Stapes surgery in osteogenesis imperfecta: retrospective analysis of 18 operated ears.

PURPOSE: To evaluate short and long term results of stapes surgery in patients with osteogenesis imperfecta (OI), METHODS: Retrospective case series of 18 primary stapes surgeries performed on 11 hearing-impaired OI patients with evidence of stapes fixation, in a Tertiary referral center. We analysed pre-operative and post-operative hearing results at 1 month and at least 1 year RESULTS: The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10 dB in 46% of the cases and to within 15 dB in 92% of the cases at 1-year follow-up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4 kHz) was 18.4 dB at 1 month and 22.4 dB at 1 year. CONCLUSION: Stapes surgery in OI gives good results with few complications in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease.

Alteration of Swallowing and Ventilation Coordination in Respiratory Diseases in Small Mammals.

In human and animal, swallowing and ventilation are coordinated to avoid laryngeal penetration or tracheal aspiration, and respiration is interrupted during swallowing and resumes in the expiratory phase. Some respiratory diseases may alter this coordination due to impaired lung function. The aim of this study was to evaluate swallowing-ventilation coordination in unrestrained rats with pulmonary emphysema or with pulmonary fibrosis. Two protocols were performed in 38 male Sprague-Dawley rats (2-3 months, 250-300 g): pulmonary emphysema and pulmonary fibrosis. Ventilation during swallowing and swallowing-ventilation coordination were recorded using a whole-body plethysmography and video recordings. The rats were free to take water after 24 h without drinking. Compared to control groups, swallowing frequency was significantly higher in rats with pulmonary emphysema and there was a decrease of swallows during expiration in rats with pulmonary emphysema and in rats with pulmonary fibrosis. In this study, we show that respiratory diseases are associated with changes in swallowing-ventilation coordination and hypothesize that they increase the risk of aspiration.

Clofarabine in combination with a standard remission induction regimen (cytosine arabinoside and idarubicin) in patients with previously untreated intermediate and bad-risk acute myelogenous leukemia (AML) or high-risk myelodysplastic syndrome (HR-MDS): phase I results of an ongoing phase I/II study of the leukemia groups of EORTC and GIMEMA (EORTC GIMEMA 06061/AML-14A trial).

This study aims to determine the maximum tolerated dose (MTD) of clofarabine combined with the EORTC-GIMEMA 3 + 10 induction regimen (idarubicin + cytosine arabinoside) in adults with untreated acute myelogenous leukemia or high-risk myelodysplastic syndrome. In this phase I trial, 25 patients (median age 56 years) received 5 days of clofarabine as 1-h infusion (arm A) or push injection (arm B) at the dose level of 5 × 10 or 5 × 15 mg/m(2)/day in an algorithmic dose escalation 3 + 3 design. A consolidation course (intermediate dose cytosine arabinoside, idarubicin) was planned for patients in complete remission (CR). Primary endpoint was safety and tolerance as measured by dose limiting toxicity (DLT); secondary endpoints were response rate, other grade III/IV toxicities, and hematological recovery after induction and consolidation. Five DLTs were observed (in arm A: one DLT at 10 mg/m(2)/day, three at 15 mg/m(2)/day; in arm B: one DLT at 15 mg/m(2)/day). Three patients receiving 15 mg/m(2)/day were withdrawn due to adverse events not classified as DLT. Prolonged hypoplasia was observed in five patients. CR + complete remission with incomplete recovery were achieved in 21 patients (11/12 (92 %) receiving clofarabine 10 mg/m(2)/day; 10/13 (77 %) receiving clofarabine 15 mg/m(2)/day). Clofarabine, 5 × 10 mg/m(2)/day, resulted in one DLT and no early treatment withdrawals. MTD of clofarabine combined with cytosine arabinoside and idarubicin is 5 × 10 mg/m(2)/day.

Laryngeal graft after total laryngectomy in humans: A SWiM analysis.

