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1.
Otol Neurotol ; 45(5): 542-548, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38511274

ABSTRACT

OBJECTIVE: To compare recidivism rates, audiometric outcomes, and postoperative complication rates between soft-wall canal wall reconstruction (S-CWR) versus bony-wall CWR (B-CWR) with mastoid obliteration (MO) in patients with cholesteatoma. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary neurotologic referral center. PATIENTS: Ninety patients aged ≥18 years old who underwent CWR with MO, either S-CWR or B-CWR, for cholesteatoma with one surgeon from January 2011 to January 2022. Patients were followed postoperatively for at least 12 months with or without second-look ossiculoplasty. INTERVENTIONS: Tympanomastoidectomy with CWR (soft vs. bony material) and mastoid obliteration. MAIN OUTCOME MEASURES: Recidivism rates; conversion rate to CWD; pre- versus postoperative pure tone averages, speech reception thresholds, word recognition scores, and air-bone gaps; postoperative complication rates. RESULTS: Middle ear and mastoid cholesteatoma recidivism rates were not significantly different between B-CWR (17.3%) and S-CWR (18.4%, p = 0.71). There was no significant difference in pre- versus postoperative change in ABG (B-CWR, -2.1 dB; S-CWR, +1.6 dB; p = 0.91) nor in the proportion of postoperative ABGs <20 dB (B-CWR, 41.3%; S-CWR, 30.7%; p = 0.42) between B-CWR and S-CWR. Further, there were no significant differences in complication rates between B-CWR and S-CWR other than increased minor TM perforations/retractions in B-CWR (63% vs. 40%, p = 0.03). CONCLUSIONS: Analysis of recidivism rates, audiometric outcomes and postoperative complications between B-CWR with MO versus S-CWR with MO revealed no significant difference. Both approaches are as effective in eradicating cholesteatoma while preserving relatively normal EAC anatomy and hearing. Surgeon preference and technical skill level may guide the surgeon's choice in approach.


Subject(s)
Cholesteatoma, Middle Ear , Mastoid , Mastoidectomy , Humans , Male , Female , Retrospective Studies , Middle Aged , Cholesteatoma, Middle Ear/surgery , Adult , Mastoid/surgery , Mastoidectomy/methods , Treatment Outcome , Tympanoplasty/methods , Postoperative Complications/epidemiology , Plastic Surgery Procedures/methods , Aged , Ear Canal/surgery , Young Adult , Audiometry, Pure-Tone , Recurrence
3.
Harefuah ; 162(7): 424-427, 2023 Aug.
Article in Hebrew | MEDLINE | ID: mdl-37561031

ABSTRACT

BACKGROUND: Cochlear implants are valuable in the auditory rehabilitation of patients with severe to profound hearing loss. However, there is limited data on the outcomes of cochlear implantation in patients with Meniere's disease (MD). OBJECTIVES: In this study, we aim to evaluate the auditory outcomes of cochlear implantation in patients with MD. METHODS: A retrospective case series of patients with MD and severe to profound sensorineural hearing loss (SNHL), who underwent cochlear implantation at a tertiary academic center between 2006-2017. Patient's clinical characteristics and audiometric data were reviewed. RESULTS: The study included 20 ears in 19 patients with MD who underwent cochlear implantation with available pre- and postoperative audiometric data. There were 10 males and 9 females with a mean age of 63 years and a mean follow-up duration of 70.8 months. Pre- and post-implant CNC word recognition scores were 18.31% and 66.89%, respectively (p<0.001). Pre- and post-implant AzBio and/or HINT sentence recognition scores were 12.25% and 68.28% in quiet, respectively (p<0.001), and 18.25% and 63.43% in noise, respectively (p<0.001). CONCLUSIONS: Cochlear implantation resulted in an improvement of word and sentence recognition scores in MD patients. These results support the role of cochlear implants in the auditory rehabilitation of MD. DISCUSSION: Dr. Samy received research support from Cochlear Corporation.


