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1.
Artículo en Inglés | MEDLINE | ID: mdl-38829556

RESUMEN

BACKGROUND: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. OBJECTIVE: To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. STUDY DESIGN: Retrospective medical record review. MATERIAL AND METHODS: Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. RESULTS: A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. CONCLUSION: Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.

2.
Eur Arch Otorhinolaryngol ; 281(3): 1195-1203, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37665344

RESUMEN

PURPOSE: The aim of this study was to evaluate the safety and surgical outcome of superior petrosal vein (SPV, Dandy's vein) sacrifice in translabyrinthine approach (TLA) for resection of vestibule schwannoma (VS) as compared with SPV preservation, with further investigation of preoperational factors associated with the implement of SPV sacrifice. METHODS: The authors prospectively collected data from patients surgically treated for VS through TLA between June 2021 and April 2022 at the Gruppo Otologico. RESULTS: There were 30 and 49 patients in SPV sacrifice and preservation groups, respectively. SPV sacrifice group had significantly larger tumor size (2.46 vs. 1.40 cm), less percentage of solid tumor (26.7% vs. 83.7%), higher incidence of brainstem compression (80% vs. 26.5%), and higher percentage of facial numbness (20.0% vs. 4.1%) than SPV preservation group. Gross total resection (GTR) rates were 73.3% after SPV sacrifice and 87.8% after SPV preservation. Facial nerve preservation rates were similar. No complication related with SPV sacrifice was observed. Logistic regression analysis showed tumor size and complete solid consistency as significant risk factors associated with SPV sacrifice. ROC curve further demonstrated tumor size as a fair predictor (AUC = 0.833), with optimum cutoff value of 1.68 cm. CONCLUSION: SPV sacrifice via TLA as needed is a safe and effective maneuver for removal of relatively large VS. Tumor size and consistency can be used as a guidance in preoperational decision-making, with cutoff value of 1.68 cm and cystic formation as predictive indicators.


Asunto(s)
Neuroma Acústico , Humanos , Neuroma Acústico/cirugía , Neuroma Acústico/etiología , Procedimientos Neuroquirúrgicos/efectos adversos , Nervio Facial/cirugía , Factores de Riesgo , Incidencia , Estudios Retrospectivos
3.
Artículo en Inglés | MEDLINE | ID: mdl-39085472

RESUMEN

OBJECTIVES: To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas. METHODS: The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated. RESULTS: Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up. CONCLUSIONS: Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon's factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.

4.
Eur Arch Otorhinolaryngol ; 281(6): 2779-2789, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38184495

RESUMEN

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.


Asunto(s)
Progresión de la Enfermedad , Tumor del Glomo Yugular , Humanos , Tumor del Glomo Yugular/radioterapia , Tumor del Glomo Yugular/cirugía , Tumor del Glomo Yugular/patología , Tumor del Glomo Yugular/diagnóstico por imagen , Espera Vigilante , Masculino , Femenino , Neoplasias del Oído/radioterapia , Neoplasias del Oído/patología , Neoplasias del Oído/diagnóstico por imagen , Persona de Mediana Edad , Paraganglioma/radioterapia , Paraganglioma/patología , Paraganglioma/diagnóstico por imagen , Adulto
5.
Artículo en Inglés | MEDLINE | ID: mdl-39095536

RESUMEN

INTRODUCTION: Lateral skull base (LSB) and middle ear pathologies often involve the facial nerve (FN), and their treatment may require FN sacrifice. Cases with unidentifiable proximal stump or intact FN with complete FN palsy, necessitate FN anastomosis with another motor nerve in order to restore innervation to the mimicking musculature. The results of hypoglossal-to-facial nerve anastomosis (HFA) and masseteric-facial nerve anastomosis in patients with facial paralysis after middle ear and LSB surgeries, are presented and compared. METHODS: Adult patients with total definitive facial paralysis after middle ear or LSB surgery undergoing facial nerve reanimation through hypoglossal or masseteric transfer anastomosis were enrolled. The facial nerve function was graded according to the House Brackmann grading system (HB). The facial function results at 3 months, 6 months, 12 months, 18 months and at the last follow up (more than 18 months) are compared. RESULTS: 153 cases of LSB and middle ear surgery presented postoperative facial palsy and underwent facial nerve reanimation surgery with HF in 85 patients (55.5%) and MF in 68 patients (44.5%). The duration of the FN palsy before reconstructive surgery was inversely associated to better FN results, in particular with having a grade III HB (p = 0.003). Both techniques had significantly lower HB scores when an interval between palsy onset and reanimation surgery was 6 months or less (MF p = 0.0401; HF p = 0.0022). Patients who underwent a MF presented significant improvement of the FN function at 3 months from surgery (p = 0.0078). At the last follow-up, 63.6% recovered to a grade III HB and 22.7% to a grade IV. On the other hand, the first significant results obtained in the HF group were at 6 months from surgery (p < 0.0001). 67.8% of patients had a grade III HB after a HF at the last follow-up, 28.8% a grade IV. FN grading at 6 months from surgery was significantly lower in the MF group compared to the HF (p = 0.0351). The two techniques had statistically similar results at later follow-up evaluations. DISCUSSION/CONCLUTION: MF was associated to initial superior results, presenting significant facial recovery at 3 months, and significantly better functional outcomes at 6 months from surgery compared to HF. Although later results were not significantly different in this study, earlier results have an important role in order to limit the duration of risk of corneal exposure.