Evaluation of the results of laryngeal transplantation (LT) in humans. Analysis of 3 bibliographic databases with the keywords "larynx, transplantation, autograft". In total, 626 abstracts were read and 25 articles selected. The main objective was to analyze the characteristics of laryngeal transplant patients. The accessory objectives comprised analysis of operative technique, immunosuppressive treatment and results. Four articles were selected for analysis. Two patients were transplanted after total laryngectomy for laryngeal carcinoma and 2 after laryngeal trauma. Three of the 4 patients had true transplantation with arterial, venous and neural microanastomosis. Two patients were decannulated and the tracheostomy tube was maintained in the other 2. Three of the 4 patients had good-quality phonation and could feed without a gastric tube. One patient died of carcinoma progression and 1 patient had to be explanted 14 years after transplantation. The number of LTs reported is too small for scientific determination of the place of this intervention in laryngology. The published results could, at first sight, suggest that the future of LT is uncertain. However, several elements, also suggest that otolaryngologists should continue to take an interest in this technique.

Audiological phenotyping evaluation in KBG syndrome: Description of a multicenter review.

OBJECTIVES: Our objective was to reinforce clinical knowledge of hearing impairment in KBG syndrome. KBG syndrome is a rare genetic disorder due to monoallelic pathogenic variations of ANKRD11.The typical phenotype includes facial dysmorphism, costal and spinal malformation and developmental delay. Hearing loss in KBG patients has been reported for many years, but no study has evaluated audiological phenotyping from a clinical and an anatomical point of view. METHODS: This French multicenter study included 32 KBG patients with retrospective collection of data on audiological features, ear imaging and genetic investigations. RESULTS: We identified a typical audiological profil in KBG syndrome: conductive (71%), bilateral (81%), mild to moderate (84%) and stable (69%) hearing loss, with some audiological heterogeneity. Among patients with an abnormality on CT imaging (55%), ossicular chain impairment (67%), fixation of the stapes footplate (33%) and inner-ear malformations (33%) were the most common abnormalities. CONCLUSION: We recommend a complete audiological and radiological evaluation and an ENT-follow up in all patients presenting with KBG Syndrome. Imaging evaluation is necessary to determine the nature of lesions in the middle and inner ear.

Acute mastoiditis in children: 10 years experience in a French tertiary university referral center.

Acute mastoiditis have been increasingly reported in the last decade, with bacteriologic modifications and new therapeutic guidelines. This study is a retrospective review of 36 children hospitalized for acute mastoiditis in a French tertiary university referral center from 1999 to 2009, to assess incidence, bacteriology of pathogens and management. There were 10 periosteitis and 26 subperiosteal abscesses. There was a trend toward increased incidence over the past 10 years. The mean age of the patients was 31.8 months. A total of 63.2% children received 7.2 days antibiotic prior to hospitalization for acute otitis media, with sensitive pathogens in 80%. The pathogens were Streptococcus pneumoniae (36.1%), S. pyogenes A (13.9%), Staphylococcus coagulase-negative (13.9%), Pseudomonas aeruginosa (8.3%), Fusobacterium necroforum (8.3%) and Haemophiluss influenzae (2.8%). Cultures were negative in 16.7%. All patients received intravenous antibiotics. Eleven patients underwent bilateral myringotomy with or without tympanostomy tubes. Mastoidectomy was performed in 24 patients. Decrease in the length of hospitalization and delay from admission to surgery were significantly correlated. We observed a trend in the increase of acute mastoiditis at our center. The pathogens were dominated by S. pneumoniae. F. necroforum and P. aerguginosae were pathogens found in children over 2 years of age. Mastoidectomy was performed in 92.3% of subperiostal abscesses. However, there has been a trend toward conservative nonsurgical treatment in recent reported studies and further prospective studies are warranted to evaluate the long-term sequelae.

Ruptured dural arteriovenous fistula and sinus venous thrombosis following surgical resection of a vestibular schwannoma: Case report and review of the literature.

