Sinonasal and ear manifestations of Erdheim-Chester disease.

To calculate the prevalence of sinonasal and ear involvement in an Erdheim-Chester disease (ECD) population, to describe the different ear, nose and throat (ENT) manifestations and to study the association between ENT involvement, other organ involvement, and BRAF mutations. We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty-two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses. Trial registration: #2011-A00447-34.

Long-Term Outcomes in Head and Neck Paragangliomas Managed with Intensity-Modulated Radiotherapy.

OBJECTIVES: Head & Neck Paragangliomas have been historically relying on surgery mostly, with worsened quality of life and major sequelae. Conventional external radiation therapy seems to offer an equivalent control rate with a low toxicity profile. The aim of this study was to assess the safety and efficiency of intensity-modulated radiation therapy in Head & Neck paragangliomas. METHODS: This is a retrospective monocentric study conducted in a referral center, including all patients treated with IMRT, whether as an exclusive or post-operative treatment for a tympanic and jugular, carotid, or vagal paraganglioma. Data collection was performed through the manuscript and computerized medical files, including consultation, operative, imaging, pathological analyses, delineation, and treatment planning reports. Success was defined as the complete or partial regression or stabilization without progression, or relapse in accordance with the RECIST criteria. Acute toxicities and long-term sequelae were assessed. RESULTS: Our cohort included 39 patients included between 2011 and 2021: 18 patients treated for a TJ PG (45.9%), 11 patients for a carotid PG (28.4%), and 9 for a vagal PG (23.1%). Twenty-nine patients had IMRT as an exclusive treatment (74.4%), whereas 10 patients had a post-operative complementary treatment (25.6%). Median follow-up in our cohort was 2318 days (average = 2200 days, 237-5690, sd = 1281.9). Among 39 patients, 37 were successfully controlled with IMRT (94.8%), and the toxicity profile was low without any major toxicity. CONCLUSION: IMRT seems an ideal treatment, whether exclusive or post-operative for Head & Neck paragangliomas. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:607-614, 2023.

Antibiotics in mastoid and epitympanic obliteration with S53P4 bioactive glass: A retrospective study.

Objective: The role of antibiotics in ear surgery is still controversial. The aim of this study was to assess their need in cholesteatoma surgery when performing obliteration with S53P4 bioactive glass, a biocompatible material with antibacterial properties. Methods: This retrospective cohort study was conducted in a tertiary referral center between January 2017 and May 2019. Sixty-nine consecutive patients, who underwent surgery for cholesteatoma removal and/or rehabilitation of canal-wall-down mastoidectomy with mastoid and epitympanic obliteration using S53P4 granules were included. Before 2019, antibiotics were routinely used (group "w/AB"). Patients received intravenous antibiotics during surgery, oral treatment was continued for 7 days and topical antibiotics for 1 month. After 2019, no antibiotics were administered (group "w/oAB"). The primary outcome was the occurrence of early surgical site infection. Secondary outcomes were late infection, anatomic and functional results at 3 and 12 months. Results: Twenty-three patients were included in group "w/oAB" and 46 in group "w/AB", with no significant differences in demographics, medical history or follow-up. Five ears (22%) in group "w/oAB" developed an early infection compared with 2 (4%) in group "w/AB" (p = .03). The relative risk was 6.11, 95CI%[1.09;31.96]. Infections were successfully treated with antibiotics, and no patient underwent surgical removal of the granules. No late infections or complications were observed. There was no difference in graft failure or air-bone gap closure at 1 year. Conclusion: Peri-/post-operative antibiotics prevent early infection in obliteration surgery with S53P4 granules. Infections can be treated medically without complications or require removal of the implanted material. Level of evidence: 4.

Nasal and Pharyngeal Coil Extrusion After Embolization of an Internal Carotid Artery Pseudoaneurysm.

This case report describes a man in his 20s with a medical history of embolization of the right internal carotid artery who presented to the ears, nose, and throat emergency department with a wire in his mouth, throat discomfort, and irritating cough.

A New Sensitive Test Using Virtual Reality and Foam to Probe Postural Control in Vestibular Patients: The Unilateral Schwannoma Model.

