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Capillary hemangiomas are benign vascular lesions that are common in head and neck, but hemangiomas of jugular foramen and temporal bone are rare with only a few cases reported in the literature. We present a case report of this rare disease entity highlighting the subtle radiographic nuances that can benefit clinicians when encountered with similar unusual clinical scenario. Although radiographic features of capillary hemangioma can be distinctive, they are not specific. In this case, the lack of significant involvement of the jugular bulb and the absence of the typical pattern of osseous erosion of the jugular carotid spine led to the alternative diagnosis of a rare capillary hemangioma of the jugular foramen.
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Hemangioma Capilar , Foramina Yugular , Paraganglioma , Hemangioma Capilar/diagnóstico por imagen , Hemangioma Capilar/cirugía , Humanos , Hueso Temporal/patologíaRESUMEN
BACKGROUND: A variety of neurosensory symptoms including tinnitus have been associated with COVID-19 infection. While most cases of tinnitus are associated with hearing loss, here we report a case of severe tinnitus following COVID-19 infection with normal thresholds through 8000 Hz. CASE REPORT: A 49-year-old male presented with new onset severe tinnitus following COVID-19 infection. Tinnitus was bilateral, constant and nonpulsatile. Audiometric evaluation revealed normal threshold through 8000 Hz, with mild hearing loss at 16,000 Hz. Conservative measures including masking strategies failed to mitigate symptoms. A trial of gabapentin 300 mg twice per day improved tinnitus with no notable side effects. CONCLUSION: This patient may represent a subpopulation of patients who suffer from severe tinnitus following COVID-19 infection in the setting of largely normal hearing. The pathophysiology may be distinct from the more common hearing loss associated tinnitus and perhaps neuromodulators may play a larger role in mitigating tinnitus in this patient subset.
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COVID-19/complicaciones , Antagonistas de Aminoácidos Excitadores/uso terapéutico , Gabapentina/uso terapéutico , Acúfeno/tratamiento farmacológico , Acúfeno/virología , Humanos , Masculino , Persona de Mediana Edad , SARS-CoV-2RESUMEN
PURPOSE: To clarify the need for post-operative radiation treatment in skull base chondrosarcomas (SBCs). METHODS: A retrospective analysis of patients with grade I or II SBC. Patients were divided according to post-surgical treatment strategies: (A) planned upfront radiotherapy and (B) watchful waiting. Tumor control and survival were compared between the treatment groups. The median follow-up after resection was 105 months (range, 9-376). RESULTS: Thirty-two patients (Grade 1, n = 16; Grade 2, n = 16) were included. The most frequent location was petroclival (21, 64%). A gross total resection (GTR) was achieved in 11 patients (34%). Fourteen (44%) underwent upfront radiotherapy (group A) whereas 18 (56%) were followed with serial MRI alone (group B). The tumor control rate for the entire group was 77% and 69% at 10- and 15-year, respectively. Upfront radiotherapy (P = 0.25), extent of resection (P = 0.11) or tumor grade (P = 0.83) did not affect tumor control. The majority of Group B patients with recurrent tumors (5/7) obtained tumor control with repeat resection (n = 2), salvage radiotherapy (n = 2), or a combination of both (n = 1). The 10-year disease-specific survival was 95% with no difference between the group A and B (P = 0.50). CONCLUSION: For patients with grade I/II SBC, a reasonable strategy is deferral of radiotherapy after maximum safe resection until tumor progression or recurrence. At that time, most patients can be successfully managed with salvage radiotherapy or surgery. Late recurrences may occur, and life-long follow-up is advisable.
