Place of Tubomanometry in Patulous Eustachian Tube Diagnosis.

OBJECTIVE: The aim of our study was to describe the diagnostic performances of tubomanometry (TMM) and to determine tubomanometric parameter thresholds for the diagnosis of patulous eustachian tube (PET). STUDY DESIGN: We performed a retrospective, monocentric study, including patients treated for PET vs control group. SETTING: This study was performed at the Otolaryngology Department of a tertiary-care hospital in the south of France. METHODS: We collected epidemiologic and clinical data, as well as adjusted opening latency index ("R"-index), rhinopharyngeal pressure threshold of tubal opening (Po), and velar contraction index (IVC) on TMM. Receiver operating characteristic (ROC) curves were used for determination of R index and Po thresholds. RESULTS: Twenty-one patients (26 patulous ears) and 14 controls (24 normal ears) were included. The R index values and Po values were significantly lower in the PET group vs controls (0.46 vs 0.80, respectively; P < .05 for R index and 13.89 vs 26.42 mbar, respectively; P < .05 for Po). No significant difference was reported between the 2 groups on IVC measurement (P = .784). After ROC curve analysis, R index was the most discriminating factor to classify PET patients with 89% specificity and 76% sensitivity with a threshold ≤0.6. Po value ≤10 mbar could support this diagnosis with more than 83% specificity. CONCLUSION: TMM is a reliable noninvasive method for positive diagnosis of PET. TMM could provide an accurate positive PET diagnosis and an objective evaluation for PET management.

Navigation strategies in patients with vestibular loss tested in a virtual reality T-maze.

During navigation, humans mainly rely on egocentric and allocentric spatial strategies, two different frames of reference working together to build a coherent representation of the environment. Spatial memory deficits during navigation have been repeatedly reported in patients with vestibular disorders. However, little is known about how vestibular disorders can change the use of spatial navigation strategies. Here, we used a new reverse T-maze paradigm in virtual reality to explore whether vestibular loss specifically modifies the use of egocentric or allocentric spatial strategies in patients with unilateral (n = 23) and bilateral (n = 23) vestibular loss compared to healthy volunteers (n = 23) matched for age, sex and education level. Results showed that the odds of selecting and using a specific strategy in the T-maze were significantly reduced in both unilateral and bilateral vestibular loss. An exploratory analysis suggests that only right vestibular loss decreased the odds of adopting a spatial strategy, indicating an asymmetry of vestibular functions. When considering patients who used strategies to navigate, we observed that a bilateral vestibular loss reduced the odds to use an allocentric strategy, whereas a unilateral vestibular loss decreased the odds to use an egocentric strategy. Age was significantly associated with an overall lower chance to adopt a navigation strategy and, more specifically, with a decrease in the odds of using an allocentric strategy. We did not observe any sex difference in the ability to select and use a specific navigation strategy. Findings are discussed in light of previous studies on visuo-spatial abilities and studies of vestibulo-hippocampal interactions in peripheral vestibular disorders. We discuss the potential impact of the history of the disease (chronic stage in patients with a bilateral vestibulopathy vs. subacute stage in patients with a unilateral vestibular loss), of hearing impairment and non-specific attentional deficits in patients with vestibular disorders.

Quantitative biomarkers allow the diagnosis of head and neck paraganglioma on multiparametric MRI.

