Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

Low-Grade Surface Osteosarcoma of the Temporal Bone in Paediatric Patients: A Case Report and Literature Review.

PURPOSE OF THE STUDY: Primary osteosarcoma of the temporal bone is an exceedingly rare pathology in the paediatric population. As of now, only 3 cases have been reported in the English literature. We describe the additional case of a 16-year-old girl with an osteosarcoma of the mastoid bone. This study aims to report a rare paediatric case of low-grade surface osteosarcoma of the temporal bone. MATERIALS AND METHODS USED: A literature review was performed to better understand paediatric osteosarcomas of the head and neck region, to optimize their investigation, to describe their histopathological and radiological characteristics, and to establish the optimal modalities of medical and surgical treatments. The research of previous published data was done using PubMed and Embase library with the keywords mentioned below. RESULTS: The patient presented with a rapidly progressive left retroauricular lesion over a 3-week period. Radiological studies demonstrated aggressive and invasive features. An open biopsy followed and confirmed the diagnosis of a low-grade surface osteosarcoma. In accordance with the multidisciplinary team, we decided to perform a complete surgical resection with wide surgical margins. We did not administer any adjuvant therapies. A control computed tomography (CT) scan obtained 26 months postoperatively still showed no signs of recurrence. CONCLUSION: Osteosarcomas are aggressive malignant neoplasms found in the head and neck region in only 6% to 10% of cases. They represent approximately 1% of head and neck cancers, and these are generally high-grade lesions. Temporal bone involvement is rare, particularly for low-grade lesions in paediatric patients. In addition to reporting the fourth paediatric case of primary temporal bone osteosarcoma, this study describes its specific clinical, histopathological, and radiological findings, to improve the management and the prognostic of patients affected with this particular clinical entity.

Recurrent dacryostenosis as initial presentation of sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease with an unknown etiology. It most commonly affects young and middle-aged females. It can affect any organ, but mostly lung, skin, and eyes. Up to half of patients are asymptomatic and the disease is often detected incidentally on abnormal chest radiography. We report the case of a 31-year-old male with bilateral recurrent dacryostenosis. The nasolacrimal obstruction was the initial manifestation of systemic sarcoidosis.

Facial nerve outcome with a peroperative stimulation threshold under 0.05 mA.

OBJECTIVES/HYPOTHESIS: To evaluate the facial nerve outcome postoperatively of schwannoma vestibular surgery with a stimulation threshold (ST) lower than 0.05 mA. STUDY DESIGN: Retrospective chart review. METHODS: A total of 106 patients had undergone schwannoma vestibular surgery between 2002 and 2008 in a tertiary center. The ST near the brainstem was obtained after the tumor removal. Facial nerve function was evaluated according to the House-Brackmann (HB) scale immediately postoperatively and at 1 year. The results were compared among the different STs used: <0.05 mA, 0.05 mA, and >0.05 mA. RESULTS: There were 50 patients who had an ST <0.05 mA, 45 patients who had an ST of 0.05 mA, and 11 patients who had a ST >0.05 mA. Immediately postoperatively, for all of these groups we had a preservation of facial nerve function (HB I-II) in 94%, 80%, and 36% of patients, respectively (P < .0001). At 1 year in the same groups the preservation of the facial nerve function was 100%, 93%, and 82% of patients, respectively (P < .01). CONCLUSIONS: A proximal ST of <0.05 mA is a better predictor of facial nerve function immediately postoperatively.