Dural arteriovenous fistula (DAVF) is a rare vascular malformation. Strong evidence suggests that the development of DAVF in adults is acquired and multifactorial. The link between cerebral venous thrombosis and DAVF is probably explained by dynamic changes in the venous drainage pattern. We report the case of a 34-year-old man admitted to the emergency department for seizure and headaches. The patient had a medical history of right vestibular schwannoma resection 9 months earlier, complicated by untreated asymptomatic sigmoid sinus thrombosis. At admission, CT scan revealed a spontaneous temporal intracerebral hemorrhage associated with ventricular hemorrhage due to the rupture of a DAVF diagnosed by complementary CT angiography. External ventricular drainage was performed in emergency, followed by endovascular exclusion of the DAVF. Good neurological outcome was achieved, with complete exclusion of the vascular malformation. This clinical case underlines the absence of guidelines on the use of anticoagulation drugs to treat postoperative venous sinus thrombosis and to potentially prevent DAVF as a late complication following cerebellopontine angle surgery.

Short-term hearing outcome of malleus removal for surgery: An observational cohort study.

OBJECTIVES: The main goal of the present study was to assess hearing outcome for malleus removal in cholesteatoma surgery compared to a group with malleus conservation. The secondary aim was to compare the auditory involvement of the stapes between the two groups. MATERIAL AND METHODS: A single-center observational study included adult patients operated on by ossiculoplasty for acquired cholesteatoma between 2015 and 2019. Endpoints comprised improvement in pure-tone average air-bone gap (PTA-ABG) and air-bone gap (ABG) at conversational frequencies and, independently, per frequency. 136 patients were included: 95 with conserved malleus (M+) and 41 with malleus removal (M(). Mean time to audiometric follow-up was 9 months in both groups. RESULTS: The PTA-ABG improvement was 4.4±12.6dB for the M+ group and 3.8±13.4dB for the M- group, with no significant significance (P=0.8). Better results (not exceeding 7.5dB) were found for the M+ group at 2 and 8kHz (P=0.3 and P=0.052 respectively). Presence or absence of the stapes did not affect the results in either group. CONCLUSION: Those results suggest a negligible role of the malleus in early hearing outcome of tympanoplasty for cholesteatoma. A slight improvement was observed in the M+ group at 2 and 8kHz, but its interpretation remains uncertain.

[Role of hypnotherapy in the treatment of debilitating tinnitus]. / Intérêt de l'hypnose dans le traitement des acouphènes invalidants.

OBJECTIVE: Hypnotherapy is currently used for tinnitus therapy in our university hospital. The aim of this study was to evaluate its efficacy. MATERIAL AND METHODS: This study was performed on 110 patients suffering from distressing tinnitus. They were treated during five sessions with hypnotherapy, supplemented by instruction on self-hypnotherapy. A subjective evaluation was done by the practitioner at the end of the sessions of hypnotherapy. Then a questionnaire on psychologic distress (Wilson 1991) was sent retrospectively to the patients. RESULTS: We received 65 responses which were used for this study. Before treatment, the mean value of the Wilson score was 54 (28-104). After treatment, it was: 31 (0-86). 69% of the patients felt an improvement > or = 5 points Wilson score. These results were compared with the evaluation carried out by the practitioner at the end of the sessions of hypnosis. There was a "significant correlation" between the evaluation of the felt benefice, analyzed by the practitioner at the end of the sessions of hypnosis, and by the patient questioned long after the treatment. These results had significant correlation with the evaluation made by the therapist at the end of the five sessions of hypnotherapy. They show, how effective (68% improvement) this therapeutic approach can be. CONCLUSION: Hypnotherapy can be regarded as an effective treatment against distressing tinnitus.

[Vocal cord dysfunction in athletes: Interest of rehabilitation to effort]. / Dysfonction des cordes vocales chez le sportif : intérêt du reconditionnement à l'effort.

INTRODUCTION: Vocal Cord Dysfunction (VCD) is a syndrome characterized by paradoxical adduction of the vocal folds during breathing. Its non-specific clinical manifestations frequently lead to misdiagnosis and delay in its treatment. The treatment of VCD is not pharmacological but rehabilitative and remains poorly appreciated. OBSERVATION: In this clinical case we describe a 16-year-old female judoka who presented with effort intolerance associated with occasional dyspnea, which had suddenly worsened over the preceding few months so that in now produced sudden respiratory difficulty, mainly during intense and sudden efforts. After a period where her symptoms were confused with asthma, she was diagnosed with exercise-induced VCD. Her treatment was aimed to: (a) rehabilitate respiratory mechanics in order to eliminate abdominal-thoracic asynchrony, (b) rehabilitate naso-nasal breathing, (c) train her to control abdominal-diaphragmatic breathing at rest and then during effort, (d) train her to use ventilatory control as soon as prodromal symptoms appear in order to prevent the development of stridor or complete closure of the vocal folds (at rest and then progressively during exercise). CONCLUSION: Following the implementation of these rehabilitation strategies to correct her ventilatory and dyskinetic issues, the patient no longer develops respiratory discomfort during exercise, including during judo competitions.

