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1.
Otol Neurotol ; 41(2): e201-e207, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31923158

RESUMO

OBJECTIVE: Intraoperative far-field auditory brainstem response (ABR) and direct cochlear nerve action potential monitoring using neural response imaging (NRI) are techniques for monitoring the cochlear nerve during vestibular schwannoma (VS) surgery. A new paradigm has developed where the cochlear nerve is anatomically preserved during tumor removal to facilitate cochlear implantation in select circumstances. This report describes the use of an unmodified commercial cochlear implant (CI) to elicit electrically-evoked direct cochlear nerve and far-field ABR monitoring to evaluate the status of the cochlear nerve during tumor resection in real time. PATIENTS: Adult female with unilateral, sporadic VS. INTERVENTION(S): Cochlear implantation followed by translabyrinthine resection of VS in single operation. MAIN OUTCOME MEASURES: During tumor resection intra-cochlear electrodes were used to deliver electrical stimulation and measure NRI or the cochlear nerve action potential. Electrically-evoked ABR (eABR) was measured using surface electrodes and wave V was monitored (far-field ABR) during surgery. RESULTS: A 61-year-old female was evaluated for a unilateral, enlarging intracanalicular VS with asymmetric SNHL. The patient opted for microsurgery and due to her bilateral hearing loss was a candidate for CI. Cochlear implantation with an unmodified, commercially available lateral wall electrode was performed. The remainder of the approach and tumor resection was performed under continuous eABR and NRI monitoring. Gross total resection was achieved with intact eABR and NRI at the conclusion of the case. Changes in ABR and NRI consistently recovered after modifying dissection strategy. The patient reported auditory percepts with her cochlear implant postoperatively. CONCLUSIONS: This report demonstrates the feasibility of using real-time NRI and eABR with a CI to facilitate preservation of the cochlear nerve during VS microsurgery. Using this method to mitigate cochlear nerve trauma during microsurgery may preserve the option of CI for hearing rehabilitation.

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.


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
3.
Otol Neurotol ; 41(3): e317-e321, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31834875

RESUMO

OBJECTIVE: Describe the first case of cochlear implantation (CI) for auditory rehabilitation of a patient with craniometaphyseal dysplasia (CMD) and progressive mixed hearing loss. PATIENTS: A 65-year-old woman with known autosomal dominant CMD presented with progressive mixed hearing loss and declining benefit from conventional hearing aids. Computed tomography and magnetic resonance imaging revealed hyperostosis of the entire craniofacial skeleton. Hearing evaluation demonstrated pure-tone thresholds in the profound range bilaterally by air conduction, and bone conduction thresholds that matched aided thresholds for her left ear, though testing was somewhat limited by inability to mask at high air-conduction thresholds. CI candidacy testing confirmed poor word and sentence scores in the right ear. INTERVENTION: Due to the inability to access the cochlea via a conventional mastoidectomy and facial recess approach, cochlear implantation via a postauricular subtotal petrosectomy approach with ear canal overclosure was performed. MAIN OUTCOME MEASURES: Post-implantation word and sentence testing. RESULTS: Despite extensive internal auditory canal stenosis, the patient demonstrated excellent early speech understanding results 5 weeks after device activation. Postimplantation audiologic evaluation showed thresholds between 20 and 30 dB HL from 250 to 6000 Hz. Word and sentence testing scores were 76% Consonant-nucleus-consonant in quiet (up from 2% preoperatively) and 77% AzBio sentences in quiet (up from 10% preoperatively). CONCLUSION: This report describes the first description of CI for CMD. Despite the extensive radiologic abnormalities, the patient has demonstrated excellent benefit from implantation. Further study of rare temporal bone dysplasias, such as CMD, is critical to better characterize the progression of otologic disease and determine optimal treatment.

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): 1287-1291, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31644474

RESUMO

OBJECTIVE: To report the use of multi-frequency intra-cochlear electrocochleography (ECOG) in monitoring and optimizing electrode placement during cochlear implant surgery. An acoustic pure tone complex comprising of 250, 500, 1000, and 2000 Hz was used to elicit ECOG, or more specifically cochlear microphonics (CMs), responses from various locations in the cochlea. The most apical cochlear implant electrode was used as the recording electrode. STUDY DESIGN: Clinical capsule report. SETTING: Tertiary academic referral center. RESULTS: ECOG measurements were performed during cochlear implant surgery in an adult patient with significant residual acoustic hearing. The 500, 1000, and 2000 Hz CM tracings from the most apical electrode showed an amplitude peak at three different instances during the early phase of cochlear implant electrode insertion. These results are consistent with the tonotopic organization of the cochlea. During final electrode placement a slight advancement of the electrode array resulted in a correlated decrease in 250, 500, and/or 1000 Hz CM amplitude. The electrode array was retracted and repositioned which resulted in a recovery of CM amplitude. Intraoperative CM thresholds revealed a correlation of r = 0.87 with preoperative audiometric thresholds. CONCLUSION: We present a report on simultaneous multi-frequency ECOG monitoring during cochlear implant surgery. Multi-frequency ECOG can be used to differentiate between electrode trauma and the advancement of the apical electrode beyond the CM source in the cochlea.