PURPOSE: The aim of this study is to identify quantitative MR biomarkers in head and neck paragangliomas. METHODS: The study was approved by an institutional review board. A retrospective review of patients with head and neck paragangliomas (HNPGL) evaluated by time-resolved MRA sequences between 2009 and 2019 was performed. A control group investigated during the same period was analyzed, including nerve sheath tumors and metastatic lymph nodes from squamous cell carcinomas or undifferentiated nasopharyngeal cancer (UCNT). A gold standard was obtained for all cases. Semi-quantitative parameters of enhancement were extracted from time-intensity curves on time-resolved MRA sequences and diffusion weighted imaging/DWI was assessed for each lesion. RESULTS: Sixty head and neck paragangliomas (HNPGLs) were included from 50 patients. The control group consisted of 30 parapharyngeal space lesions (27 patients), which included nerve sheath tumors (n = 12) and metastatic lymph nodes (n = 18) from squamous cell carcinomas or UCNT. PGLs showed a shorter time-to-peak value compared to other groups, measured at 25.0 +/- 29 sec. The wash-in and wash-out ratios were also significantly higher for PGLs, respectively measured at 5.34 ± 2.99 (p < 0,001) and 1.24 ± 0.80 (p < 0.001). On DWI sequences, the mean ADC value for PGLs (1.17 ± 0.19 10^-3 mm2/s) was significantly different than the other tumor groups (p < 0.001). HNPGLs were clearly distinguishable from other tumors on classification with regression tree based on TTP and ADC values. These distinct group features were also consistent on principal component analysis. CONCLUSION: Our study identifies a multiparametric signature for disease subtyping, providing a strong impetus for switching from qualitative to quantitative analysis of deep soft-tissue tumors of the neck.

Sunnybrook Facial Grading System: Intra-rater and Inter-rater Variabilities.

OBJECTIVES: Evaluate intra-rater and inter-rater variabilities of the Sunnybrook Facial Grading System (SFGS) and explore potential factors of variability. STUDY DESIGN: Prospective test of hypothesis. SETTING: University tertiary referral center. PARTICIPANTS/METHODS: Facial video recordings of 20 patients with variable degrees of peripheral facial palsy (PFP) were anonymized then presented to 31 independents raters in 2 trials. The raters were senior and junior professionals involved in the management of PFP: ENT specialists, physiotherapists, and speech therapists. The SFGS was used for grading paralysis. MAIN OUTCOME MEASURE: Intra-rater and inter-rater variabilities were estimated by intraclass correlation coefficient (ICC [95% confidence interval]) for the composite score and the three subscores of the SFGS. Factors of variability studied were: rater professions and rater experience (senior vs junior). RESULTS: For the total population, the intra-rater ICC was 0.915[0.900-0.929] for the composite score considered to represent almost perfect repeatability. Repeatability was important for symmetry at rest (0.694[0.646-0.737]), almost perfect for voluntary movements (0.903[0.886-0.918]), and important to almost perfect for synkinesis (0.810[0.778-0.838]). The inter-rater ICC for the composite score was 0.847[0.755-0.923] indicating important to almost perfect agreement between all raters. Agreement between raters was important to almost perfect for voluntary movements (0.839[0.746-0.919]), but moderate to important for symmetry at rest (0.553[0.408-0.730]) and weak to important for synkinesis (0.476[0.333-0.666]). Some variability was found between raters groups; however, repeatability and agreement were good for all raters. CONCLUSIONS: The SFGS is a reproducible scale. It can be used with good reproducibility by both novices and experts, and by all professionals involved in the management of PFP.

Syngeneic Transplantation of Rat Olfactory Stem Cells in a Vein Conduit Improves Facial Movements and Reduces Synkinesis after Facial Nerve Injury.

BACKGROUND: Posttraumatic facial paralysis is a disabling condition. Current surgical management by faciofacial nerve suture provides limited recovery. To improve the outcome, the authors evaluated an add-on strategy based on a syngeneic transplantation of nasal olfactory stem cells in a rat model of facial nerve injury. The main readouts of the study were the recording of whisking function and buccal synkinesis. METHODS: Sixty rats were allocated to three groups. Animals with a 2-mm facial nerve loss were repaired with a femoral vein, filled or not with olfactory stem cells. These two groups were compared to similarly injured rats but with a faciofacial nerve suture. Olfactory stem cells were purified from rat olfactory mucosa. Three months after surgery, facial motor performance was evaluated using video-based motion analysis and electromyography. Synkinesis was assessed by electromyography, using measure of buccal involuntary movements during blink reflex, and double retrograde labeling of regenerating motoneurons. RESULTS: The authors' study reveals that olfactory stem cell transplantation induces functional recovery in comparison to nontransplanted and faciofacial nerve suture groups. They significantly increase (1) maximal amplitude of vibrissae protraction and retraction cycles and (2) angular velocity during protraction of vibrissae. They also reduce buccal synkinesis, according to the two techniques used. However, olfactory stem cell transplantation did not improve axonal regrowth of the facial nerve, 3 months after surgery. CONCLUSIONS: The authors show here that the adjuvant strategy of syngeneic transplantation of olfactory stem cells improves functional recovery. These promising results open the way for a phase I clinical trial based on the autologous engraftment of olfactory stem cells in patients with a facial nerve paralysis.

