Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use.

The objective of this study is to evaluate the safety and efficacy of a new transcutaneous bone-conduction implant (BCI BB) in patients with conductive and mixed hearing loss or with single-sided deafness (SSD), 1 year after surgical implantation. The study design is multicentric prospective, intra-subject measurements. Each subject is his/her own control. The setting is nine university hospitals: 7 French and 2 Belgian. Sixteen subjects with conductive or mixed hearing loss with bone-conduction hearing thresholds under the upper limit of 45 dB HL for each frequency from 500 to 4000 Hz, and 12 subjects with SSD (contralateral hearing within normal range) were enrolled in the study. All subjects were older than 18 years. The intervention is rehabilitative. The main outcome measure is the evaluation of skin safety, audiological measurements, benefit, and satisfaction questionnaires with a 1-year follow up. Skin safety was rated as good or very good. For the mixed or conductive hearing loss groups, the average functional gain (at 500 Hz, 1, 2, 4 kHz) was 26.1 dB HL (SD 13.7), and mean percentage of speech recognition in quiet at 65 dB was 95 % (vs 74 % unaided). In 5/6 SSD subjects, values of SRT in noise were lower with BB. Questionnaires revealed patient benefit and satisfaction. The transcutaneous BCI is very well tolerated at 1-year follow up, improves audiometric thresholds and intelligibility for speech in quiet and noise, and gives satisfaction to both patients with mixed and conductive hearing loss and patients with SSD.

Post-traumatic balance disorder.

The causes of balance disorder are many and various, and the subjective syndrome of cranial trauma patients is diagnosed by elimination. Progress in otoneurologic functional exploration and brain imaging, however, now generally allow this functional complaint to be given an objective basis. In recent years, new diagnoses have improved recognition of such pathologies in the appraisal of corporal injury for compensation purposes. The present article seeks to detail etiology and, by a review of the literature, to determine factors liable to influence management and appraisal in particular.

Olfactory stem cells reveal MOCOS as a new player in autism spectrum disorders.

With an onset under the age of 3 years, autism spectrum disorders (ASDs) are now understood as diseases arising from pre- and/or early postnatal brain developmental anomalies and/or early brain insults. To unveil the molecular mechanisms taking place during the misshaping of the developing brain, we chose to study cells that are representative of the very early stages of ontogenesis, namely stem cells. Here we report on MOlybdenum COfactor Sulfurase (MOCOS), an enzyme involved in purine metabolism, as a newly identified player in ASD. We found in adult nasal olfactory stem cells of 11 adults with ASD that MOCOS is downregulated in most of them when compared with 11 age- and gender-matched control adults without any neuropsychiatric disorders. Genetic approaches using in vivo and in vitro engineered models converge to indicate that altered expression of MOCOS results in neurotransmission and synaptic defects. Furthermore, we found that MOCOS misexpression induces increased oxidative-stress sensitivity. Our results demonstrate that altered MOCOS expression is likely to have an impact on neurodevelopment and neurotransmission, and may explain comorbid conditions, including gastrointestinal disorders. We anticipate our discovery to be a fresh starting point for the study on the roles of MOCOS in brain development and its functional implications in ASD clinical symptoms. Moreover, our study suggests the possible development of new diagnostic tests based on MOCOS expression, and paves the way for drug screening targeting MOCOS and/or the purine metabolism to ultimately develop novel treatments in ASD.

Treatment of a mastoid defect by free anterolateral thigh flap.

