Otology Neurotology 2020-12-01

A Systematic Review of Cochlear Implantation in Temporal Bone Fractures and the Significance of Otic Capsule Involvement

Cowan, Brandon; Oska, Sandra; Arianpour, Khashayar; Svider, Peter F.; Bojrab, Dennis II; Hong, Robert S.

Publicatie 01-12-2020


Objective: Head trauma can cause deafness in cases with and without a skull base fracture. Anatomic disruption can occur with skull base fractures, but inner ear structures can also be damaged by the concussive forces of the trauma even without fracture. It is thought that negative prognostic indicators for successful cochlear implantation (CI) for hearing rehabilitation include fractures involving the otic capsule or ossification of the cochlea. This review evaluates success of CI in posttrauma patients.Data Sources: PubMed/MEDLINE, Cochrane, and Embase.Study Selection: Search terms used were “cochlear implant” AND (“trauma” OR “fracture”). Studies including preoperative computed tomography or magnetic resonance imaging and patient-level outcome data were included.Data Extraction: Study design, perioperative and postoperative outcomes, and complications were reported. Success rates of CI were compared using χ2 analysis.Data Synthesis: Twenty-three studies encompassing 88 patients who experienced posttraumatic deafness and received subsequent CI were included. CI was reported as successful for 28 of 34 patients (82%) with otic capsule-involving fracture, as compared with 17 of 22 (77%) in those who had an otic capsule-sparing temporal bone fracture, and 15 of 17 (88%) of patients without evidence of fracture (i.e., concussive inner ear injury).Conclusions: In patients with posttraumatic hearing loss, success rates do not significantly differ between cases that do or do not involve fractures of the otic capsule. CI for posttraumatic hearing loss is generally successful.

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Noise Damage Accelerates Auditory Aging and Tinnitus: A Canadian Population-Based Study

Jafari, Zahra; Copps, Thomas; Hole, Glenn; Kolb, Bryan E.; Mohajerani, Majid H.

Publicatie 01-12-2020


Objective: Age-related hearing loss (ARHL) is the third most challenging disability in older adults. Noise is a known modifiable risk factor of ARHL, which can drive adverse health effects. Few large-scale studies, however, have shown how chronic noise exposure (CNE) impacts the progression of ARHL and tinnitus.Study Design: Retrospective large-scale study.Setting: Audiology clinical practice.Patients: In this study, 928 individuals aged 30-100 years without (n=497) or with the experience of CNE (n=431) were compared in their hearing assessments and tinnitus. In order to only investigate the impact of CNE on ARHL and tinnitus, people with other risk factors of hearing loss were excluded from the study.Intervention: Diagnostic.Main Outcome Measures: Noise damage was associated with a greater ARHL per age decades (pure-tone average(PTA)0.5-4kHz alterations 19.6-70.8 dB vs. 8.0-63.2 dB, ≤0.001), an acceleration of developing a significant ARHL at least by two decades (PTA0.5-4kHz 33.4 dB at 50-59yr vs. 28.2 dB at 30-39yr, ≤0.001), and an increased loss of word recognition scores (total average 84.7% vs. 80.0%, ≤0.001). Significant noise-associated growth in the prevalence of tinnitus also was shown, including more than a triple prevalence for constant tinnitus (28.10% vs. 8.85%, ≤0.001) and near to a double prevalence for intermittent tinnitus (19.10% vs. 11.10%, ≤0.001). Noise also resulted in the elevation of the static compliance of the tympanic membrane throughout age (total average 0.61 vs. 0.85 mmho, ≤0.001).Conclusions: Our findings emphasize the significant contribution of CNE in auditory aging and the precipitation of both ARHL and tinnitus.

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Ear Preference and Interaural Threshold Asymmetry

Chang, Jolie L.; Huwyler, Camille M.; Cueva, Kristine L.; Henderson-Sabes, Jennifer; Cheung, Steven W.

Publicatie 01-12-2020


Objective: To define the relationships among ear preference strength, audiometric interaural asymmetry magnitude, and hearing impairment.Study Design: Prospective, cross-sectional.Setting: Academic audiology clinic.Patients: Adults.Interventions: Diagnostic.Main Outcome Measures: Patient-reported ear preference strength using a seven-category preference (no preference; left or right somewhat, strongly, or completely) scheme, hearing disability level on the Speech, Spatial, and Qualities of Hearing scale, and audiometric interaural threshold asymmetry were analyzed in three study cohorts: 1) normal hearing (thresholds ≤ 25 dB, n = 66), 2) symmetric hearing loss (any single threshold > 25 dB, n = 81), and 3) asymmetric hearing loss (maximum average interaural threshold difference at any two adjacent frequencies (IThrDmax2)≥ 15 dB, n = 112).Results: Receiver operating characteristic curves for somewhat, strongly, and completely ear preference levels using IThrDmax2 cutoff values at 15, 30, and 45 dB showed good to excellent classifier performance (all curve areas ≥ 0.84). The mapping of ear preference strength to the most likely IThrDmax2 range by odds ratio analysis demonstrated: no preference (< 15 dB), somewhat (15–29 dB), strongly (30–44 dB), and completely (≥ 45 dB). Complete dependence on one ear was associated with the most severe degradation in spatial hearing function.Conclusion: Categorical ratings of ear preference strength may be mapped to ranges of audiometric threshold asymmetry magnitude and spatial hearing disability level. Querying ear preference strength in routine clinical practice would enable practitioners to identify patients with asymmetric hearing more expeditiously and promote timely evaluation and treatment.

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Changes in the Auditory Association Cortex in Dementing Illnesses

Aylward, Alana; Auduong, Priscilla; Anderson, Jeffrey S.; Zielinski, Brandon A.; Wang, Angela Y.; Weng, Cindy; Foster, Norman L.; Gurgel, Richard K.

Publicatie 01-12-2020


Objective: To evaluate the relationship between degree of cognitive impairment and gray-matter density changes in the auditory cortex.Study Design: Retrospective case-control.Patients: Six hundred sixty-three patients of a tertiary referral center cognitive disorders clinic.Intervention: Magnetic resonance imaging.Main Outcome Measures: Ratios of gray matter density of the primary auditory cortex (A1) to whole brain and auditory association cortex (AAC) to whole brain in patients with Alzheimers disease (AD) compared with mild cognitive impairment (MCI) and patients with a mini-mental state exam (MMSE) scores ≤25 versus >25.Results: After multivariate analysis, a statistically significant difference between AAC to brain ratios for patients with a MMSE ≤25 (n = 325) compared with >25 (n = 269) was found, with values −0.03 (95% CI −0.04 to −0.02, p < 0.0001) on the left and −0.04 (95% CI −0.06 to −0.03, p < 0.0001) on the right. The adjusted average difference of left and right AAC to brain ratios between AD patients (n = 218) compared with MCI patients (n = 121) was also statistically significant, at −0.03 (95% CI −0.05 to −0.01, p = 0.004) and −0.05 (95% CI −0.07 to −0.03, p < 0.0001), respectively. There was no statistically significant difference in the left or right A1 to brain ratios between the MMSE groups or between the AD and MCI groups.Conclusions: The AAC for patients with MMSE ≤25 and for those with AD shows decreased gray matter density when compared with patients with better cognitive function. No difference was detected in A1, raising the possibility that patients may have intact neural hearing, but impaired ability to interpret sounds.

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Increase Risk of Dementia in Patients With Sudden Hearing Loss: A Population-Based Cohort Study With 7-Year Follow-Up in Taiwan

Lin, Ching-Chun; Lin, Herng-Ching; Chiu, Hung-Wen

Publicatie 01-12-2020


Objective: To investigate the prevalence and risk of subsequent dementia in subjects with sudden hearing loss during a 7-year follow-up period through comparisons with cohorts matched by sex, age group, and year of index date.Study Design: A retrospective matched-cohort study.Setting: The Longitudinal Health Insurance Database 2000 (LHID2000) in Taiwan.Patients: This study included a total of 11,148 subjects, including 1,858 in the study group and 9,290 in the comparison cohort group.Intervention(s): None.Main Outcome Measure(s): We analyzed the differences in sociodemographic characteristics and comorbidities between subjects with sudden hearing loss and the comparison cohort group. Then, we estimated the risk of dementia and also plotted the survival outcomes to evaluate differences in dementia-free survival rates between the two groups.Results: The dementia incidence rates per 1000 person-years were 20.45 and 8.15 for the subjects with sudden hearing loss and comparison cohorts, respectively. When we adjusted for the subjects’ characteristics, the hazard ratio for dementia was 1.69 (95% confidence interval CI = 1.06–2.68, p < 0.01) for subjects with sudden hearing loss compared with comparison cohorts during the follow-up period, and subjects with sudden hearing loss had lower 7-year dementia-free survival rates compared with comparison cohorts by using a log-rank test. Furthermore, male subjects with sudden hearing loss had a higher risk of dementia (adjusted hazard ratio HR = 2.11) than did the male comparison cohorts.Conclusions: This study revealed a relationship between sudden hearing loss and dementia in an Asian country. The risk of dementia was higher among patients with sudden hearing loss compared with matched cohorts during the 7-year follow-up period.

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Does Calcium Dobesilate Have Therapeutic Effect on Gentamicin-induced Cochlear Nerve Ototoxicity? An Experimental Study

Çinar, Zehra; Edizer, Deniz Tuna; Yigit, Özgür; Altunay, Zeynep Önerci; Gül, Mehmet; Atas, Ahmet

Publicatie 01-12-2020


Hypothesis: The ototoxic effects of aminoglycosides are well known. Gentamicin carries a substantial risk of hearing loss. Gentamicin is widely used to combat life-threatening infections, despite its ototoxic effects. Calcium dobesilate is a pharmacologically active agent used to treat many disorders due to its vasoprotective and antioxidant effects. We investigated the therapeutic role of calcium dobesilate against gentamicin-induced cochlear nerve ototoxicity in an animal model.Methods: Thirty-two Sprague Dawley rats were divided into four groups: Gentamicin, Gentamicin + Calcium Dobesilate, Calcium Dobesilate, and Control. Preoperative and postoperative hearing thresholds were determined using auditory brainstem response thresholds with click and 16-kHz tone-burst stimuli. Histological analysis of the tympanic bulla specimens was performed under light and transmission electron microscopy. The histological findings were subjected to semiquantitative grading, of which the results were compared between the groups.Results: Gentamicin + Calcium Dobesilate group had, on average, 27 dB better click-evoked hearing than Gentamicin group (p  0.01). Histologically examining the Control and Calcium Dobesilate groups revealed normal ultrastructural appearances. The Gentamicin group showed the most severe histological alterations including myelin destruction, total axonal degeneration, and edema. The histological evidence of damage was significantly reduced in the Gentamicin + Calcium Dobesilate group compared with the Gentamicin group.Conclusion: Adding oral calcium dobesilate to systemic gentamicin was demonstrated to exert beneficial effects on click-evoked hearing thresholds, as supported by the histological findings.