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Condrosarcoma , Neoplasias de la Base del Cráneo , Condrosarcoma/diagnóstico por imagen , Condrosarcoma/radioterapia , Condrosarcoma/cirugía , Estudios de Seguimiento , Humanos , Estadificación de Neoplasias , Estudios Retrospectivos , Base del Cráneo , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Vestibular schwannomas (VS) present at variable size with heterogeneous symptomatology. Modern treatment paradigms for large VS include gross total resection, subtotal resection (STR) in combination with observation, and/or radiation to achieve optimal function preservation, whereas treatment is felt to be both easier and safer for small VS. The objective is to better characterize the presentation and surgical outcomes of large and small VS. METHODS: We collected data of patients who had surgically treated VS with a posterior fossa diameter of 4.0 cm or larger (large tumor group, LTG) and smaller than 1.0 cm in cisternal diameter (small tumor group, STG). Statistical significance was defined as p < 0.05. RESULTS: LTG included 48 patients (average tumor size: 44.9 mm) and STG 38 (7.9 mm). Patients in STG presented more frequently with tinnitus and sudden hearing loss. Patients in LTG underwent more STR than STG (50.0% vs. 2.6%, p < 0.0001). LTG had more complications (31.3% vs. 13.2%, p = 0.049). Postoperative facial nerve function in STG was significantly better than LTG. STG had better hearing preoperatively (p < 0.0001) and postoperatively than LTG (p = 0.0002). Postoperative headache was more common in STG (13.2% vs. 2.1%, p = 0.045). The rate of recurrence/progression needing treatment was not statistically different between the groups (12.5% in LTG vs. 7.9% in STG, p = 0.49). Those patients who required periprocedural cerebrospinal fluid diversion had higher risk of infection (20.8% vs 4.8%, p = 0.022). CONCLUSION: Large and small VS present differently. LTG showed more unsatisfactory outcomes in facial nerve function and postoperative hearing despite maximal efforts undertaken toward function-preservation strategy; however, similar tumor control was achieved.
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Neuroma Acústico , Cefalea , Audición , Humanos , Recurrencia Local de Neoplasia , Neuroma Acústico/cirugía , Procedimientos NeuroquirúrgicosRESUMEN
OBJECTIVE: To test the hypothesis that severe to profound preoperative hearing loss predicts less acute postoperative vestibulopathy following microsurgical removal of vestibular schwannoma (VS) allowing for earlier postoperative mobilization and hospital discharge. METHODS: Patients with VS who underwent microsurgery and were found to have preoperative severe to profound hearing loss (pure tone average [PTA]â¯>â¯70â¯dB HL) were matched 1:1 by age and tumor size to a group of randomly selected controls with preoperative serviceable hearing. RESULTS: A total of 57 patients met inclusion criteria and were matched to controls. Median age at the time of microsurgery was 56â¯years. The median PTA and WRS for cases were 91â¯dB HL (interquartile range [IQR] 78-120) and 0% (IQR 0-0), respectively. Median tumor size was 14.2â¯mm (IQR 10.9-20.9). A total of 35 (61%) patients exhibited nystagmus after surgery associated with acute vestibular deafferentation. Median time to ambulation in the hallway was 2â¯days. Controls exhibited similar tumor size (12.7â¯mm, pâ¯=â¯0.11) and age (57â¯years, pâ¯=â¯0.52). Preoperative hearing loss did not predict severity or duration of postoperative nystagmus or days to discharge; however, those with Class D hearing exhibited a shorter time to ambulation (pâ¯=â¯0.04). CONCLUSION: Following microsurgical removal of VS, preoperative profound hearing loss was associated with a shorter time to postoperative mobilization; however, there were no observed associations with duration or severity of nystagmus and time to hospital discharge. Although not a predictor of nystagmus, preoperative profound hearing loss may portend quicker recovery from clinically significant postoperative vestibulopathy.