INTRODUCTION: Free anterolateral thigh (ALT) flap constitutes an alternative to latissimus dorsi and radial forearm flaps for head and neck reconstruction. The authors report a case of mastoid reconstruction in a patient with multi-operated cholesteatoma associated with tubal dysfunction. CASE REPORT: This patient presented a fistula of the lateral semicircular canal and invagination of retroauricular soft tissues responsible for non-compensated almost complete areflexia with anacusis. She did not present any signs of cholesteatoma recurrence, but chronic inflammation of the mastoidectomy cavity. The head and neck procedure consisted of translabyrinthine labyrinthectomy: resection of the atrophic retroauricular skin, resection of the vestibular neurosensory tissue and obliteration of the mastoidectomy cavity. An ALT flap measuring 5×5 cm, anastomosed to the superior thyroid artery and facial vein, was used to cover the defect. Careful defatting of the flap allowed filling of the defect, while providing a sufficient quantity of appropriate tissue. The postoperative course was uneventful and the patient was considerably improved. The only donor site sequelae consisted of a thin linear scar. CONCLUSION: The free anterolateral thigh flap, a reliable, polyvalent flap that can be shaped as needed and which is associated with minimal donor site morbidity, constitutes a good alternative for head and neck reconstruction.

Vestibular compensation following vestibular neurotomy.

OBJECTIVES: Four studies assessing vestibular compensation in Menière's disease patients undergoing unilateral vestibular neurotomy, using different analysis methods, are reviewed, with a focus on the different strategies used by patients according to their preoperative sensory preference. MATERIAL AND METHODS: Four prospective studies performed in a university tertiary referral center were reviewed, measuring the pattern of vestibular compensation in Menière's disease patients before and after unilateral vestibular neurotomy on various assessment protocols: postural syndrome assessed on static posturography and gait analysis; perceptual syndrome assessed on subjective visual vertical perception; and oculomotor syndrome assessed on ocular cyclotorsion. RESULTS: Vestibular compensation occurred at variable intervals depending on the parameter investigated. Open-eye postural control and gait/walking returned to normal one month after neurotomy. Fine balance analysis found that visual perception of the vertical and ocular cyclotorsion impairment persisted at long-term follow-up. Clinical postural disturbance persisted only when visual afferents were cut off (eyes closed). These impairments were the expression of a postoperative change in postural strategy related to the new use of visual and non-visual references. CONCLUSIONS: Understanding pre-operative interindividual variation in balance strategy is critical to screening for postural instability and tailoring vestibular rehabilitation.

Effects of haptic supplementation on postural stability in unilateral vestibular loss patients.

The effects of haptic supplementation on postural stability during upright standing with vision were investigated in 11 unilateral vestibular loss (UVL) patients and compared to 12 age-marched controls. All the participants were instructed to grip a fixed or mobile stick. In the mobile conditions, the lower extremity of the stick was in contact with a slippery or a rough surface. Postural stability was assessed by the root mean square (RMS) and the velocity of the center of foot pressure (CoP) displacements in both antero-posterior (AP) and medio-lateral (ML) directions. Results showed that RMS was larger in UVL patients than in aged-matched participants in AP direction. However, in the mobile stick conditions, only the rough surface improved postural stability. In addition, RMS was more sensitive to haptic supplementation than CoP velocity. The present study confirms the importance of haptic information in the multi-sensory (re) weighting process underlying postural control in UVL patients.

Mini-invasive surgery of infratemporal fossa schwannomas.

BACKGROUND: Infratemporal fossa schwannomas are benign, encapsulated tumours of the trigeminal nerve limited to the infratemporal fossa. Because of the complications and significant morbidity associated with traditional surgical approaches to the infratemporal fossa, which include facial nerve dysfunction, hearing loss, dental malocclusion and cosmetic problems, less invasive alternatives have been sought. METHODS: This paper reports two cases of infratemporal fossa schwannomas treated in 2012 using mini-invasive approaches. The literature regarding different infratemporal fossa approaches was reviewed. RESULTS: The first schwannoma was 30 mm in size and was removed completely by a preauricular subtemporal approach. The second one was 25 mm in size and was removed completely using a purely transnasal endoscopic approach. In both cases, there were no intra-operative or post-operative complications. CONCLUSION: These two approaches allow non-invasive and wide exposure of the infratemporal fossa as compared to classical approaches. Surgical approach should be selected according to the tumour's anatomical location with respect to the maxillary sinus posterior wall. The preauricular subtemporal approach is recommended for tumours localised posterolaterally with respect to the maxillary sinus posterior wall. Medial and anterior tumours near the maxillary sinus posterior wall can be best removed using a transnasal endoscopic approach.