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Management of Severe Cochlear Implant Infections—35 Years Clinical Experience

Weder, Stefan; Shaul, Chanan; Wong, Amy; O’Leary, Stephen; Briggs, Robert J.

Publicatie 01-12-2020


Objective: Infectious complications occurring in cochlear implant (CI) recipients is of potentially major impact. A better understanding of severe infections in this cohort is necessary.Design: Single-center, retrospective cohort study. Level of Evidence 2B.Setting: Single-center, retrospective cohort study at a tertiary referral hospital.Participants and interventions: We included all patients who received a CI at our institution between 1983 and end of 2018 (4,622 implantations).Main Outcomes: Prevalence, incidence, risk factors, and functional outcomes in severe implant infections.Results: There was an overall prevalence of 0.65% of severe CI infections. The cumulative incidence decreased after the year 2000, with lower infection rates with newer implant models. Patients with local risk factors were more susceptible to implant infection. In most patients, delayed re-implantation was successful. Speech-perception after re-implantation was comparable to pre-revision performance.Conclusions: Modified implant design and improved surgical technique has led to a decrease in the prevalence and incidence of infected implants. In severe implant infections, active surgical and antimicrobial management is required, to achieve good long-term results.

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Aligning Hearing Aid and Cochlear Implant Improves Hearing Outcome in Bimodal Cochlear Implant Users

Holtmann, Laura Christine; Janosi, Anna; Bagus, Heike; Scholz, Tim; Lang, Stephan; Arweiler-Harbeck, Diana; Hans, Stefan

Publicatie 01-12-2020


Objective: Patients with unilateral deafness and residual hearing on the contralateral ear can benefit from a cochlear implant (CI) on one side and a hearing aid (HA) on the other. However, hearing improvement among these patients is heterogenous. Interindividual differences in bimodal benefit may be caused by a mismatch of CI and HA. The aim of this study was to clinically apply a HA fitting strategy and to evaluate hearing outcome with and without a dedicated bimodal fitting formula.Study Design: Prospective non-randomized study.Setting: Tertiary referral center.Patients: Twelve patients using a CI processor and a conventional HA were enrolled. Before and after the new HA had been adjusted to the patient and linked to the CI, pure-tone audiometry and localization tests were performed. Speech perception was determined in quiet and noise. Tests were repeated after 6 and 12 weeks. To evaluate the subjective listening comfort two questionnaires (Oldenburg Inventory and HISQUI19) were assessed.Intervention: Therapeutic.Results Main outcome measure: Word recognition in quiet, sentence recognition in noise. Speech perception in noise improved significantly: directed suppression of noise helped to segregate the target speech signal from a mixture of sounds or competing speakers. Evaluation of the questionnaires revealed a positive subjective hearing experience compared with patients’ initial settings of the devices.Conclusion: By linking CI and HA hearing and speech perception can be improved. However, good counselling at the outset is essential to obtain enhanced outcome.

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Bimodal Stimulation in Children With Bilateral Profound Sensorineural Hearing Loss: A Suitable Intervention Model for Children at the Early Developmental Stage

Chen, Ying; Huang, Meiping; Li, Bei; Wang, Zhaoyan; Zhang, Zhihua; Jia, Huan; Hu, Lingxiang; Cai, Yun; Li, Yun; Huang, Zhiwu; Wu, Hao

Publicatie 01-12-2020


Objective: To evaluate the auditory and speech benefit of bimodal stimulation for prelingual deafened cochlear implantation recipients.Study Design: Retrospective and comparative study.Setting: Tertiary referral center.Patients: Fifty-six children with bilateral prelingual profound sensorineural hearing loss were enrolled, including 28 consecutive children with unilateral cochlear implantation (CI group), and 28 consecutive children with bimodal stimulation (BI group) who used an additional hearing aid (HA) in the contralateral ear.Main Outcome Measures: Hearing assessments included the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and Categories of Auditory Performance (CAP). Speech evaluations included the Meaningful Use of Speech Scale (MUSS), and Speech Intelligibility Rating (SIR). These measurements were evaluated at the first mapping of cochlear implants and 0.5, 1, 3, 6, 12, 18, 24 months after. Data were analyzed by repeated measures analysis.Results: The mean ages of BI and CI groups were similar (17.6 ± 6.87 vs 19.0 ± 8.10 months, p = 0.497). The initial scores for hearing and speech assessments showed no differences between the two groups, apart from IT-MAIS (2.46 ± 0.631 in BI group vs 0.50 ± 0.279 in CI group, p = 0.004). The auditory and speech development over time were different in the two groups as seen in IT-MAIS (p < 0.001), CAP (p = 0.029), MUSS (p < 0.001), and SIR (p < 0.001). A continuing but stable difference was observed in CAP, MUSS, and SIR at 3, 18, and 12 months after the first mapping, respectively. In addition, the BI group had better IT-MAIS scores at 3 and 6 months compared with the CI group; however, the difference was not significant after 12 months.Conclusion: Bimodal stimulation is beneficial for prelingually deafened CI recipients who have minimal contralateral residual hearing when bilateral CIs are not available. Hearing aid use in the contralateral ear might be recommended for children after unilateral cochlear implantation to facilitate the development of auditory and speech skills.

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Assessment of Angular Insertion-Depth of Bilateral Cochlear Implants Using Plain X-ray Scans

Sokolov, Meirav; Zavdy, Ofir; Raveh, Eyal; Ulanovski, David; Attias, Yoseph; Hilly, Ohad

Publicatie 01-12-2020


Objective: To evaluate in cochlear implant patients, the feasibility and reliability of angular depth of insertion (aDOI) measurements using plain x-ray scans.Study Design: Retrospective study where three observers independently evaluated and compared intraoperative anterior-posterior and oblique x-ray scans.Setting: A tertiary pediatric medical center.Patients: Included were 50 children (100 ears) who underwent bilateral simultaneous cochlear implantation during 2008 to 2015.Main Outcome Measures: Inter-rater agreement of aDOI measured in plain x-ray scans; effect of head position on measured aDOI; and symmetry of aDOI between patients’ ears in bilateral simultaneous cochlear implantations.Results: Differences in the average aDOI measurements among the three observers ranged between 2 and 7 degrees. There was high inter-rater agreement (R = 0.99, p < 0.01) among all observers, and strong correlations between each pair of observers (0.92–0.99). Head rotation of 45 degrees (between the two views) resulted in a median difference in aDOI of 14 degrees, with excellent correlation among the observers. The rate of asymmetry was high, with a median difference of 39 degrees and up to 220 degrees between ears.Conclusions: Assessment of aDOI using intraoperative plain x-rays is efficient and reliable. The effect of head positioning on measurement is small. Further studies are needed to evaluate the effect of aDOI and insertion symmetry on functional outcomes.

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When Opposites Attract: Pediatric Cochlear Implantation in the Setting of Cerebrospinal Fluid Shunts

Selleck, Anne Morgan; Park, Lisa R.; Brown, Kevin D.

Publicatie 01-12-2020


Objective: The Food and Drug Administration (FDA) has recently raised concern regarding the safety of cochlear implantation in the setting of programmable cerebrospinal fluid shunts. The purpose of this study was to evaluate the outcomes and complications of cochlear implantation in children who have a cerebrospinal fluid shunt.Study Design: Retrospective chart review.Setting: Tertiary academic referral center.Patients: Twenty pediatric subjects with a cerebrospinal fluid shunt and cochlear implant (CI).Intervention: Cochlear implantation in the setting of a cerebrospinal fluid shunt.Main Outcome Measures: Primary outcome measures included descriptive data regarding age at implantation, etiology of hearing loss, medical management, complications, and speech perception outcomes.Results: The average age of CI candidacy was 30 months with an average 21.5 months delay to implantation. In 45% of cases the laterality of the shunt determined the ear to be implanted. Three of the subjects required a surgical intervention on the shunt before cochlear implantation. Three subjects had a concurrently programmable shunt and activated CI. Two of the three subjects had no complications as a result of the two devices; however, the third subject had significant interactions requiring multiple revision surgeries. For those with the cognitive ability to perform open set, recoded speech perception, the average postoperative Consonant Nucleus Consonant word score in the best aided condition was 65.2% (n = 5).Conclusions: Children with a cerebrospinal fluid shunt are viable candidates for cochlear implantation, although they often require additional procedures and considerations before and after implantation.

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Audiologic Outcomes of Cochlear Implantation in Cochlear Malformations: A Comparative Analysis of Lateral Wall and Perimodiolar Electrode Arrays

Schwartz, Nofrat; Brown, Kevin D.; Park, Lisa R.

Publicatie 01-12-2020


Objective: Cochlear implantation in children with inner ear malformations has been shown to be beneficial. The aims of this study are to evaluate open set word recognition outcomes among children with cochlear implants who have cochlear malformations, and to further assess if either the lateral wall (LW) or perimodiolar (PM) electrode arrays confer any performance outcome advantages.Study Design: Retrospective case series.Setting: Tertiary referral center.Patients: Pediatric cochlear implant recipients with cochlear malformations who were implanted at our institution within the last 10 years and had speech perception scores were eligible for inclusion in the study. Potential participants were excluded if they had less than 1 year of listening experience with the cochlear implant or suspected cochlear nerve deficiency.Intervention: None.Main Outcome Measure: Most recent consonant-nucleus-consonant word score.Results: ANOVA analysis demonstrated that the type of cochlear malformation was significantly associated with speech perception outcome (p = 0.006). Those with IP2 malformations had significantly better word recognition outcomes than the remaining cochlear malformations. Array type (LW or PM) was not associated with better word recognition outcomes in long-term follow-up of patients with IP2 malformations (p = 0.13).Conclusions: In children who have cochlear malformations, cochlear implantation results in varying word recognition outcomes based on the type of malformation. While the participants in this study demonstrated postoperative open set word recognition skills, those with IP2 malformations demonstrated the most benefit. Electrode type was not found to significantly impact outcomes in this cohort.