6.
Audiol Neurootol ; 28(1): 12-21, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36228574

RESUMEN

INTRODUCTION: This work aimed to study the management of vestibular schwannoma (VS) patients with normal hearing (NH). METHODS: A retrospective study was undertaken in a Quaternary referral center for skull base pathologies. Among 4,000 VS patients 162 met our strict audiological criteria for NH. These patients were divided into 2 management groups, wait and scan (W&S) (45/162, 25%) and operated patients (123/162, 75%), and 6 patients were included in both groups. RESULTS: Our management strategy achieved the goals for treatment of VS. First goal, all tumors were completely removed except for 2 intentional residuals. Second goal, facial nerve (FN) function preservation (House Brackmann I, II, and III) was 95.9%. Third goal, possible hearing preservation (HP) attempts occurred in (50/122) (40.9%) with an HP rate in 44% of the patients. Additionally, there were only 2 cases of postoperative complications with no CSF leakage. The prospect of HP in NH patients did not differ with respect to tumor size. However, patients with normal preoperative ABR seemed to have better chances of HP and good FN function and vice versa. HP rate was superior for the MCFA as opposed to the RS + RLA. W&S group demonstrated hearing stability in 88.9% of the patients and FN function stability of HB I in 100% of the patients. CONCLUSIONS: Surgical resection is a reasonable and definitive management option for VS with NH. Nevertheless, choosing to manage cases with observation remains an appropriate management option for NH patients. ABR might be considered as an adjuvant tool indicating better prognosis for HP.


Asunto(s)
Neuroma Acústico , Humanos , Neuroma Acústico/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Audición/fisiología , Pronóstico , Complicaciones Posoperatorias , Nervio Facial/cirugía
7.
Eur Arch Otorhinolaryngol ; 280(7): 3485-3488, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37020047

RESUMEN

OBJECTIVE: Report a case of localized necrotizing meningoencephalitis as the cause of functional hearing loss after cochlear implant (CI) surgery. CASE REPORT: A 12-year-old with bilateral CI presented to our quaternary center due to severe functional hearing loss after 11 years since left ear CI surgery. CT with contrast was conducted showing a CPA tumor-like mass. Pre-operative computed tomography (CT) scans and magnetic resonance imaging (MRI) performed at the age of 1 year showed no inner ear abnormalities and in particular no evidence of a tumor in the cerebellopontine angle (CPA). CONCLUSION: Following removal of the CI and the mass, histopathological, immunohistochemical and cultural examinations revealed a necrotizing meningoencephalitis, with the CI electrode as the focus.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Funcional , Meningoencefalitis , Neuroma Acústico , Humanos , Niño , Implantes Cocleares/efectos adversos , Pérdida Auditiva Funcional/cirugía , Implantación Coclear/efectos adversos , Implantación Coclear/métodos , Neuroma Acústico/cirugía , Neuroma Acústico/patología , Imagen por Resonancia Magnética/métodos , Meningoencefalitis/diagnóstico , Meningoencefalitis/etiología , Meningoencefalitis/cirugía
8.
Eur Arch Otorhinolaryngol ; 279(12): 5655-5665, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35767053