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Neoplasias del Oído/cirugía , Ambulación Precoz , Pérdida Auditiva/etiología , Microcirugia/métodos , Neuroma Acústico/cirugía , Procedimientos Quirúrgicos Otológicos/métodos , Vestíbulo del Laberinto/cirugía , Neoplasias del Oído/complicaciones , Femenino , Predicción , Audición , Pérdida Auditiva/diagnóstico , Pérdida Auditiva/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Neuroma Acústico/complicaciones , Alta del Paciente , Complicaciones Posoperatorias , Periodo Preoperatorio , Índice de Severidad de la Enfermedad , Factores de Tiempo , VértigoRESUMEN
OBJECTIVE: Electrode impedances play a critical role in cochlear implant programming. It has been previously shown that impedances rise during periods of non-use, such as the post-operative recovery period. Then when the device is activated and use is initiated, impedances fall and are typically stable. In this study, we report a new pattern where electrode impedances increase with device use and decrease with device rest. DESIGN: Electrode impedances were measured three to four times every day over a span of 1-3 months for two cochlear implant patients. STUDY SAMPLE: Two patients with a Nucleus cochlear implant participated in this study. RESULTS: Both subjects in this study show wide fluctuations in electrode impedances. By taking serial electrode impedance measurements throughout a day of use, we observe that electrode impedances consistently increase with device use and decrease with device rest. CONCLUSION: In this study, we report two cases of electrode impedances increasing as a function of device use. Numerous management strategies were employed to reduce this effect but none prevailed; a clear pathophysiologic mechanism remains elusive. Further study into the cause of this electrode impedance pattern is warranted to establish a management strategy for these cochlear implant users.
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Implantación Coclear , Implantes Cocleares , Cóclea , Impedancia Eléctrica , Estimulación Eléctrica , HumanosRESUMEN
OBJECTIVES: Describe the presentation and treatment of a patient who suffered a penetrating cervical trauma resulting in occlusion of the vertebral and internal carotid arteries. METHODS: The electronic medical record was used to collect information pertaining to the patient's clinical history. RESULTS: A 20-year-old male suffered a unique penetrating neck injury resulting in simultaneous injuries to the internal carotid and vertebral arteries as demonstrated by pre-operative angiography. Combined endovascular and open surgical approaches were utilized to successfully manage the vascular injuries prior to foreign body extraction. CONCLUSION: Complex penetrating cervical trauma is best managed with a multidisciplinary and multimodality approach. In appropriately selected patients, pre-operative angiography is a critical diagnostic modality that can prevent life-threatening hemorrhage following foreign body extraction.
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Traumatismos de las Arterias Carótidas/etiología , Laceraciones/etiología , Traumatismos del Cuello/etiología , Traumatismos Vertebrales/diagnóstico , Traumatismos Vertebrales/etiología , Arteria Vertebral/lesiones , Heridas Penetrantes/etiología , Traumatismos de las Arterias Carótidas/diagnóstico , Traumatismos de las Arterias Carótidas/cirugía , Arteria Carótida Interna , Vértebras Cervicales , Humanos , Laceraciones/diagnóstico , Laceraciones/cirugía , Masculino , Traumatismos del Cuello/diagnóstico , Traumatismos del Cuello/cirugía , Traumatismos Vertebrales/cirugía , Heridas Penetrantes/diagnóstico , Heridas Penetrantes/cirugía , Adulto JovenRESUMEN
PURPOSE: Prior studies evaluating Eustachian tube physiology, baseline middle ear pressure (MEP), and the effects of continuous positive airway pressure (CPAP) have been performed on awake patients. No study to date has specifically investigated MEP during sleep despite the fact that the average individual spends a third of their lifetime sleeping. The primary objectives of the current study are to quantify normal physiologic MEP during sleep and to evaluate the effects of escalating CPAP levels. MATERIALS AND METHODS: Prospective observational study at a tertiary academic referral center evaluating serial tympanometry on sleeping adult patients during polysomnography. MEP was recorded awake, at 1-hour intervals during diagnostic polysomnography, and at all CPAP levels during titration. Changes in MEP with duration of sleep and escalating CPAP levels were analyzed. RESULTS: Ten adults were included (4 females; 6 males; mean age 58years). The mean MEP while awake was 3 decapascals (daPa). The mean MEP during sleep without CPAP rose steadily from 14 daPa at 1hour to 41 daPa at 4hours (r=0.52; p<0.001). The mean MEP during sleep at a CPAP level of 5cm of water was 54 daPa. The mean MEP rose steadily with increasing CPAP levels, and was 104 daPa at 10cm of water, (r=0.82; p<0.001). The mean MEP during sleep without CPAP was 26 daPa, which was significantly lower than the mean MEP during sleep with CPAP between 5-10cm H2O (p<0.01). CONCLUSIONS: MEP naturally increases with duration of sleep. CPAP therapy causes a supraphysiologic elevation in MEP that rises with increasing pressure levels. These findings may help guide future studies examining the safety of CPAP following otologic surgery and the potential therapeutic benefit in patients with chronic middle ear disease.