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Curvilinear Multiplanar Reconstruction to Predict Useful Length and Diameter of Cochlear Lumen for Cochlear Implantation

Guigou, Caroline; Schein, Antoine; Trouilloud, Pierre; Lalande, Alain; Hussain, Raabid; Bozorg Grayeli, Alexis

Publicatie 01-12-2020


Objective: Evaluate the useful length and the diameter of the cochlear lumen (CL) using routine imaging before cochlear implantation to study inter-individual variability and its impact on the insertion depth of the electrode carrier (EC).Study design: Prospective cross-sectional study.Setting: Tertiary referral center.Patients: Thirty-one preoperative and postimplantation temporal bone CT scans were analyzed by two investigators.Intervention: Images were analyzed via orthogonal multiplanar reconstruction (Osirix) to measure the lengths of the entire CL and the basal turn. By means of curvilinear reconstruction, the CL was unfolded and the diameters of the CL and of the EC were measured every 2 mm from the round window (RW) to the apex.Results: Very high-inter individual variability was found for the length of the basal turn (RSD > 1000%), the entire CL length (RSD > 800%), and the CL diameter at the RW (RSD > 600%). CL diameter was not correlated to the CL length. The inserted EC/total visible CL length ratio was 1.0 ± 0.12. Reliability of the measures was acceptable for the CL length and the diameter at 16 mm from the RW (Crohnbachs alpha > 0.7, n = 31).Conclusion: CL length and diameter can be directly measured in a reliable manner by commercially available tools. These parameters potentially influence the EC insertion and should be assessed before cochlear implant surgery.

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The Utility of Numeric Grading Scales of Middle Ear Risk in Predicting Ossiculoplasty Hearing Outcomes

Judd, Ryan T.; Imbery, Terence E.; Gluth, Michael B.

Publicatie 01-12-2020


Objective: To assess the usefulness of numeric grading scales of middle ear risk in predicting ossiculoplasty hearing outcomes.Study Design: Retrospective review.Setting: Tertiary care, academic medical center.Patients: Adults and children undergoing ossiculoplasty between May 2013 and May 2019 including: synthetic ossicular replacement prosthesis, autograft interposition, bone cement repair, and mobilization of lateral chain fixation.Intervention: Cases were scored via middle ear risk index (MERI), surgical prosthetic infection tissue eustachian tube (SPITE) method, and ossiculoplasty outcome scoring parameter (OOPS) scale. Preoperative and postoperative hearing outcomes were recorded.Main Outcome Measure: Statistical correlation between risk score and postoperative pure-tone average air-bone gap (PTA-ABG).Results: The 179 included cases had average pre and postoperative PTA-ABGs of 30.3dB (standard deviation SD 12.7) and 20.3dB (SD 11.1), respectively. Mean MERI, SPITE, and OOPS scores were 4.5 (SD 2.3), 2.8 (SD 1.7), and 3.1 (SD 1.8), respectively. Statistically significant correlations with hearing outcome were noted for all three methods (MERI r = 0.22, p = 0.003; OOPS r = 0.19, p = 0.012; SPITE r = 0.27, p  30dB) outcomes; only low SPITE scores predicted excellent (PTA-ABG < 10dB) outcomes (odds ratio OR 0.74 Confidence Interval: 0.57 – 0.97, p = 0.032).Conclusions: Significant weak correlations between each middle ear risk score and hearing outcomes were encountered. Although only the SPITE method predicted postoperative PTA-ABG, it was not overwhelmingly superior. Current grading scale selection may be justified by familiarity or ease of use.

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The Treatment of Cholesteatomas Involving the Antrum and Mastoid Using Transcanal Underwater Endoscopic Ear Surgery

Chen, Yichao; Hu, Junjiao; Liu, Wei; Wang, Qin; Li, Yajun; Peng, Anquan; Zhang, Zhiwen

Publicatie 01-12-2020


Objective: The aim of this study was to evaluate the feasibility and safety of transcanal underwater endoscopic bone resection (TUEBR) of the external auditory canal (EAC) for the management of cholesteatoma involving the antrum and mastoid.Study Design: Retrospective case review.Setting: Tertiary referral center.Patients: Pediatric and adult patients with primary cholesteatoma extending to the antrum and mastoid who underwent transcanal endoscopic ear surgery (TEES) with TUEBR between March 2016 and June 2017.Intervention: A rigid 2.7 mm diameter, 18 cm length Hopkins-rod telescope with an endoscopic sheath was inserted in the EAC and continuously perfused with saline during the dissection. TUEBR was performed to expose extensive cholesteatoma by using a high speed drill with curved burrs and a protected shaft. Next, removal of visible disease, reconstruction of the resected EAC, ossiculoplasty, and tympanoplasty were accomplished with TEES.Results: There were no intra- or postoperative severe complications such as facial palsy and inner ear injury except one patient suffering from secondary labyrinthitis. There was a negative linear relationship (r = –0.909) between the procedure time and procedure number of TUEBR. There was a weak relationship (r = 0.224) between the procedure time of TUEBR and the degree of the extension of cholesteatoma into the antrum and mastoid. There were two cases with residual cholesteatoma at 12 and 22 months follow-up postoperatively.Conclusion: TUEBR is a safe and efficient technique for the resection of EAC bone and transcanal exposure of extensive cholesteatoma that would otherwise require mastoid dissection.

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Bilateral Sequential Spontaneous Otogenic Pneumocephalus, Lessons in Pathophysiology, and Management of a Rare Case

Shapiro, Scott B.; Lipschitz, Noga; Tumlin, Parker; Krueger, Bryan; Forbes, Jonathan; Samy, Ravi N.

Publicatie 01-12-2020


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 patients 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.

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Predicting Recidivism for Acquired Cholesteatoma: Evaluation of a Current Staging System

Angeli, Simon; Shahal, David; Brown, Clifford Scott; Herman, Bjorn

Publicatie 01-12-2020


Objective: Assess the utility and prognostic capabilities of the European Academy of Otology and Neurotology (EAONO) and Japanese Otological Society (JOS) cholesteatoma classification system, specifically for retraction pocket cholesteatoma.Study Design: Retrospective.Setting: Tertiary referral hospital.Patients: Adults and children with retraction pocket cholesteatoma.Interventions: Primary and planned second-look tympanoplasty with mastoidectomy.Main Outcome Measures: Incidence of recurrent or residual cholesteatoma at planned second-look surgery. Independent variables of age, gender, size of canal defect, and mucosa status were assessed. Additionally, the cholesteatoma classification, stage, and extent according to the EAONO/JOS system were recorded during the primary surgery.Results: A total of 125 cases were included. Twelve (9.6%) cases had recidivism over an average time of 7.5 months: the recurrence rate was 4% (n = 5), residual rate was 5% (n = 6), and one patient had both recurrent and residual disease (0.8%). Residual cholesteatoma occurred more frequently in children (p = 0.04, RR = 7.9 1.0, 63.6). Supratubal recess (S1) disease was associated with both recurrent cholesteatoma (p = 0.04, RR = 5.9 1.3, 27.2) and recidivism (p = 0.01, RR = 4.2 1.5, 11.9). Larger canal defects also showed an association with residual disease (p = 0.017).Conclusion: Younger patients and those with large ear canal defects tend to have residual disease at second-look surgery. Supratubal recess disease is also associated with recurrence. Despite the utility of the EAONO/JOS classification and staging system for cholesteatoma description and type, the prognostic value remains uncertain.

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Postoperative Course of Pars Flaccida Cholesteatoma Patients With Habitual Sniffing

Endo, Shiori; Mizuta, Kunihiro; Takahashi, Goro; Arai, Maki; Nakanishi, Hiroshi; Takizawa, Yoshinori; Hosokawa, Kumiko; Yamatodani, Takashi; Misawa, Kiyoshi; Hosokawa, Seiji; Mineta, Hiroyuki

Publicatie 01-12-2020


Objective: To investigate the effects of habitual sniffing on the postoperative course of pars flaccida cholesteatoma.Study Design: Retrospective case series study.Setting: University hospital.Patients: Forty-nine patients (53 ears) with pars flaccida cholesteatoma and history of habitual sniffing before the initial operation.Interventions: Patients were divided into a “sniffing cessation group” characterized by sniffing cessation and a “continual sniffing group” characterized by continuation of sniffing despite instructions for conscious cessation.Main Outcome Measures: Hearing level, tympanic membrane findings, tympanograms, mastoid cell development before the operation, and pneumatization 1 year postoperatively.Results: The sniffing cessation and continual sniffing groups comprised 35 patients (38 ears) and 14 patients (15 ears), respectively. The average postoperative hearing was slightly better in the continual sniffing group. In the sniffing cessation group, retractions were evident in significantly fewer cases. Retractions were observed in all continual sniffing group cases, with a high percentage of severe retractions, wherein the bottom was not visible. Type A tympanogram was predominant in the sniffing cessation group. Mastoid cell development was not significantly different between the two groups. Satisfactory pneumatization was significantly more common in the sniffing cessation group (Fishers exact test, ∗∗p < 0.005).Conclusion: Conscious cessation of the sniffing habit could reduce the risk of postoperative retraction and improve pneumatization in patients with pars flaccida cholesteatoma. The presence or absence of the sniffing habit after surgery is a defining factor in postoperative prognosis (retraction, recurrence), and may be a determinant for decisions regarding surgical approach.

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Feasibility and Efficacy of Vibrant Soundbridge Short Process Coupler in Patients With Aural Atresia

Alzhrani, Farid; Halawani, Roa; Yousef, Medhat

Publicatie 01-12-2020


Objectives: Different options are available for hearing restoration in patients with aural atresia. Middle ear implantation with the Vibrant Soundbridge (VSB) has been used successfully in many patients with aural atresia with conductive or mixed hearing loss. The aim of the current study was to assess the safety and the efficiency of VSBs coupled to the short process of the incus in patients with aural atresia with conductive or mixed hearing loss.Methods: The study was a retrospective chart review conducted at a tertiary university hospital. Audiological and surgical data from six ears with aural atresia implanted with VSBs coupled to the short process of the incus were analyzed. The audiological results were compared with previously reported results obtained after the utilization of different coupling techniques.Results: All six atretic ears were successfully implanted using the short process coupler, and there were no major complications. Postoperative bone conduction thresholds did not differ substantially from preoperative thresholds. The respective mean preoperative pure-tone average 4, speech reception threshold, and word recognition score values were 56.7 dB, 61.7 dB, and 44%. Postoperative pure-tone average 4 thresholds (25 ± 3.5 dB) and speech reception thresholds (20.8 ± 8 dB) were significantly improved. The maximum postoperative word recognition score achieved was 100%. The audiological outcomes obtained were similar to previously reported outcomes after classic stapes placement.Conclusion: Coupling of the floating mass transducer to the short process of the incus in patients with aural atresia resulted in significant audiological improvements that were comparable to previously reported improvements after placement via other methods.

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Congenital Hearing Loss Is Associated With a High Incidence of Central Nervous System Abnormalities

Sayyid, Zahra N.; Boldt, Brian; Mokhtari, Tara E.; Schoppy, Krista; Yeom, Kristen W.; Cheng, Alan G.