RESUMEN

PURPOSE: Report our experience in the management of posterior petrous surface meningiomas (PPSMs), and identify features that affect hearing, facial nerve (FN) function, and control of the disease. METHODS: Retrospective case series of 131 patients surgically managed for PPSMs. FN status, hearing and tumour radicality were assessed and compared between patients with tumours of different locations (Desgeorges classification) and internal auditory canal involvement (IAC). RESULTS: At the time of surgery 74.8% of patients had a hearing loss. Hearing was mostly unserviceable in tumors attached to the meatus. Pure tone audiometry did not correlate to IAC extension, while speech discrimination scores were statistically worse when the tumor occupied the IAC (unpaired t test, p = 0.0152). Similarly, extrameatal tumors undergoing removal by otic preserving techniques maintained postoperative hearing, whereas hearing worsened significantly in tumors involving the IAC (paired t test, p = 0.048). The FN was affected preoperatively in 11.4% of cases. Postoperative FN palsy was significantly correlated to the IAC involvement (Fisher's exact test, p = 0.0013), while it was not correlated to tumor size. According to the Desgeorges classification, a postoperative FN palsy complicated the majority of anteriorly extending tumors and, two-fifths of meatus centred tumors. 75% of posterior located tumors had a postoperative FN grade I HB. CONCLUSIONS: Since the involvement of the IAC by the tumor affects both hearing and FN function, the IAC is of primary importance in PPSMs and should be studied and addressed as much as the tumor location in the CPA.


Asunto(s)
Parálisis Facial , Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/cirugía , Meningioma/patología , Estudios Retrospectivos , Hueso Petroso/cirugía , Hueso Petroso/patología , Parálisis Facial/patología , Neoplasias Meníngeas/cirugía , Neoplasias Meníngeas/patología
9.
Neurosurg Rev ; 44(4): 2119-2131, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32860105

RESUMEN

(1) To study the overall outcomes of patients surgically treated for large/giant vestibular schwannomas (VSs) and (2) to identify and analyze preoperative and intraoperative prognostic factors influencing facial nerve (FN) outcome. A retrospective clinical study was conducted at a quaternary referral otology and skull-base center. A total of 389 cases were enrolled. The inclusion criteria were patients with tumor > 30 mm undergoing surgery with a minimum follow-up of 12 months. Neurofibromatosis-II, previous radiotherapy, revision surgeries, preoperative FN House-Brackmann (HB) grade > I, partial resections, incomplete records, or those lost to follow-up for a minimum period of 1 year were excluded. In addition, partial resections and cases where FN was sacrificed intraoperatively were also excluded and were analyzed separately. The mean duration of symptoms was 35.4 months, pronounced more in elderly (58.3 months) than in younger individuals (28.4 months). Mean tumor diameter was 36 mm and 52.7% was cystic. Total resection (TR), near total resection (NTR), and subtotal resections (STR) were achieved in 77.4%, 9.5%, and 13.2% of cases, respectively. Regrowth was observed only after STR (19.6%). Good (HB I-II), moderate (HB III), and poor (HB IV-VI) FN functions were observed in 36.8%, 51.7%, and 11.6% cases, respectively. Younger individuals underwent TR in 259 (86.9%) cases against 42 (46.2%) in elderly individuals. Non-total resections (NTR/STR) were performed in 49 (53.8%) cases in elderly as against 39(13.1%) in younger individuals. Good FN outcome was observed in 28 (57.1%) cases of non-total resections in elderly as against 13 (33.3%) cases in younger individuals. On multiple logistic regression analysis, size of the tumor, preoperatively prolonged duration of symptoms, profound deafness, and antero-superiorly located FN with respect to the tumor played a detrimental role in the final facial nerve outcome postoperatively. On the contrary, in large tumors (3-3.9 cm), presence of vertigo/disequilibrium had a relatively better impact on final FN outcome. Partial resections accounted for 41(7.8%) cases and FN was interrupted in 71(13.6%) cases in total. Factors detrimental to better FN outcome were giant VSs (> 4 cm), antero-superiorly located FN intratumorally, preoperatively prolonged duration of symptoms, and profound deafness. In large tumors (3-3.9 cm), presence of vertigo/disequilibrium had a better impact on FN outcome. Understandably, cases with TR in comparison with NTR/STR had worse FN outcomes. In comparison with younger patients, elderly patients underwent higher NTR/STR resulting in better FN outcomes. The above factors can be used as prognosticators for patient counseling and surgical decision making.


Asunto(s)
Nervio Facial , Neuroma Acústico , Nervio Facial/cirugía , Traumatismos del Nervio Facial , Humanos , Neuroma Acústico/cirugía , Procedimientos Neuroquirúrgicos , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Resultado del Tratamiento
10.
Eur Arch Otorhinolaryngol ; 278(7): 2297-2304, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-32889625

RESUMEN

OBJECTIVE: Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). STUDY DESIGN: Retrospective case study and review of the literature. SETTING: Tertiary referral center. PATIENTS: Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. INTERVENTION(S): Therapeutical. MAIN OUTCOME MEASURE(S): Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. RESULTS: Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). CONCLUSIONS: Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences.


