Predictors of hearing functional outcome following surgery for cerebellopontine angle meningioma.

OBJECTIVE: Cerebellopontine angle (CPA) meningiomas can affect hearing function and require expeditious treatment to prevent permanent hearing loss. The authors sought to determine the factors associated with functional hearing outcome in CPA meningioma patients treated with surgery and/or radiation therapy in the form of either stereotactic radiosurgery or stereotactic radiation therapy. METHODS: Consecutive patients with CPA meningiomas who had presented at our hospital from 2008 to 2018 were identified through retrospective chart review. Hearing function (as defined by pure tone average (PTA) and speech discrimination score (SDS) on Audiogram) was assessed before and after surgery for CPA meningioma. Audiograms with PTA > 50 dB and SDS < 69% were defined as poor hearing functional outcome. Multivariable Cox Proportional Hazards Regression Model was used to assess the associations between pre-operative hearing functional assessment and post-operative hearing functional outcomes. RESULTS: The study cohort included 31 patients (80.6% females, with a mean age of 61.3 ± 15.2 years) with a median clinical follow-up of 5 months (range: 1 week-98 months). The mean pre-operative PTA and SDS were 23.8 ± 11.2 dB and 64.4 ± 22.2% respectively. At the last visit, there was significant hearing recovery, with an improvement of 29.7 ± 18.0 dB (p < 0.001) and 87.6 ± 17.8% (p < 0.001) in PTA and SDS respectively. After adjusting for age, gender, tumor volume, location, and tumor classification, Multivariable Cox Proportional Hazards Regression Model was conducted which revealed that patients undergoing surgery through retro sigmoid approach [Hazards Ratio (HR): 32.1, 95% Confidence Interval (CI): 2.11-491.0, p = 0.01] and gross total resection (GTR) (HR: 2.99, 95% CI: 1.09-9.32, p = 0.05) had significantly higher risk of poor hearing functional outcome compared to petrosal approach and near/subtotal resection. Moreover, patients with poor preoperative hearing had 85% higher chance of poor hearing functional outcome postoperatively (HR: 0.15, 95%CI: 0.03-0.59, p = 0.007). CONCLUSION: Postoperative improvement in hearing is a reasonable expectation following surgery for CPA meningioma. Preoperative hearing, surgical approach and extent of surgical resection are predictive factors of postoperative hearing function outcome and can therefore aid in identification of patients at higher risk of hearing loss.

Evaluation of Cholesteatoma.

Evaluation of cholesteatoma depends on clinical history and examination, with microscope and/or endoscope. A history of hearing loss with a chronic draining ear, refractory to ototopical medication, raises suspicion for cholesteatoma. Symptoms of Eustachian tube dysfunction or prior ear surgery including ear tubes should be elicited. Inflammation can be severe and should be suppressed if possible. Once cholesteatoma is diagnosed or strongly suspected, further workup includes audiometry prior to surgical excision. Imaging may supplement the workup and is especially helpful if there are concerning features including vertigo, third window symptoms, asymmetric bone line, facial nerve weakness, or for anticipatory guidance.

Audiologic Consequences of Gunshot Wounds to the Temporal Bone Show No Relation to Concomitant Neurologic or Vascular Injuries.

OBJECTIVES: To evaluate audiologic consequences of gunshot wounds (GSWs) to the temporal bone (TB), and to correlate hearing outcomes with neurologic and vascular injuries adjacent to the temporal bone. STUDY DESIGN: Retrospective case series. SETTING: University-based level-one trauma center. METHODS: Retrospective review of 35 patients surviving TB ballistic injury, 2012 to 2021. Main outcomes were audiologic results. Demographics, concomitant injuries, CT, and interventions were reviewed. RESULTS: Mean age was 30.7 years; 80% male. Seventeen patients (48.6%) underwent audiologic testing. Mean pure tone average (PTA) was 75 ± 35 dB, bone line average 41 ± 26 dB, and speech discrimination score (SDS) 60 ± 43%. Nineteen (54.3%) demonstrated facial nerve injury (FNI), who were more likely to show SNHL especially anacusis, though their mean PTA and SDS were not statistically different from those without (P = .30 and .47, respectively). Radiographic review of those with sensorineural loss (SNHL, 6/17) revealed otic capsule-disrupting fracture (n = 2), pneumolabyrinth (n = 2), intracranial hemorrhage (n = 3). Those with mixed loss (6/17) showed otic capsule-sparing fracture (n = 6), EAC injury (n = 5), ossicular discontinuity (n = 2), and intracranial hemorrhage (n = 4). Two with mastoid tip fractures alone had normal audiograms. Audiometric outcomes were not predicted by concomitant CSF leak, spinal injuries, vascular injuries, cranial neuropathies, or traumatic brain injury. CONCLUSIONS: All patterns of hearing loss-conductive, sensorineural, mixed and normal-may be seen following TB ballistic injuries. Trauma severe enough to disrupt the facial nerve is more likely to cause anacusis. However, all should be formally evaluated, since ballistic injuries complicated by neurologic or vascular damage do not necessarily correlate with worse audiologic outcomes, while patients with minimal fractures may demonstrate losses.