Publicatie 01-12-2020


Objective(s): To assess the incidence of central nervous system abnormalities in pediatric subjects with sensorineural hearing loss (SNHL).Methods: One hundred forty-three pediatric subjects evaluated for SNHL at a single academic center from 2007 to 2014 were included and divided into eight diagnosis groups based on etiology of SNHL. One hundred forty-three age- and gender-matched control subjects with no known brain-related pathology or history of hearing loss were included as healthy controls for comparison. Two neuroradiologists independently evaluated magnetic resonance imaging (MRI) and computed tomography (CT) scans for each subject. Comparison of abnormal cerebral development was performed using an ordinal logistic regression model. Concordance between CT and MRI of the temporal bone was assessed using the kappa statistic.Results: The etiologies of hearing loss in our cohort were 37.8% genetic, 12.6% infectious, 1.4% ototoxin-induced, and 48.3% idiopathic. Brain MRI revealed cerebral developmental abnormalities in defined regions in >30% of the SNHL cohort, significantly more than in normal-hearing pediatric controls. The Sylvian fissure, Virchow-Robin spaces, and lateral ventricles were most commonly affected. In the temporal bone, the percentage of subjects with concordant findings on CT and MRI was ≥92% across all anatomical structures.Conclusion: MRI revealed a high incidence of intracranial abnormalities, suggestive of aberrant development of auditory and nonauditory neural structures associated with SNHL. CT and MRI share a high degree of concordance in detecting temporal bone anomalies. Inclusion of MRI as part of the workup of congenital SNHL may facilitate the detection of developmental anomalies of the brain associated with SNHL.

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Posttraumatic Cranial Suture Diastasis in Pediatric Temporal Bone Fractures

Reeve, Nathaniel H.; Kahane, Jacob B.; Miller, Jordan; Cross, Chad; Young, Allen; Ng, Matthew

Publicatie 01-12-2020


Objective: Only a handful of case reports exist describing posttraumatic sutural diastasis in the calvarium and none report concurrent temporal bone involvement. We aim to describe diastasis along the temporal bone suture lines in the setting of temporal bone trauma and to identify clinical sequelae.Study Design: Retrospective case review.Setting: Tertiary Level 1 trauma center.Patients: Forty-four patients aged 18 and younger who suffered a temporal bone fracture from 2013 to 2018 were identified. Diastasis and diastasis with displacement at the occipitomastoid, lambdoid, sphenosquamosal and petro-occipital sutures, and synchondroses were determined.Main Outcome Measures: The presence of temporal bone suture and synchondrosal diastasis following temporal bone trauma. Diastasis was defined as sutural separation of a distance greater than 1 mm in comparison to the contralateral side.Results: Using our diastasis diagnostic criteria, diastasis occurred in 41.5% of temporal bone fractures. Transverse fracture types were significantly associated with diastasis (p ≤ 0.001). Lower Glasgow Coma Scale (GCS) and loss of consciousness (LOC) were associated with the presence of diastasis with displacement and diastasis (p = 0.034 and p = 0.042, respectively). Otic capsule violation was more common in fractures with diastasis but did not reach statistical significance. There were two cases of cerebrospinal fluid otorrhea and three deaths in cases that featured diastasis.Conclusion: Our findings indicate that diastasis is a positive predictor for higher disruptive force injuries and more severe outcomes and complications. Posttraumatic temporal bone suture diastasis may represent a separate clinico-pathologic entity in addition to the usual temporal bone fracture classification types.

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Changes in Bone Conduction Implant Geometry Improve the Bone Fit in Mastoids of Children and Young Adults

Wenzel, Claudia; Schilde, Sebastian; Plontke, Stefan K.; Rahne, Torsten

Publicatie 01-12-2020


Objectives: In 2012 the first active bone conduction implant was introduced, but did not fit into the mastoids of some adults and many children. Thus, a geometry change of the transducer was proposed (BCI 602). In this study, we aimed to determine whether these changes improved the mastoid cavity fit of the implant in children and young adults.Design: We retrospectively analyzed computed tomography scans of 151 mastoids from 81 children and adolescents (age range, 5 mo to 20 yr) and 52 control mastoids from 33 adults. After three-dimensional reconstruction of the temporal bone from computed tomography, we virtually implanted the BCI 602 into the mastoids, and compared the bone fit with that of the BCI 601.Results: The BCI 602 could be virtually implanted in 100% of patients ≥12 years old, while the BCI 601 transducer could be completely embedded in the bone of only 70% of these mastoids. Moreover, virtual implantation of the BCI 602 was possible in 75% of children 3 to 5 years of age, while the BCI 601 did not fit in the mastoids of any patients under 5 years old without the use of lifts.Conclusions: Compared to the BCI 601, placement of the BCI 602 allegedly requires less bone removal. The newer BCI 602 transducer is more likely than its predecessor to be completely accommodated in the mastoid bone among all age groups and indications. Preoperative planning is still recommended to avoid exposure of delicate structures.

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"Transmastoid Labyrinthectomy for Menières Disease: Experience and Outcomes"

Bergmark, Regan W.; Semco, Robert S.; Abdul-Aziz, Dunia; Rauch, Steven D.

Publicatie 01-12-2020


Objective: To characterize presurgical symptoms and treatment history and postoperative course in patients with medically recalcitrant Menières disease undergoing transmastoid labyrinthectomy in the post-intratympanic gentamicin era.Study Design: Retrospective case series.Setting: Tertiary academic medical center.Patients: All patients who underwent transmastoid labyrinthectomy for medically recalcitrant Menières disease in 2003 to 2019 by the senior author.Interventions: Review of patients’ medical records for: preoperative history of drop attacks, gentamicin injections, endolymphatic sac decompression or vestibular neurectomy, preoperative audiograms, length of hospital stay, postoperative complications, and persistent symptoms or challenging recovery.Main Outcome Measures: Presurgical clinical history and proximal postoperative outcomes.Results: Seventy-two patients with a mean age of 56.7 (standard deviation SD 10.7) were included. All cases were unilateral. Forty-three patients (59.7%) suffered from drop attacks. Sixty-two (86.1%) had failed sufficient symptom control with gentamicin injections. The mean preoperative word recognition score was 36.4% (SD 23.7) versus 95.1% (SD 8.5) in the contralateral ear. The mean pure-tone average (PTA) of the ipsilateral ear before surgery was 65.5 dB (SD 18.0) versus 16.2 (SD 13.5) for the contralateral ear. Mean hospital stay was 2.0 days (SD 0.87 days, range of 1–5 d). Three patients (4.2%) had prolonged postoperative vertigo.Conclusions: Transmastoid labyrinthectomy at our center is performed for unilateral Menières disease, generally when intratympanic gentamicin has failed. A majority of surgical patients suffer from drop attacks preoperatively. Hospital stay is typically brief.

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The Clinical Spectrum of Dizziness in Sleep Apnea

Foster, Carol A.; Machala, Maria

Publicatie 01-12-2020


Objective: Sleep apnea has been associated with dizziness, but the clinical characteristics of this dizziness have not been fully described. This study evaluated clinical descriptions of dizziness, vestibular diagnoses, and the response to treatment of dizzy patients with sleep apnea.Patients: All subjects had a new diagnosis of sleep apnea with a confirmatory pulse oximetry or polysomnogram and received treatment for sleep apnea through our center. Fifty-two patients met these criteria, with a mean age of 55 years and a mean BMI of 31.Intervention: Continuous positive airway pressure or uvulopalatopharyngoplasty.Main Outcome Measures: Reported symptoms of dizziness and vestibular diagnoses were collected retrospectively in all patients. Nineteen patients with a complete resolution of dizziness with treatment of sleep apnea on mean follow-up of 4 years were used to define the clinical syndrome and their symptoms were compared with the remainder of the group.Results: Repeated spells of sudden momentary vertigo were common. Vestibular migraine, Menières disease, and sudden sensorineural hearing loss occurred in a much higher frequency than in the general population.Conclusions: Brief spells of nonpositional vertigo that recur throughout the day, phenotypically similar to vestibular paroxysmia, responded to treatment of sleep apnea, and could represent a new vestibular entity. The greatly elevated risk of migraine, Menières disease and sudden sensorineural hearing loss are likely due to enhanced vascular risks associated with sleep apnea. A history of snoring should be sought in all dizzy patients.

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Effect of Aging and Direction of Impulse in Suppression Head Impulse Test

Lee, Jung-Yup; Kim, Min-Beom

Publicatie 01-12-2020


Objective: To identify differences in the vestibulo-ocular reflex (VOR) gain value and the peak saccade velocity in the suppression video head impulse test paradigms according to the age of the subject and the direction of the impulse.Study Design: Retrospective chart analysis.Setting: Tertiary referral hospital.Patients/Interventions: Between October 2017 and May 2019, we enrolled subjects who had previous histories of dizziness but no dizziness over the last 1 month.Main Outcome Measure: We conducted cervical vestibular-evoked myogenic potential and caloric tests, as well as video head impulse tests. We excluded the subjects who had abnormal cervical vestibular-evoked myogenic potential results (asymmetry ratio of greater than 30%) and abnormal caloric test results (caloric paresis of greater than 25%).Results: We included 647 subjects aged 10 to 87 years. The mean VOR gain and peak saccade velocity were maintained in subjects less than 70 years old (VOR gain, 0.991 ± 0.08, peak saccade velocity, 348.47 ± 142.32). However, the decreases in VOR gain and peak saccade velocity were significant in subjects over 70 years old (VOR gain, 0.928 ± 0.09, peak saccade velocity, 315.51 ± 0.09; p < 0.001). The mean VOR gain of the rightward impulse (1.00 ± 0.09) was higher than the leftward impulse (0.96 ± 0.08, p < 0.001).Conclusions: Both the VOR gain and peak saccade velocity of suppression video head impulse test paradigms declined with increasing age over 70 years. In addition, the VOR gain of the rightward impulse was higher than the leftward impulse in the right-eye recordings.

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Aberrant AICA Injury During Translabyrinthine Approach

Bauer, Ashley M.; Angster, Kristen; Schuman, Ari D.; Thompson, Byron Gregory; Telian, Steven A.

