Recurrent facial palsy: A CARE case report.

INTRODUCTION: Middle-ear adenoma is a rare benign endocrine tumor with slow progression, and can, in very rare cases, lead to recurrent peripheral facial palsy. CASE REPORT: A young man experienced three episodes of right peripheral facial palsy of incremental intensity, suggestive of barotrauma. CT and MRI found a tissue mass in the tympanic cavity, and biopsy diagnosed middle-ear adenoma. Electroneuromyography found 50% impairment of facial function. Closed right tympanoplasty with complete tumor resection enabled complete recovery of facial function within 1 month. DISCUSSION: Middle-ear adenoma is diagnosed on histology, as imaging on MRI can be non-specific, mimicking chronic otitis. Facial involvement is rare and is due to edematous compression of the vasa nervorum. Treatment is surgical, and follow-up should be prolonged. Palsy assessment on electroneuromyography indicates the urgency of treatment.

Surgical treatment of cholesteatomatous labyrinthine fistula by hydrodissection.

Middle ear cholesteatoma is a destructive lesion that can potentially erode the bone. Cholesteatoma is a clinical diagnosis in the vast majority of cases. Patients must be systematically investigated for the presence of signs suggestive of complications, the most common of which is labyrinthine fistula. However, the clinical features of labyrinthine fistula are inconstant and the fistula sign may sometimes be negative. CT scan of the petrous temporal bone is performed systematically to specify the site and extension of the cholesteatoma, and to assess the extent of osteolysis that can result in exposure of the membranous labyrinth. Surgical treatment has three main objectives: complete resection of the cholesteatoma, which is the only way to avoid residual cholesteatoma, prevention of recurrence by an adapted, preferably one-step, technique, and restoration of good quality hearing. Hydrodissection of the cholesteatoma matrix in the presence of labyrinthine fistula is a simple technique that can achieve the three main general objectives of cholesteatoma surgery.

How to do and why perform the skull vibration-induced nystagmus test.

The skull vibration-induced nystagmus test (SVINT) is a global vestibular test stimulating otoliths and semicircular canals at a frequency of 100Hz, not modified by vestibular compensation, which may reveal vibration-induced nystagmus (VIN). Bone-conducted vibration applied to the mastoid processes and the vertex instantaneously induces predominantly low-velocity (∼10°/s) horizontal nystagmus, with rapid phases beating away from the affected side in patients with unilateral vestibular loss (UVL). VIN starts and stops immediately with stimulation, is continuous, reproducible, beats in the same direction irrespective of which mastoid process is stimulated, with no or little habituation. The SVINT acts like a vestibular Weber test. In peripheral UVL, the SVINT is a good marker of vestibular asymmetry and demonstrates pathological nystagmus beating towards the healthy side in 90% of cases of vestibular neuritis, 71% of cases of Menière's diseases and 44 to 78% of vestibular schwannomas. In superior semicircular canal dehiscence, VIN usually beats towards the affected side due to facilitation of bone conduction related to the presence of a third window. Stimulation of the vertex is more effective than in UVL patients, with sensitivity extending to higher frequencies, up to 700Hz. Observation of vibration-induced nystagmus then reveals equally represented vertical, torsional, and horizontal components beating towards the affected ear, suggesting dominant, but not exclusive, stimulation of the dehiscent superior semicircular canal.

The French Cochlear Implant Registry (EPIIC): Cochlear implant candidacy assessment of off-label indications.

OBJECTIVE: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label. MATERIAL AND METHODS: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded. RESULTS: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients. CONCLUSION: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid.

Glial heterotopia of the middle ear.

INTRODUCTION: Glial heterotopia is defined as the presence of normal glial tissue in an abnormal site. CASE REPORT: We report the case of a 63-year-old man who presented with right hearing loss and tinnitus. ENT examination, computed tomography and magnetic resonance imaging showed signs of chronic otitis media. Right mastoidectomy demonstrated a solid mass of the right mastoid and middle ear with no associated dehiscence of the tegmen tympani. Histological examination of the mass revealed normal glial tissue. DISCUSSION: Glial heterotopia is a rare entity, which must be distinguished from encephalocele. It is usually an incidental surgical finding when the surgeon observes a mass of the mastoid and middle ear not communicating with the central nervous system. Histological examination confirms the diagnosis by revealing normal glial tissue in the ear.

Persistent stapedial artery in PHACE syndrome.

INTRODUCTION: PHACE syndrome is characterized by posterior fossa malformations, haemangioma, arterial anomalies, coarctation of the aorta, and eye abnormalities. CASE REPORT: We present the case of a 6-year-old girl followed since birth for PHACE syndrome and left hemifacial haemangioma, who presented with left hearing loss. Computed tomography scan showed left persistent stapedial artery (PSA). DISCUSSION: Two types of arterial anomalies may be observed in PHACE syndrome: persistence of embryonic arteries and anomalies of cerebral arteries. PSA can be observed in the context of PHACE syndrome. Children with PHACE syndrome require regular audiometric follow-up to detect hearing loss and avoid its consequences on speech and language development.

Role of near-infrared fluorescence imaging in the resection of metastatic lymph nodes in an optimized orthotopic animal model of HNSCC.

OBJECTIVES: To study the role of near-infrared fluorescence imaging in the detection and resection of metastatic cervical lymph nodes in head and neck cancer. MATERIALS AND METHODS: CAL33 head and neck cancer cells of human origin were implanted in the oral cavity of nude mice. The mice were followed up after tumor resection to detect the development of lymph node metastases. A specific fluorescent tracer for αvß3 integrin expressed by CAL33 cells was injected intravenously in the surviving mice between the second and the fourth month following tumor resection. A near-infrared fluorescence-imaging camera was used to detect tracer uptake in metastatic cervical lymph nodes, to guide of lymph-node resection for histological analysis. RESULTS: Lymph node metastases were observed in 42.8% of surviving mice between the second and the fourth month following orthotopic tumor resection. Near-infrared fluorescence imaging provided real-time intraoperative detection of clinical and subclinical lymph node metastases. These results were confirmed histologically. CONCLUSION: Near infrared fluorescence imaging provides real-time contrast between normal and malignant tissue, allowing intraoperative detection of metastatic lymph nodes. This preclinical stage is essential before testing the technique in humans.