Delays in Treatment for Temporal Encephaloceles and Cerebrospinal Fluid Leaks.

OBJECTIVE: Lateral skull base defects (LSBD) pose a diagnostic challenge; however, early recognition and treatment are important to avoid sequelae. This study examines the impact of health care disparities associated with time to diagnosis and treatment for patients with LSBD. STUDY DESIGN, SETTING, PATIENTS, INTERVENTION, OUTCOME MEASURES: Multi-institutional retrospective cohort study at four U.S. tertiary centers from 2000 to 2022. Adult patients with a primary diagnosis of CSF leak or encephalocele were included. Multivariate regressions used to analyze how age, sex, race/ethnicity, insurance, language, zip code, distance to medical center, referral patterns, diagnostic workup, and clinical course affected time to diagnosis and treatment. RESULTS: In 127 patients with LSBD, mean time to treatment of CSF leak or encephalocele was 13.9 months. On average, patients waited 10.6 months from initial assessment to diagnosis and saw 2 providers prior to diagnosis. Approximately 91% (115) of patients had a CT scan, and 75% (95) had an MRI. Imaging did not influence time to treatment. Older age, public insurance, and number of providers seen were associated with delays. Non-English speakers (5% of 127) encountered treatment delays, although this was not statistically significant. Fifty-eight (46%) people had private insurance. The average traveled distance for care was 62.6 miles. Clinical presentation, race, zip code, imaging, myringotomy, beta-2 transferrin, and ED workup were not found to be associated with delays to care. CONCLUSION: There are significant delays in diagnosis and management of LSBD. Referral patterns did influence care. Health care disparities did not impact care; however, disparities and language barriers need to be studied further to determine contributions to delays in care.

Management of Foreign Bodies in the Ear Canal.

Using the appropriate instruments and techniques for removing a foreign body from the external auditory canal facilitates the procedure and reduces the risk of complications. Most ear foreign bodies can be addressed on a nonurgent basis, but batteries and caustic materials warrant prompt removal. Referral to an otolaryngologist should be considered for difficult cases or after a failed attempt.

Cochlear implantation through intracochlear fibrosis: A comparison of surgical techniques.

OBJECTIVE: While the implications of ossification on cochlear implantation (CI) have been extensively described, there is a paucity of data regarding the fibrotic stage. We examined the outcomes of different insertion techniques for managing intracochlear fibrosis. STUDY DESIGN: Retrospective review of case series with case-control comparison. SETTING: University-based tertiary-referral otology-neurotology practice. PATIENTS: Between 2009 to 2020, 384 patients underwent CI. Of those, 7 patients (8 ears) demonstrated intracochlear fibrosis. INTERVENTIONS: CI performed 1-4 months following meningitis/labyrinthitis and 12-24 months after idiopathic sudden SNHL. Fibrosis removal (38%) or dilation (63%) permitted implantation. A styleted-electrode was used in 63% due to dense fibrosis. MAIN OUTCOME MEASURES: Postoperative audiometry with CI in place, additional comparisons with audiometric outcomes in age-matched controls. RESULTS: Full insertion achieved in all except one ear with partial ossification. Mean ipsilateral pure tone average (PTA) improved to 29 ± 15 dB and speech discrimination to 72 ± 28%. Fibrosis removal vs. dilation resulted in no PTA differences (p = 0.76). Poorest outcomes occurred with the longest time to surgery. CONCLUSIONS: Good CI audiologic outcomes in the setting of cochlear fibrosis can be achieved and are independent of technique. Instead, they vary with time to implantation. Every attempt should be made to intervene as early as possible.