Subject(s)
Cochlear Implantation , Cochlear Implants , Meniere Disease , Speech Perception , Male , Female , Humans , Middle Aged , Cochlear Implantation/methods , Meniere Disease/complications , Meniere Disease/surgery , Retrospective Studies , Treatment Outcome
4.
Otol Neurotol ; 44(6): e428-e434, 2023 07 01.
Article in English | MEDLINE | ID: mdl-37315233

ABSTRACT

OBJECTIVE: To characterize the viability and volume of autologous free fat grafts over time, determine clinical/patient factors that may affect free fat graft survival and assess the clinical impact of free fat graft survival on patient outcomes in the translabyrinthine approach for lateral skull base tumor resection. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary neurotologic referral center. PATIENTS: Forty-two adult patients who underwent translabyrinthine craniotomy for resection of a lateral skull base tumor with the mastoid defect filled by autologous abdominal fat graft and subsequently underwent more than one postoperative magnetic resonance imaging (MRI) scans of the brain. INTERVENTIONS: Mastoid obliteration with abdominal fat after craniotomy, postoperative MRI. MAIN OUTCOME MEASURES: Rate of fat graft volume loss, fraction retention of original fat graft volume, initial fat graft volume, time to steady-state fat graft retention, rate of postoperative cerebrospinal fluid (CSF) leak, and/or pseudomeningocele formation. RESULTS: Patients were followed postoperatively with MRI for a mean of 31.6 months with a mean of 3.2 postoperative MRIs per patient. Initial graft size was a mean of 18.7 cm3 with a steady-state fat graft retention of 35.5%. Steady-state graft retention (<5% loss per year) was achieved at a mean of 24.96 months postoperatively. No significant association was found in multivariate regression analysis of clinical factors impact on fat graft retention and CSF leak/pseudomeningocele formation. CONCLUSIONS: In the use of autologous abdominal free fat graft for filling mastoid defects after translabyrinthine craniotomy, there is a logarithmic decline in fat graft volume over time, reaching steady state in 2 years. Rates of CSF leak or pseudomeningocele formation were not significantly affected by initial volume of the fat graft, rate of fat graft resorption, nor the fraction of original fat graft volume at steady state. In addition, no analyzed clinical factors significantly influenced fat graft retention over time.


Subject(s)
Skull Base Neoplasms , Adult , Humans , Retrospective Studies , Cerebrospinal Fluid Leak , Brain , Magnetic Resonance Imaging
5.
Laryngoscope ; 133(11): 2856-2867, 2023 11.
Article in English | MEDLINE | ID: mdl-37078512

ABSTRACT

OBJECTIVE: Exclusive endoscopic (EETTA) and expanded (ExpTTA) transcanal transpromontorial approaches have shown promising results for treating internal auditory canal (IAC) lesions. We reviewed the literature to answer the question: "Do EETTA and ExpTTA achieve high rates of complete resection and low rates of complications in treating patients with IAC pathologies?" DATA SOURCES: PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched. REVIEW METHODS: Studies reporting EETTA/ExpTTA for IAC pathologies were included. Indications and techniques were discussed and meta-analyzed rates of outcomes and complications were obtained with random-effect model meta-analyses. RESULTS: We included 16 studies comprising 173 patients, all with non-serviceable hearing. Baseline FN function was mostly House-Brackmann-I (96.5%; 95% CI: 94.9-98.1%). Most lesions were vestibular/cochlear schwannomas (98.3%; 95% CI: 96.7-99.8%) of Koos-I (45.9%; 95% CI: 41.3-50.3%) or II (47.1%; 95% CI: 43-51.1%). EETTA was performed in 101 patients (58.4%; 95% CI: 52.4-64.3%) and ExpTTA in 72 (41.6%; 95% CI: 35.6-47.6%), achieving gross-total resection in all cases. Transient complications occurred in 30 patients (17.3%; 95% CI: 13.9-20.5%), with meta-analyzed rates of 9% (95% CI: 4-15%), comprising FN palsy with spontaneous resolution (10.4%; 95% CI: 7.7-13.1%). Persistent complications occurred in 34 patients (19.6%; 95% CI: 17.1-22.2%), with meta-analyzed rates of 12% (95% CI: 7-19%), comprising persistent FN palsy in 22 patients (12.7%; 95% CI: 10.2-15.2%). Mean follow-up was 16 months (range, 1-69; 95% CI: 14.7-17.4). Post-surgery FN function was stable in 131 patients (75.8%; 95% CI: 72.1-79.5%), worsened in 38 (21.9%; 95% CI: 18.8-25%), and improved in 4 (2.3%; 95% CI: 0.7-3.9%), with meta-analyzed rates of improved/stable response of 84% (95% CI: 76-90%). CONCLUSION: Transpromontorial approaches offer newer routes for IAC surgery, but their restricted indications and unfavorable FN outcomes currently limit their use. Laryngoscope, 133:2856-2867, 2023.