6.
Otol Neurotol ; 40(9): 1224-1229, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31469794

RESUMO

OBJECTIVE: Previous research has shown that tumor growth during observation of small-to-medium sized sporadic vestibular schwannomas (VSs) occurs almost exclusively within 3 to 5 years following diagnosis. This has led some to consider ending surveillance after this interval. This study seeks to characterize a cohort of patients with tumors that exhibited late growth. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Adults with sporadic VSs who initially elected observation with serial magnetic resonance imaging (MRI) surveillance. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Linear tumor growth was measured in accordance with AAO-HNS reporting guidelines. Delayed growth was defined as growth ≥2 mm in linear diameter that was first detected 5 years or more from the initial MRI. RESULTS: From a total of 361 patients, 172 experienced tumor growth during the interval of observation. Fourteen of these 172 patients (8.1%) experienced late growth occurring at 5 years or beyond. Among patients with delayed growth, the fastest growth rate after extended quiescence was 1.33 mm/yr, and the longest delay before tumor growth detection was 11.1 years. Additional treatment was recommended for six (42.9%) of the patients with delayed growth. Of 68 tumors that remained in the IAC, 11 (16.2%) demonstrated delayed growth. Of 66 tumors that presented in the CPA, 2 (3.0%) demonstrated delayed growth. Initial size was larger for tumors demonstrating early growth compared with those with delayed growth. For tumors within the IAC, those with early growth had a significantly higher median growth rate than those with delayed growth (1.40 vs. 0.45 mm/yr, p < 0.001). CONCLUSIONS: Delayed growth encompassed 8.1% of growing VSs and 3.9% of all observed tumors. Patients with delayed growth exhibited slower growth rates compared with those who were diagnosed with growth early in their observation course. These findings support the need for lifelong surveillance of untreated VSs given the possibility of clinically significant delayed growth. Increasing the time interval between MRI studies after 5 years is a reasonable concession to balance practicalities of cost and convenience with risk of delayed of tumor growth.

7.
Otol Neurotol ; 40(9): 1230-1236, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31469795

RESUMO

OBJECTIVE: The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. STUDY DESIGN: Blinded, retrospective review of imaging and audiometry. SETTING: Tertiary center. PATIENTS: Adults with JP. INTERVENTION(S): None. MAIN OUTCOME MEASURES: Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. RESULTS: Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p < 0.0001). Regression analysis demonstrates that enhancement within the CA is associated with a BCPTA difference of 19.4 dB HL (p = 0.0006). CONCLUSIONS: Cochlear aqueduct involvement by JP is associated with SNHL in the absence of inner ear fistula, vestibulocochlear nerve involvement, or brainstem compression. Correlation with operative findings or histopathologic evidence of tumor involvement may validate this intriguing imaging finding.

8.
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.

9.
J Med Case Rep ; 13(1): 215, 2019 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-31303177

RESUMO

BACKGROUND: Recurrent respiratory papillomatosis is a chronic disease of viral origin affecting the larynx, trachea, and lower airways. Inverted papilloma, most commonly originating from the lateral nasal wall, is typically a single, expansile, locally aggressive tumor that remodels bone around the site of origin. CASE PRESENTATION: We report a case of histopathologically proven inverted papilloma occurring in a 50-year-old Caucasian man with recurrent respiratory papillomatosis affecting his nasal cavity, larynx, and trachea. This constitutes the first report of nasal involvement in recurrent respiratory papillomatosis. Viral in situ hybridization studies demonstrated evidence of human papillomavirus in both the septum and middle turbinate subsites. Repeat nasal excision with margin analysis is planned. CONCLUSIONS: This report emphasizes the importance of considering a broad differential diagnosis in patients with papillomata, and obtaining comprehensive histopathologic evaluation of lesions in multiple subsites in order to rule out inverted papilloma or overt malignant transformation, particularly if high-risk human papillomavirus (HPV) subtypes are identified. LEVEL OF EVIDENCE: 4.