Management of the facial nerve following temporal bone ballistic injury.

Objective: To understand the patterns of temporal bone fracture and facial nerve injury from ballistic trauma. Study Design: Retrospective case series. Methods: Retrospective review of 42 patients evaluated following temporal bone ballistic injury at a single institution, university-based level-one trauma center between 2012 and 2021. Demographics, facial nerve status, CT images, interventions, complications, and outcomes were reviewed. Results: Mean age 30.3 years (range 5-58 years); 79% male. Racial demographics reflected the surrounding community. Seven mortalities occurred. Nineteen patients (54%) demonstrated facial nerve injury. Of those, 13/19 displayed immediate paralysis, 1 delayed, 5 unknown (due to altered mental status). On consultation, House-Brackmann grade 6 paralysis was common (13/19). Fracture was otic capsule-sparing in 17/19 (90%), universally comminuted, with significant disruption along the mastoid tip (16/19), external auditory canal (EAC) (15/19), and periauricular soft tissues (13/19). Nine patients underwent surgical intervention: Transmastoid facial nerve decompression to remove compressive bony spicules (n = 5); eye protection surgery (n = 3); and peripheral facial nerve exploration (n = 1), noting transection at the pes. One required middle cranial fossa and transmastoid repair of cerebrospinal fistulae in setting of severe meningitis. House-Brackmann scores improved in 80% following transmastoid nerve decompression despite CT evidence of likely additional injury in its extratemporal course. Conclusions: Common patterns of temporal bone fracture seen in blunt trauma (longitudinal/transverse, otic capsule-sparing/disrupting) were not found in patients with ballistic facial nerve injury. Rather, injury was commonly apparent in the EAC, mastoid tip, and periauricular soft tissues. Clinicians should have high suspicion for extratemporal facial nerve injury following ballistic trauma.

Conversations in Cochlear Implantation: The Inner Ear Therapy of Today.

As biomolecular approaches for hearing restoration in profound sensorineural hearing loss evolve, they will be applied in conjunction with or instead of cochlear implants. An understanding of the current state-of-the-art of this technology, including its advantages, disadvantages, and its potential for delivering and interacting with biomolecular hearing restoration approaches, is helpful for designing modern hearing-restoration strategies. Cochlear implants (CI) have evolved over the last four decades to restore hearing more effectively, in more people, with diverse indications. This evolution has been driven by advances in technology, surgery, and healthcare delivery. Here, we offer a practical treatise on the state of cochlear implantation directed towards developing the next generation of inner ear therapeutics. We aim to capture and distill conversations ongoing in CI research, development, and clinical management. In this review, we discuss successes and physiological constraints of hearing with an implant, common surgical approaches and electrode arrays, new indications and outcome measures for implantation, and barriers to CI utilization. Additionally, we compare cochlear implantation with biomolecular and pharmacological approaches, consider strategies to combine these approaches, and identify unmet medical needs with cochlear implants. The strengths and weaknesses of modern implantation highlighted here can mark opportunities for continued progress or improvement in the design and delivery of the next generation of inner ear therapeutics.

The Role of Intra-Operative Duplex Ultrasonography Following Translabyrinthine Approach for Vestibular Schwannoma.

