A sudden bilateral hearing loss caused by inner ear hemorrhage.

INTRODUCTION: Labyrinthine hemorrhage is a rare cause of sudden deafness and generally concerns only on one side. CASE SUMMARY: An 84-year-old man with a past medical history of myelomonocytic chronic leukemia (CMML) suffered from sudden bilateral hearing loss associated with vertigo. The audiogram revealed a left cophosis and a right profound deafness. Videonystagmography showed a left vestibular deficit. The MRI showed a spontaneous strong T1 weighted signal in the left and right labyrinths, corresponding to a bilateral inner ear hemorrhage (IEH). Dizziness resolved rapidly following vestibular physiotherapy, in contrast to hearing which did not improve at all and let the patient isolated in his environment. The patient successfully underwent cochlear implantation so that he could communicate. DISCUSSION: Most IEHs are unilateral and due to anticoagulants treatments and hematological diseases. Only rare cases have described bilateral labyrinth hemorrhage. This is the first case reported of bilateral labyrinth hemorrhage due to CMML.

Implication of the SH3TC2 gene in Charcot-Marie-Tooth disease associated with deafness and/or scoliosis: Illustration with four new pathogenic variants.

The autosomal recessive demyelinating form of Charcot-Marie-Tooth can be due to SH3TC2 gene pathogenic variants (CMT4C, AR-CMTde-SH3TC2). We report on a series of 13 patients with AR-CMTde-SH3TC2 among a French cohort of 350 patients suffering from all type of inheritance peripheral neuropathy. The SH3TC2 gene appeared to be the most frequently mutated gene for demyelinating neuropathy in this series by NGS. Four new pathogenic variants have been identified: two nonsense variants (p.(Tyr970*), p.(Trp1199*)) and two missense variants (p.(Leu1126Pro), p.(Ala1206Asp)). The recurrent variant p.Arg954* was present in 62%, and seems to be a founder mutation. The phenotype is fairly homogeneous, as all these patients, except the youngest ones, presented scoliosis and/or hearing loss.

Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on cervical lymphatic malformation in adults and children: Diagnosis.

OBJECTIVES: The authors present the guidelines of the French Society of Otorhinolaryngology (SFORL) for the diagnosis of cervical lymphatic malformation in adults and children. METHODS: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group, and finalized in a coordination meeting. Guidelines were graded A, B, C or expert opinion, by decreasing level of evidence. RESULTS: The SFORL recommends that complete ENT examination should be performed to identify lesions at high risk of complication or associated with poor prognosis. In case of diagnostic doubt, especially in latero-cervical or oral floor lesions, fine-needle aspiration cytology should be performed before therapeutic decision-making. One or more validated classifications should be used to assess treatment efficacy and monitor progression. The reliability of antenatal diagnosis should be ensured by associating MRI to ultrasound. In antenatal diagnosis, the locoregional extension of the cervical lymphatic malformation should be evaluated accurately for prognosis, and associated malformations should be screened for, to guide treatment options.

[Transcient ischemic attack, a rare manifestation of Eagle syndrome]. / Accident ischémique transitoire, une manifestation rare du syndrome d'Eagle.

INTRODUCTION: Clinical presentation of Eagle syndrome (ES) is very variable and non-specific, making its diagnosis difficult. It is usually limited to pain. Transient neurological manifestations are exceptional. We report one case in which the diagnosis of ES has been made based on neurological events occurring during left anterolateral head bending, without pain. OBSERVATION: A 47-year-old man presented with transient neurological events progressing since two years, half-right body paresthesia and reduced field of vision on the left side type, triggered by left anterolateral head flexion and regressive in neutral position. Transcranial Doppler and CT angiography of the supra-aortic trunks were performed in neutral position and in right and left head rotation that showed a disruption of the left sylvian flow and an extrinsic compression of the left internal carotid artery, due to a musculoskeletal impediment involving the lower end of the temporal styloid process. Complete recovering was achieved after surgical resection of this process. A control CT angiography confirmed the cessation of the compression. DISCUSSION: ES has non-specific and highly variable clinical manifestations making diagnosis difficult or leading to misdiagnosis. ES should be considered in any transient neurological deficit, especially when occurring during head rotation. Treatment relies on surgical resection of the excessively long styloid process.