Vestibular schwannomas (VS) are benign tumors of the vestibular nerve that may trigger hearing loss, tinnitus, rotatory vertigo, and dizziness in patients. Vestibular and auditory tests can determine the precise degree of impairment of the auditory nerve, and superior and inferior vestibular nerves. However, balance is often poorly quantified in patients with untreated vestibular schwannoma, for whom validated standardized assessments of balance are often lacking. Balance can be quantified with the EquiTest. However, this device was developed a long time ago and is expensive, specific, and not sensitive enough to detect early deficits because it assesses balance principally in the sagittal plane on a firm platform. In this study, we assessed postural performances in a well-defined group of VS patients. We used the Dizziness Handicap Inventory (DHI) and a customized device consisting of a smartphone, a mask delivering a fixed or moving visual scene, and foam rubber. Patients were tested in four successive sessions of 25 s each: eyes open (EO), eyes closed (EC), fixed visual scene (VR0), and visual moving scenes (VR1) delivered by the HTC VIVE mask. Postural oscillations were quantified with sensors from an android smartphone (Galaxy S9) fixed to the back. The results obtained were compared to those obtained with the EquiTest. Vestibulo-ocular deficits were also quantified with the caloric test and vHIT. The function of the utricle and saccule were assessed with ocular and cervical vestibular-evoked myogenic potentials (o-VEMPs and c-VEMPs), respectively. We found that falls and abnormal postural oscillations were frequently detected in the VS patients with the VR/Foam device. We detected no correlation between falls or abnormal postural movements and horizontal canal deficit or age. In conclusion, this new method provides a simpler, quicker, and cheaper method for quantifying balance. It will be very helpful for (1) determining balance deficits in VS patients; (2) optimizing the optimal therapy indications (active follow-up, surgery, or gamma therapy) and follow-up of VS patients before and after treatment; (3) developing new rehabilitation methods based on balance training in extreme conditions with disturbed visual and proprioceptive inputs.

Chronic oropharyngeal pain and medical nomadism in an Eagle's syndrome patient: a case report.

BACKGROUND: Elongation of the styloid process associated with oropharyngeal pain and dysphagia is known as Eagle's syndrome, a condition whose pathophysiology is still a matter of debate. Given its low prevalence and complex symptomatology, this syndrome is often misdiagnosed, leading to chronic pain and medical nomadism. A 51-year-old woman of African origin with 3-year history of left-side oropharyngeal pain and worsening dysphagia consulted several health professionals. Medical and surgical treatments, including a sinus surgery and the extraction of three healthy teeth, did not improve her symptoms. Evaluation in an orofacial pain clinic revealed an asymmetrically elongated styloid process. Surgical shortening of the elongated styloid process provided complete pain relief and recovery of normal swallowing function. CONCLUSION: Based on this case report, the pathophysiology of Eagle's syndrome is discussed, and the need for specific follow-up in a subpopulation of patients with asymptomatic styloid process elongation is highlighted.

Outcomes and prognostic factors of facial nerve repair: a retrospective comparative study of 31 patients and literature review.

PURPOSE: To evaluate functional results of facial nerve repair by direct nerve suture or grafting, compare results between a traumatic and a tumoral group and identify prognostic factors. METHODS: A retrospective monocentric cohort study was studied in a university ENT department. Thirty-one patients who benefited from facial nerve suture or grafting, with at least 12 months postoperative follow-up were included. Patients were divided into two groups according to the lesion type: traumatic (accident of the public road or iatrogenic) and tumoral. Preoperative data studied were sex, side, etiology documented by CT and/or MRI, facial palsy duration and grade according to House Brackmann grading system. Intraoperative data included: surgeon, age of patient, surgical technique, graft type, use of biological glue, facial nerve derivation, lesioned site. Postoperative data included: histological diagnosis, radiotherapy history, time to onset of the first signs of reinnervation, follow-up duration, and final facial function. RESULTS: Success rate, including grade III and IV, was 68% in the whole cohort, 79% in the traumatic group and 59% in the tumoral group. Patients presenting with facial palsy evolving less than 6 months had better recovery results than those evolving longer than 6 months (p = 0.02). No other prognostic factors were identified. CONCLUSIONS: The best outcome that can be achieved by suture or grafting of the facial nerve in traumatic or tumoral lesions is a grade III. Preoperative facial palsy duration is a prognostic factor and must be considered when establishing an operative indication.

Oro-facial motor assessment: validation of the MBLF protocol in facial palsy.