Objective: Sigmoid sinus (SS) stenosis is a complication of translabyrinthine approach. Velocity changes in the SS measured by intra-operative doppler ultrasound may help in identifying patients at risk for sinus occlusion. Patients: SS velocity was measured using doppler ultrasound prior to opening dura and again prior to placement of the abdominal fat graft. Intervention: Data collected included: patient age, surgical side, sinus dominance, tumor volume, intra-operative doppler ultrasound measurements, post-operative venous sinus imaging, anticoagulation, and morbidities and mortalities. Main Outcome Measure: SS patency and velocity. Results: Eight patients were included in the analysis (22 to 69 years). Four had left-sided and four had right-sided craniotomies. Sigmoid sinuses were either right-side dominant or co-dominant. The mean velocity ± standard deviation (SD) prior to dura opening and abdominal fat packing was 23.2 ± 11.3 and 25.5 ± 13.9 cm/s, respectively, p = 0.575. Post-operative Magnetic Resonance Venography (MRV) imaging showed four sigmoid sinus occlusions; seven patients showed sigmoid sinus stenosis, and one internal jugular vein occlusion. One patient had post-operative Computed Tomography Venography (CTV) only. Of the four patients with MRV occlusions, CTVs were performed with three showing occlusion and all four-showing stenosis. One patient with internal jugular vein occlusion on MRV received warfarin anticoagulation. There was one cerebrospinal fluid leak requiring ear closure, one small cerebellar infarct, and one with facial nerve palsy (House-Brackman Grade 3). Conclusion: SS velocity changes before and after tumor resection were not predictive of sinus occlusion. We hypothesize that sinus occlusion may be caused by related factors other than thrombosis, such as external compression of the sinus secondary to abdominal fat grafting.

Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease.

OBJECTIVE: To describe the clinical presentation of patients with isolated saccular endolymphathic hydrops (EH) detected. STUDY DESIGN: Clinical case series. SETTING: University-based tertiary referral center. PATIENTS: All subjects presenting with vertigo or hearing loss who had isolated saccular EH detected. INTERVENTION: High-resolution delayed-contrast magnetic resonance imaging (MRI) conducted between November 2015 and November 2016. MAIN OUTCOME MEASURES: Audiovestibular testing results and analysis of clinical histories. RESULTS: Isolated saccular EH was detected in 18 subjects. Sixteen met criteria for definite Menière's disease (MD, n = 12) or delayed endolymphatic hydrops (DEH, n = 4). One had a history of sudden sensorineural hearing loss (SSNHL) and 3 years after MRI developed recurrent vertigo characteristic of DEH. One patient had a history of atypical DEH (Tumarkin falls without vertigo following SSNHL). Four patients had Tumarkin falls. Most (83%) demonstrated mild-to-severe low-frequency fluctuating loss, and six (33.3%) had a history of ipsilateral sudden profound SNHL. Nine of the 17 (53%) patients tested had an ipsilateral caloric paresis ranging from 26 to 67%. Ipsilateral vestibular-evoked myogenic potentials showed reduced or absent responses in 5 of the 17 tested (29%). CONCLUSIONS: The full spectrum of MD may be associated with saccular hydrops. We propose that MD and DEH often begin in the saccule, and MRI may provide clues to the pathophysiology of MD. Saccular hydrops was present in one patient with SSNHL who did not develop vertigo spells until 3 years after MRI, indicating that saccular hydrops may be the first manifestation of MD or DEH.

Phase II randomized study of preoperative calcitriol to prevent hypocalcemia following thyroidectomy.

BACKGROUND: A prospective, stratified, randomized, double-blind, placebo-controlled study was conducted to observe the impact of preoperative calcitriol supplementation on serum calcium levels following total thyroidectomy. METHODS: Subjects were randomized 1:1 to receive 1 µg calcitriol or placebo for 1 week preceding thyroidectomy. The primary outcome measure was change in serum calcium from baseline to 18 h post-thyroidectomy. Subjects were also assessed for incidence of symptomatic hypocalcemia, length of stay, readmission for hypocalcemia, and intravenous calcium supplementation. RESULTS: Forty-seven patients underwent thyroidectomy; 23 received preoperative calcitriol supplementation, and 24 received placebo. Repeated measures regression demonstrated no difference in postoperative serum calcium over time (p = 0.22). There were no occurrences of hypocalcemia, intravenous calcium supplementation, or readmission in either group. No difference was observed in length of stay (p = 0.38). One patient in the calcitriol group developed Grade 3 hypercalcemia. CONCLUSIONS: Preoperative calcitriol supplementation had no impact on postoperative serum calcium levels compared to placebo.

Anatomic Assessment of the Limits of an Endoscopically Assisted Retrolabyrinthine Approach to the Internal Auditory Canal.