Assuntos
Neoplasias Nasais/complicações , Papiloma Invertido/complicações , Infecções por Papillomavirus/complicações , Infecções Respiratórias/complicações , Transformação Celular Neoplásica/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Septo Nasal/patologia , Septo Nasal/cirurgia , Neoplasias Nasais/patologia , Neoplasias Nasais/cirurgia , Papiloma Invertido/patologia , Papiloma Invertido/cirurgia , Papillomaviridae/isolamento & purificação , Conchas Nasais/patologia , Conchas Nasais/cirurgia
10.
J Neurol Surg B Skull Base ; 80(Suppl 3): S286, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31143593

RESUMO

Objectives This video describes the surgical indications, relevant anatomy, and surgical steps of routine translabyrinthine surgery for gross total resection of sporadic vestibular schwannoma. Design The procedure is presented through a surgical instructional video. Setting The surgery took place at tertiary skull base referral center. Parcipant A 47-year-old patient reported with nonserviceable hearing, frequent episodes of vertigo, recurrent severe headache, and a small unilateral right sided vestibular schwannoma. Results Gross total resection with preservation of facial nerve function was achieved. Conclusion This instructional video documents the surgical steps and relevant anatomy for translabyrinthine resection of vestibular schwannoma. The link to the video can be found at: https://youtu.be/CJ2vKMLs7aI .

11.
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.

12.
J Neurol Surg Rep ; 80(1): e10-e13, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30941279

RESUMO

Objectives To present a rare case of traumatic facial neuroma involving the geniculate ganglion and review relevant literature. Patient Thirty-year-old man. Intervention Microsurgical resection via combined mastoid-middle fossa approach with great auricular nerve interpositional graft. Main Outcome Measures Patient demographics and pre- and postoperative facial nerve function. Results A 30-year-old man with a reported history of prior Bell's palsy developed progressive complete (House-Brackmann VI) right facial paralysis following blunt trauma. Imaging was strongly suggestive of a geniculate ganglion hemangioma. As the patient had no spontaneous improvement in his poor facial function over the course of 9 months, he underwent resection of the facial nerve lesion with great auricular nerve graft interposition via a combined mastoid-middle fossa approach. Histopathology demonstrated disorganized fascicles, with axonal clustering reminiscent of sprouting/regeneration following trauma. No cellular proliferation or vascular malformation was present. Conclusion Traumatic facial nerve neuromas can occur following temporal bone trauma and can closely mimic primary facial nerve tumors. Akin to the management of geniculate ganglion hemangioma and schwannoma, preoperative facial function largely dictates if and when surgery should be pursued.

13.
Otol Neurotol ; 40(1): 103-107, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30015753

RESUMO

OBJECTIVES: Catecholamine-secreting jugular paragangliomas (JPs) represent a rare subset of head and neck paragangliomas that may present with hypertension, arrhythmia, or syncopal episodes. Subtotal resection to protect critical neurovascular structures may result in persistent catecholamine excess from residual tumor. Herein, we report our experience with stereotactic radiosurgery (SRS) for salvage treatment of catecholamine-secreting JP following subtotal microsurgical resection. PATIENTS: Adult patients treated with SRS after subtotal microsurgical resection of catecholamine-secreting JP. INTERVENTIONS: SRS. MAIN OUTCOME MEASURES: Post-treatment catecholamine and metanephrine levels, clinical outcomes, and tumor control. RESULTS: Of 85 patients with JPs treated with primary or salvage radiosurgery between 1990 and 2017, 2 (2%) harbored nonmalignant secreting tumors. Patient 1 developed catecholamine excess with elevated norepinephrine (NE) at 475 mcg/24 hours (normal < 80 mcg/24 h). Following subtotal resection, she developed catecholamine excess with radiographic evidence of tumor growth and therefore underwent SRS. Three years post-SRS and beyond, catecholamine levels remained normalized (NE 62 mcg/24 h at 10 yr) and tumor volume remained stable on serial MRI studies over the 17-year follow-up period.Patient 2 developed symptomatic arrhythmia and was found to have a JP. Urine norepinephrine (NE) was elevated at 213 mcg/24 hours. She underwent nerve-sparing subtotal resection and upfront SRS was used to treat residual tumor. Twenty months following SRS, the area of residual JP had not enlarged in size and catecholamine levels remained normal (NE 46 mcg/24 h). CONCLUSIONS: Radiosurgery can be used in salvage treatment of catecholamine-secreting JP, providing durable tumor control and resolution of catecholamine excess. For patients with large catecholamine-secreting JP and normal lower cranial nerve function, aggressive nerve-sparing subtotal resection with adjuvant radiosurgery may offer a low-morbidity alternative to gross total resection. Further study of this subset of patients is warranted to substantiate these promising, yet preliminary findings.