Publicatie 01-12-2020


Objective: To define a complication of the translabyrinthine surgical approach to the posterior fossa related to a rare variant of the anterior inferior cerebellar artery (AICA) that penetrated into the petrous temporal bone.Patient: A healthy 59-year-old male with a unilateral sporadic vestibular schwannoma.Intervention: The patient elected to undergo a translabyrinthine approach for resection of a vestibular schwannoma. An aberrant loop of AICA was encountered during the temporal bone dissection within the petrous portion of the temporal bone.Outcomes: The patient suffered a presumed ischemic insult resulting in a fluctuating ipsilateral facial paresis and atypical postoperative nystagmus.Results: MRI demonstrated an ischemic lesion in the vascular distribution of the right anterior-inferior cerebellar artery, including the lateral portion of the right cerebellar hemisphere, middle cerebellar peduncle, and bordering the right cranial nerve VII nucleus. His functional recovery was excellent, essentially identical to the anticipated course in an otherwise uncomplicated surgery.Conclusions: This case highlights the irregular anatomy of the AICA as well as the importance of thorough neurological exams in the postsurgical lateral skull base patient.

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Can Dizziness Handicap Inventory and Activities-Specific Balance Confidence Scores Assess Vestibular Loss After Vestibular Schwannoma Excision?

Erbele, Isaac D.; MacDowell, Sara G.; Arriaga, Moisés A.

Publicatie 01-12-2020


Objective: Evaluate and compare the Dizziness Handicap Inventory with Activities-specific Balance Confidence scores shortly after vestibular schwannoma excision.Study Design: Retrospective database review.Setting: Tertiary care center.Patients: Adults undergoing vestibular schwannoma excision between January 2015 and December 2019.Intervention: Diagnostic, therapeutic, and rehabilitative.Main Outcome Measures: Postoperative change in Dizziness Handicap Inventory scores and Activities-specific Balance Confidence scores 2 to 3 weeks after surgical intervention in relation to preoperative vestibular testing.Results: A total of 49 patients met inclusion criteria. The average change in the Dizziness Handicap Inventory was 6 (p = 0.07, 95% CI 0–13). This was weakly correlated to preoperative caloric testing values (r = −0.31, p = 0.03), but not cervical vestibular evoked myogenic potentials (cVEMP) values (r = −0.17, p = 0.23). The average change in Activities-specific Balance Confidence was −10% (p = 0.007, 95% CI −3 to −17%). This change was moderately correlated with preoperative caloric values (r = 0.42, p = 0.006), but it was not correlated with cVEMP (r = 0.07, p = 0.66).Conclusions: In vestibular schwannoma patients, factors other than preoperative vestibular function likely affect postoperative Dizziness Handicap Inventory and Activities-specific Balance Confidence scores. The change in Activities-specific Balance Confidence was slightly more consistent with expected physiological vestibular loss, and it represents another tool in a multidisciplinary vestibular evaluation of the postoperative patient.

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"Intraoperative Electrocochleography of Posterior Fossa Tumors Producing Menières Syndrome"

Varadarajan, Varun V.; Riggs, Willian J.; Hiss, Meghan M.; Moberly, Aaron C.; Dodson, Edward E.; Adunka, Oliver F.; Mattingly, Jameson K.

Publicatie 01-12-2020


Objectives: Intraoperative electrocochleography (ECochG) has provided insight regarding inner ear pathophysiology during neurotologic procedures. In this study, intraoperative ECochG findings are reported in patients who presented with episodic aural and vestibular symptoms during resection of posterior fossa neoplasms.Patients: Three patients with episodic vertigo who underwent resection of posterior fossa tumors.Intervention: Intraoperative ECochG was performed before and after tumor resection with the active electrode at the round window. Acoustic stimuli consisted of click and tone bursts presented in alternating polarity.Main Outcome Measure: ECochG responses including summation potential (SP), action potential (AP), and SP:AP ratio values to evaluate for endolymphatic hydrops.Results: All subjects presented with asymmetric sensorineural hearing loss (SNHL), episodic vertigo, and tinnitus. Subject 1 was a 63-year-old woman who underwent left translabyrinthine excision of an endolymphatic sac (ELS) tumor and demonstrated no measurable responses until fenestration of the lateral semicircular canal, suggesting severe hydrops relieved by labyrinthotomy. Subject 2 was a 44-year-old woman who underwent right ELS tumor resection and exhibited an elevated SP:AP ratio. Subject 3 was a 55-year-old woman who underwent right retrolabyrinthine resection of a meningioma and exhibited robust responses without hydrops.Conclusions: Endolymphatic hydrops secondary to mechanical obstruction by a posterior fossa neoplasm may be demonstrated using intraoperative ECochG. Immediate improvement of hydrops may not be demonstrated after tumor resection.

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Increasing Frailty, Not Increasing Age, Results in Increased Length of Stay Following Vestibular Schwannoma Surgery

Casazza, Geoffrey C.; McIntyre, Matthew K.; Gurgel, Richard K.; McCrary, Hilary C.; Shelton, Clough; Couldwell, William T.; Bowers, Christian A.

Publicatie 01-12-2020


Objective: Understand the frailty of vestibular schwannoma surgical patients and how frailty impacts clinical course.Study Design: Retrospective Cohort.Setting: Single-tertiary academic hospital.Patients: All patients undergoing vestibular schwannoma surgery.Intervention: The modified frailty index (mFI) was calculated for all patients undergoing surgery for vestibular schwannoma between 2011 and 2018. Patient demographics and medical history, perioperative course, and postoperative complications were obtained from the medical record.Main Outcome Measures: The primary endpoint was hospital length of stay (LOS). Secondary endpoint was postoperative complications. Basic statistical analysis was performed including multivariate linear regressions to determine independent predictors of LOS.Results: There were 218 patients included and the mean age was 48.1 ± 0.9 (range 12–77). One-hundred ten patients were male (50.5%). The mean ICU LOS was 1.6 ± 0.1 days while mean total hospital LOS was 4.3 ± 0.2. There were 145 patients (66.5%) who were robust (nonfrail) with an mFI of 0, while 73 (33.5%) had an mFI of ≥1. Frailty (mFI≥2) was associated with longer hospital LOS compared with the prefrail (p = 0.0014) and robust (p = 0.0004) groups, but was not associated with increased complications (OR = 1.3; 95% CI: 0.5–3.7; p = 0.5925) or ICU LOS (p > 0.05). In multivariate analysis, increased mFI, and NOT increased age, was an independent risk factor for increased hospital LOS (p = 0.027).Conclusion: Increasing frailty, and not increasing age, is an independent risk factor for longer hospital LOS, but not for increased postoperative complications. Patients’ frailty status may be useful preoperatively in counselling patients about postoperative expectations and frail vestibular schwannoma patients may require increased health spending costs given their increased hospital LOS.

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Endoscopic Decompression of the Labyrinthine Segment of the Facial Nerve

Cornu, Nicolas; Marchioni, Daniele; Eliezer, Michael; Verillaud, Benjamin; Hautefort, Charlotte; Green, Andrew; Marc, Morgane; Bresson, Damien; Froelich, Sebastien; Herman, Philippe; Kania, Romain

Publicatie 01-12-2020


Objective: To describe the outcome and feasibility of an exclusive endoscopic transcanal transpromontorial approach (ETTA) for decompression of the labyrinthine segment of the facial nerve (LSFN).Patient: A 60-year-old man with a left-sided transverse fracture of temporal bone involving the LSFN, resulting in a grade VI House–Brackmann (HB) facial palsy, associated with ipsilateral total sensorineural hearing loss.Intervention: Surgical decompression of the LSFN by ETTA.Main Outcome Measure: The patient underwent ETTA which allowed complete exposure and decompression of the LSFN.Results: One year postoperatively, the patient had recovered with House–Brackmann grade II facial function.Conclusion: ETTA can be considered a valuable and appropriate technique for posttraumatic decompression of LSFN, associated with unilateral total sensorineural hearing loss. The procedure resulted in significant facial nerve function improvement. ETTA should be considered both a scarless, mastoid conserving and less invasive surgical technique for posttraumatic LSFN decompression associated with pre-existing cochlear impairment.

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MITF Is Mutated in Type 1 Waardenburg Syndrome With Unusual Phenotype

Li, Wu; Feng, Yong; Chen, Hongsheng; He, Chufeng; Mei, Lingyun; Liu, Xue Zhong; Men, Meichao

Publicatie 01-12-2020


Background: Waardenburg syndrome (WS) is a rare disorder characterized by varying combinations of sensorineural hearing loss and abnormal pigmentation of the hair and skin. WS is classified into four subtypes (WS1–WS4) based on additional symptoms. Dystopia canthorum is a hallmark of WS type 1. There are two genes linked to WS type 1, including PAX3 and EDNRB.Objective: This study aimed to investigate the genetic etiology of WS type 1 in a pair of twins from China with profound hearing loss, blond hair and eyebrows, dystopia canthorum, and brown irides.Methods: The target capture sequencing and Whole-exome sequencing were performed to detect mutations in WS-related genes.Results: A novel de novo frameshift mutation, p.L341Rfs∗18 in MITF was identified in the twins. Hearing thresholds showed substantial improvements following cochlear implantation with a pure-tone average of 30 dB in free-field conditions.Conclusions: The study showed the new genotype–phenotype correlations of MITF to WS type 1. Further molecular analysis is necessary to reappraise the current classification on WS.

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Absence of Endolymphatic Sac Ion Transport Proteins in Large Vestibular Aqueduct Syndrome—A Human Temporal Bone Study

Eckhard, Andreas H.; Bächinger, David; Nadol, Joseph B. Jr.

Publicatie 01-12-2020


Hypothesis: Epithelial ion transport pathologies of the endolymphatic sac (ES) are associated with large vestibular aqueduct syndrome (LVAS).Background: LVAS is defined by the pathognomonic features of a widened bony vestibular aqueduct (VA) and an enlarged ES. The underlying cause of its associated cochleovestibular symptoms remains elusive. Disturbances in epithelial ion transport in the enlarged ES, affecting inner ear fluid regulation, were proposed as a possible pathophysiology. However, although respective epithelial ion transport pathologies have been demonstrated in the enlarged ES from transgenic LVAS mouse models, these pathologies have not been investigated in human LVAS cases.Methods: Histological and immunohistochemical analysis of the enlarged ES epithelium in postmortem temporal bones from two individuals with a clinical diagnosis of LVAS.Results: The enlarged ES epithelium demonstrated an overall atypical epithelial differentiation and a lack of the immunolocalization of signature ion transport proteins. Notably, in both cases, a rudimentary branch of the ES with a typically differentiated ES epithelium was present.Conclusions: The described cellular and molecular pathologies of the enlarged ES in humans provide evidence of epithelial transport pathology as one potential cause of cochleovestibular symptoms in LVAS. The present findings also emphasize the clinical relevance of already established LVAS mouse models.