Head and neck lymphatic malformation and treatment: Clinical study of 23 cases.

OBJECTIVES: Head and neck lymphatic malformation is a rare benign tumor, mainly affecting children under 1 year of age. Total resection is often difficult, and recurrence is frequent. Functional and esthetic sequelae are a major issue. MATERIALS AND METHODS: A single-center retrospective study recruited patients with head and neck lymphatic malformation managed surgically, with or without associated sclerosis by alcohol, bleomycin or aetoxisclerol, between January 1, 2004 and December 31, 2013. Local control, recurrence and complications rates were analyzed, as were swallowing disorder, tracheostomy and impaired phonation. RESULTS: Twenty-three patients, with a mean age of 15.80 years, were included. Location was suprahyoid in 17 cases (73.91%) and infrahyoid in 6 (26.09%). There were 11 macrocysts (47.82%), 2 microcysts (8.70%) and 10 mixed lesions (43.48%). Three patients showed spontaneous resolution; 12 patients (52.17%) underwent a single surgical procedure, 2 (8.7%) multiple procedures, 2 (8.7%) 1 surgical procedure and 1 sclerosis, 2 (8.7%) 1 surgical procedure and multiple scleroses, and 2 (8.7%) multiple procedures and multiple scleroses. The local control rate was 69.56%. There were 8 recurrences (34.78%), all in suprahyoid microcystic or mixed lesions. There were 2 complications (8.7%): 1 severe upper cervical edema following sclerosis of the floor of the mouth, and 1 postsurgical palsy of the marginal mandibular branch of the facial nerve. In 1 case (4.35%), a nasogastric tube was required for 6 days, without tracheostomy. CONCLUSION: Recurrence was frequent, with non-negligible functional and esthetic impact, especially in case of suprahyoid and microcystic lesion.

[Nasal tumours of the threshold and vestibule]. / Les tumeurs du seuil et du vestibule narinaire.

The nasal vestibule tumors are not very frequent. The pronostic can be severe if the first treatment is not effective. The authors present a personnal nine pateints' serie. They point out the clinical aspect, the evolutivity of these tumors and more specially the local extension which can produce recurrencies. The differents aspects of the treatment are analysed and more specially the surgical reconstruction procedures. reconstruction.

[Radiation-induced sarcoma of the head and neck]. / Sarcomes radio-induits de la tête et du cou.

INTRODUCTION: Radiation-induced sarcoma (RIS) of the head and neck is a late and rare complication of cancer treatment by radiation therapy. It occurs mostly within the limits of the irradiated area. Its incidence is increasing and related to an improved survival rate of treated patients. But it does not rule out the use of radiotherapy in the treatment of this cancer. OBSERVATION: We report two cases of sarcoma having appeared in the irradiated area, in patients treated by adjuvant radiotherapy for head and neck neoplasm. DISCUSSION: The prognosis for this sarcoma depends mainly on how early the diagnosis is made and the quality of surgical resection. Adjuvant chemotherapy or radiotherapy can be considered. Ongoing research on the expression of RIS genes could soon lead to new treatments.

Isolated hypoglossal palsy due to cervical osteophyte.

INTRODUCTION: Isolated hypoglossal nerve palsy is rare, and etiological diagnosis is difficult. We report a case of isolated hypoglossal compression by a cervical osteophyte in the hypoglossal canal exit. CASE STUDY: An 86-year-old woman with history of cervical spondylotic myelopathy consulted for a lesion of the free edge of the tongue with impaired elocution. Clinical examination found a bite lesion on the right free edge of the tongue with right lingual amyotrophy and associated left deviation on retraction. Isolated right hypoglossal palsy was diagnosed. Skull base CT found a cervical osteophyte compressing the hypoglossal nerve at the exit from the right hypoglossal canal. Surgery was contra-indicated by the patient's general health status. No motor recovery was observed at 6 months' follow-up, but the elocution disorders regressed under speech therapy. CONCLUSION: Hypoglossal palsy is infrequent, but generally a sign of skull base pathology. History-taking and careful examination guide rational selection of the radiological examinations required for etiological diagnosis.