Endoscopic revision of external dacryocystorhinostomy failure.

OBJECTIVE: To evaluate the usefulness of endoscopic analysis and surgery of the lacrimal sac in cases of external dacryocystorhinostomy (DCR) failure. MATERIAL & METHOD: In a retrospective study, 17 endoscopic procedures in 17 patients during 11 years with recurrent lacrimal obstruction after external DCR were performed. Endoscopic analysis and procedures were carried out with a routine silicone tube catheterization for 3 to 6 months. RESULTS: In 13 patients, scar tissue was the cause of the obstacle, while in 3 patients an unsuitable location of the ostia and in 1 case an inflammatory polyp were found. After a long-term follow-up (56 months), the epiphora was controlled in 94% of the cases. The mean delay between the first DCR and the recurrence of epiphora was 22 months. CONCLUSION: A persistent or recurrent epiphora can be explored after an external procedure and treated by endoscopic procedure. The endonasal approach for DCR was considered safe, and effective particularly in patients with unsuccessful external DCR.

The Regenerative Effect of Trans-spinal Magnetic Stimulation After Spinal Cord Injury: Mechanisms and Pathways Underlying the Effect.

Spinal cord injury (SCI) leads to a loss of sensitive and motor functions. Currently, there is no therapeutic intervention offering a complete recovery. Here, we report that repetitive trans-spinal magnetic stimulation (rTSMS) can be a noninvasive SCI treatment that enhances tissue repair and functional recovery. Several techniques including immunohistochemical, behavioral, cells cultures, and proteomics have been performed. Moreover, different lesion paradigms, such as acute and chronic phase following SCI in wild-type and transgenic animals at different ages (juvenile, adult, and aged), have been used. We demonstrate that rTSMS modulates the lesion scar by decreasing fibrosis and inflammation and increases proliferation of spinal cord stem cells. Our results demonstrate also that rTSMS decreases demyelination, which contributes to axonal regrowth, neuronal survival, and locomotor recovery after SCI. This research provides evidence that rTSMS induces therapeutic effects in a preclinical rodent model and suggests possible translation to clinical application in humans.

Retropharyngeal lipostructure in the treatment of velopharyngeal insufficiency: A prospective study and update.

INTRODUCTION: Retropharyngeal lipostructure is a recent procedure in velopharyngeal insufficiency (VPI), offering an effective alternative to heavier surgery. OBJECTIVES: To update and assess retropharyngeal lipostructure as a treatment for VPI in the University Hospital Center of Rouen (France). TYPE OF STUDY: Single-center prospective study, from May 2012 to May 2014. PATIENTS AND METHODS: Six patients (4 girls, 2 boys) presenting with VPI were treated by retropharyngeal lipostructure. Age at surgery ranged between 6 and 12 years. Four of the patients bore a 22q11 microdeletion. Treatment was indicated in case of Borel-Maisonny type 2b (n=2) or 2m (n=4) despite well-conducted speech therapy and of≥50% velopharyngeal sphincter closure on nasal endoscopy. Patients were assessed preoperatively and at 3 months, by a multidisciplinary team. Borel-Maisonny type was assessed by a speech therapist. Nasality was measured on assisted vocal evaluation (EVA®). Sphincter closure was assessed on dynamic MRI. RESULTS: Between 6 and 8cm3autologous fat was injected. At 3months, 4 children showed 1-grade improvement in Borel-Maisonny type. Nasality decreased systematically, from a mean 14.5% preoperatively to 10.5% postoperatively. MRI showed improvement in all cases, with complete closure in occlusive vowels in 3 children. CONCLUSION: EVA® and MRI provide precise objective assessment of VPI. Retropharyngeal lipostructure is a simple, relatively non-invasive, reproducible technique, providing good results in VPI.

Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI.

PURPOSE: The purpose of this study was to assess whether the volume of the vestibular endolymphatic space correlates with the degree of hearing loss using heavily T2-weighted fast imaging employing steady-state acquisition with cycle phase (FIESTA-C) MRI. MATERIALS AND METHODS: A total of 23 patients with vestibular schwannoma, as diagnosed on typical image findings, who underwent FIESTA-C MRI were included. There were 13 women and 10 men with a mean age of 63.5±9.3 (SD) years (range: 49-88years). Two radiologists independently evaluated the volume of the utricle and saccule. Correlation between tumor volume, vestibular endolymphatic space volume and degree of hearing loss - as evaluated with the levels of pure-tone average and speech recognition threshold - were searched for. RESULTS: The mean saccular, utricular and tumor volumes were 3.17±1.1 (SD) mm3 (range: 1.45-5.7mm3), 14.55±5 (SD) mm3; (range: 6.6-23.9mm3) and 17.4±5.5 (SD) mm3; (range: 8.3-25.4mm3), respectively. There was a moderate correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of pure-tone average (rho=0.5; P=0.015) and speech recognition threshold (rho=0.58; P=0.004). There were no significant correlations between saccular and tumor volumes and the degree of hearing loss. CONCLUSION: The volume of the utricle in patients with obstructive vestibular schwannoma moderately correlates with the degree of hearing loss.

[Comparison of two antibioprophylaxis protocols in ear surgery]. / Comparaison de deux protocoles d'antibioprophylaxie dans la chirurgie de l'oreille.

INTRODUCTION: During surgical procedure, antibioprophylaxis is known to decrease bacterial proliferation and limit postoperative complications such as infections. In France, antibiotic prescription guidelines have been established for ear surgery, but applied with discrepancies. The purpose of the study was to evaluate the necessity of antibioprophylaxis in ear surgery. MATERIAL AND METHODS: Retrospective study of two consecutives series of ear surgery with two different antibioprophylaxis protocols. In the first series (n=100), antibioprophylaxis by amoxicillin and clavulanic acid was given only in cases of chronic otitis media with otorrhea and cholesteatoma (contaminated surgery). In the second series (n=107), no antibiotic was administered. The number of infected complications was evaluated by reviewing medical charts. RESULTS: The percentage of infected complications was 5% versus 6.5% in the second series (with no antibioprophylaxis), for all types of ear surgery, 9.4% versus 4.2% after ear contaminated surgery. No statistical difference was observed between the two series. CONCLUSION: In ear surgery, postoperative infected complications do not more frequently occur without antibioprophylaxis, either in otorrhea or in cholesteatoma surgery.

[Laryngeal schwannomas]. / Schwannomes du larynx.

Laryngeal schwannoma is a rare benign tumour of the larynx. Schwannomas derive from the Schwann cells. The majority occurs in supraglottic area and may arise at any age. Magnetic resonance imaging (MRI) is the best imaging tool for suggesting the diagnosis in the appropriate clinical context. Conservative surgery is the treatment of choice. These tumors have to be distinguished from neurofibromas. We report 2 cases of laryngeal schwannoma. The first one concerned an 8-month-old baby with a laryngeal stridor history which seemed to be related to laryngomalacia. The second case was a 22-year-old man with a 5-year history of dysphonia. On nasofibroscopic examination, a sub mucosal mass enlarged the posterior part of the right false vocal cord in both cases. Both CT scan and MRI sequences showed a well margined lesion in the same place. Histological biopsies identified a schwannoma. External surgeries were subsequently and successfully performed. Diagnosis (imaging), specific treatment (endoscopic or external surgery), and possible association of laryngeal schwannomas are discussed.

Modified first or second cervical nerve transplantation technique for the treatment of recurrent laryngeal neuropathy in horses.