Assuntos
Tumor do Glomo Jugular/cirurgia , Neoplasia Residual/radioterapia , Radiocirurgia , Adulto , Progressão da Doença , Feminino , Tumor do Glomo Jugular/patologia , Tumor do Glomo Jugular/radioterapia , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Terapia de Salvação , Resultado do Tratamento , Carga Tumoral
14.
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
15.
J Neurol Surg B Skull Base ; 79(5): 489-494, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30210977

RESUMO

Objectives To describe transnasal Eustachian tube (ET) occlusion with a liquid embolic solution for lateral skull base cerebrospinal fluid (CSF) leaks. Design A lateral skull base CSF fistula model was developed by the authors using fresh cadaveric heads. Using a transtympanic needle, regulated pressurized pigmented saline was continuously instilled into the middle ear space and visualized endoscopically in the nasopharynx. An angioembolization catheter was then placed through the cartilaginous ET orifice just medial to the bony ET. Under endoscopic and fluoroscopic guidance, a column of liquid embolic agent was deployed into the bony ET segment up to the middle ear space. Setting Tertiary care academic center. Participants Cadaveric specimens. Main Outcome Measures Cessation of CSF flow after occlusion at supraphysiologic pressures. Results In two cadavers, a CSF fistula model was developed and endoscopic visualization of irrigant flow into the nasopharynx was confirmed. Fluoroscopy provided adequate anatomic views of the ET and middle ear, in addition to dynamic views of embolization. Cessation of flow after occlusion was successfully achieved with pressures up to 25 mm Hg, which exceeds normal physiological intracranial pressure. Conclusion Eustachian tube occlusion with a liquid embolic solution is feasible in a novel cadaveric CSF leak model. In the future, this relatively short, straightforward procedure may become an outpatient alternative to manage intermittent or low-flow CSF fistulae following lateral skull base surgery.

17.
J Neurosurg ; : 1-9, 2018 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-29652232

RESUMO

OBJECTIVEThe morbidity of gross-total resection of jugular paraganglioma (JP) is often unacceptable due to the potential for irreversible lower cranial neuropathy. Stereotactic radiosurgery (SRS) has been used at the authors' institution since 1990 for the treatment of JP and other benign intracranial tumors. Conventional means of assessing tumor progression using linear measurements or elliptical approximations are imprecise due to the irregular shape and insinuating growth pattern of JP. The objective of this study was to assess long-term tumor control in these patients by using slice-by-slice 3D volumetric segmentation of serial MRI data.METHODSRadiographic data and clinical records were reviewed retrospectively at a single, tertiary-care academic referral center for patients treated from 1990 to 2017. Volumetric analyses by integration of consecutive tumor cross-sectional areas (tumor segmentation) of serial MRI data were performed. Tumor progression was defined as volumetric growth of 15% or greater over the imaging interval. Primary outcomes analyzed included survival free of radiographic and clinical progression. Secondary outcomes included new or worsened cranial neuropathy.RESULTSA total of 85 patients were treated with Gamma Knife radiosurgery (GKRS) for JP at the authors' institution over the last 27 years. Sixty patients had pretreatment and serial posttreatment contrast-enhanced MRI follow-up suitable for volumetric analysis. A total of 214 MR images were analyzed to segment tumor images in a slice-by-slice fashion to calculate integral tumor volume. The median follow-up duration was 66 months (range 7-202 months). At 5 years the tumor progression-free survival rate was 98%. Three tumors exhibited progression more than 10 years after GKRS. Estimated survival free of radiographic progression rates (95% confidence interval [CI]; n = number still at risk) at 5, 10, and 15 years following radiosurgery were 98% (95% CI 94%-100%; n = 34), 94% (95% CI 85%-100%; n = 16), and 74% (95% CI 56%-98%; n = 6), respectively. One patient with tumor progression required treatment intervention using external beam radiation therapy, constituting the only case of clinical progression. Two patients (3%) without preexisting lower cranial nerve dysfunction developed new ipsilateral vocal fold paralysis following radiosurgery.CONCLUSIONSSRS achieves excellent long-term tumor control for JP without a high risk for new or worsened cranial neuropathy when used in primary, combined modality, or recurrent settings. Long-term follow-up is critical due to the potential for late radiographic progression (i.e., more than 10 years after SRS). As none of the patients with late progression have required salvage therapy, the clinical implications of this degree of tumor growth have yet to be determined.