Subject(s)
Ear, Inner , Neuroma, Acoustic , Humans , Retrospective Studies , Ear, Inner/surgery , Ear, Inner/pathology , Neuroma, Acoustic/surgery , Neuroma, Acoustic/pathology , Endoscopy/methods , Paralysis
6.
Oper Neurosurg (Hagerstown) ; 24(5): 556-563, 2023 05 01.
Article in English | MEDLINE | ID: mdl-36701659

ABSTRACT

BACKGROUND: Cerebrospinal fluid rhinorrhea after temporal bone surgery involves drainage from the Eustachian tube (ET) into the nasopharynx, causing significant patient morbidity. Variable anatomy of the ET accounts for failures of currently used ET obliteration techniques. OBJECTIVE: To describe the surgical anatomy of the ET and examine possible techniques for ET closure through middle fossa (MF) and transmastoid approaches. METHODS: We described the surgical anatomy of the ET from the MF and transmastoid approaches in 5 adult cadaveric heads, measuring morphometric and surgical anatomy parameters and establishing targets for definite ET obliteration. RESULTS: The osseous ET measured an average of 19.53 mm (±1.56 mm), with a mean diameter of 2.24 mm (±0.29 mm). The shortest distance between the greater superficial petrosal nerve and the ET junction was 6.61 mm (±0.61 mm). Shortest distances between the ET junction and the foramen spinosum and posterior border of the foramen ovale were 1.09 mm (±0.24 mm) and 2.03 mm (±0.30 mm), respectively. Closure of the cartilaginous ET may be performed by folding it in on itself, securing it by packing, suturing, or surgical clip ligation. CONCLUSION: Definite obliteration of the cartilaginous ET appears feasible and the most definite approach to eliminate egress of cerebrospinal fluid to the nasopharynx using the MF approach. This technique may be used as an adjunct to skull base procedures where ET closure is planned.


Subject(s)
Cerebrospinal Fluid Rhinorrhea , Eustachian Tube , Adult , Humans , Eustachian Tube/surgery , Eustachian Tube/anatomy & histology , Skull Base/surgery , Skull Base/anatomy & histology , Cerebrospinal Fluid Rhinorrhea/etiology , Neurosurgical Procedures/adverse effects , Cadaver
7.
Ann Otol Rhinol Laryngol ; 132(8): 976-979, 2023 Aug.
Article in English | MEDLINE | ID: mdl-36154465

ABSTRACT

OBJECTIVES: The neurotologic literature commonly describes venous sinus thrombosis as a complication of mastoiditis. However, thrombosis of the internal carotid artery in the setting of mastoiditis is rarely described. We aim to document a case of carotid artery thrombosis in a patient presenting with mastoiditis. METHODS: We describe this case and review relevant literature. RESULTS: A renal transplant patient was transferred to our hospital with a left middle cerebral artery (MCA) infarct due to acute mastoiditis. Examination demonstrated middle ear effusion and radiologic workup confirmed mastoid infection adjacent to the site of arterial thrombosis. During cortical mastoidectomy and facial recess approach to the middle ear, the petrous carotid bone was found to be dehiscent with pneumatization of the petrous apex. Thrombosis was found to resolve following surgery, IV antibiotics and anticoagulation. Clinically, his focal neurological deficits improved. Proximity of the infectious process to an exposed petrous carotid artery supports the hypothesis that this patient's thrombus was a product of infectious spread and extra-luminal compression. CONCLUSION: To our knowledge, this is the first report of MCA infarction due to petrous ICA arterial thrombus in the setting of mastoid infection. The patient's immunocompromised state may have predisposed and contributed to the adverse outcome. We advocate for aggressive management of acute mastoiditis in the immunocompromised to prevent or manage complications (such as venous thrombophlebitis as well as ICA thrombus) as these patients don't show typical signs of infection and inflammation.