BACKGROUND: In horses, the only established method for reinnervation of the larynx is the nerve-muscle pedicle implantation, whereas in human medicine, direct nerve implantation is a standard surgical technique for selective laryngeal reinnervation in human patients suffering from bilateral vocal fold paralysis. OBJECTIVES: (1) To describe a modified first or second cervical nerve transplantation technique for the treatment of recurrent laryngeal neuropathy (RLN) in horses and (2) evaluate the outcomes of reinnervation using direct nerve needle-stimulation of the first cervical nerve and exercising endoscopy before and after surgery. STUDY DESIGN: Case series. METHODS: Nerve transplantation surgery, in which the first or second cervical nerve is tunnelled through the atrophied left cricoarytenoideus dorsalis muscle, was performed in combination with ipsilateral laser ventriculocordectomy. Ultrasound-guided stimulation of the first cervical nerve at the level of the alar foramen was used to confirm successful reinnervation post-operatively. Exercising endoscopy was performed before and after surgery. The exercising RLN grade of the left arytenoid was blindly determined at the highest stride frequency for each examination. RESULTS: Surgery was performed in 17 client-owned animals with RLN. Reinnervation was confirmed by nerve stimulation and subsequent arytenoid abduction observed in 11 out of 12 cases between 4 and 12 months post-operatively. Fourteen horses had exercising endoscopy before and after surgery. Nine horses had an improved exercising RLN grade, four horses had the same exercising grade and one horse had a worse exercising grade after surgery. MAIN LIMITATIONS: A sham-operated control group was not included and follow-up beyond 12 months and objective performance data were not obtained. CONCLUSIONS: The modified first or second cervical nerve transplantation technique, using tunnelling and direct implantation of the donor nerve into the cricoarytenoideus dorsalis muscle, resulted in reinnervation in 11 out of 12 cases and improved exercising grade in 9 out of 14 horses within 12 months after surgery.

Treatment of unilateral vocal fold paralysis with ansa cervicalis to recurrent nerve anastomosis in a young adolescent: European case report.

BACKGROUND: Laryngeal re-innervation in paediatric unilateral vocal fold paralysis is a relatively new treatment option, of which there has been little reported experience in Europe. METHODS: In this European case report of a 13-year-old boy with dysphonia secondary to left-sided unilateral vocal fold paralysis after cardiac surgery, the patient underwent re-innervation using an ansa cervicalis to recurrent laryngeal nerve transfer, in combination with fat augmentation, after 12 years of nerve denervation. Perceptual analysis data, and acoustic and laryngoscopy recordings were acquired pre-operatively, and at one and two years post-operatively. RESULTS: The patient's perceptual voice quality was improved. He experienced subjective improvement and is very satisfied with the result. As expected, laryngoscopy at one and two years after surgery showed no physiological mobility of the vocal fold concerned, but improved closure during phonation was achieved. Electromyography showed evidence of re-innervation. CONCLUSION: Laryngeal re-innervation could be considered as a treatment option for unilateral vocal fold paralysis in children and adolescents, even after a long-term delay.

Mylohyoid motor-evoked potentials relate to swallowing function after chronic stroke dysphagia.

This work aimed to study mylohyoid motor-evoked potentials (MHMEPs) and examined if it is related to chronic stroke dysphagia. Conduction time (CT) and amplitudes of the right and left MHMEPs in response to focal cortical magnetic stimulations over affected and unaffected hemispheres were recorded in 16 stroke patients with aspiration (n = 9) or residue (n = 7) and compared with eight control patients. In control group, MHMEPs were present on ipsilateral and contralateral sides after stimulation of both hemispheres and permitted to determine a dominant hemisphere. In stroke patients, after stimulation of the affected hemisphere, ipsilateral MEPs had a longer CT and lower amplitudes in patients with aspiration compared with patients with residue or control patients (P < 0.05). Contralateral CT was not different between the three groups, but amplitudes were lower in patients with residue and aspiration than in control patients (P < 0.01). In the unaffected hemisphere, MHMEPs were present, and not different between the three groups for the ipsilateral side and amplitudes were decreased in contralateral side in patients with residue. In conclusion, MHMEP alterations of the affected hemisphere related to chronic stroke dysphagia severity and were closed to normal in the unaffected hemisphere.