Objective Data regarding the surgical advantages and anatomic constraints of a hearing-preserving endoscopic-assisted retrolabyrinthine approach to the IAC are scarce. This study aimed to define the minimum amount of retrosigmoid dural exposure necessary for endoscopic exposure of the IAC and the surgical freedom of motion afforded by this approach. Methods Presigmoid retrolabyrinthine approaches were performed on fresh cadaveric heads. The IAC was exposed under endoscopic guidance. The retrosigmoid posterior fossa dura was decompressed until the fundus of the IAC was exposed. Surgical freedom of motion at the fundus was calculated after both retrolabyrinthine and translabyrinthine approaches. Results The IAC was entirely exposed in nine specimens with a median length of 12 mm (range: 10-13 mm). Complete IAC exposure could be achieved with 1 cm of retrosigmoid dural exposure in eight of nine mastoids. For the retrolabyrinthine approach, the median anterior-posterior surgical freedom was 13 degrees (range: 6-23 degrees) compared with 46 degrees (range: 36-53 degrees) for the translabyrinthine approach ( p = 0.014). For the retrolabyrinthine approach, the median superior-inferior surgical freedom was 40 degrees (range 33-46 degrees) compared with 47 degrees (range: 42-51 degrees) for the translabyrinthine approach ( p = 0.022). Conclusion Using endoscopic assistance, the retrolabyrinthine approach can expose the entire IAC. We recommend at least 1.5 cm of retrosigmoid posterior fossa dura exposure for this approach. Although this strategy provides significantly less instrument freedom of motion in both the horizontal and vertical axes than the translabyrinthine approach, it may be appropriate for carefully selected patients with intact hearing and small-to-medium sized tumors involving the IAC.

Clinical Significance of Middle Cerebellar Peduncle Ischemia After Translabyrinthine Vestibular Schwannoma Resection.

OBJECTIVE: To assess clinical symptoms, signs, and radiographic evolution of middle cerebellar peduncle (MCP) diffusion restriction (DR) abnormalities following vestibular schwannoma (VS) resection. STUDY DESIGN: Retrospective chart and imaging review. SETTING: Tertiary-referral neurotology and neurosurgery practice. PATIENTS: All consecutive patients who underwent translabyrinthine VS resection over a 2-year period (August 2017-May 2019). INTERVENTION: Translabyrinthine craniotomy for VS resection. MAIN OUTCOME MEASURES: Magnetic resonance imaging (MRI) obtained on postoperative day 1 were reviewed for DR within the pons and cerebellum, with 3 months follow-up MRI to assess for evolution of these vascular changes. RESULTS: Of the 31 patients who met inclusion criteria, MRI demonstrated MCP DR consistent with acute ischemia in 29% (9/31). Of those, two showed corresponding T2 signal abnormalities on follow up MRI consistent with cerebrovascular accident (CVA) within the MCP. Both had severe gait ataxia and dysmetria requiring acute rehabilitation admission and significantly larger tumors (p = 0.02). The remaining seven were asymptomatic, and DR abnormality resolved without lasting radiographic changes. Brainstem compression was present in 100% of patients with postoperative MCP DR (mean MCP ipsilateral:contralateral ratio 0.59 ±â€Š0.19), and 68.1% of those without (mean MCP ratio 0.71 ±â€Š0.25), a difference that was not statistically significant (p = 0.14). In the two patients with CVA, MCP asymmetry persisted, whereas the asymmetry resolved in all others. CONCLUSIONS: Asymptomatic acute MCP ischemia discovered incidentally does not require intervention. However, when the ischemic area is large and patients are symptomatic, especially if an acute rehabilitation admission is required, surgeons should suspect true CVA.

Hypercoagulability in Vestibular Schwannoma Surgery.

OBJECTIVE: Patients with vestibular schwannoma who harbor a genetic predisposition for venous thromboembolism require special consideration when determining optimal therapeutic management. The primary objective of the current study was to provide recommendations on treatment of hypercoagulable patients with vestibular schwannoma through a case series and review of the literature. PATIENTS: Two patients who underwent resection of vestibular schwannomas. INTERVENTIONS: Surgical resection and diagnostic testing. MAIN OUTCOME MEASURES: Postoperative venous thromboses. RESULTS: One patient who underwent resection of vestibular schwannoma and suffered several postoperative thrombotic complications consistent with a clinical thrombophilia. One patient with known Factor V Leiden deficiency who underwent resection of vestibular schwannoma followed by postoperative chemoprophylaxis with a direct factor Xa inhibitor and experienced an uneventful postoperative course. CONCLUSIONS: In patients with a known propensity for venous thromboembolism, the skull base surgeon should consider nonsurgical management. If the patient undergoes surgical resection, we recommend careful effort to minimize trauma to the sigmoid sinus. In addition, the surgeon may consider retrosigmoid or middle fossa approaches. Best practice recommendations include the use of pneumatic compression devices, early ambulation, and consideration of postoperative prophylactic anticoagulation in patients with a known genetic predisposition.