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Pentoxifylline and Tocopherol in the Management of Temporal Bone Osteoradionecrosis: A Case Series

Lovin, Benjamin D.; Choi, Jonathan S.; Lindquist, Nathan R.; Phan, Jack; Gidley, Paul W.; Nader, Marc-Elie

Publicatie 01-12-2020


Objective: Temporal bone osteoradionecrosis (TBORN) is a rare, chronic complication of head and neck radiation. Initial treatment consists of conservative management, with surgical resection of necrotic bone indicated for cases of severe, symptomatic, or progressive disease. Pentoxifylline-tocopherol (PENTO) has demonstrated usefulness for osteoradionecrosis of other head and neck subsites. Herein, we report five TBORN cases utilizing this protocol.Study Design: Retrospective case series.Setting: Tertiary referral center.Patients: This case series describes five TBORN cases in which the PENTO protocol was used in conjunction with conservative management. All patients were women and average age was 61 ± 8 years.Intervention: All patients received a daily dose of 800 mg of pentoxifylline and 1 g of tocopherol. Four of the five patients received systemic and/or ototopical antibiotics as an antimicrobial regimen before and/or during the PENTO protocol.Main Outcome Measures: Details regarding the total duration of protocol, improvement in symptoms, exposed bone and radiographic changes, and duration until first improvement of exposed bone were collected retrospectively.Results: The average duration of PENTO protocol was 302 ± 166 days. Four of the five (80%) patients demonstrated a decrease in exposed ear canal bone. Three of the five (60%) patients had stable or improvement in otologic symptoms of TBORN. One patient progressed to diffuse TBORN. The average duration until first improvement in exposed bone was 193 ± 137 days.Conclusions: The PENTO protocol may be a useful adjunct to conservative measures in the management of localized TBORN. We recommend trialing the protocol for at least 12 months.

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A Qualitative Appraisal of Neurotology Fellowship Websites

Hakimi, Amir A.; Loanzon, Roberto S.; Azhdam, Ariel M.; Liu, Yuan; Simental, Alfred

Publicatie 01-12-2020


Objective: To assess the accessibility and breadth of online information regarding neurotology fellowship programs by appraising individual fellowship websites as well as two popular online databases.Methods: The American Neurotology Society Program Information Page (ANSPIP), the American Medical Associations Fellowship and Residency Electronic Interactive Database (FREIDA), and three online search engines (Google, Yahoo, Bing) were assessed for accessibility to individual fellowship websites. Each programs ANSPIP data sheet and fellowship-specific website were then evaluated for the presence of 18 characteristics of interest to the neurotology fellowship applicant.Results: All three search engines yielded 23 (96%) Accreditation Council for Graduate Medical Education (ACGME)-accredited neurotology fellowship websites. Searching “Neurotology fellowships” in Google, Yahoo, and Bing required exploring up to nine pages of search results to identify all of the ACGME-accredited program-specific websites. Direct links to program websites were found on 12 (55%) and 3 (13%) of the ANSPIP and FREIDA fellowship pages, respectively. Of the 18 characteristics of interest, the ANSPIP data sheet and individual program websites on average delineated 8.0 and 7.8 characteristics, respectively. Ninety-one percent of fellowship websites included program coordinator information, general program description, and breadth of surgical exposure. In contrast, work/life balance, postfellowship placement, and current fellow(s) were least commonly displayed (4%, 9%, 13% respectively). One-hundred percent of ANSPIP data sheets included program coordinator information, number of fellows, and affiliated hospital(s), whereas none of the pages included selection criteria, application requirements, description of location, or work/life balance.Conclusion: Although most neurotology fellowship programs have websites or ANSPIP data sheets, many of them lack information that has been previously demonstrated to be valued by applicants. Furthermore, incongruence of information between these sources may lead to confusion, applicant stress, and reflect poorly on fellowship programs. Perhaps a standardized list of ACGME-required data points to be posted on websites would facilitate the application process.

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Facial Paralysis in a Babylonian Plaque

Fulco, William J.; Levy, Sabrina L.; Canalis, Rinaldo F.

Publicatie 01-12-2020


Hypothesis: A terra cotta plaque LMU 2551 from the Neo-Babylonian period (c.629-539 BCE), housed in the museum of the Archaeology Center at Loyola Marymount University, Los Angeles, is a representation of right peripheral facial paralysis.Background: Ancient representations of pathology are rare and often difficult to identify. This is particularly true of Assyrian-Babylonian cultures where, despite numerous surviving medical texts, artistic examples of disease are almost non-existent.Methods: Precise caliper measurements and archaeological analysis of LMU 2551 were used to confirm the authors’ hypothesis.Results: The facial distortions portrayed in LMU 2551 are not accidental. Measurements show a pronounced asymmetry of the lower face where the length from the mid-philtrum to the oral commissure and from the lateral edge of the ala nasi to the mid-ipsilateral nasolabial fold are twice as long in the left than in the right side. The left eye is closed, whereas the right is widely open.Conclusion: The described plaque is among the oldest representations of facial paralysis on record. It correlates with contemporary Babylonian texts describing neurological disorders but its function is unknown.

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Endoscopic Tympanoplasty in the Treatment of Chronic Otitis Media

Cavaliere, Matteo; De Luca, Pietro; Scarpa, Alfonso; Cuofano, Rossella; Cassandro, Claudia; Panetti, Maria; Panetti, Giuseppe

Publicatie 01-12-2020


No abstract available

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Authors’ Reply to the Letter to the Editor: “Endoscopic Tympanoplasty in the Treatment of Chronic Otitis Media” a Comment to the Article: “The Management of Tympanic Membrane Perforation with Endoscopic Type I Tympanoplasty”

Bianconi, Luca; Di Maro, Flavia; Gazzini, Luca; De Rossi, Stefano; Sacchetto, Luca; Carner, Marco; Marchioni, Daniele

Publicatie 01-12-2020


No abstract available

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Letter to the Editor Regarding “Improving Microscopic Imaging in Otology and Neurotology”

Kram, Yoseph A.; Chen, Brian

Publicatie 01-12-2020


No abstract available

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Response to Letter to the Editor Regarding “Improving Microscopic Imaging in Otology and Neurotology”

Saadi, Robert; Liaw, Jeffrey; Baker, Aaron; Isildak, Huseyin

Publicatie 01-12-2020


No abstract available

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"Otitis Media Complicated by Lateral Sinus Thrombosis and Citellis Abscess"

Catalano, Dominic J.; Puccinelli, Cassandra L.; Lane, John I.; Carlson, Matthew L.

Publicatie 01-12-2020


No abstract available

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Concurrent Spindle Cell Hemangioma and Schwannoma of the Jugular Foramina

Lovin, Benjamin D.; Appelbaum, Eric N.; Patel, Akash J.; Sweeney, Alex D.

Publicatie 01-12-2020


No abstract available

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Temporal Bone Histopathology Case of the Month: Small Cell Neuroendocrine Carcinoma of the External Auditory Canal and Middle Ear

Orozco, Francis Reyes; Choi, Janet S.; Correa, Adrian J.; Voelker, Courtney C. J.

Publicatie 01-12-2020


No abstract available

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Trans-canal Endoscopic Repair of Cerebrospinal Fluid (CSF) Leak Following Vestibular Schwannoma Resection

Manzoor, Nauman F.; Nassiri, Ashley M.; Rivas, Alejandro

Publicatie 01-12-2020


Video Summary:http://links.lww.com/MAO/B51

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Introduction to the Special Issue, “Proceedings of the 8th Quadrennial International Conference on Vestibular Schwannoma and Other Cerebellopontine Angle Tumors”

Carlson, Matthew L.; Link, Michael J.; Driscoll, Colin L. W.; Haynes, David S.

Publicatie 01-12-2020


No abstract available

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Natural History of Facial Weakness Following Surgery of the Cerebellopontine Angle: A Tertiary Care Cohort

Luryi, Alexander L.; Babu, Seilesh; Michaelides, Elias M.; Bojrab, Dennis I.; Kveton, John F.; Hong, Robert S.; Jacob, Jeffrey T.; Schutt, Christopher A.

Publicatie 01-12-2020


Objective: Facial function is a key outcome in surgery of the cerebellopontine angle (CPA). This study describes the natural history of facial weakness after CPA surgery.Study design and setting: Retrospective study of two tertiary centers.Patients and intervention: Patients undergoing surgery for CPA tumors from 2003 to 2017 with preoperatively normal facial function and without subsequent surgical or adjuvant therapy.Main outcome measure: Serial facial nerve examinations using the House–Brackmann (HB) scale.Results: Of 301 patients examined, 149 (49.5%) had facial weakness postoperatively and 74.5% of these occurred within 24 hours. Of patients with HB-1 function within 24 hours after surgery, 95.3% had HB-1 function and 100% had HB-3 or better function at last follow-up. Of patients with HB-4 or worse function within 24 hours after surgery, 22.6% ultimately achieved HB-1 function and 83.9% ultimately achieved HB-3 or better function. By 180 days postoperatively, 83.7% of patients’ facial function had stabilized. At last follow-up, 3.0% of patients had facial function poorer than HB-3, which was associated with subtotal resections (12.9% vs. 4.4% for near total and 0.6% for gross total resections, p = 0.002), aspirin use (10.6% vs. 1.6%, p = 0.001), larger tumors (p < 0.0005), longer surgery (p < 0.002), and immediate versus delayed postoperative facial weakness (p = 0.002).Conclusions: Facial function over time after CPA surgery is presented. While facial function immediately after surgery correlates with future function, delayed weakness or improvement is common. These data inform patient counseling both preceding and following surgery.

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Immunophenotype of Vestibular Schwannomas

Bi, Wenya Linda; Gupta, Saksham; Mei, Yu; Abdulmohsen, Sally Al; Giantini Larsen, Alexandra; Unadkat, Prashin; Ramkissoon, Shakti; Abedalthagafi, Malak; Dunn, Ian F.