Asunto(s)
Neoplasias del Oído , Saco Endolinfático , Enfermedad de von Hippel-Lindau , Neoplasias del Oído/cirugía , Saco Endolinfático/diagnóstico por imagen , Saco Endolinfático/cirugía , Humanos , Recurrencia Local de Neoplasia , Estudios Retrospectivos
11.
Eur Arch Otorhinolaryngol ; 278(9): 3193-3202, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-32979119

RESUMEN

OBJECTIVE: Temporal bone squamous cell carcinoma (TBSCC) is a rare, aggressive tumor. Surgery, alone or combined with radiotherapy, represent the mainstay of treatment. To report our experience in the treatment of TBSCC and evaluate the disease-specific survival, identifying the factors influencing this outcome. MATERIALS AND METHODS: A retrospective study was performed on 66 patients between 1993 and 2018. Patients were staged according to the University of Pittsburgh-modified TNM staging system. Nine cases (13.6%) were Stage I, 7 cases (10.6%) Stage II, 20 cases (30.3%) Stage III and 30 cases (45.5%) Stage IV. Twenty-four patients underwent lateral temporal bone resection (LTBR) and 42 patients underwent subtotal temporal bone resection (STBR). RESULTS: One hundred percent of Stage I and II patients showed no evidence of disease (NED) after a median follow-up of 101 months (range 1-289 months). NED resulted in 88.2% of Stage III (mean follow-up 80.3 months; range 8-257) and 46.4% of stage IV (mean follow-up 50.6 months; range 3-217). Pittsburgh Stage or involvement of mastoid, facial nerve, medial wall of the middle ear, temporomandibular joint and middle fossa dura emerged as negative prognostic factors. The highest mortality rate occurred in the first 2 years after treatment, due to local recurrence. CONCLUSIONS: Prognosis of TBSCC can be excellent in early stage tumors, employing a LTBR. In more advanced cases, prognosis is poor. STBR with adjuvant radiotherapy represents the treatment of choice, offering acceptable survival rates. Given the rarity of the pathology, many controversies still exist concerning optimal management.


Asunto(s)
Carcinoma de Células Escamosas , Recurrencia Local de Neoplasia , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/cirugía , Humanos , Recurrencia Local de Neoplasia/patología , Estadificación de Neoplasias , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Hueso Temporal/patología , Hueso Temporal/cirugía , Resultado del Tratamiento
12.
Audiol Neurootol ; 25(6): 323-335, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32474562

RESUMEN

OBJECTIVES: To assess the validity of the subtotal petrosectomy (STP) technique in problematic cases of cochlear implant (CI) surgery, and review indications, outcomes, and related controversies. STUDY DESIGN: This is a retrospective review of data from a private quaternary referral center of otology and skull base surgery. PATIENTS AND METHODS: A review of patients who underwent CI with STP (STP-CI) as the leading approach was performed. Demographics, indications, surgical details, and main outcomes were evaluated. The surgeries performed were usually single-stage procedures encompassing a comprehensive mastoidectomy, blind sac closure of the external auditory canal (EAC), and mastoid obliteration with autologous fat. RESULTS: A total of 107 cases were included. Mean follow-up was 7.1 years (range 1-13 years). The most frequent indication for STP-CI was chronic otitis media with/without cholesteatoma (32.7%), followed by open mastoid cavity (26.1%), and cochlear ossification (17.7%). Other difficult conditions where STP facilitates successful implantation include inner-ear malformations, temporal-bone trauma, unfavorable anatomic conditions, and revision surgery. A planned staged procedure was performed in 3 cases. The rate of major complications was 5.6% (n = 6). Three patients developed postauricular wound dehiscence which eventually resulted in device extrusion. No cases of recurrent/entrapped cholesteatoma, EAC breakdown, or meningitis were encountered. This is the largest single-center series of STP-CI reported in the literature. CONCLUSIONS: When CI is intended in technically challenging cases or associated with a high risk of complications, STP is effective and reliable. Safe implantation and excellent long-term outcomes can be achieved provided surgical steps are properly followed. Single-stage procedures can be performed in most cases, even when there is active middle-ear disease.