A wider influence: The impact of formal humanitarian otology training on otology-neurotology fellows.

OBJECTIVES: Long-term commitment to humanitarian surgical outreach requires the opportunity, resources, and time to participate, but perhaps more importantly, it requires a preceding successful outreach experience. The Accreditation Council for Graduate Medical Education (ACGME) expects physician trainees to achieve six Core Competencies: patient care, medical knowledge, interpersonal and communication skills, professionalism, practice-based learning and improvement, and systems-based practice. This study investigates the challenges and benefits of a supervised humanitarian experience with a focus on ACGME Core Competencies, future global outreach, and commitment to education. STUDY DESIGN: Physician survey and program curriculum review. METHODS: Participants in a supervised humanitarian educational experience at a single Otology-Neurotology Fellowship Program between the years of 2006 and 2019 were surveyed. Barriers to participation, challenges, benefits, prior and ongoing humanitarian efforts, and education endeavors were discussed. RESULTS: Challenges including time away from fellowship, logistical difficulties with Resident Review Committee recognition of operative case volume, and civil unrest were encountered. Benefits within all six ACGME Core Competencies were achieved. International collaboration with local providers, patients, and families with diverse socioeconomic and cultural backgrounds allowed bidirectional education while striving for safe, innovative solutions in resource-poor environments. CONCLUSIONS: A supervised education experience with a sustainable humanitarian otology program offers Neurotology fellows an early positive experience near the end of their formal training. ACGME Professionalism goals were achieved through building collegial relationships with local otolaryngologists and staff, while fostering a sense of responsibility to assist those in developing nations. Participation correlated with an ongoing commitment to humanitarian efforts and education post-fellowship. LEVEL OF EVIDENCE: 4.

Congenital Cholesteatoma of the Sphenoid, Occipital, and Temporal Bones: 54-Year Follow Up.

OBJECTIVE: To provide long-term follow up of a unique patient with history of massive petrous apex congenital cholesteatoma. PATIENT: 75-year-old man who presented at age 18 with left Gradenigo-like syndrome. INTERVENTION: Staged left radical mastoidectomy and open transsphenoidal marsupialization. Followed with routine in-office cholesteatoma debridement. MAIN OUTCOME MEASURES: Bony erosion on computed tomography (CT), monitoring for new symptoms. RESULTS: Interval development of sensorineural component of hearing loss due to cochlear fistulization. Very slow skull base bony erosion continues without neck destabilization. No new or recurrent cranial neuropathies have developed. CONCLUSIONS: Marsupialized cholesteatoma may be followed with routine debridement and interval imaging for monitoring over many decades.

Failure to close the gap: Concomitant superior canal dehiscence in otosclerosis patients.

OBJECTIVE: Superior semicircular canal dehiscence (SSCD) causing conductive hearing loss with present reflexes is a known reason for stapes surgery failure. However, concomitant SSCD and otosclerosis occur rarely. We present a case series of SSCD diagnosed in positively identified otosclerosis patients. METHODS: Retrospective review of clinical case series in two tertiary-referral neurotologic practices was performed. Clinical histories, operative findings, audiograms, and computed tomography (CT) images were reviewed for patients diagnosed with both SSCD and otosclerosis. Patients with present stapedial reflexes were excluded. Comprehensive literature review was performed. RESULTS: Four cases were identified. Three reported improved hearing with partial or complete closure of the air-bone gap (ABG), including one patient with far-advanced otosclerosis. One patient had no improvement in pure tone average or ABG. One patient reported worsening of preoperative autophony and pulsatile tinnitus, but none developed new postoperative third-window symptoms. CTs demonstrated both SSCD and otosclerosis. CONCLUSION: Failure to close the ABG in patients with positively identified otosclerosis intraoperatively may be due to SSCD as an occult concomitant diagnosis. SSCD should be considered as a possible cause of persistent conductive hearing loss after stapes surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1023-1027, 2020.