Publicatie 01-12-2020


Background: Vestibular schwannomas exhibit a uniquely variable natural history of growth, stability, or even spontaneous regression. We hypothesized that a transitory population of immune cells, or immunomodulation of tumors cells, may influence the growth pattern of schwannomas. We therefore sought to characterize the impact of the immune microenvironment on schwannoma behavior.Methods: Forty-eight vestibular schwannomas with preoperative magnetic resonance imaging and 11 with serial imaging were evaluated for presence of immune infiltrates (including the pan-leukocyte marker Cluster of Differentiation (CD)45, CD4 and CD8 T-cell, and CD68 and CD163 macrophages) as well as expression of immunomodulatory regulators (Programmed Death Ligand 1 (PD-L1), Programmed Death Ligand 2 (PD-L2), LAG-3, TIM-3, V-domain Ig Suppressor of T cell Activation). Maximal diameter, volume, and recurrence were annotated.Results: Vestibular schwannomas were characterized by diverse signatures of tumor infiltrating leukocytes and immunomodulatory markers. The median tumor volume was 4.7 cm3 (Interquartile Range (IQR) 1.0–13.0) and maximum diameter was 2.3 cm (IQR 1.5–3.2). Among tumors with serial imaging, the median volumetric growth was 0.04 cm3/mo (IQR 0.01–0.18). Tumor volume and maximum diameter demonstrated strong concordance (R2 = 0.90; p < 0.001). Vestibular schwannoma volume was positively associated with CD4, CD68, and CD163, but not CD8, immune infiltration (all p < 0.05). Tumor growth was positively associated with CD163 and PD-L1 (both p < 0.05). Further, CD163 modified this effect: the relationship between PD-L1 and growth strengthened with increasing CD163 infiltration (R2 = 0.81, p = 0.007). No other immune cell types modified this relationship. These associations were inconsistently observed for maximum diameter and linear growth.Conclusion: Vestibular schwannomas demonstrate variable expression of immune regulatory markers as well as immune infiltrates. Tumor size is associated with immune infiltrates and tumor growth is associated with PD-L1, especially in the presence of M2-subtype macrophages. Volumetric measures may associate with the biological signature more accurately than linear parameters. Future exploration of the role of immune modulation in select schwannomas will further enhance our understanding of the biology of these tumors and suggest potential therapeutic avenues for control of tumor growth.

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Reduced Spread of Electric Field After Surgical Removal of Intracochlear Schwannoma and Cochlear Implantation

Wagner, Luise; Plontke, Stefan K.; Fröhlich, Laura; Rahne, Torsten

Publicatie 01-12-2020


Objective: The primary aim of the study was to explore whether reduced spread of electrical field is observed after partial or subtotal cochleoectomy and cochlear implantation compared with standard cochlear implantation. Secondarily, the influence on speech perception was explored comparing both groups.Study Design: Nonconcurrent cohort study.Setting: Monocentric study at a tertiary referral center.Patients: Twenty adult cochlear implant (CI) users after tumor resection with cochleoectomy of varying extent and 20 electrode-matched CI users with standard electrode insertion.Interventions: Partial and subtotal cochleoectomy for tumor removal and CI.Outcome Measures: Trans-impedance, electrically evoked compound action potentials, and word recognition were measured. Relative impedance was computed as a function of distance between the stimulation and recording electrode.Results: Trans-impedance was smaller and more homogeneous in patients with partial or subtotal cochleoectomy than in the control group. In the tumor group, the mean relative impedance decreased to 0.20 (standard deviation SD = 0.03) at a distance of 1 electrode and to 0.25 (SD = 0.04) in the control group. After excluding seven patients with a second tumor in the internal auditory canal or cerebellopontine angle, with transmodiolar tumors, after near total cochleoectomy, or only extended cochleostomy, word recognition was 61% (SD = 19%) at 3 months and 75% (SD = 19%) at 12 months after activation of the audio processor in the tumor group. At 12 months, it was significantly (p < 0.05) better than in the control group (3 mo: 45%, SD = 25%; 12 mo: 53%, SD = 26%). A smaller trans-impedance is associated with a better word recognition.Conclusion: We conclude that the surgical technique used for CI surgery after subtotal cochleoectomy reduces the spread of the electric field and overcomes the potential drawbacks in structure preservation associated with that technique.

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Vestibular Schwannoma Practice Patterns: An International Cross-specialty Survey

Macielak, Robert J.; Driscoll, Colin L.W.; Link, Michael J.; Haynes, David S.; Lohse, Christine M.; Carlson, Matthew L.

Publicatie 01-12-2020


Objective: To assess vestibular schwannoma (VS) practice patterns among providers.Study Design: Cross-sectional survey.Setting: 8th Quadrennial International Conference on Vestibular Schwannoma and Other CPA Tumors.Subjects: Clinicians who specialize in the management of VS.Main Outcome Measures: Responses to questions on the management and anticipated outcomes of VS for a series of common clinical scenarios were compared by specialty (otolaryngology versus neurosurgery), level of experience, scope of practice (surgery versus radiation and surgery), and geographic location of practice (United States versus international).Results: Responses from 110 participants were analyzed. Overall, 53% of respondents were otolaryngologists, 60% had greater than 10 years of experience, and 57% practiced within the United States. In total, 86% of respondents would pursue initial observation for themselves if diagnosed with a 4 mm distal intracanalicular VS; however, practicing radiosurgeons were more likely to select stereotactic radiosurgery for this scenario compared with providers who solely practice surgery (14 versus 0%; p = 0.032). Otolaryngologists and neurosurgeons alike report that radiosurgery should not be considered a long-term hearing preservation strategy.Otolaryngologists were more optimistic regarding microsurgical hearing preservation outcomes for small distal intracanalicular tumors compared with neurosurgeons (11 versus 3% selected a high likelihood of maintaining class A/B hearing; p = 0.007). Ninety-five percent of respondents prioritized facial nerve outcome over complete disease removal in the context of microsurgical resection of large tumors.Conclusions: Management decision-making and expected outcomes for various clinical scenarios were largely similar among providers; however, variances in several key clinical areas exist. This study points to the feasibility of developing a widely accepted consensus statement among VS experts across specialties.

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Long-term Outcomes of Gamma Knife Radiosurgery for Treating Vestibular Schwannoma With a Lower Prescription Dose of 12\u200aGy Compared With Higher Dose Treatment

Kawashima, Mariko; Hasegawa, Hirotaka; Shin, Masahiro; Takahashi, Wataru; Shinya, Yuki; Iwasaki, Shinichi; Kashio, Akinori; Nakatomi, Hirofumi; Saito, Nobuhito

Publicatie 01-12-2020


Objective: Gamma knife radiosurgery (GKRS) is commonly used to treat vestibular schwannomas (VSs). The risk of complications from GKRS decreases at lower doses, but it is unknown if long-term tumor control is negatively affected by dose reduction.Study Design: This was a retrospective case review and analysis of patient data.Setting: Tertiary referral center.Patients: Patients with VSs who underwent GKRS between 1990 and 2007 at the authors’ institution.Intervention(s): The subjects were divided into two cohorts based on the prescribed doses of radiation received: a 12 Gy cohort (96 patients) with a follow-up period of 124 months and a >12 Gy cohort (118 patients) with a follow-up period of 143 months.Main Outcome Measures: Tumor control rates at 10 to 15 years, frequency of facial and trigeminal nerve complications, and hearing function.Results: The 10 to 15-year tumor control rates were 95% in the 12 Gy cohort and 88% in the > 12 Gy cohort, but the differences were not significant. Compared with the >12 Gy cohort, facial and trigeminal nerve deficits occurred significantly less frequently in the 12 Gy cohort, with the 10-year cumulative, permanent deficit-free rates being 2% and 0%, respectively. Multivariate analyses revealed that treatment doses exceeding 12 Gy were associated with a significantly higher risk for cranial nerve deficits. The percentage of subjects retaining pure-tone average ≤ 50 dB at the final follow-up did not significantly differ between the cohorts (12 Gy cohort, 30% and >12 Gy cohort, 33%; p = 0.823).Conclusions: Dose reduction to 12 Gy for GKRS to treat VSs decreased facial and trigeminal nerve complications without worsening tumor control rates.

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Radiomics-Based Prediction of Long-Term Treatment Response of Vestibular Schwannomas Following Stereotactic Radiosurgery

Langenhuizen, Patrick P. J. H.; Zinger, Svetlana; Leenstra, Sieger; Kunst, Henricus P. M.; Mulder, Jef J. S.; Hanssens, Patrick E. J.; de With, Peter H. N.; Verheul, Jeroen B.

Publicatie 01-12-2020


Objective: Stereotactic radiosurgery (SRS) is one of the treatment modalities for vestibular schwannomas (VSs). However, tumor progression can still occur after treatment. Currently, it remains unknown how to predict long-term SRS treatment outcome. This study investigates possible magnetic resonance imaging (MRI)-based predictors of long-term tumor control following SRS.Study Design: Retrospective cohort study.Setting: Tertiary referral center.Patients: Analysis was performed on a database containing 735 patients with unilateral VS, treated with SRS between June 2002 and December 2014. Using strict volumetric criteria for long-term tumor control and tumor progression, a total of 85 patients were included for tumor texture analysis.Intervention(s): All patients underwent SRS and had at least 2 years of follow-up.Main Outcome Measure(s): Quantitative tumor texture features were extracted from conventional MRI scans. These features were supplied to a machine learning stage to train prediction models. Prediction accuracy, sensitivity, specificity, and area under the receiver operating curve (AUC) are evaluated.Results: Gray-level co-occurrence matrices, which capture statistics from specific MRI tumor texture features, obtained the best prediction scores: 0.77 accuracy, 0.71 sensitivity, 0.83 specificity, and 0.93 AUC. These prediction scores further improved to 0.83, 0.83, 0.82, and 0.99, respectively, for tumors larger than 5 cm3.Conclusions: Results of this study show the feasibility of predicting the long-term SRS treatment response of VS tumors on an individual basis, using MRI-based tumor texture features. These results can be exploited for further research into creating a clinical decision support system, facilitating physicians, and patients to select a personalized optimal treatment strategy.

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Predicting Long-Term Facial Nerve Outcomes After Resection of Vestibular Schwannoma

Tawfik, Kareem O.; Alexander, Thomas H.; Saliba, Joe; Mastrodimos, Bill; Cueva, Roberto A.

Publicatie 01-12-2020


Objectives: 1) Describe the effect of tumor size on facial nerve (FN) outcomes after microsurgical resection of vestibular schwannoma (VS).2) Describe the effect of surgical approach, preoperative radiation, and early postoperative facial function on long-term FN outcomes.Study Design: Retrospective analysis.Setting: Tertiary referral center.Patients: Adult (≥18 yr) patients underwent translabyrinthine or retrosigmoid VS resection by a single neurotologist and single neurosurgeon between February 2008 and December 2017.Main Outcome Measures: Long-term FN outcomes (≥12 mo) according to House–Brackmann (HB) grade.Results: During the study period, 350 patients underwent VS resection, of whom 290 met inclusion criteria. Translabyrinthine surgery was performed in 54% (n = 158) and retrosigmoid in 45% (n = 131). One patient underwent a combined approach. Among patients who underwent retrosigmoid approach, none had a tumor more than 30 mm. Gross total resection was achieved in 98% (n = 283). Long-term HB1–2 function was achieved in 90% (n = 261). On univariate analysis, tumor size (per cm increase), history of preoperative radiation, and worse HB score at discharge predicted worse FN function. Multivariate analysis showed that tumor size (per cm increase) and history of radiation were independent predictors of FN function. For patients with tumors less than 30 mm, multivariate analysis of tumor size and surgical approach was performed; tumor size remained predictive of worse FN function (odds ratio OR 2.362, p = 0.0035), whereas surgical approach was not significantly predictive (p = 0.7569).Conclusion: Tumor size and history of radiation predict long-term FN function after VS resection. When accounting for tumor size, the translabyrinthine and retrosigmoid approaches yield equivalent FN results.