Switching from a percutaneous to a transcutaneous bone anchored hearing system: the utility of the fascia temporalis superficialis pedicled flap in case of skin intolerance.

OBJECTIVES: To present the option to use a Fascia Temporalis Superficialis pedicled flap for the implantation of a transcutaneous magnetic osseointegrated auditory implant in selected revision procedures. BACKGROUND: Osseointegrated auditory implants represent a reliable option for patients. Skin intolerance and aesthetic issue are two clear limitations of percutaneous system. Magnetic transcutaneous devices have been developed to overcome such drawbacks. The current new experience of such a device shows that they are well tolerated in primary cases. However, switching from a previous percutaneous system raises the question of skin tolerance. Hence, previous implanted abutments with skin penetration and skin reduction are more likely cause to skin intolerance and complication. METHODS: We described here the option to use a Fascia Temporalis Superficialis flap to cover the implanted magnet. CONCLUSION: When switching from percutaneous osseointegrated auditory implant to transcutaneous system in case of high risk of skin intolerance, this procedure may provide sufficient tissue coverage to prevent skin intolerance, pain and delayed extrusion.

Vestibular compensation and vestibular rehabilitation. Current concepts and new trends.

The aim of this review is to present the current knowledge of the mechanisms underlying the vestibular compensation and demonstrating how the vestibular rehabilitation is conducted to help the recovery of balance function. Vestibular rehabilitation is based on improving the natural phenomenon called vestibular compensation that occurs after acute vestibular disturbance or chronic and gradual misbalance. Central compensation implies three main mechanisms namely adaptation, substitution and habituation. The compensation, aided by the rehabilitation aimed to compensate and/or to correct the underused or misused of the visual, proprioceptive and vestibular inputs involved in the postural control. As the strategy of equilibration is not corrected, the patient is incompletely cured and remains with inappropriate balance control with its significance on the risk of fall and impact on quality of life. The vestibular rehabilitation helps to correct inappropriate strategy of equilibrium or to accelerate a good but slow compensation phenomenon. Nowadays, new tools are more and more employed for the diagnosis of vestibular deficit (that may include various sources of impairment), the assessment of postural deficit, the control of the appropriate strategy as well to facilitate the efficiency of the rehabilitation especially in elderly people.

Late pneumolabyrinth after undiagnosed post-traumatic perilymphatic fistula. Case report illustrating the importance of systematic emergency management.

INTRODUCTION: Temporal bone fracture is a common complication of high-energy cranial trauma. Labyrinth involvement is rare, but there is a risk of perilymphatic rupture that is often underestimated on initial clinical examination due to the predominance of neurological and/or somatic symptoms. CASE REPORT: A patient presented with overlooked perilymphatic fistula, decompensated by hyperpressure effort due to poorly adapted management. DISCUSSION: Following a review of the literature on post-traumatic pneumolabyrinth, overall management (from diagnosis to treatment, via prevention advice) was analyzed. A constructive critique of the patient's treatment was thus made. CONCLUSION: We argue for a systematic management protocol in cranial trauma with temporal bone fracture, comprising ENT examination, millimetric-scale cross-sectional imaging of the fracture site, and standardized counseling to prevent late complications.

Transoral surgical treatment of Eagle's syndrome: case report and review of literature.

Eagle's syndrome is a symptomatic abnormal length of the styloid process and/or a calcification of the stylohyoid ligament. Diagnosis is based on common pharyngeal symptoms and is confirmed by radiologic examination, more particularly CT-scan. Medical and surgical treatments are described. Medical treatment consists in repeated local anaesthetics, steroid infiltration or analgesics administration. It is purely symptomatic and has no long-term effectiveness. Surgical treatment remains the treatment of choice and consists of removal of the abnormal process. Both transoral and external approaches have been described. The advantages of the transoral approach is a shorter operative time, the absence of aesthetic prejudice and a lesser risk of neurovascular injury. Few post operative complications have been described (surgical cervical emphysema, cervical swelling, trismus and moderate breathing difficulty) but none of the studies reported neurovascular injury, deep cervical infection nor long-term complication. We present the technique, a case report and a review of the literature of the transoral approach.