Transoral robotic surgery for head and neck cancer: a series of 17 cases.

OBJECTIVES: Transoral robotic surgery (TORS) is an innovative surgical technique indicated for resection of selected head and neck cancers. The authors report their experience and discuss the indications, advantages and disadvantages of this technique. MATERIALS AND METHODS: Seventeen patients were operated by TORS in the Limoges University Hospital ENT department between March 2010 and January 2011. RESULTS: Tumour sites were the aryepiglottic fold (n=3), piriform sinus (n=2), lateral pharyngeal wall (n=3), posterior pharyngeal wall (n=2), base of tongue (n=3), vallecula (n=1), epiglottis (n=2) and arytenoid (n=2). One patient had two primary sites treated by TORS. This series comprised two stage I (11.7%), seven stage II (41.2%), six stage III (35.4%) and two stage IVa tumours (11.7%). Mean TORS set-up and operating times were 20.5 and 39.7 minutes, respectively. No major intraoperative complication was observed. One patient was reoperated on D5 for bleeding. Fifteen patients had clear surgical margins. Swallowing was restored on D2 for nine patients. The mean length of hospital stay was 10 days. Seven patients received adjuvant radiotherapy, seven patients were treated by chemoradiotherapy and three patients were submitted to simple clinical surveillance. CONCLUSION: TORS appears to provide new treatment options for head and neck cancers by extending the indications for endoscopic resection for selected cases of head and neck cancers. It allows effective cancer resection under excellent operating conditions with low morbidity and improved functional recovery. This new treatment modality needs to be evaluated in relation to other open surgery, endoscopic laser and chemoradiotherapy techniques.

Eustachian tube melanoma treated by double surgical approach.

OBJECTIVES: We report a case of an eustachian tube melanoma treated by a double surgical approach. MATERIAL AND METHODS: A 56-year-old man consulted for a recurrent left serous otitis media. RESULTS: CT-scan and MRI showed a lesion filling completely the middle ear, extending along the Eustachian tube up to the nasopharynx, with enhancement after injection. The biopsy of the lesion confirmed the diagnosis of malignant melanoma. After left internal carotid artery occlusion, the removal of the lesion was performed by a double surgical approach: combination of an endoscopic transnasal and a retroauricular approach. Radiotherapy was adjuvant therapy. CONCLUSION: Eustachian tube melanoma is a rare occurrence. Surgical treatment must be radical, what represents a challenge considering the localization; it can be obtained with the double surgical approach proposed in our case report.

[A new look on septoplasties: an anatomo-clinical study and surgical procedures of the 4 main septoplasties]. / Les septoplasties revisitées: étude anatomo-clinique et technique chirurgicale des 4 principales septoplasties.

The aim of a nasal septum surgery is functional and aesthetic aims. With a semiologic study the authors analyse four kinds of septoplasties which allows to correct the main septal deviations: erndoscopic septoplasty for posterior nasal obstruction, Cottle's septoplasty for septum's luxation and deviation on the premaxilla area, septoplasty with spreader grafts for dorsum cartilage deviations, extracorporeal septoplasty with a new septum cartilage frame for the complex deviations. The authors emphasize on the help given by videoendoscopy during the surgical procedures.

Mobile-bearing insert translational and rotational kinematics in a PCL-retaining total knee arthroplasty.