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The Effect of Tumor Size on Likelihood of Hearing Preservation After Retrosigmoid Vestibular Schwannoma Resection

Tawfik, Kareem O.; Alexander, Thomas H.; Saliba, Joe; Mastrodimos, Bill; Cueva, Roberto A.

Publicatie 01-12-2020


Objectives: 1) Describe the effect of tumor size on the likelihood of hearing preservation after retrosigmoid approach for resection of vestibular schwannoma (VS).2) Describe the effect of preoperative hearing status on the likelihood of hearing preservation.Study Design: Retrospective chart review.Setting: Tertiary referral center.Patients: Adult (18 years or older) patients underwent retrosigmoid VS resection and postoperative audiometry between 2008 and 2018 and had a preoperative word recognition score (WRS) of at least 50%. Patients with a history of neurofibromatosis 2, radiation, or previous resection were excluded.Interventions: All patients underwent retrosigmoid VS resection with attempted hearing preservation.Main Outcome Measures: WRS of at least 50%.Results: Data from 153 patients were analyzed. Mean age was 50.8 (±11.3) years and mean tumor size 14 (±6) mm. Hearing was preserved and lost in 64 (41.8%) and 89 (58.2%) patients, respectively. Hearing preservation rates were higher for intrameatal tumors than for tumors with extrameatal extension (57.6% versus 29.4%, p = 0.0005). On univariate and multivariate regression analysis, tumor size (per mm increase) was a negative predictor of hearing preservation (odds ratio OR 0.893, p = 0.0002 and 0.841, p = 0.0005, respectively). Preoperative American Academy of Otolaryngology–Head & Neck Surgery Hearing Class was also predictive of hearing preservation (p = 0.0044). Class A hearing (compared with class B hearing) was the strongest positive risk factor for hearing preservation (OR 3.149, p = 0.0048 and 1.236, p = 0.0005, respectively).Conclusion: Small tumor size and preoperative class A hearing are positive predictors of hearing preservation in patients undergoing the retrosigmoid approach for VS resection.

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The Experience of a Facial Nerve Unit in the Treatment of Patients With Facial Paralysis Following Skull Base Surgery

Lassaletta, Luis; Morales-Puebla, José Manuel; González-Otero, Teresa; Moraleda, Susana; Roda, José María; Gavilán, Javier

Publicatie 01-12-2020


The management of facial paralysis following skull base surgery is complex and requires multidisciplinary intervention. This review shows the experience of a facial nerve (FN) unit in a tertiary university referral center. A multidisciplinary approach has led to the breaking of some old treatment paradigms. An overview of five FN scenarios is presented. For each setting a contemporary approach is proposed in contrast to the established approach. 1) For patients with an anatomically preserved FN with no electrical response at the end of surgery for vestibular schwannoma, watchful waiting is usually advocated. In these cases, reinforcement with an interposed nerve graft is recommended. 2) In cases of epineural FN repair, with or without grafting, and a poor expected prognosis, an additional masseter-to-facial transfer is recommended. 3) FN transfer, mainly hypoglossal-to-facial and masseter-to facial, are usually chosen based on the surgeons’ preference. The choice should be based on clinical factors. A combination of techniques improves the outcome in selected patients. 4) FN reconstruction following malignant tumors requires a combination of parotid and temporal bone surgery, involving different specialists. This collaboration is not always consistent. Exposure of the mastoid FN is recommended for lesions involving the stylomastoid foramen, as well as intraoperative FN reconstruction. 5) In patients with incomplete facial paralysis and a skull base tumor requiring additional surgery, consider an alternative reinnervation procedure, “take the FN out of the equation” before tumor resection. In summary, to achieve the best results in complex cases of facial paralysis, a multidisciplinary approach is recommended.

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Skull Base Training and Mentorship

Haynes, David S.; Roser, Florian; Brackmann, Derald E.; van Loveren, Harry R.

Publicatie 01-12-2020


Objective: To review teaching and mentoring techniques of experienced skull base surgeons and educatorsStudy Design: Expert commentary.Setting: 8th Quadrennial International Conference on vestibular schwannoma and other CPA tumors, panel on teaching, and mentoring.Main Outcome Measures: Experiences and opinions of experienced skull base surgeons, both neurosurgeons and neurotologists, presented and discussed at the conference.Conclusions: Obtaining surgical mastery is essential for the teachers of skull base surgery. Hard work and practice with immediate and constant feedback on performance is an essential component to success. Creating a patient-centered culture that encourages academic achievement is an accelerator for success of a training program. Both the mentor and the mentee must play an intentional and active role to maximize learning.

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Evolving Role of Non-Total Resection in Management of Acoustic Neuroma in the Gamma Knife Era

Luryi, Alexander L.; Kveton, John F.; Babu, Seilesh; Bojrab, Dennis I.; Michaelides, Elias M.; Schutt, Christopher A.

Publicatie 01-12-2020


Objective: To examine patients with residual tumor after vestibular schwannoma (VS) resection with focus on need for further therapy, including stereotactic radiosurgery (SRS) and revision surgery.Study Design and Setting: Retrospective review at two tertiary otology referral centers.Patients and Intervention: Patients undergoing primary surgery for VS from 2007 to 2017.Main Outcome Measure: Degree of resection and need for further treatment.Results: Of 289 patients undergoing surgery, 38 (13.1%) underwent subtotal resections (<95% of tumor resected) and 77 (26.6%) underwent near-total resections (≥95% but <100%). Patients with any residual tumor had larger tumors preoperatively (mean estimated volume 6.3 cm3 versus 2.1 cm3, p < 0.0005) but were otherwise clinically and demographically similar to the population as a whole. Further treatment (surgery or SRS) was needed in 4.6, 14.3, and 50.0% of patients after gross total, near-total, and subtotal resections, respectively (p < 0.0005). Patients undergoing additional therapy had larger residual tumors (median post- to preoperative estimated volume ratio 0.09 versus 0.01, p < 0.0005). Patients undergoing subtotal and near-total resections had poorer facial function at ultimate follow up than those undergoing gross total resections (p = 0.001), likely due to larger tumors and more difficult resections. Literature review revealed higher rates of gross total resection as well as facial palsy in the pre-SRS era.Conclusion: Residual tumor following VS resection is more common today than in the pre-SRS era. Availability of SRS may encourage leaving residual tumor intraoperatively to preserve neural structures. Current surgical strategies decrease surgical morbidity but necessitate further treatment in over 10% of cases.

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Working Toward Consensus on Sporadic Vestibular Schwannoma Care: A Modified Delphi Study

Carlson, Matthew L.; Link, Michael J.; Driscoll, Colin L.W.; Haynes, David S.; Billings, Heather A.; Lohse, Christine M.; Hall, Elissa R.; Agazzi, Siviero; Barker, Frederick G. III; Brackmann, Derald E.; Cueva, Roberto A.; Golfinos, John G.; Gurgel, Richard K.; Kondziolka, Douglas; Kutz, J. Walter Jr; Neff, Brian A.; Sheehan, Jason P.; Van Gompel, Jamie J.; Yu, Chung Ping

Publicatie 01-12-2020


Objective: To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma.Study Design: Modified Delphi method.Methods: The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round.Results: Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus.Conclusion: This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

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Epidemiology and Diagnostic Characteristics of Vestibular Schwannomas—Does Gender Matter?

Reznitsky, Martin; Petersen, Mette Marie Babiel Schmidt; West, Niels; Stangerup, Sven-Eric; Cayé-Thomasen, Per

Publicatie 01-12-2020


Introduction: A number of epidemiological studies have reported data on, e.g., tumor size and hearing at diagnosis for patients with a vestibular schwannoma (VS), whereas only a few have touched upon the potential significance of sex. The aim of this report is thus to present gender-specific data on incidence and age, tumor localization, tumor size, and hearing loss at diagnosis.Material and Methods: Since 1976, various data for all patients diagnosed with a sporadic VS in Denmark have been entered prospectively into a national database. Data on sex, incidence, age, tumor localization, tumor size, and hearing (discrimination) were extracted for the period 1976 to 2015.Results: Over the 40 years, 3,637 cases were diagnosed, of which 1,804 were women (50%) and 1,833 men (50%). For both sexes, an increasing incidence of tumors with a steadily decreasing size was found. Age was increasing and hearing at diagnosis was increasingly better.Previously, women had more extrameatal and thus larger tumors. During the most recent decade, more tumors were found in men and hearing at diagnosis was better for women, whereas age and tumor size were comparable.Conclusion: The data on gender-specific epidemiology and diagnostic characteristics show varying trends over the 40-year-period spanned. For both sexes, an increasing incidence of tumors with a steadily decreasing size was found. Age is increasing and hearing at diagnosis is increasingly better. More extrameatal and thus larger tumors were previously found in women. Contemporarily, more vestibular schwannomas are found in men and diagnostic hearing is better in women.

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A Journey Through 100 Years of Vestibular Schwannoma Surgery at Mayo Clinic: A Historical Illustrative Case Series

Huang, Alice E.; Marinelli, John P.; Link, Michael J.; Boes, Christopher J.; Carlson, Matthew L.

Publicatie 01-12-2020


The present state of vestibular schwannoma (VS) management is the product of over a century of technical progress by revolutionary surgeons who transformed a once perilous operation. At the beginning of the 1900s, patients who did not succumb to their disease were treated exclusively with surgery, which itself was almost assuredly devastating. Through the pioneering work of surgeons such as Harvey Cushing, Walter Dandy, William House, and others, safer surgical approaches were established with concurrent advances in neuromonitoring, neuroanesthesia, radiology, and adoption of the operating microscope. Owing to refinements in radiosurgical treatment and a greater understanding of the natural history of disease, there has been a dramatic shift toward more conservative management in recent years. For more than 100 years, the Mayo Clinic in Rochester, MN, has maintained an active and uninterrupted VS practice with activities that are well documented and preserved through the Mayo Clinic historical archives. We herein report representative early cases to illustrate the fascinating evolution in VS surgery over the past century at a single-tertiary referral center. Original clinical, imaging, pathology, and operative reports are presented from each era of management. To accurately portray the medical context of each era, antiquated terminology is intentionally preserved as originally transcribed.

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