[Middle ear barotraumas due to rhinopharyngeal scar tissue: tubomanometry diagnostic and therapeutic contribution]. / Barotraumatismes de l'oreille moyenne par brides rhinopharyngées: intérêt diagnostique et thérapeutique de la tubomanométrie.

OBJECTIVE: Evaluation of tubomanometry contribution to diagnosis middle ear barotraumas in relation with rhinopharyngeal scar tissue, and contribution to check postoperative effectiveness of scar tissue surgical resection. METHODS: Clinical cases study of two stewardesses who have lost their flight fitness consecutively to barotraumatic otitis during landing, engendered by rhinopharyngeal scar tissue. RESULTS: Pre-operative tubomanometric parameters were abnormal in both cases: decrease of intratympanic pressure in one case, variability of tube opening latency index and lengthening of intratympanic pressure rising time in the other case. Surgical section during endonasal endoscopy results in initially abnormal tubomanometric parameters normalization, allowing resumption of flight fitness without any barotrauma. CONCLUSION: Some abnormal tubomanometric parameters help to establish causality link between middle ear barotrauma and rhinopharyngeal scar tissue for which surgical section is thus indicated. Post-operative tubomanometric parameters normalization prove surgical effectiveness. Then, flight fitness could then be restored.

Titanium ossicular chain replacement prostheses: prognostic factors and preliminary functional results.

OBJECTIVE: To assess the efficiency of ossiculoplasty procedures with the Kurz titanium ossicular prosthesis and evaluate prognostic factors for the functional results. METHODS: Retrospective chart reviews were performed for ossiculoplasty involving Kurz titanium prostheses between 2006 and 2009 in the ENT Head and Neck Department of the hôpital Nord, Marseille, France. RESULTS: The population studied was 70 patients, with 37 procedures using the partial (PORP) and 33 the total ossicular replacement prosthesis (TORP). Mean follow-up was 9 months. Pre- and postoperative audiological parameters on four frequency averages (0.5, 1, 2, and 3kHz) were compared according to AAO-HNS guidelines. A postoperative air-bone gap (ABG)≤20dB was obtained in 71.43% of the patients (86.49% for PORP, and 54.55% for TORP). The mean change in ABG was 12.45dB in cholesteatomatous otitis versus 13.41dB in non-cholesteatomatous otitis. CONCLUSION: Increasing the length of the ossicular prosthesis, especially TORP, may improve postoperative functional results.

Comparison of [¹¹¹In]pentetreotide-SPECT and [¹8F]FDOPA-PET in the localization of extra-adrenal paragangliomas: the case for a patient-tailored use of nuclear imaging modalities.

AIMS AND METHODS: The aim of this prospective study was to compare the diagnostic value of [¹8F]FDOPA-PET and [¹¹¹In]pentetreotide-SPECT somatostatin receptor scintigraphy (SRS) in patients with nonmetastatic extra-adrenal paragangliomas (PGLs). Twenty-five consecutive unrelated patients who were known or suspected of having nonmetastatic extra-adrenal PGLs were prospectively evaluated with SRS and [¹8F]FDOPA-PET. ¹³¹I-MIBG and [¹8F]FDG-PET were added to the work-up in patients with a personal or familial history of PGL, predisposing mutations, abdominal PGLs, metanephrine hypersecretion and abdominal foci on SRS and/or [¹8F]FDOPA-PET. RESULTS: SRS correctly detected 23/45 lesions of which 20 were head or neck lesions (H&N) and 3 were abdominal lesions. [¹8F]FDOPA-PET detected significantly more lesions than SRS (39/45, P < 0·001). Both SRS and ¹8F-DOPA-PET detected significantly more H&N than abdominal lesions (66·7% vs 20%, P = 0·003 and 96·7% vs 67%, P = 0·012, respectively). In two patients with the succinate dehydrogenase D (SDHD) mutation, [¹8F]FDOPA-PET missed five abdominal PGLs which were detected by the combination of SRS, [¹³¹I]MIBG and [¹8F]FDG-PET. A lesion-based analysis using a forward stepwise logistic regression model demonstrates that size ≤ 10 mm (P = 0·002) and abdominal lesions (P = 0·031) were independently associated with "[¹8F]FDOPA-PET diagnosis only". In turn, a previous history of surgery and/or the presence of germline mutation was associated with lower lesion size (P = 0·001). CONCLUSIONS: The sensitivity of SRS for localizing parasympathetic PGLs is lower than originally reported, and [¹8F]FDOPA-PET is better than SRS for localizing small lesions. SRS should be replaced by [¹8F]FDOPA-PET as the first-line imaging procedure in H&N PGL, especially in patients at risk of multifocal disease (predisposing mutations and or previous history of surgery).