Facial Nerve Canal Paraganglioma.

We report the case of a 72-year-old woman presenting with a progressive left peripheral facial paralysis and a facial canal mass extending through the stylomastoid foramen along the posterior edge of the parotid gland. On MRI, the early and intense enhancement was highly suggestive of paraganglioma but could not rule out a nonossifying hemangioma. Laboratory analysis showed normal plasma metanephrines. On F-FDOPA PET/CT, the mass exhibited a typical paraganglioma feature with a marked tumor uptake. Our case demonstrates that F-FDOPA plays a vital role in this rare entity and can avoid any further confirmatory invasive procedure.

The Effects of Hypoxia on Middle Ear Pressure Regulation.

Background: Middle ear (ME) pressure regulation has been suggested as a physiological mechanism that maintains pressure equilibrium between the ME and the ambient environment. This mechanism would be based on a complex sensorineural reflex loop composed of mechanoreceptors, an integrative center, and efferent neural pathways. Our aim was to demonstrate that hypoxic conditions, which would inhibit mechanoreceptors in general, similarly participate in the inhibition of the opening of the Eustachian tube (ET), and thus, to suggest that such receptors are involved in the overall regulation of ME pressure. Materials and Methods: Among 14 healthy volunteers, tubomanometry was performed in normoxia followed by hypoxia, and 3 parameters were evaluated for each ear under each condition, allowing the evaluation of the reactivity of the system: ET opening latency index (R), the Index of Velum Contraction (IVC), and the latency of pressure instauration (C2-C1). Results: Hypoxia induced a significant increase in the opening latency index of ET opening, without deleterious effects on the quality (IVC) and latency (C2-C1) of soft palate contraction. Conclusions: Our study supports the theory of a sensorineural reflex loop and provides evidence for the existence of mechanoreceptors, whose function is modified by changes in oxygen partial pressure, able to collect information on pressure variations between the ME and the external environment.

Does NF2 status impact the results of combined surgery and adjunctive Gamma Knife surgery for large vestibular schwannomas?

In order to verify whether neurofibromatosis type 2 (NF2) could influence the oncological and functional outcome in large vestibular schwannoma (VS) surgery, we compared a group of NF2 patients operated with a facial nerve-sparing technique to a group of sporadic VSs of similar volume that underwent the same treatment regimen in the same period. Single-center retrospective cohort study about 12 consecutive NF2 and 69 non-NF2 patients operated on for large VS between September 2006 and November 2016. After resection, patients were allocated to an upfront Gamma Knife surgery policy of the tumor residue. At last follow-up examination, the facial nerve function was good (House-Brackmann grades I or II) in 92% of the NF2 and 83% of the non-NF2 patients (p = .90). The median volume of tumor residue was .92 cc in the NF2 group and .54 cc in the non-NF2 group (p = .14). Tumor control was achieved in 83% and 81% of cases in the NF2 and the non-NF2 populations, respectively, with a mean follow-up of 73 months in both groups. The 1-, 5-, and 7-year progression-free survival were 92%, 83%, and 83% respectively in the NF2 group, and 99%, 83%, and 80% in the non-NF2 group (p = .96). Our analysis of 12 NF2 and 69 non-NF2 patients operated on by the same surgical team with the same treatment regimen did not show any functional or tumor control difference between those groups.

Incidental Effect of Long-Term Tacrolimus Treatment on Sporadic Vestibular Schwannoma Volume Shrinkage and Clinical Improvement.