Subject(s)
Carotid Artery Thrombosis , Mastoiditis , Otitis Media , Thrombosis , Humans , Mastoiditis/complications , Mastoiditis/diagnosis , Carotid Artery Thrombosis/complications , Carotid Artery Thrombosis/drug therapy , Anti-Bacterial Agents/therapeutic use , Petrous Bone/diagnostic imaging , Thrombosis/complications , Thrombosis/drug therapy , Otitis Media/complications
8.
Curr Opin Otolaryngol Head Neck Surg ; 30(5): 309-313, 2022 Oct 01.
Article in English | MEDLINE | ID: mdl-36036530

ABSTRACT

PURPOSE OF REVIEW: Preservation of hearing is one of the tenets of vestibular schwannoma management. In recent years, cochlear implants have been employed with increasing use in patients who have suffered profound sensorineural hearing loss due to the natural history of vestibular schwannoma or due to injury to neurovascular anatomy at time of surgical resection. RECENT FINDINGS: Cochlear implantation has been found to be an effective modality for hearing restoration following vestibular schwannoma. Simultaneous cochlear implantation has been employed by an increased number of centers around the world and has been shown to provide restoration of open set speech perception and return of binaural hearing. Ongoing use of electrically evoked auditory brainstem response (ABR) has improved our detection of viable cochlear nerves and provided insight into those who would benefit from this procedure. Finally, minimally invasive approaches to the internal auditory canal and intralabyrinthine tumors have been described. These methods frequently employ simultaneous cochlear implantation and have emphasized that hearing preservation remains possible with surgical excision despite the location of the tumor. SUMMARY: Cochlear implantation is an effective modality for hearing restoration following vestibular schwannoma excision.


Subject(s)
Cochlear Implantation , Cochlear Implants , Hearing Loss, Sensorineural , Neuroma, Acoustic , Cochlear Nerve , Hearing , Humans
9.
Otol Neurotol ; 43(4): e529, 2022 04 01.
Article in English | MEDLINE | ID: mdl-35213481

ABSTRACT

Vestibular nerve section (VNS) is a surgical intervention with hearing preservation used for the treatment of Menière's disease after conservative medical therapy has failed (1,2). With the recent rise in less invasive treatments such as intratympanic gentamicin, VNS has been performed less frequently (3). The middle cranial fossa (MCF) approach for VNS is an uncommon approach due to its technical difficulty. However, it can provide the best distinction of internal auditory canal contents compared with retrosigmoid and retrolabyrinthine approaches. Several advancements in the MCF approach have been described, including the use of intraoperative facial nerve monitoring with electromyography, early removal of the temporal lobe retractor, and the use of an ultrasonic bone aspirator for internal auditory canal decompression (4-6). We demonstrate a case study with a step-by-step approach to successfully sectioning the superior and inferior vestibular nerves while utilizing these advancements and avoiding facial and cochlear nerve injury via the MCF (7). SDC video link: http://links.lww.com/MAO/B409.


Subject(s)
Ear, Inner , Meniere Disease , Cranial Fossa, Middle/surgery , Ear, Inner/surgery , Humans , Meniere Disease/surgery , Neurosurgical Procedures , Vestibular Nerve
10.
Otol Neurotol ; 43(3): 289-294, 2022 03 01.
Article in English | MEDLINE | ID: mdl-35147603

ABSTRACT

OBJECTIVE: Examine the effect of preoperative bone conduction pattern on outcomes of stapedotomy/stapedectomy. STUDY DESIGN: Retrospective case series. SETTING: Tertiary-care academic medical center. PATIENTS: Patients who underwent stapedotomy or stapedectomy from 2013 to 2019. INTERVENTIONS: Primary small-fenestra stapedotomy or partial stapedectomy. MAIN OUTCOME MEASURES: Association between preoperative bone conduction patterns and hearing after stapes surgery. RESULTS: Complete audiometric data were available for 137 patients who had surgery. The mean preoperative air-bone gap (ABG) was 26.8 dBHL. The ABG was closed to less than 20 and 10 dBHL in 88.7 and 65.2% of patients, respectively. A notch at 2000 Hz was present in 32.1% of operated ears and was rarely found at other frequencies. There was no statistically significant association between the presence of a notch and hearing outcomes. The slope of the bone conduction line had no association with hearing outcomes, though an increased bone conduction PTA compared with the contralateral ear was associated with ABG closure less than 10 dBHL and overclosure (odds ratio: 2.14, p = 0.027 and odds ratio: 2.20, p = 0.04). CONCLUSION: In properly selected otosclerosis patients, depressions in bone conduction other than near 2000 Hz are rare and hearing outcomes are generally favorable regardless of the preoperative bone conduction pattern. Despite the association with otosclerosis, the presence of a notch at 2000 Hz is not associated with better hearing outcomes with surgery.