[An unusual fester of the peri-auricular area: pathomimia in ENT practice]. / A propos d'une ulcération péri-auriculaire atypique: la pathomimie en pratique ORL.

We report a case of a patient who self-injured her ear and peri-auricular skin. Self-mutilation is an unusual behaviour in ENT practice. We give some elements for the diagnosis and treatment, and we emphasize the importance of the psychiatric treatment. The suicidal risk makes the vital prognosis uncertain.

[Paraganglioma of the cerebellopontine angle: report of two cases]. / Paragangliomes de l'angle ponto-cérébelleux: à propos de deux cas.

Paragangliomas of the cerebellopontine angle are exceptional tumours. We report two cases of paragangliomas of the cerebellopontine angle. To our knowledge, including these two cases, only five cases have been reported in the international literature. The emerging field of genetic study of these tumours will prove critical for their diagnosis and prognosis. The presence of paraganglioma in this area is explained by an abnormal embryologic migration of paraganglionic cells into the cerebellopontine angle, associated with tumorogenesis. These tumors can be associated with others cervico-facial tumours and to genomic abnormalities. The preoperative diagnosis of these tumours is very difficult, with clinical signs like hearing loss, vertigo and tinnitus are very ambiguous. Radiologic assessment of the cerebellopontine angle is also vague. The final diagnosis is only made possible peroperatively and by the post-operative histopathologic study. ENT and neurosurgeons should be awarded of the possible existence of paragangliomas of the cerebellopontine angle, and the necessity of a systemic and genetic assessment.

[Contribution of 18FDG PET/CT post treatment surveillance of head and neck squamous cell carcinoma]. / Intérêt de la TEP au 18FDG couplée à la TDM dans la surveillance post-thérapeutique des carcinomes épidermoïdes des voies aérodigestives supérieures.

OBJECTIVE: To evaluate the contribution of 18FDG positron emission tomography for the post treatment surveillance of head and neck carcinomas. METHODS: : This prospective study included 61 patients with advanced squamous cell carcinoma of the upper airways and/or digestive tract and who underwent curative treatment. Patients underwent a standard workup (physical examination, CT scan and panendoscopy) and a PET/CT image fusion 3 months after the end of treatment. The follow-up was 6 months minimum in all patients after this workup. PET/CT was evaluated in terms of: primary tumor, cervical lymph nodes, metastases and overall patient assessment. RESULTS: : For diagnosis of the primary tumor, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of PET/CT were respectively 86.7%, 82.6%, 62%, 95% and 83.6%. Values for cervical lymph nodes were: 100%, 98.2%, 80%, 100% and 98.3%. Values for metastases were: 100%, 92.2%, 66.7%, 100% and 93.2%. Values for overall patient assessment were: 88.8%, 78.1%, 64%, 94.1% and 81.4%. Performances of PET/CT were better than standard workup in 22% of patients. CONCLUSION: PET/CT contributes useful information in this indication, particularly an excellent negative predictive value.