Asunto(s)
Colesteatoma/cirugía , Cóclea/cirugía , Implantación Coclear/métodos , Implantes Cocleares , Otitis Media/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Hueso Temporal/cirugía , Resultado del Tratamiento , Timpanoplastia , Adulto Joven
13.
Audiol Neurootol ; 24(3): 117-126, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31266017

RESUMEN

OBJECTIVE: To evaluate the audiological aspects of vestibular schwannoma (VS) patients with normal hearing. STUDY DESIGN: Retrospective study. SETTING: Quaternary referral center for skull base pathologies. PATIENTS: The records on 4,000 patients who had been diagnosed with VS between 1986 and December 2017 were retrospectively reviewed. The patients included in the study were the ones who complied with the strict audiological normality criteria, as follows: a pure tone hearing threshold (at the 6-octave-spaced frequencies from 250 to 8,000 Hz) ≤25 dBHL; a word recognition score >90%; and interaural differences ≤10 dB at each frequency. INTERVENTIONS: Auditory brainstem response (ABR) testing and radiological imaging. MAIN OUTCOME MEASURES: The incidence of normal objective hearing among VS patients, and the diagnostic utility of the ABR and the effect of tumor size and site on the response. RESULTS: The incidence of normal hearing among VS patients was 4.2%. Tinnitus and vertigo were the most common symptoms across tumor grades; 5.6% of the tumors were large and giant tumors. The ABR yielded a sensitivity of 73.6%, with a false negative rate of 26.3% using a cutoff point of 0.2 ms for interaural latency differences. CONCLUSIONS: The diagnosis of VS should not be based on audiometric thresholds alone. Alarming signs of VS should be clear to the physician in order not to miss or delay the diagnosis of the disease. The ABR is useful in the diagnosis of VS, but normal results do not exclude the occurrence of the disease in patients with normal hearing.


Asunto(s)
Umbral Auditivo/fisiología , Potenciales Evocados Auditivos del Tronco Encefálico/fisiología , Audición/fisiología , Neuroma Acústico/fisiopatología , Audiometría de Tonos Puros , Bases de Datos Factuales , Humanos , Neuroma Acústico/complicaciones , Estudios Retrospectivos , Acúfeno/etiología , Acúfeno/fisiopatología , Vértigo/etiología , Vértigo/fisiopatología
18.
Acta Neuropathol ; 135(5): 779-798, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29305721

RESUMEN

Tumours can be viewed as aberrant tissues or organs sustained by tumorigenic stem-like cells that engage into dysregulated histo/organogenetic processes. Paragangliomas, prototypical organoid tumours constituted by dysmorphic variants of the vascular and neural tissues found in normal paraganglia, provide a model to test this hypothesis. To understand the origin of paragangliomas, we built a biobank comprising 77 cases, 18 primary cultures, 4 derived cell lines, 80 patient-derived xenografts and 11 cell-derived xenografts. We comparatively investigated these unique complementary materials using morphofunctional, ultrastructural and flow cytometric assays accompanied by microRNA studies. We found that paragangliomas contain stem-like cells with hybrid mesenchymal/vasculoneural phenotype, stabilized and expanded in the derived cultures. The viability and growth of such cultures depended on the downregulation of the miR-200 and miR-34 families, which allowed high PDGFRA and ZEB1 protein expression levels. Both tumour tissue- and cell culture-derived xenografts recapitulated the vasculoneural paraganglioma structure and arose from mesenchymal-like cells through a fixed developmental sequence. First, vasculoangiogenesis organized the microenvironment, building a perivascular niche which in turn supported neurogenesis. Neuroepithelial differentiation was associated with severe mitochondrial dysfunction, not present in cultured paraganglioma cells, but acquired in vivo during xenograft formation. Vasculogenesis was the Achilles' heel of xenograft development. In fact, imatinib, that targets endothelial-mural signalling, blocked paraganglioma xenograft formation (11 xenografts from 12 cell transplants in the control group versus 2 out of 10 in the treated group, P = 0.0015). Overall our key results were unaffected by the SDHx gene carrier status of the patient, characterized for 70 out of 77 cases. In conclusion, we explain the biphasic vasculoneural structure of paragangliomas and identify an early and pharmacologically actionable phase of paraganglioma organization.