Sensitivity of High-Resolution Computed Tomography in Otosclerosis Patients undergoing Primary Stapedotomy.

OBJECTIVE: To determine the incidence of abnormal otospongiotic or otosclerotic findings on high-resolution computed tomography (HRCT) as read by local radiologists in patients with surgically-confirmed otosclerosis. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary-referral private otology-neurotology practice. PATIENTS: Adults (>18 years old) with surgically-confirmed otosclerosis between 2012 and 2017 with a HRCT performed preoperatively. INTERVENTION: Preoperative HRCT then stapedotomy. MAIN OUTCOME MEASURES: Positive identification and location of radiographic otosclerosis as reported by the local radiologist. We then correlated the CT with surgical location as documented at time of surgery. Audiometry, demographic data, intraoperative findings, and surgical technique were secondarily reviewed. RESULTS: Of the 708 stapes surgeries were performed during the study time frame. Preoperative HRCT scans were available for 68 primary stapedotomy surgeries performed in 54 patients. Otosclerosis was reported in 20/68 (29.4%). Following a negative report by the local radiologist, a re-review by the surgeon and/or collaborating neuroradiologist confirmed otosclerosis in 12/48 additional cases (25.0%). There was an overall sensitivity of 47.1%. Intraoperatively, cases with negative reads tended to have more limited localization at the ligament (8.7%) or anterior crus (39.1%), compared with positive reads, which demonstrated more extensive involvement, with bipolar foci (30.0%) or diffuse footplate manifestations (20.0%) more common. Acoustic reflexes were characteristically absent. CONCLUSIONS: While HRCT may aid in the diagnosis of otosclerosis and rule out concomitant pathology in certain cases of clinical uncertainty or unexplained symptoms, its sensitivity for otosclerosis remains low. HRCT should not be relied upon to diagnose routine fenestral otosclerosis.

Radiation-Induced Malignant Peripheral Nerve Sheath Tumor of the Vagus Nerve Following Radiation Treatment of Cervical Paraganglioma.

Radiation-induced sarcoma is a known but rare complication of radiation treatment for skull base paraganglioma. We present the cases of a female patient with multiple paraganglioma syndrome treated with external beam radiation treatment who presented 4 years later with a malignant peripheral nerve sheath tumor of the vagus nerve.

Effect of Vestibular Schwannoma Size and Nerve of Origin on Posterior External Auditory Canal Sensation: A Prospective Observational Study.

OBJECTIVE: Posterior external auditory canal (EAC) hypesthesia (Hitselberger's sign) has been previously described to occur in all vestibular schwannomas (1966) but has not been studied since. We hypothesized that sensory loss may be related to tumor size and sought to determine if this clinical sign could predict preoperative characteristics of vestibular schwannomas, intraoperative findings, and/or surgical outcomes. STUDY DESIGN: Prospective observational study. SETTING: Tertiary referral center. PATIENTS: Twenty-five consecutive patients who underwent surgery for vestibular schwannoma. INTERVENTION: Patients were tested for the presence of EAC hypesthesia or anesthesia. MAIN OUTCOME MEASURES: Preoperative, intraoperative, and postoperative findings were recorded, including facial nerve function, hearing function, tumor size, tumor nerve of origin, and extent of resection. RESULTS: Twelve patients (48%) demonstrated either posterior EAC hypesthesia (11 patients) or anesthesia (1 patient). Sensory loss was a significant predictor of size (tumor maximal diameter) (p = 0.004). Median tumor diameter was 1.7 cm in the cohort with intact sensation versus 2.9 cm in the cohort with sensory loss. Patients with sensory loss were also significantly more likely to be associated with a superior vestibular nerve origin tumor (p = 0.01). Preoperative sensory loss did not significantly predict postoperative facial outcome (p = 0.10). CONCLUSION: Neurological exam findings may be overlooked in the workup of brain tumors. Posterior EAC hypesthesia is a predictor of tumor size and superior vestibular nerve origin. These findings may have implications for patient selection, particularly with the middle cranial fossa approach. Furthermore, given this relationship with tumor size, this clinical biomarker should be studied as a potential predictor of tumor growth.