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Pruebas de Impedancia Acústica/métodos , Oído Medio/fisiopatología , Respiración con Presión Positiva/métodos , Apnea Obstructiva del Sueño/terapia , Sueño/fisiología , Adulto , Anciano , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Posicionamiento del Paciente , Polisomnografía/métodos , Estudios Prospectivos , Valores de Referencia , Medición de Riesgo , Apnea Obstructiva del Sueño/diagnóstico , Centros de Atención TerciariaRESUMEN
PURPOSE: 1. Describe the presentation, imaging, and outcome of two cases of paraganglioma of the facial canal at our institution. 2. Summarize existing literature to better understand this lesion. 3. Clarify terminology. METHODS: Retrospective chart review at single tertiary academic referral center. Literature review using the PubMed electronic database. RESULTS: There are 12 cases of histologically-proven paraganglioma of the facial canal published in the English literature. We present two additional cases that were encountered at our institution. We also include three additional cases from a separate institution that have been accepted for publication in a different journal. We found that patients most commonly present with slowly progressive facial paralysis; though paralysis can be acute. The second most common symptom was pulsatile tinnitus, which was the only symptom in one patient. Radiographically, tumor location was in the descending segment in all but one case. The mass was often centered directly over the stylomastoid foramen, mimicking a parotid mass. We found circumferential widening of the proximal fallopian canal, and a "moth-eaten" bony destruction distally on CT imaging in several patients. In patients with poor facial nerve function (HB IV-VI), complete tumor removal with nerve sacrifice followed by great auricular or sural nerve grafting was performed with acceptable facial function results. CONCLUSIONS: Paraganglioma of the facial canal remains a rare etiology of progressive facial paralysis. The lesions are found in the distal descending segment, or centered over the stylomastoid foramen 94% of the time. In patients with poor facial function, surgical removal with nerve sacrifice, followed by great auricular or sural nerve grafting, yields an acceptable functional result.
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Neoplasias de los Nervios Craneales/diagnóstico , Enfermedades del Nervio Facial/diagnóstico , Paraganglioma/diagnóstico , Anciano , Neoplasias de los Nervios Craneales/patología , Neoplasias de los Nervios Craneales/cirugía , Progresión de la Enfermedad , Enfermedades del Nervio Facial/patología , Enfermedades del Nervio Facial/cirugía , Parálisis Facial/etiología , Femenino , Humanos , Persona de Mediana Edad , Paraganglioma/patología , Paraganglioma/cirugía , Acúfeno/etiología , Tomografía Computarizada por Rayos XRESUMEN
PURPOSE: Supramaximal facial nerve stimulation is an applied current sufficient to evoke a maximal electromyographic response of facial musculature. It is used during cerebellopontine angle surgery for prognostication of postoperative nerve function. We utilized a rat model to examine safe parameters for intracranial electrical stimulation. MATERIALS AND METHODS: Intracranial facial nerve stimulation with electromyographic monitoring of 14 rats was performed. Supramaximal current level was determined and 50 additional pulses of supramaximal (4 rats), 3 times supramaximal (4), 10 times supramaximal (3), or zero (3) current were applied. To monitor progression of facial nerve injury, video recordings of vibrissae movements and eye closure were captured at 1, 3 and 28 days after surgery; animals were sacrificed on day 28, when nerve morphometry was performed. RESULTS: One rat in the supramaximal stimulation group (of 4), and one rat in the 10 times supramaximal stimulation group (of 3) demonstrated persistent impairment of facial nerve function as evidenced by decreased amplitude of vibrissae sweeping and eye closure impairment. The remainder of rats in all experimental groups demonstrated symmetric and normal facial nerve function at all time points. CONCLUSIONS: A novel animal model for supramaximal stimulation of the rat intracranial facial nerve is described. A small proportion of animals demonstrated functional evidence of nerve injury postoperatively. Function was preserved in some animals after stimulation with current order of magnitude higher than supramaximal levels. Further study with this model is necessary to definitively isolate the effects of surgical trauma from those of supramaximal electrical stimulation.