OBJECTIVE: We describe the effect of long-term tacrolimus delivery on sporadic vestibular schwannoma (VS). PATIENT: A 48-year-old woman who was diagnosed a right VS. INTERVENTION: Long-term tacrolimus delivery following liver transplant. MAIN OUTCOME MEASURES: Clinical examination, audiometric test, and gadolinium-contrasted magnetic resonance imaging. RESULTS: During the reported follow-up period, the patient felt hearing improvement especially in noisy environment. This was consistent with a clear audiometric improvement. Over a 26 months follow-up period, we noticed a 50% volume regression and a 40% decrease in the intensity of enhancement of the tumor after gadolinium injection. CONCLUSION: Tacrolimus, an immunosuppressant used to prevent rejection in organ transplant, could lead to VS volume shrinkage and hearing improvement. Tacrolimus could be an interesting new therapeutic weapon, especially for VS in Neurofibromatosis type 2.

Exploring the link between tumour metabolism and succinate dehydrogenase deficiency: A 18 F-FDOPA PET/CT study in head and neck paragangliomas.

OBJECTIVES: Nuclear imaging findings by virtue of phenotyping disease heavily depend on genetic background, metabolites, cell membrane specific targets and signalling pathways. PPGL related to succinate dehydrogenase subunits mutations (SDHx mutations) are less differentiated than other subgroups and therefore may lack to concentrate 18 F-FDOPA, a precursor of catecholamines biosynthesis. However, this 18 F-FDOPA negative phenotype has been reported mostly in SDHx-PPGL of sympathetic origin, suggesting that both genotype status and location (from sympathetic vs parasympathetic paraganglia; adrenal vs extra-adrenal) could influence 18 F-FDOPA uptake. The aim of this study was to test if SDHx drives 18 F-FDOPA uptake in presence of normal epinephrine/norepinephrine concentrations. DESIGN: Retrospective study PATIENTS: A cohort of 86 head and neck PPGL patients (including three metastatic) with normal metanephrines underwent 18 F-FDOPA PET/CT. The relationships between 18 F-FDOPA uptake and tumour genotype were evaluated. RESULTS: In nonmetastatic HNPGL (50 non-SDHx/33 SDHx), no significant difference was observed between these two groups for SUVmax (P = .256), SUVmean (P = .188), MTV 42% (P = .596) and total lesion uptake (P = .144). Metastatic HNPGL also had high elevated uptake values. CONCLUSIONS: Our results suggest that SDH deficiency or metastatic behaviour have no influence on 18 F-FDOPA uptake in HNPGL probably due to their very-well differentiation status, even at metastatic stage. The potential prognosticator value of 18 F-FDOPA uptake would need to be further explored in the setting of metastatic PPGL of sympathetic origin.

Unilateral vestibular deafferentation impairs embodied spatial cognition.

A growing number of studies indicate that cognitive complaints are common in patients with peripheral vestibular disorders. A better understanding of how vestibular disorders influence cognition in these patients requires a clear delineation of the cognitive domains affected by vestibular disorders. Here, we compared the consequences of left and right vestibular neurectomy on third-person perspective taking-a visuo-spatial task requiring mainly own-body mental imagery, and on 3D objects mental rotation imagery-requiring object-based mental imagery, but no perspective taking. Patients tested 1 week after a unilateral vestibular neurectomy and a group of age- and gender-matched healthy participants played a virtual ball-tossing game from their own first-person perspective (1PP) and from the perspective of a distant avatar (third-person perspective, 3PP). Results showed larger response times in the patients with respect to their controls for the 3PP taking task, but not for the 1PP task and the 3D objects mental imagery. In addition, we found that only patients with left vestibular neurectomy presented altered 3PP taking abilities when compared to their controls. This study suggests that unilateral vestibular loss affects mainly own-body mental transformation and that only left vestibular loss seems to impair this cognitive process. Our study also brings further evidence that vestibular signals contribute to the sensorimotor bases of social cognition and strengthens the connections between the so far distinct fields of social neuroscience and human vestibular physiology.

Predictive Factors of Early Postoperative and Long-Term Facial Nerve Function After Large Vestibular Schwannoma Surgery.