BACKGROUND: Oro-facial myofunctional praxis assesses the muscular coordination and the degree of motor impairment of the lingual, mandibular and facial muscles necessary for articulation, mimicry and swallowing. PURPOSE: The objective of this study was to create and validate the MBLF protocol, a French oro-facial myofunctional assessment in order to quantify patient's impairment and to specify the motor and functional deficit for an adapted management. METHODS: The MBLF was validated against the Sunnybrook Facial Grading System (criterion validity). The construct validity was tested by comparing healthy subjects (n = 102) from patients with facial palsy (n = 60). Internal and external consistency of face symmetry were reported. Normative data was provided. RESULTS: There was a statistically significant correlation between the MBLF protocol and the Sunnybrook Facial Grading System [F(59) = 310.51, p < 0.001, R2 = 0.843]. Significant differences were observed in Student's t test between healthy volunteers and patients with facial palsy [t(74.13) = 14,704, p < 0.001, r = 0.863]. A significant effect of the severity grades of facial palsy on the MBLF_TOTAL scores was found [F(158) = 268.469, p < 0.001]. The more severe the facial palsy, the lower the motor scores were. CONCLUSION: This MBLF French validation provides a baseline for comparing and quantifying the performance of subjects. The MBLF protocol is valid for assessing facial symmetry in peripheral facial palsy. A prospective study is needed to validate its role in dynamic evaluation of facial palsy.

Management of tegmen defects with mastoid and epitympanic obliteration using S53P4 bioactive glass.

OBJECTIVE: To evaluate the surgical results and complications in a cohort of patients operated on to repair a tegmen bony defect using either transmastoid approach with obliteration using S53P4 bioactive glass granules (TMA-O), or the classic middle cranial fossa approach (MCFA). STUDY DESIGN: A retrospective monocentric study. METHODS: Twenty-five cases (24 patients) were included. Data regarding patient demographics, etiology, intraoperative findings, complications, recurrences, audiometric data, and follow-up were analyzed. RESULTS: Seven patients were operated with MCFA and 17 patients with TMA-O. One patient was operated on using a combined approach (MCFA + TMA-O). In the preoperative HRCT scan, the size of the defect was estimated to be 6 ± 3.8 mm in the TMA-O group and 6 ± 3.5 mm in the MCFA group (P = .969). Intraoperatively, in the MCFA group, the location of the defect was mostly anterior (86%) with an intact ossicular chain (86%). A discontinuous chain was observed in 15 patients (88%) in the TMA-O group. The mean follow-up time was 22 ± 14 months in the TMA-O group and 24 ± 15 months in the MCFA group (P = .762). In both groups, there were no early postoperative complications or recurrences during follow-up. CONCLUSION: Repair of a tegmen bony defect with S53P4 bioactive glass granules seems to be safe and effective, limiting the use of the middle cranial fossa approach to cases with epitympanic defects and with an intact ossicular chain, and it could be used whatever the size of the defect and/or the presence of meningoencephalocele. LEVEL OF EVIDENCE: 4.

Imaging Criteria to Predict Surgical Difficulties During Stapes Surgery.

BACKGROUND AND PURPOSE: Stapes surgery for otosclerosis can be challenging if access to the oval window niche is restricted. The aim of this study was to determine the accuracy of the computed tomographic (CT) scan in the evaluation of anatomical distances, and to analyze its reliability in predicting surgical technical difficulties. MATERIAL AND METHODS: A total of 96 patients (101 ears) were enrolled in a prospective study between 2012 and May 2015. During surgery, we evaluated the distance D1 between the stapes and the facial nerve, distance D2 between the promontory and the facial nerve after ablation of the superstructure, and the intraoperative discomfort of the surgeon. On preoperative CT scans, we measured the width and depth of the oval window niche, and the angle formed by two axes starting from the center-point of the footplate, the first tangential to the superior wall of the promontory, and the second tangential to the inferior wall of the fallopian canal. RESULTS: Intraoperative distances D1 and D2 were correlated with the width of the oval window and with the facial-promontory angle measured on imaging. CT scan measurements of the facial-promontory angle and width of the oval window were associated with the degree of discomfort of the surgeon. The cut-off threshold for intraoperative subjective discomfort was computed as 1.1 mm for the width of the oval window niche, with a sensibility of 71% and a specificity of 84%. CONCLUSION: Preoperative imaging analysis of the oval window width and the facialpromontory angle can predict operative difficulty in otosclerosis surgery.