18.
Otol Neurotol ; 39(1): 99-105, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29194225

RESUMO

OBJECTIVE: To describe audiometric outcomes following stereotactic radiosurgery (SRS) for jugular paraganglioma (JP). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Patients with pretreatment serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] Class A or B) and serial post-SRS audiometric follow-up who underwent Gamma Knife SRS for JP between 1990 and 2017. INTERVENTION(S): Gamma Knife SRS. MAIN OUTCOME MEASURES: Progression to nonserviceable hearing; correlation between baseline hearing and treatment parameters with audiometric outcomes. RESULTS: Of 85 patients with JP who underwent SRS during the study period, 35 (66% female, median age 53) had pretreatment serviceable hearing and serial post-treatment audiometry available for review. Median tumor volume at the time of treatment was 7,080 mm, median cochlear point dose was 5.8 Gy (interquartile range [IQR] 4.1 to 7.3 Gy), and median marginal and maximum tumor doses were 16 and 32 Gy, respectively. After a median follow-up of 37 months (IQR 16 to 77 mo), the median change in pure-tone average and speech discrimination score in the treated ear was -1.2 dB HL/yr (IQR -4.5 to 0.3) and 0%/yr (IQR 0-3.5%), compared with 0.07 dB HL/yr (IQR -0.03 to 0.12) and 0 %/yr (IQR 0 to 0%) in the contralateral untreated ear. Seven patients developed nonserviceable hearing (AAO-HNS Class C or D) at a median of 13.2 months following SRS (IQR 4.8 to 24 mo). Among those who maintained serviceable hearing, median audiometric follow-up was 42 months (IQR 18 to 77 mo). The Kaplan-Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. CONCLUSION: The short- and intermediate-term risk of progression to nonserviceable hearing following SRS for JP is low. Data regarding the impact of cochlear dose from the vestibular schwannoma literature should not be freely applied to JP, since the impact of SRS parameters on hearing preservation seems to be less significant.


Assuntos
Tumor do Glomo Jugular/cirurgia , Radiocirurgia/métodos , Adulto , Idoso , Progressão da Doença , Feminino , Tumor do Glomo Jugular/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Radiocirurgia/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
19.
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
20.
Otolaryngol Head Neck Surg ; 156(5): 946-951, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28418817

RESUMO

Objective To define relationships between the frontal sinus opening, ostia of other frontal recess cells, and endoscopic landmarks and to develop a clinically useful framework to guide frontal sinus surgery. Study Design Retrospective review. Setting Tertiary care academic referral center. Methods Adult patients with computed tomography (CT) without sinonasal pathology were included. Virtual endoscopy (using OsiriX) and corresponding CT reconstructions were used to identify all visible ostia in the frontal recess and characterize their positions in spaces between the uncinate/agger nasi (U), bulla ethmoidalis (EB), and middle turbinate (MT). Results Two hundred sides in 100 patients (median age 51 years, 62% female) were analyzed. The "center" of each map was defined as the intersection of spaces between U, EB, and MT. The frontal sinus opening was in the "center" in 53% of frontal recesses, lateral to this position in 29%, and anterior in 11%. When the frontal sinus opening was at the "center," anterior ostia drained frontal Kuhn T cells in 51% and intersinus septal cells in 23%. The skull base attachment of the apical strut of the uncinate process demarcated medial and lateral within the space between U and EB, with the opening to the frontal sinus medial in 68% and lateral in 31%. Left-right asymmetry in frontal sinus openings was noted in 46% of patients. Conclusion Combining preoperative imaging and knowledge of these anatomic relationships may facilitate more efficient frontal outflow tract identification and instrumentation. This represents the first and largest description of ostial configurations relative to endoscopic structural landmarks. LEVEL OF EVIDENCE: 4.


Assuntos
Pontos de Referência Anatômicos/cirurgia , Seio Frontal/diagnóstico por imagem , Seio Frontal/cirurgia , Tomografia Computadorizada Espiral/métodos , Terapia de Exposição à Realidade Virtual/métodos , Centros Médicos Acadêmicos , Adulto , Idoso , Estudos de Coortes , Endoscopia/métodos , Feminino , Humanos , Imageamento Tridimensional/métodos , Masculino , Pessoa de Meia-Idade , Cuidados Pré-Operatórios/métodos , Prognóstico , Estudos Retrospectivos , Medição de Risco , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
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