Subject(s)
Otosclerosis , Stapes Surgery , Bone Conduction , Hearing , Humans , Otosclerosis/complications , Otosclerosis/surgery , Retrospective Studies , Stapes , Treatment Outcome
11.
Ann Otol Rhinol Laryngol ; 131(3): 277-280, 2022 Mar.
Article in English | MEDLINE | ID: mdl-34049445

ABSTRACT

OBJECTIVE: Examine the effects of a multi-disciplinary skull base conference (MDSBC) on the management of patients seen for skull base pathology in a neurotology clinic. METHODS: Retrospective case review of patients who were seen in a neurotology clinic at a tertiary academic medical center for pathology of the lateral skull base and were discussed at an MDSBC between July 2019 and February 2020. Patient characteristics, nature of the skull base pathology, and pre- and post-MDSBC plan of care was categorized. RESULTS: A total of 82 patients with pathology of the lateral skull base were discussed at a MDSBC during an 8-month study period. About 54 (65.9%) had a mass in the internal auditory canal and/or cerebellopontine angle while 28 (34.1%) had other pathology of the lateral skull base. Forty-nine (59.8%) were new patients and 33 (40.2%) were established. The management plan changed in 11 (13.4%, 7.4-22.6 95% CI) patients as a result of the skull base conference discussion. The planned management changed from some form of treatment to observation in 4 patients, and changed from observation to some form of treatment in 4 patients. For 3 patients who underwent surgery, the planned approach was altered. CONCLUSIONS: For a significant proportion of patients with pathology of the lateral skull base, the management plan changed as a result of discussion at an MDSBC. Although participants of a MDSBC would agree of its importance, it is unclear how an MDSBC affects patient outcomes.


Subject(s)
Clinical Decision-Making , Neuroma, Acoustic/diagnosis , Neuroma, Acoustic/surgery , Patient Care Team/organization & administration , Skull Base Neoplasms/diagnosis , Skull Base Neoplasms/surgery , Humans , Retrospective Studies
12.
Med Clin North Am ; 105(5): 799-811, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34391534

ABSTRACT

A focused history, otoscopic and tuning fork examination and formal hearing testing are the diagnostic pillars for the workup of hearing loss and tinnitus. The causes of hearing loss and tinnitus are varied and range from relatively common age-related hearing loss to rare tumors of the brain and skull base. In this chapter, the authors explain the diagnostic workup of hearing loss and tinnitus, review the pathophysiology of the most common causes, and describe the treatments available.


Subject(s)
Hearing Loss/physiopathology , Tinnitus/physiopathology , Audiometry , Cochlear Implants , Hearing Aids , Hearing Loss/diagnosis , Hearing Loss/etiology , Hearing Loss/therapy , Hearing Loss, Conductive/diagnosis , Hearing Loss, Conductive/physiopathology , Hearing Loss, Conductive/therapy , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Sensorineural/therapy , Hearing Tests , Humans , Primary Health Care , Severity of Illness Index , Tinnitus/diagnosis , Tinnitus/etiology , Tinnitus/therapy
13.
Med Clin North Am ; 105(5): 901-916, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34391542

ABSTRACT

Vertigo is defined as the illusion of internal or external motion. The evaluation of a patient with vertigo in the primary care setting should not necessarily focus on providing a specific diagnosis. Rather, the physician should aim to localize the lesion. This practice streamlines the workup of patients. This article provides detailed information regarding appropriate organ system-based clinical history and the clinical workup of vertigo. Additional signs and symptoms that can facilitate appropriate referral and treatment are highlighted. Although disorder-specific treatments exist the mainstay of therapy for vertigo-induced pathology is physical therapy.