INTRODUCTION: Total knee prostheses with a mobile-bearing insert were developed to provide nonconstrained joint range of motion while reducing friction forces. The purpose of this study was, based on weightbearing X-rays, to evaluate the mobility of the polyethylene tibial insert in relation to the femoral and tibial components. We studied the results of a cementless total knee arthroplasty (TKA) retaining the posterior cruciate ligament (PCL), with a mobile-bearing platform in rotation and anteroposterior translation (Innex Anterior-Posterior Glide, Zimmer) with a mean 23-month follow-up duration after surgery. HYPOTHESIS: Both anterior-posterior tibiofemoral translation and intraprosthetic axial rotation occur between the mobile polyethylene insert and the tibial endplate. MATERIAL AND METHOD: In a series of 51 primary TKA, the three-dimensional (3D) kinematics of the femoral, tibial, and mobile insert components were determined using a computerized matching system between the prosthetic 3D models and the radiographic images of the implants on three lateral follow-up weightbearing knee X-rays: films were taken in full extension, at 45 degrees flexion, and at maximum flexion. RESULTS: There was a statistically significant increase in the internal rotation of the mobile tray with flexion, (up to a mean -3+/-3 degrees between the femoral box and the mobile tray [p<0.0001] and up to a mean -5+/-7 degrees between the tibial tray and the mobile tray [p<0.0001]). The mobile tray did not translate in relation to the tibial endplate from extension to 45 degrees flexion (0+/-2 mm [range: -5 to 6 mm]). However, from 45 degrees to maximum flexion, a statistically significant mean 1+/-2 mm (range: -2 to 9 mm) of anterior translation (p<0.0001) was found. DISCUSSION: The extent of insert mobility varies from one study to another. Some have reported relatively limited mobility stemming from a superior surface that is not highly congruent, (thus allowing anterior-posterior and mediolateral translation through gliding of the femur in contact with the insert). Other studies have reported mobile-bearing tray mobility in relation to the tibial endplate and minimal rotation at the femoral component level. In this series of PCL retaining TKA with a mobile-bearing platform, the mobile-bearing platform showed a progressive increase in internal rotation during flexion. Most of this rotational mobility occurred between the mobile platform and the tibial endplate, confirming our hypothesis. However, with flexion, the femoral component increased its mobility relatively to the platform. During flexion, an anterior-posterior translation occurred between the femoral implant and the tibial insert, and between the tibial insert and the tibial endplate, but the direction of the mobile tibial insert translation remained unpredictable with this nonconstrained implant design used. LEVEL OF EVIDENCE: Level IV. Prospective non-controlled therapeutic study.

Kinematics of a cementless mobile bearing posterior cruciate ligament-retaining total knee arthroplasty.

Mobile bearing (MB) total knee arthroplasty (TKA) was developed to provide low contact stress and unconstrained joint motion. We studied a consecutive series of 41 knees with mobile-bearing, posterior cruciate-retaining (CR) TKAs to determine if kinematics resembled normal knees or if kinematics changed over time. Patients were studied at 3 and 21 months average follow-up with weight-bearing radiographs at full extension, 30 degrees flexion and maximum flexion. Shape-matching techniques were used to measure TKA kinematics. Implant hyperextension, maximum flexion and total ROM increased with follow-up. Tibial rotation and condylar translations did not change with time. The medial condyle did not translate from extension to 30 degrees, but translated 5 mm anteriorly from 30 degrees to maximum flexion. Lateral condylar translation was 3 mm posterior from extension to 30 degrees, with no translation from 30 degrees to maximum flexion. Tibiofemoral kinematics in CR-MB-TKAs were stable over time, but did not replicate motions observed in healthy knees. The mobile tibial insert showed rotation and translation at both follow-up examinations, but the patterns of translation were not predictable.

[Patellar fracture after anterior cruciate ligament reconstruction using a bone patellar tendon bone transplant: a comparative study of two harvesting techniques]. / Fracture de la patella après prélèvement os-tendon patellaire-os pour ligamentoplastie de reconstruction du ligament croisé antérieur : incidence du type de prélèvement.

PURPOSE OF THE STUDY: We studied the incidence and the impact of patellar fracture after anterior cruciate ligament (ACL) reconstruction, comparing two harvesting techniques. MATERIAL AND METHODS: Series A included Kenneth Jones ligamentoplasties (n=1234). The distal and central part of the patella were harvested using an oscillating saw and a gouge. Series B included 676 Mac InJones reconstructions performed during the same period. The patellar cut was done from porximal to distal its attachment were harvested with a manual wire saw. The bony harvesting site was filled with cancellous bone. RESULTS: There were three postoperative transversal patellar fractures (0.24%), all in series A. Functional outcome was disappointing but there was no impact on knee stability. DISCUSSION: The fact that the Mac InJones technique does not involve a transversal cut would apparently prevent secondary fracture.