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Estimulación Eléctrica/métodos , Músculos Faciales/fisiopatología , Traumatismos del Nervio Facial/terapia , Nervio Facial/fisiopatología , Animales , Modelos Animales de Enfermedad , Electromiografía , Músculos Faciales/inervación , Traumatismos del Nervio Facial/fisiopatología , Masculino , Contracción Muscular , Ratas , Ratas Sprague-DawleyRESUMEN
OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.
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Hiperacusia , Mioclonía , Tensor del Tímpano , Humanos , Anciano , Hiperacusia/cirugía , Tensor del Tímpano/cirugía , Mioclonía/etiología , Mioclonía/cirugía , Complicaciones Posoperatorias/etiología , Masculino , Oído Medio/cirugía , Tendones/cirugía , Procedimientos de Cirugía Plástica/métodos , Resultado del TratamientoRESUMEN
Epidermoid tumors are benign, slow-growing lesions, originating from misplaced ectodermal cells that become trapped during neural tube closure.1 The cerebellopontine angle (CPA) is the most common intracranial location for epidermoid tumors, accounting for approximately 60% of cases.2 Treatment of epidermoid tumors consists of surgical resection, with the goal of gross total resection.3 Here, we describe the case of a patient with a large epidermoid tumor at the CPA causing near-complete hearing loss, who remarkably experienced full recovery of hearing after resection of the tumor. The patient is a 37-year-old woman who presented to our clinic with a CPA tumor causing severe hearing loss consisting of class D hearing and a word recognition score of 5% on audiological examination. Radiographically, the tumor demonstrated significant mass effect on the right cranial nerves VII and VIII with prominent extension into the internal auditory canal. Given the patient's profound hearing loss, she consented to receive a right retrosigmoid craniotomy for resection of the lesion. Although cranial nerves VII and VIII were heavily invested in the tumor, we were able to systematically resect the lesion from the CPA and internal auditory canal, and achieve a gross total resection. Histological examination confirmed the diagnosis of an epidermoid tumor. Remarkably, the patient's audiogram at 3-month follow-up demonstrated complete recovery of hearing in her right ear with a word recognition score of 100% and normal hearing sensitivity across all tested frequencies.
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OBJECTIVE: Comprehensively assess the prevalence of monopolar electrosurgery-related device complications among cochlear implant (CI) recipients. STUDY DESIGN: Multifaceted retrospective review and survey. SETTING: Tertiary medical center. METHODS: Multifaceted approach including: (i) review of the current literature; (ii) historical review of institutional data from an academic, tertiary CI center; (iii) review of industry data provided by 3 Food and Drug Administration-approved CI manufacturers; and (iv) survey of high-volume CI centers. RESULTS: Literature review identified 9 human studies, detailing 84 devices with 199 episodes of device-cautery exposure. From studies reporting on patients records, no implant showed evidence of damage after exposure. One cadaveric study using dental cautery reported 1 episode of device damage. Review of institutional records did not identify any CI damage in 84 instances of exposure. Data from the 3 major implant manufacturers showed a single report of damage that could be reasonably linked to monopolar electrosurgery, out of a possible 689,426 CIs. Last, a survey of 8 high-volume CI centers did not identify any adverse events associated with monopolar cautery. CONCLUSION: These data estimate the risk of adverse device-related events or tissue injury to be extraordinarily low. Short of operating in immediate proximity to the CI (ie, the ipsilateral temporoparietal scalp), these data indicate that monopolar electrosurgery can be used in the body and the head-and-neck of CI recipients with nominal risk. These findings may guide decision-making in cases that are optimally or preferably performed with monopolar electrocautery and can be used to counsel CI patients following inadvertent exposures.