Subject(s)
Vertigo/pathology , Diagnosis, Differential , Humans , Physical Examination , Physical Therapy Modalities , Primary Health Care , Vertigo/diagnosis , Vertigo/etiology , Vertigo/therapy
14.
Laryngoscope Investig Otolaryngol ; 6(2): 320-324, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33869764

ABSTRACT

OBJECTIVES: Determine the proportion of patients starting the cochlear implant evaluation (CIE) process proceeding to cochlear implantation.Determine which patient factors are associated with undergoing cochlear implantation. METHODS: Retrospective case series of all patients scheduled for a CIE within a tertiary academic neurotology practice between January 1, 2014 and April 30, 2016. Management pathways of patients undergoing CIE were examined. RESULTS: Two hundred thirty-seven adult patients were scheduled for CIE during the study period. Two hundred twenty-six patients started the evaluation process, and 203 patients completed full evaluation. Of patients that completed CIE, 166/203 (82%) met criteria for implantation and 37/203 (18%) did not meet criteria. Fifty-nine patients out of 166 patients (36%) meeting criteria did not receive implants and 107/166 (64%) underwent implantation, yielding an overall implantation rate of 47% (107/226) among patients scheduled for CIE. Common reasons for deferring CI among candidates included failure to show up for preoperative appointment (24%), choosing hearing aids as an alternative (22%), patient refusal (21%) and insurance issues (17%). Overall, CIE led to a new adjunctive hearing device (CI or hearing aid) in 113 (113/203, 56%) cases. CONCLUSION: Fifty-six (113/203) percent of patients who underwent CIE at an academic medical center underwent CI surgery or received an adjunctive hearing device, but 36% (59/166) of candidates did not receive a CI. Patients who forewent CI despite meeting candidacy criteria did so due to cost/insurance issues, or due to preference for auditory amplification rather than CI. LEVEL OF EVIDENCE: 4.

15.
Otol Neurotol ; 42(7): e925-e929, 2021 08 01.
Article in English | MEDLINE | ID: mdl-33710161

ABSTRACT

OBJECTIVE: Traditionally, auditory brainstem implants (ABIs) have been placed via the translabyrinthine or retrosigmoid approaches. In select patients, a modified extended middle cranial fossa (xMCF) approach with tentorial ligation may be advantageous for vestibular schwannoma (VS) resection and auditory rehabilitation. This manuscript describes the application of this modification of the MCF approach for simultaneous VS resection and ABI placement. PATIENTS: Patients with neurofibromatosis type 2, profound bilateral sensorineural hearing loss, single functioning sigmoid/jugular venous system, and giant (>4 cm) VS. INTERVENTIONS: Simultaneous VS resection and ABI placement via a modified xMCF approach with tentorial ligation. MAIN OUTCOME MEASURES: Extent of tumor removal and brainstem decompression, access to lateral recess of the fourth ventricle, functional hearing improvement, surgical complications. RESULTS: Two patients met indications and underwent surgery. There were no immediate or delayed surgical complications. Both had subtotal tumor removal with significant decompression of the brainstem and ABI placement. One patient achieved voice and environmental sound awareness at 35 to 55 dbHL across frequencies. The second patient presented with failure to thrive and multiple lower cranial neuropathies in addition to the above-listed indications. She was hospitalized multiple times after surgery due to failure to thrive and recurrent aspiration pneumonia. Her device was never activated, and she expired 1 year after surgery. CONCLUSIONS: The xMCF with tentorial ligation is an additional approach for tumor resection and ABI placement in selected patients with neurofibromatosis type 2. Future studies will further define when this approach is most applicable as well as the challenges and pitfalls.


Subject(s)
Auditory Brain Stem Implants , Neurofibromatosis 2 , Brain Stem , Cranial Fossa, Middle/surgery , Female , Hearing Loss, Bilateral , Humans , Neurofibromatosis 2/surgery , Treatment Outcome
16.
Int J Mol Sci ; 22(2)2021 Jan 12.
Article in English | MEDLINE | ID: mdl-33445724

ABSTRACT

Patients diagnosed with neurofibromatosis type 2 (NF2) are extremely likely to develop meningiomas, in addition to vestibular schwannomas. Meningiomas are a common primary brain tumor; many NF2 patients suffer from multiple meningiomas. In NF2, patients have mutations in the NF2 gene, specifically with loss of function in a tumor-suppressor protein that has a number of synonymous names, including: Merlin, Neurofibromin 2, and schwannomin. Merlin is a 70 kDa protein that has 10 different isoforms. The Hippo Tumor Suppressor pathway is regulated upstream by Merlin. This pathway is critical in regulating cell proliferation and apoptosis, characteristics that are important for tumor progression. Mutations of the NF2 gene are strongly associated with NF2 diagnosis, leading to benign proliferative conditions such as vestibular schwannomas and meningiomas. Unfortunately, even though these tumors are benign, they are associated with significant morbidity and the potential for early mortality. In this review, we aim to encompass meningiomas and vestibular schwannomas as they pertain to NF2 by assessing molecular genetics, common tumor types, and tumor pathogenesis.


