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31725593

RESUMO

OBJECTIVE: To ascertain long-term hearing outcomes in patients with serviceable hearing following microsurgical resection of sporadic vestibular schwannoma (VS). STUDY DESIGN: Retrospective cohort. SETTING: Tertiary academic referral center. PATIENTS: Forty-three adult subjects with unilateral sporadic VS who had serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) on initial postoperative audiogram following microsurgical resection between 2003 and 2016 with a minimum of two postoperative audiograms available for review. INTERVENTION: Surgical treatment with a retrosigmoid or middle cranial fossa approach. MAIN OUTCOME MEASURE: Rate of maintaining serviceable hearing, as estimated using the Kaplan-Meier method, in accordance with the 1995 and 2012 AAO-HNS guidelines on reporting hearing outcomes. RESULTS: The median immediate postoperative pure-tone average (PTA) and word recognition score (WRS) were 31 dB and 95%, respectively. At last follow-up, the median PTA was 38 dB with a median change of 5 dB from initial postoperative audiogram, and the median WRS was 90% with a median change of 0% from initial postoperative audiogram. Eight patients developed non-serviceable hearing at a median of 4.1 years following microsurgical resection (interquartile range, 2.9-7.0). The median duration of hearing follow-up for the 35 patients who maintained serviceable hearing was 3.1 years (interquartile range, 2.2-7.5). Tumor control was achieved in 41 (95%) patients. The rate of maintaining serviceable hearing at 5 years was 81%. CONCLUSION: Microsurgical resection provides excellent tumor control and durable long-term hearing in those with AAO-HNS class A or B hearing postoperatively. The paradigm of proactive microsurgical resection-when the tumor is small and hearing is good-hinges on the surgeon's ability to preserve residual hearing in a very high percentage of cases at or near preoperative hearing levels to maintain an advantage over conservative observation with regard to long-term hearing preservation.

5.
Otol Neurotol ; 40(10): e1012-e1017, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31634279

RESUMO

OBJECTIVE: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation. SETTING: Two tertiary care centers. PATIENTS: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two. MAIN OUTCOME MEASURE(S): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%. RESULTS: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95-100; 206), 78% (72-85; 98), 68% (60-77; 39), 60% (50-73; 17), and 44% (29-67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44-2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66-0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72-3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67-0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing. CONCLUSION: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

6.
Ear Nose Throat J ; : 145561319869609, 2019 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-31569975
7.
Ear Nose Throat J ; : 145561319849010, 2019 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-31064246
8.
J Neurol Surg B Skull Base ; 80(2): 169-177, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30931225

RESUMO

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

9.
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
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