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Implantación Coclear , Implantes Cocleares , Humanos , Electrocirugia/efectos adversos , Implantes Cocleares/efectos adversos , Electrocoagulación , Implantación Coclear/efectos adversos , CauterizaciónRESUMEN
OBJECTIVE: To describe the experience and results from coordinated and closely scheduled radiosurgery and cochlear implantation (CI) in a vestibular schwannoma (VS) cohort. PATIENTS: Patients with VS who underwent radiosurgery followed by CI on the same or next day. INTERVENTIONS: Interventions included sequential radiosurgery and CI. MAIN OUTCOME MEASURES: Tumor control defined by tumor growth on posttreatment surveillance and audiometric outcomes including consonant-nucleus-consonant words and AzBio sentences in quiet. RESULTS: In total, six patients were identified that met the inclusion criteria, with an age range of 38 to 69 years and tumor sizes ranging from 2.0 to 16.3 mm. All patients successfully underwent radiosurgery and CI on the same or immediately successive day. Postoperatively, all patients obtained open-set speech recognition. Consonant-nucleus-consonant word scores ranged from 40 to 88% correct, and AzBio scores ranged from 44 to 94% correct. During posttreatment magnetic resonance imaging surveillance, which ranged from 12 to 68 months, all tumors were noted to be adequately visualized, and no tumor progression was noted. CONCLUSION: Coordinated radiosurgery and CI can be safely performed in patients with VS on the same or next day, serving to decrease burden on patients and increase access to this vital rehabilitative strategy.
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Implantación Coclear , Implantes Cocleares , Neuroma Acústico , Radiocirugia , Percepción del Habla , Humanos , Adulto , Persona de Mediana Edad , Anciano , Implantación Coclear/métodos , Neuroma Acústico/cirugía , Radiocirugia/métodos , Estudios Retrospectivos , Audiometría , Resultado del TratamientoRESUMEN
The leading cause of trigeminal neuralgia (TGN) relies on the microvascular conflict between the superior cerebellar artery (SCA) loop and the dorsal root entry zone of the trigeminal nerve (TN). However, lesions along the TN have been described as a possible cause of TGN for direct mass effect or indirect vascular transposition. Thus, the surgical approach to TGN in patients harboring cerebellopontine angle or Meckel's cave tumor should be methodically chosen. The retrosigmoid (RS) approach with suprameatal extension offers direct access to the TN in both its cisternal and Meckel's cave segment, allowing optimal TN decompression from vascular and tumoral components. Although the RS approach with suprameatal extension has been described in numerous studies,1-4 videos detailing its key steps in addressing a multicomponent TGN are lacking. In this video, we highlight the case of a 46 year-old woman with 6 months of medically refractory typical TGN with a right en plaque meningioma involving the petrous bone, petroclival junction, Meckel's cave, and tentorium. In addition, magnetic resonance imaging was suspicious for a compressive SCA loop over the dorsal root entry zone. The patient underwent a RS approach with suprameatal extension for subtotal resection of the tumor and microvascular decompression of the TGN. The patient recovered with no complications and TGN resolved.