Subject(s)
Brain Neoplasms/pathology , Meningeal Neoplasms/pathology , Meningioma/pathology , Neurofibromatosis 2/pathology , Neuroma, Acoustic/pathology , Animals , Apoptosis/genetics , Brain Neoplasms/genetics , Cell Proliferation/genetics , Humans , Meningeal Neoplasms/genetics , Meningioma/genetics , Mutation/genetics , Neurofibromatosis 2/genetics , Neuroma, Acoustic/genetics
17.
Otol Neurotol ; 42(1): e75-e81, 2021 01.
Article in English | MEDLINE | ID: mdl-32947493

ABSTRACT

OBJECTIVE: Compare outcomes of middle cranial fossa approach (MCF) to vestibular schwannoma (VS) resection in patients 60 years of age and older to patients under 60. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Charts of 216 consecutive VS patients over 18 years of age were reviewed to identify 67 patients who underwent MCF approach to VS resection between 2006 and 2017. INTERVENTION(S): Age at time of surgery. MAIN OUTCOME MEASURE(S): Measured outcomes included postoperative hearing results, facial nerve function, length of hospital stay, wound complications, cerebrospinal fluid leak, myocardial infarction, cerebrovascular accident, seizure, deep vein thrombosis, 30-day readmission, and return to operating room. RESULTS: Sixty-seven patients underwent VS resection via MCF approach including 16 patients > = 60 years (mean 64.4 SD 3.3) and 51 patients < 60 years (mean 45.7 SD 10.2). Between these two groups, there were no differences in sex, tumor laterality, tumor size (10.4 mm versus 9.8 mm, p = 0.6), or other demographic characteristics. Postoperatively, there were no differences between groups in complication rates. Rates of HB 1 or 2 facial nerve function were similar (93.8% versus 88.2%, p = 0.7) as were rates of maintenance of class A or B hearing (58.3% versus 44.4%, p = 0.7). CONCLUSIONS: Patients over 60 undergoing MCF for VS resection experienced similar rates of postoperative complications, facial nerve outcomes, and hearing preservation compared with younger patients. MCF for VS may be considered in the older population. Further research is warranted to evaluate appropriate limitations for this approach based on age.


Subject(s)
Cranial Fossa, Middle , Neuroma, Acoustic , Adolescent , Adult , Cranial Fossa, Middle/surgery , Facial Nerve , Hearing , Humans , Neuroma, Acoustic/surgery , Postoperative Complications/epidemiology , Retrospective Studies , Treatment Outcome
18.
Otol Neurotol ; 42(1): 47-50, 2021 01.
Article in English | MEDLINE | ID: mdl-33165156

ABSTRACT

BACKGROUND: Traditional paradigms of care recommend close audiology follow-up and regular speech perception outcomes assessment indefinitely for cochlear implant (CI) recipients after device activation. However, there is scant published data on actual compliance with this paradigm in clinical practice. METHODS: A multi-center cochlear implant database was queried to identify follow-up rates after cochlear implantation. Follow-up rates where speech perception outcomes assessment occurred at 1-, 3-, 6-, 12-, 18-, and 24-months post-activation were determined by tabulating observed follow-up divided by expected follow-up (O/E ratio) expressed as a percentage. To determine all-cause audiology follow-up rates (with or without testing speech perception outcomes assessment), the database patients from two participating centers (one private practice and one academic center) were similarly analyzed using electronic health record (EHR) data to calculate O/E rates where audiology follow-up occurred for any reason. RESULTS: O/E follow-up rates where speech perception outcomes assessment occurred was 42, 40, 31, 29, 5, and 22% for 1-, 3-, 6-, 12-, 18-, and 24-months post-activation, respectively (n = 2,554). All-cause audiology follow-up rates (with or without speech perception outcomes assessment) using EHR-confirmed data from two individual centers were 97, 94, 81, 66, 41, and 35% at 1-, 3-, 6-, 12-, 18-, and 24-months post-activation visits, respectively (n = 118). CONCLUSIONS: Compliance with audiology follow-up and speech perception outcomes assessment is generally low and decreases significantly as time post-activation increases. Future paradigms of care for CI should be designed recognizing the significant attrition that occurs with CI follow-up.