Asunto(s)
Antineoplásicos/uso terapéutico , Neoplasias de Cabeza y Cuello/tratamiento farmacológico , Neoplasias de Cabeza y Cuello/fisiopatología , Mesilato de Imatinib/uso terapéutico , Paraganglioma/tratamiento farmacológico , Paraganglioma/fisiopatología , Animales , Antineoplásicos/farmacología , Línea Celular , Neoplasias de Cabeza y Cuello/genética , Neoplasias de Cabeza y Cuello/patología , Humanos , Mesilato de Imatinib/farmacología , Ratones Endogámicos NOD , Ratones SCID , MicroARNs/metabolismo , Células-Madre Neurales/efectos de los fármacos , Células-Madre Neurales/metabolismo , Células-Madre Neurales/patología , Organogénesis/efectos de los fármacos , Organogénesis/fisiología , Paraganglioma/genética , Paraganglioma/patología , Cultivo Primario de Células , Microambiente Tumoral/efectos de los fármacos , Microambiente Tumoral/fisiología , Ensayos Antitumor por Modelo de Xenoinjerto
19.
Eur Arch Otorhinolaryngol ; 275(5): 1059-1068, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-29504040

RESUMEN

OBJECTIVES: To evaluate incidence, demographics, surgical, and radiological correlates of incomplete and false tract electrode array insertions during cochlear implantation (CI). To evaluate outcomes in patients with incomplete electrode insertion (IEI). STUDY DESIGN: Retrospective analysis. SETTING: Otology and skull base center. PATIENTS AND METHODS: Charts of 18 patients (19 ears) with incomplete or false tract insertions of the electrode array were evaluated who underwent CI, with at least 1 year follow-up (from 470 cases). Demographic findings, etiologies, pre-operative radiologic findings, operative records, post-operative plain radiographic assessment for extent of electrode insertion, and switch-on mapping were evaluated. Audiological outcomes were evaluated using maximum and last recorded vowel, word, sentence, and comprehension scores for patients with IEI. RESULTS: Incidence of insertional abnormalities was 4.25% with 17 instances of incomplete and 2 cases of insertion into superior semicircular canal. Mean age and duration of deafness were 55.18 ± 4.62 and 22.12 ± 5.71 years. Etiologies in the IEI group were idiopathic, otosclerosis, meningitis, chronic otitis media (COM), temporal bone fractures, and Neurofibromatosis-2. 29.4% cases had cochlear luminal obstruction. Mean radiological and active electrophysiological length of insertion was 20.49 ± 0.66 and 19.49 ± 0.88 mm, respectively. No significant correlation was observed between audiological outcomes and insertional length except in time to achieve maximum word scores (p = 0.04). Age at implantation had significant correlations with last recorded word and comprehension scores at mean follow-up of 42.9 months, and with time to achieve maximum auditory scores. CONCLUSIONS: IEI during cochlear implantation using straight electrodes can occur with or without cochlear luminal obstruction. Age plays an important role in the auditory rehabilitation in this patient subset.


Asunto(s)
Implantación Coclear/efectos adversos , Sordera/cirugía , Errores Médicos/efectos adversos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Implantación Coclear/instrumentación , Implantación Coclear/métodos , Implantes Cocleares , Sordera/diagnóstico , Electrodos Implantados , Femenino , Estudios de Seguimiento , Humanos , Masculino , Errores Médicos/estadística & datos numéricos , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
20.
Eur Arch Otorhinolaryngol ; 274(2): 695-700, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27637751

RESUMEN

To investigate the surgical and audiological outcomes of an installation of a bone-anchored hearing system (BAHS) procedure without tissue reduction using Ponto implants and abutments. Retrospective consecutive case series. Forty-nine patients, 18 years or older and eligible for treatment with a bone-anchored hearing system with tissue preservation surgery, were included in the study. Following a systematic scheme for medical outcomes, we collected the data regarding surgical intervention, quality of life (GBI), skin and soft tissue reactions (Holgers grading system), pain and numbness (VAS). Hearing performance (aided thresholds and speech recognition in noise) was recorded in 20 patients. No implants were lost, skin, and soft tissue reactions were mild in 96 % of the all visits. Quality of life (GBI) generally improved in the aided condition compared to prior to implantation. Audiologically, 100 % of the 20 patients examined showed improvement of speech reception and sound field thresholds comparing aided to unaided. An average improvement of 33 dB on PTA was recorded. The study, presenting data on a large population, treated with tissue preservation and modern titanium implants, shows that this treatment is a viable solution that results in fewer complications, high degree of predictability and good audiological results.


Asunto(s)
Audífonos , Prótesis e Implantes , Implantación de Prótesis/métodos , Anclas para Sutura , Adulto , Anciano , Umbral Auditivo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Calidad de Vida , Estudios Retrospectivos , Percepción del Habla , Escala Visual Analógica , Adulto Joven
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