[Results of self-centering patellofemoral prosthesis: a retrospective study of 57 implants]. / Résultats de la prothèse fémoropatellaire autocentrique: à propos d'une série continue de 57 prothèses.

PURPOSE OF THE STUDY: The aim of this retrospective analysis was to report results obtained with a self-centering patellofemoral prosthesis. We wanted to determine whether self-centering still has indications for the treatment of patellofemoral osteoarthritis. MATERIAL AND METHODS: This was a continuous series of 57 knees operated on since 1986 in the same center for implantation of a self-centering patellofemoral prosthesis (Medinov then Depuy). Eight patients died and four were lost to follow-up. Two knees were excluded from the analysis after revision with a PFP. We report here the outcome of 43 prostheses at mean follow-up of six years two months (range 78 months to 15 years). The IKS score (200 points) and the ADL scale (in %) were recorded. The position of the prosthesis was assessed on plain X-rays. Mean age at implantation was 67.2 years. The main reasons for surgery were osteoarthritis secondary to dysplasia (60%) and primary disease (31.1%). RESULTS: At last follow-up, the IKS score was 157.2 points (range 76-195). The mean ADL score was 74.1/100 (48.8-96.3). The IKS evaluation showed good outcome in 66.7% of knees. The ADL scale gave a less satisfactory outcome: 57.7% good outcome for this scale which takes into account all knee functions for activities of daily life. Outcome was better among patients with trochlear dysplasia. Eleven patients (24.4%) had had revision surgery for total knee arthroplasty. Preoperatively, the trochlear angle was smaller in revision cases (p=0.023). In these patients, the first prosthesis was more anterior (p=0.004) with a greater horizontal axis (p=0.015). DISCUSSION: Our outcomes were less satisfactory than the average results in the literature. It must be noted however, that the concept of a good outcome depends on the scale used for assessment. We found in our series a 10% difference between the ADL scale and the IKS score. Independently of the assessment scale used, outcome was better in knees with osteoarthritis secondary to dysplasia. An analysis of the X-ray findings disclosed technical errors leading to failure. The outcome of patellofemoral prosthesis depends essentially on two factors: technical precision and patient selection. CONCLUSION: In light of these findings, we have come to limit still further the rare indications for patellofemoral prostheses. The typical indication is isolated advanced patellofemoral osteoarthritis secondary to patellofemoral dysplasia unresponsive to medical treatment in patients aged 50-70 years. Revision with a total knee arthroplasty required changing the patellar insert if worn. We have not had any particular problem with revision total knee arthroplasty after patellofemoral prosthesis.

Comparative study of knee anterior cruciate ligament reconstruction with or without fluoroscopic assistance: a prospective study of 73 cases.

INTRODUCTION: Correct placement of both tibial and femoral tunnels is one of the main factors for a favorable clinical outcome after anterior cruciate ligament (ACL) reconstruction. We used an original system of computer assisted surgery (CAS). The system, based on fluoroscopic guidance combined with special graphical software of image analyzing, showed to the surgeon, before drilling, the recommended placement of tibial and femoral tunnel centers. We compared the first anatomical and clinical results of this procedure to the usual one single incision technique. MATERIALS AND METHODS: We conducted a prospective study on 73 patients; 37 patients were operated on with CAS and 36 without CAS, by the same senior surgeon. The mean age was 27 years for both groups. Every patient was reviewed at an average of 2.2 years (range 1-4.5) by an independent observer, using IKDC scoring system, KT-1000, and passive stress radiographs. RESULTS: Time between ACL rupture and reconstruction averaged 30 months for both groups. CAS needed 9.3 min extra surgery time. Clinical evaluation was graded from A to C as per the IKDC scoring system: 67.6% A, 29.7% B, 2.7% C with CAS; and 60% A, 37.1% B, 2.9% C without CAS. IKDC subjective knee evaluation score averaged 89.7 with CAS and 89.5 without CAS. Pre operative KT-1000 maxi manual differential laxity averaged 7. At revision time, all the patients after CAS had a differential laxity less than 2 and 97.7% without CAS. Stress X-rays differential laxity averaged 2.4 mm with CAS and 3 mm without CAS. The area of dispersion of the tunnels' center was smaller on the femoral side using the CAS method. There was no statistically significant difference between both groups using IKDC score, KT-1000 and passive stress radiographs. CONCLUSIONS: The CAS method provided a more accurate and reproducible tunnels placement without clinical significant effect.