Smile Reanimation after Unilateral Facial Palsy by Lengthening Temporalis Myoplasty: Objective and Subjective Evaluation on 25 Cases.

BACKGROUND: Comparison of functional results of lengthening temporalis myoplasty relies in current practice on subjective scales. The goal of this study was to define a simple, reproducible, objective scale validated through a comparison with a subjective scale for smile symmetrization results after temporal muscle myoplasty. METHODS: A retrospective study was conducted on 25 patients having a unilateral facial palsy and rehabilitated with lengthening temporalis myoplasty. Evaluation consisted of objective measures: smile horizontal symmetry between left and right sides, vertical symmetry, and smile width on healthy and paretic sides on preoperative and postoperative photographs. Subjective scales were also used (i.e., a numeric scale and the Terzis and Noah scale) by a jury (four professionals and four nonprofessionals) and the patient himself or herself. Each evaluation was performed in three conditions: at rest, at intermediary smile, and at maximum smile. RESULTS: Comparison of objective measures on the impaired side showed a postoperative improvement in the three conditions evaluation. Reproducibility of the numeric scale was weak for evaluation at rest and fair for maximum smile evaluation (intraclass correlation coefficient of 0.57). The Terzis and Noah scale was not reproducible from one observer to another. At maximum smile, a correlation between smile symmetry in the vertical plane, smile symmetry in the horizontal plane, and professional evaluation with the numeric scale on the one hand and global patient satisfaction on the other hand was observed. CONCLUSION: Postoperative smile horizontal symmetry between left and right sides, and smile vertical symmetry, are good indicators with which to assess postoperative results of facial palsy rehabilitation.

Supramaximal stimulation during intraoperative facial nerve monitoring as a simple parameter to predict early functional outcome after parotidectomy.

CONCLUSION: A supramaximal stimulation at 2 mA during intraoperative electromyographic (EMG) facial nerve monitoring appears to be a simple and effective parameter to predict immediate postoperative injury. OBJECTIVES: To assess the role of systematic intraoperative facial nerve monitoring in predicting the early functional outcomes obtained after parotidectomy. METHODS: Data were collected from patients who underwent parotidectomy. Intraoperative EMG monitoring of the facial nerve was performed by registering two parameters, event intensity (>100 µV) and amplitude of response after a supramaximal stimulation at 2 mA, at the beginning and end of gland removal. Early postoperative clinical functional facial nerve disorder was assessed at day 2. RESULTS: Overall, 50 patients were included and an early facial dysfunction was detected in 27 cases (54%). The maximal response amplitude after supramaximal stimulation at the trunk of the facial nerve was higher in patients with normal facial function compared with those with poor outcomes at the end of surgery (p < 0.01). The postdissection to predissection ratios of maximal response amplitude, but not the stimulation thresholds, were indicative of a nerve conduction block and were significantly lower in the patient group with a poor outcome compared with the group with a normal facial outcome (p < 0.02).

Temporal breach management in chronic otitis media.

In a retrospective study performed at the Otolaryngology, Head and Neck Surgery Department, Pitié-Salpêtrière Hospital, Paris from 1991 to March 2007, we determined surgical procedures for the treatment of tegmen breaches in chronic otitis media. Forty-two cases were examined: 76% corresponded to chronic otitis media with cholesteatoma, and 24% to chronic otitis media without cholesteatoma. Twenty-eight cases were operated using a combined approach, eight cases using a single suprapetrous approach, and six cases using a transmastoid approach. A total of 33% of the cases showed a meningocele or a meningoencephalocele treated through either a combined or a suprapetrous approach. No recurrence or neural/meningeal infectious involvement was found after a mean time of 43 months in the 36 long-term follow-up cases operated through the combined or suprapetrous approaches. Two cases included in the study were a loss to follow-up. Three of the former cases had already been operated for supracentimetric fissure using lower approach. Two out of the six patients operated using lower approach presented post surgery cerebrospinal fluid otorrhea. Combined or suprapetrous approaches seem to be best adapted to the treatment of supracentimetric or recurrent tegmen breaches, as well as to the precise examination and repair of meningeal lesions. Treatment for tegmen breach can be achieved in a single intervention, even when there is an ongoing infection of the middle ear. The mastoid approach should be used only for infracentimetric defects when there is no neural/meningeal lesion.