Subject(s)
Audiology , Cochlear Implantation , Cochlear Implants , Speech Perception , Follow-Up Studies , Humans
19.
Otol Neurotol ; 41(10): 1387-1390, 2020 12.
Article in English | MEDLINE | ID: mdl-33044334

ABSTRACT

OBJECTIVE: Report the details of an unusual case of initially unilateral intradural spontaneous otogenic pneumocephalus in which the patient developed contralateral pneumocephalus after surgical repair of temporal bone and dural defects on the initial side. Discuss the lessons learned concerning management and pathophysiology of this rare case. PATIENT: A 73-year-old male presenting with bilateral retro-orbital pain and headache was found to have unilateral intradural pneumocephalus in the temporal lobe with an adjacent defect in the temporal bone. INTERVENTION: Surgical repair of the initial defect in the temporal bone and dura via a middle cranial fossa approach with obliteration of the mastoid cavity. RESULTS: The patient's symptoms and degree of pneumocephalus on first affected side decreased initially after surgery. Two weeks after surgery he developed worsening pneumocephalus on the contralateral side in the temporal and frontal lobes with weakness and mild somnolence. This side was repaired via a similar technique without mastoid obliteration. Two weeks after surgery the patient recovered to normal level of consciousness with mild residual weakness. CONCLUSIONS: Spontaneous otogenic intradural pneumocephalus is an extremely rare but serious condition related to defects in the temporal bone and dura. The precise mechanism by which this occurs is poorly understood. This unusual case where there were bilateral defects presenting with spontaneous otogenic pneumocephalus in sequence illustrates that it represents a rare sequelae of processes which thin the skull base. These processes should be addressed in addition to acute management of the defect and intracranial air.


Subject(s)
Pneumocephalus , Aged , Cranial Fossa, Middle , Humans , Male , Pneumocephalus/diagnostic imaging , Pneumocephalus/etiology , Pneumocephalus/surgery , Skull Base/diagnostic imaging , Skull Base/surgery , Temporal Bone/diagnostic imaging , Temporal Bone/surgery , Temporal Lobe
20.
Otol Neurotol ; 41(9): e1154-e1157, 2020 10.
Article in English | MEDLINE | ID: mdl-32925860

ABSTRACT

OBJECTIVE: Compare outcomes for patients who were seen via telemedicine visits after otology/neurotology surgical procedures with those who were seen in traditional office visits. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care academic medical center. PATIENTS: Inclusion criteria were patients who underwent otologic/neurotologic surgery and were seen in at least one telemedicine visit for postoperative care. INTERVENTION (S): Telemedicine visit for postoperative care. MAIN OUTCOME MEASURE (S): Comparison of the number of patient calls, unplanned visits, and delayed complications between telemedicine patients and controls matched for age, procedure, and American Society of Anesthesiologists (ASA) status. RESULTS: Eighteen patients were seen in telemedicine visits postoperatively after the following otologic/neurotologic surgical procedures: cochlear implant surgery (10), vestibular schwannoma resection (4), combined middle fossa and transmastoid cerebrospinal fluid (CSF) leak repair (2), middle fossa repair of superior canal dehiscence (1), and stapedectomy (1). All telehealth visits were for the second postoperative visit. The mean number of patient calls after surgery was 2.3 for the telemedicine group and 1.9 for matched controls which was not a statistically significant difference (p = 0.466). Both groups each had two patients who went to the emergency room, with one in each group being admitted. There was one delayed complication in the control group. CONCLUSION: Our preliminary experience suggests telemedicine may be used safely in caring for patients after otologic/neurotologic surgery, though it may be associated with more patient phone calls. Further study and experience, including assessing patient satisfaction, will clarify the future role of telemedicine in otology and neurotology.


Subject(s)
Neurotology , Otologic Surgical Procedures , Telemedicine , Cerebrospinal Fluid Leak , Humans , Retrospective Studies
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