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OBJECTIVES: Describe a single institution's cochlear implant outcomes for patients with inner ear schwannomas (IES) in the setting of various tumor management strategies (observation, surgical resection, or stereotactic radiosurgery [SRS]). STUDY DESIGN: Single-institution retrospective review. PATIENTS: Patients diagnosed with isolated, sporadic IES who underwent cochlear implantation (CI). INTERVENTIONS: CI with or without IES treatment. MAIN OUTCOME MEASURES: Speech perception outcomes, tumor status. RESULTS: Twelve patients with IES underwent CI with a median audiologic and radiologic follow-up of 12 months. Six patients underwent complete resection of the tumor at the time of CI, four underwent tumor observation, and two underwent SRS before CI. At 1 year after CI for all patients, the median consonant-nucleus-consonant (CNC) word score was 55% (interquartile range, 44-73%), and the median AzBio sentence in quiet score was 77% (interquartile range, 68-93%). Overall, those with surgical resection performed similarly to those with tumor observation (CNC 58 versus 61%; AzBio in quiet 74 versus 91%, respectively). Patients who underwent tumor resection before implantation had a wider range of speech performance outcomes compared with patients who underwent tumor observation. Two patients had SRS treatment before CI (10 months previous and same-day as CI) with CNC word scores of 6 and 40%, respectively. CONCLUSIONS: Patients with IES who underwent CI demonstrated similar speech performance outcomes (CNC 56% and AzBio 82%), when compared with the general cochlear implant population. Patients who underwent either tumor observation or surgical resection performed well after CI.
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OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. INTERVENTIONS: CI or ABI ipsilateral to vestibular schwannoma. MAIN OUTCOME MEASURES: Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. RESULTS: Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. CONCLUSION: Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.
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Implantación Auditiva en el Tronco Encefálico , Implantes Auditivos de Tronco Encefálico , Implantación Coclear , Implantes Cocleares , Neurofibromatosis 2 , Neuroma Acústico , Percepción del Habla , Humanos , Neuroma Acústico/complicaciones , Neuroma Acústico/cirugía , Neuroma Acústico/patología , Implantes Cocleares/efectos adversos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/cirugía , Neurofibromatosis 2/patología , Resultado del Tratamiento , Estudios RetrospectivosRESUMEN
OBJECTIVE: The treatment paradigm of vestibular schwannoma (VS) focuses on preservation of neurologic function, with small tumors increasingly managed with active surveillance. Often, tumor size and hearing outcomes are poorly correlated. The aim of the current work was to describe the natural history of hearing among patients with nongrowing VS during observational management. STUDY DESIGN: Historical cohort study. PATIENTS: Adults with sporadic VS. INTERVENTION: Wait-and-scan management. MAIN OUTCOME MEASURE: Maintenance of serviceable hearing (SH) after diagnosis. RESULTS: Among 228 patients with nongrowing VS, 157 patients had SH at diagnosis. Rates of maintaining SH (95% CI; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (89-98; 118), 81% (74-89; 65), and 78% (71-87; 42), respectively. Poorer hearing at diagnosis (hazard ratio [HR] per 10 dB hearing level increase in pure-tone average of 2.51, p < 0.001; HR per 10% decrease in word recognition score of 1.70, p = 0.001) was associated with increased likelihood of developing non-SH during observation. When controlling for baseline hearing status, tumors measuring 5 mm or greater in the internal auditory canal or with cerebellopontine angle extension were associated with significantly increased risk of developing non-SH (HR, 4.87; p = 0.03). At 5 years after diagnosis, 95% of patients with nongrowing intracanalicular VS measuring less than 5 mm maintained SH. CONCLUSIONS: Hearing worsens during periods of nongrowth in sporadic VS. Patients with small (<5 mm) intracanalicular tumors demonstrate robust maintenance of SH over time, reinforcing the consideration of initial observation in this patient subset.