OBJECTIVE: The preservation of acceptable facial nerve (FN) function after surgery is the key concern for most patients with vestibular schwannomas (VS). To assess predictive factors of early postoperative and long-term FN function in patients harboring large VS operated with a FN-sparing technique. METHODS: Single-center retrospective cohort study with 169 consecutive large VS operated on between January 2003 and May 2015. Clinical, radiologic, and intraoperative factors were assessed according to FN function. RESULTS: At last follow-up examination, among the 145 patients without preoperative FN palsy, FN function was good (House-Brackmann [HB] grades I or II) in 84% and moderate (HB grade III) in 15% of patients. Only 1 patient presented with poor HB grade IV function. Multivariate logistic regression model showed the mean preoperative VS extrameatal diameter as being an independent predictor of an unfavorable initial FN outcome (odds ratio [OR], 1.062; P = 0.038). Surgical anatomic preservation of the cochlear nerve was associated with better FN outcomes (OR, 0.237; P = 0.012). A history of previous surgery seemed to be related to long-term impaired FN function (OR, 71.405; P = 0.042), as well as early postoperative FN function (OR, 19.068; P = 0.000). No correlation was found between a history of previous Gamma Knife surgery treatment (P = 0.225) or the extent of resection (P = 0.438) and impaired FN outcomes. History of previous surgery was identified as an unfavorable predictive recovery factor of impaired postoperative FN function (P = 0.034). CONCLUSIONS: As long as the extent of resection or additional Gamma Knife surgery have not been identified as predictive risk factors of postoperative FN palsy, we suggest that optimal resection is the main option for patients harboring large VS.

Malignant Aspergillus flavus Otitis Externa with Jugular Thrombosis.

We report a case of malignant otitis externa with jugular vein thrombosis caused by Aspergillus flavus. Magnetic resonance imaging revealed an unusual ink smudge pattern deep in a cervical abscess. The pattern was consistent with mycetoma and may be important for diagnosing these life-threatening infections.

Validation of French versions of the Facial Disability Index and the Facial Clinimetric Evaluation Scale, specific quality of life scales for peripheral facial palsy patients.

OBJECTIVES: To translate and validate French versions of two health-related quality of life questionnaires for patients with peripheral facial palsy: Facial Disability Index (FDI) and Facial Clinimetric Evaluation (FaCE) scale. DESIGN: Prospective cohort study. SETTING: University tertiary referral centre. PARTICIPANTS: A pilot test was performed on 10 subjects (5 patients with facial palsy of more than 1-month duration and 5 normal subjects), and then 67 adult patients with facial palsy were enrolled in the validation study. MAIN OUTCOME MEASURES: Translation of the original questionnaires has followed international guidelines using a forward-backward translation method. A pilot test and a validation study based on the translated questionnaires were performed. Internal consistency, test-retest reliability, validity and responsiveness were assessed. Validity was assessed by comparing to SF-36 and Sunnybrook/House-Brackmann grading systems. Subjects answered scales twice within a one-week interval. RESULTS: Sixty-seven patients were enrolled, among which 63 completed scales one week later (retest). For physical and social functions of FDI and FaCE scores, Cronbach's α representing internal consistency was 0.88, 0.70 and 0.89, and test-retest reliability by intra-class correlation coefficients was 0.81, 0.86 and 0.89, respectively. The correlation of facial movement score of FaCE scale was good with Sunnybrook/House-Brackmann grading systems (0.73 and -0.75, P < 0.01). The correlation of social function of FaCE scale was excellent with social function of SF-36 (0.8, P < 0.01). CONCLUSIONS: French versions of FDI and FaCE scale are psychometrically valid. Both questionnaires can be used for clinical studies to assess the quality of life of patients with peripheral facial palsy.

Tumor multifocality with vagus nerve involvement as a phenotypic marker of SDHD mutation in patients with head and neck paragangliomas: A 18 F-FDOPA PET/CT study.