Effects of radiograph projection parameter uncertainty on TKA kinematics from model-image registration.

Model-image registration techniques have been used extensively for the measurement of joint kinematics in vivo. These techniques typically utilize an explicit measurement of X-ray projection parameters (principal distance, principal point), which is easily done for prospective studies. However, there is vast opportunity to derive useful information from previously collected clinical radiographic films where the projection parameters are unknown. The purpose of this study was to determine variation in measured knee arthroplasty kinematics when the X-ray projection parameters were unknown, but bounded. Based on the clinical radiographic protocol, a nominal principal point was chosen and eight additional points +/-2 and +/-5 cm in the horizontal and vertical directions were defined. Tibiofemoral kinematics were determined for all nine projection parameter sets for a series of 10 lateral radiographs. In addition, the principal distance was varied +/-15 cm and tibiofemoral kinematics were determined for these two projection sets. Measured joint kinematics varied less than 0.6 degrees and 0.4 mm for +/-2 cm variations in principal point location, and 0.7 degrees and 0.6 mm for +/-5 cm variations in principal point location. Measured joint kinematics varied less than 0.6 degrees and 0.7 mm for +/-15 cm variations in principal distance. Variation in X-ray principal point and principal distance over clinically bounded ranges has a small effect on knee arthroplasty kinematics computed from model-image registration with high-quality clinical radiographs.

[Anterior cruciate ligament revision: analysis and results from a series of 74 cases]. / Les reprises de ligamentoplastie du ligament croisé antérieur: analyse et résultats d'une série de 74 cas.

PURPOSE OF THE STUDY: This was a retrospective series of patients who underwent revision ligamentoplasty of the anterior cruciate ligament (ACL). We wanted to assess the functional outcome after this type of surgery and search for potential prognostic factors. MATERIAL AND METHODS: The series included 74 patients, mean age 34 years (range 21-59 years). The initial ligamentoplasty was performed with a synthetic ligament (n=16), an autograft (n=57) or an allograft (n=1). Differential laxity (KT-1000 maximal manual) was 7 +/- 2.5 mm. Anterior drawer was measured on the stress x-rays in 20 degrees flexion: medial 8 +/- 4.7 mm, lateral 8.3 +/- 4.9 mm. Mean time to revision surgery was 78 months. Reconstruction was performed arthroscopically in 69 knees. The implant used for the revision reconstruction was an autograft: patellar tendon (n=42), quadriceps tendon (n=15), hamstring tendon (n=13), patellar tendon and quadriceps tendon (MacInJones) (n=3), fascia lata (n=1). A meniscal tear was noted in 24 knees and a cartilage lesion in 35. RESULTS: Outcome was assessed at mean 21.2 months follow-up. The mean IKDC function score was 71.7 (range 21.8-100). 78% of patients considered their knee normal or nearly normal and 88% presented a positive Lachmann. Mean differential laxity measured with KT-1000 (maximal manual) was 2 +/- 1.7 mm. Stress x-rays revealed a mean differential laxity measured at 3.7 +/- 2.3 mm medially and 6.3 +/- 4.3 mm laterally. The presence of a meniscal lesion favored osteoarthritic degradation. Presence of chondral lesions altered the functional outcome significantly and limited resumption of sports activities. An initial repair using a synthetic ligament affected the functional outcome after revision surgery and favored or aggravated chondral lesions. DISCUSSION: The clinical results we have obtained with revision ACL ligamentoplasty are comparable to previous series reported in the literature. The functional outcome is not as good as after first-intention repair, especially if the initial plasty was done with a synthetic ligament and the knee presented meniscal or cartilage damage.