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Neuroma Acústico , Adulto , Humanos , Neuroma Acústico/patología , Estudios de Cohortes , Espera Vigilante , Audición , Pruebas Auditivas , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: A small number of cochlear implant (CI) users experience facial nerve stimulation (FNS), which can manifest as facial twitching. In some patients, this can be resolved by adjusting the electrical stimulation parameters. However, for others, facial stimulation can significantly impair CI outcomes or even prevent its use. The exact mechanisms underlying FNS are unclear and may vary among patients. DESIGN: Transimpedance measurements were used to assess lateral and longitudinal spread of current within 15 cochlea of nucleus CI recipients with FNS (13 unilateral recipients and 1 bilateral recipient). We compared the transimpedance measurements with programming parameters from clinical visits and pre- and postoperative temporal bone computed tomography (CT) scans to identify factors that may contribute to FNS in each CI ear. RESULTS: In nine ears, transimpedance curves showed inflection, which suggests a localized current sink within the cochlea. This indicates a low-impedance pathway through which current exits the cochlea and stimulates the labyrinthine segment of the facial nerve canal. Electrodes near this current sink were disabled or underfit to minimize facial stimulation. In the other seven ears, current flow peaked toward the basal end of the cochlea, suggesting that current exits through the round window or other structures near the basal end of the cochlea, stimulating the tympanic segment of the facial nerve. CONCLUSIONS: Objective transimpedance measurements can be used to elucidate the mechanisms of FNS and to develop strategies for optimizing electrical stimulation parameters and speech coding to minimize or eliminate FNS in a small subset of CI users.
Asunto(s)
Implantación Coclear , Implantes Cocleares , Humanos , Nervio Facial , Cóclea , Estimulación EléctricaRESUMEN
OBJECTIVE: The aim of this study was to assess the benefit of having preserved acoustic hearing in the implanted ear for speech recognition in complex listening environments. DESIGN: The present study included a within-subjects, repeated-measures design including 21 English-speaking and 17 Polish-speaking cochlear implant (CI) recipients with preserved acoustic hearing in the implanted ear. The patients were implanted with electrodes that varied in insertion depth from 10 to 31 mm. Mean preoperative low-frequency thresholds (average of 125, 250, and 500 Hz) in the implanted ear were 39.3 and 23.4 dB HL for the English- and Polish-speaking participants, respectively. In one condition, speech perception was assessed in an eight-loudspeaker environment in which the speech signals were presented from one loudspeaker and restaurant noise was presented from all loudspeakers. In another condition, the signals were presented in a simulation of a reverberant environment with a reverberation time of 0.6 sec. The response measures included speech reception thresholds (SRTs) and percent correct sentence understanding for two test conditions: CI plus low-frequency hearing in the contralateral ear (bimodal condition) and CI plus low-frequency hearing in both ears (best-aided condition). A subset of six English-speaking listeners were also assessed on measures of interaural time difference thresholds for a 250-Hz signal. RESULTS: Small, but significant, improvements in performance (1.7-2.1 dB and 6-10 percentage points) were found for the best-aided condition versus the bimodal condition. Postoperative thresholds in the implanted ear were correlated with the degree of electric and acoustic stimulation (EAS) benefit for speech recognition in diffuse noise. There was no reliable relationship among measures of audiometric threshold in the implanted ear nor elevation in threshold after surgery and improvement in speech understanding in reverberation. There was a significant correlation between interaural time difference threshold at 250 Hz and EAS-related benefit for the adaptive speech reception threshold. CONCLUSIONS: The findings of this study suggest that (1) preserved low-frequency hearing improves speech understanding for CI recipients, (2) testing in complex listening environments, in which binaural timing cues differ for signal and noise, may best demonstrate the value of having two ears with low-frequency acoustic hearing, and (3) preservation of binaural timing cues, although poorer than observed for individuals with normal hearing, is possible after unilateral cochlear implantation with hearing preservation and is associated with EAS benefit. The results of this study demonstrate significant communicative benefit for hearing preservation in the implanted ear and provide support for the expansion of CI criteria to include individuals with low-frequency thresholds in even the normal to near-normal range.