BACKGROUND: 18 F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the relationship between tumor characteristics and the SDHx-mutational status in a large series of patients with HNPGL evaluated by 18 F-FDOPA PET/CT. METHODS: A total of 104 patients with HNPGL (65 sporadic/39 SDHx-mutated) were included. RESULTS: In comparison to SDHB/SDC/SDHx-negative cases, patients with SDHD were younger at diagnosis and had a higher rate of multifocal, vagal, and carotid paraganglioma. In patients with SDHD, vagal paraganglia represented the primary site of tumor origin. Multicentric involvement of the vagus nerve alone or in association with other locations was found to be a typical feature of SDHD cases compared to other cases (odds ratio = 59.4). CONCLUSION: The present study shows that tumor multifocality within the vagus nerve is a phenotypic marker of SDHD mutation. This information is essential in the choice of the therapeutic strategy.

Adjunctive Gamma Knife Surgery or Wait and Scan Policy After Optimal Resection of Large Vestibular Schwannomas: Clinical and Radiologic Outcomes.

OBJECTIVES: Patients with large vestibular schwannomas (VSs) will require surgical treatment owing to the potential consequences of long tract and cranial nerve compression. We assessed the long-term clinical and radiologic outcomes of patients harboring large VSs treated with a facial nerve (FN)-sparing technique. METHODS: We performed a single-center retrospective cohort study of 169 consecutive large VSs treated surgically from January 2003 to May 2015. The postoperative volume of the tumor residue was assessed using thin-slice magnetic resonance imaging 6 months after surgery. Postoperatively, the patients were allocated to a wait and rescan (W&reS) or an upfront gamma knife surgery (GKS) policy. RESULTS: At the last follow-up examination, FN function was good (House-Brackmann grade I or II) in 84% of the patients. Of the 169 patients, 11% had undergone gross total resection, 59% near total, 21% subtotal, and 9% partial resection. In the 143 patients without gross total resection, the overall median tumor residue volume was 0.39 cm3. Of these 143 patients, 66 had been allocated to the W&reS policy and 77 to upfront GKS. Overall tumor control was achieved in 83% of cases, with a mean follow-up of 62 months. Of the 27 growing residues, 17 required salvage treatment (11% failure rate). The 1-, 5-, and 7-year progression-free survival rate was 95%, 82%, and 76% in the W&reS group and 99%, 81%, and 78% in the GKS group, respectively (P = 0.57). CONCLUSIONS: Functional nerve-sparing resection provides satisfactory FN preservation. The low probability of long-term regrowth of small remnants is an argument for a W&reS protocol. GKS is a legitimate option for salvage treatment.

Intracanalicular Vestibular Schwannomas: Initial Clinical Manifestation, Imaging Classification, and Risk Stratification for Management Proposal.

INTRODUCTION: There is no consensus about the use of observation as a therapeutic modality for intracanalicular vestibular schwannoma (ICVS). The objective of this study was to describe tumor evolution, its correlation with initial size, stage of vestibular schwannoma, clinical presentation and to propose a risk classification for tumor growth with a therapeutic decision algorithm. METHODS: Fifty-three patients with ICVS were followed prospectively from 2010 to 2015. The mean follow-up was 32 months. Patients underwent clinical examination, audiogram, magnetic resonance imaging at 6, 12, and then 12 months intervals within the first 5 years of initial diagnosis. We analyzed the patient's clinical course, audiologic changes, initial tumor location, and correlation of different parameters with ICVS growth. RESULTS: Fourteen patients (26%) deteriorated their hearing level and 17 patients (27%) showed tumor growth during the follow-up period. Growth was noted during the first year of observation in 13 patients (76.5%). Considering initial presentation: tumor size, intracanalicular staging, and hearing level, patients with larger vestibular schwannoma, extension to the fundus regardless of tumor size, higher initial pure-tone average, and speech recognition test showed a significantly higher rate of tumor growth. ICVS evolution was not correlated with hearing deterioration with time. DISCUSSION: We should consider observation a therapeutic modality. We valid the intrameatal staging as prognostic factor and propose a stratification of patients into low, moderate, or high risk for potential tumor growth to guide the initial management of ICVS.