Facial Nerve Tumors in Children: Two Clinical Cases and a Review of the Literature.

We provide an extensive review of clinical features, diagnosis, and treatment of primitive facial nerve tumors in children, and report 2 recent personal observations. We conducted a comprehensive literature search through PubMed, Medline, and ScienceDirect and collected information on patients' age, symptoms, tumor types and sites, diagnostic procedures, surgical approaches, and outcomes. Overall, we reviewed 26 pediatric cases from 20 papers. About 69.2% of children presented with some degree of facial palsy. Other symptoms included hearing loss, dizziness, and tinnitus. 84.6% of tumors were schwannomas, followed by meningiomas, epithelioid hemangioendothelioma, and germ cell tumors. The geniculate ganglion was the most commonly affected segment of the facial nerve. A total of 92.3% of children received surgery as complete or partial tumor resection. Facial nerve function improved in 26.9% of children. No tumor recurrence was reported. Facial nerve tumors are extremely rare in children but should be considered in the differential diagnosis of facial palsy, even in newborns. Audiometric and radiologic examinations are necessary; radiologic imaging allows to determine tumor localization, and the correct surgical approach surgery is suggested in almost all cases.

Current outcomes of myringoplasty in a European referral children's hospital.

OBJECTIVES: Myringoplasty is a frequently performed procedure in children, with a heterogeneous failure rate. Our study aimed to evaluate the outcome of myringoplasty in a pediatric hospital and to identify which are risk factors for reperforation or poor hearing improvement after surgery. METHODS: Preoperative and intraoperative variables between pediatric patients who had undergone myringoplasty with an intact tympanic membrane at follow-up and the cases with reperforation were compared. The same factors were investigated as potential predictors of audiological success. Pre and postoperative PTA and ABG were compared in the whole population, in structural success and failure groups and closure of ABG was calculated and used to compare the audiological outcomes between the two groups. RESULTS: Parameters that affected the postoperative integrity of TM were age, the time between diagnosis and surgery, the intraoperative status of the middle ear, and secondhand smoke exposure. Early perforations occurred mostly after surgeries performed by trainees, while late perforations were more frequently in autumn. Myringoplasty, regardless of the structural outcome, can improve the ABG and PTA. No preoperative and intraoperative parameters affected the audiological outcome. CONCLUSION: Pediatric myringoplasty is a safe and successful procedure that can improve hearing, regardless of the structural outcome. In light of our results, parameters to consider before surgery are age, the time between diagnosis and surgery, the intraoperative status of the middle ear, and secondhand smoke exposure.

Comparison of Lower Cranial Nerve Function Between Tympanojugular Paraganglioma Class C1/C2 With and Without Intracranial Extension: A Four-Decade Experience.

OBJECTIVE: To compare preoperative and postoperative lower cranial nerve (LCN) function between Class C1 and C2 tympanojugular paraganglioma (TJP) with/without intracranial intradural (Di)/extradural (De) extensions, according to the experience of a single surgeon over four decades. STUDY DESIGN: Retrospective review. SETTING: Quaternary referral center for otology and skull base surgery. MATERIAL AND METHODS: A chart review was conducted of all the patients operated for C1/C2 TJPs from September 1983 to December 2018. The tumors were classified as: Limited-Group (C1/C2 without Di/De extensions) and Extended-Group (C1/C2 with Di/De extensions). RESULTS: Of 159 patients, 107 (67.3%) were women; the mean age at surgery was 46.5 years. The Limited-Group (56.6%) comprised C1 (41.1%) and C2 (58.9%) tumors; the Extended-Group (43.4%) comprised C1+Di/De (14.5%) and C2+Di/De (85.5%) tumors. The prevalence of preoperative LCN palsy was 11.9 times higher in Extended than Limited tumors: 61.9% versus 4.9% (p < 0.05). The risk for postoperative LCN palsy was 4.7 times greater in Extended than Limited tumors: 29.2% versus 12.9%, p = 0.01. CONCLUSION: Especially in younger patients, complete removal of Limited C1/C2 tumors, before they extend intracranially, reduces the risk of dysfunctionality of LCNs and the burden of residual tumor. The incidence of new tumors increased over four decades. However, new-postoperative LCN palsy did not occur in any Limited C1/C2 tumors operated after the year 2000, and declined to less than 10% of Extended C1/C2 tumors.

Simultaneous Cochlear Implantation After Translabyrinthine Vestibular Schwannoma Resection: A Report of 41 Cases.

OBJECTIVE: To study the auditory outcome of simultaneous translabyrinthine vestibular schwannoma (VS) resection and cochlear implantation (CI) after successful cochlear nerve preservation. STUDY DESIGN: A retrospective case series and patient questionnaire. SETTING: Quaternary referral center for skull base pathologies. PATIENTS: Patients with small (<2 cm) sporadic or neurofibromatosis 2 related tumors were included in this study. INTERVENTION: Vestibular schwannoma resection + simultaneous cochlear implantation. MAIN OUTCOME MEASURE: Audiological performance postimplantation and perceived patients' benefits. RESULTS: Forty-one patients were included. Thirty-three were sporadic VS and eight were neurofibromatosis 2. Auditory perception postimplantation was achieved in 33 patients (80.5%). At the last follow-up, 20 patients (48.8%) were users and 21 (51.2%) were nonusers. In the users' group, and after 1 year of implant activation, vowel identification was 75.3%, disyllabic word recognition 54%, sentence recognition 60.7%, and common phrase comprehension 61%, whereas in the nonusers' group and after 1 year of implant activation, vowel identification was 22.9%, disyllabic word recognition 14.8%, sentence recognition 15.3%, and common phrase comprehension 14%. Sixteen users were classified into 10 high performers, three intermediate performers, and three poor performers. In the user' group, the mean postimplantation pure tone average was 63.4 dB and the mean speech discrimination score was 63.7%. CONCLUSIONS: Simultaneous CI and VS resection is a viable option with many patients achieving auditory perception and nearly half the patients are CI users at long follow-up.

Middle Ear Neuroendocrine Tumor: A Case Report and Review of the Literature in Pediatric Population.

A rare pediatric case report of middle ear neuroendocrine tumor and review of the pediatric cases reported in the literature. A 16-year-old female showed a lesion occupying the posterosuperior part of the medial third of the right external auditory canal confirmed by computed tomography scan, without clear evidence of bone erosion. The patient underwent canal wall tympanoplasty in 1 stage. No residual pathology was present after 1 month, 3-6 months, and after 1 year. There are few known pediatric cases of this disease, there is no statistically significant data for this population regarding the risk of recurrence or metastasis. Middle ear neuroendocrine tumors are rare above all in children. They are slow aggressive tumors but they can recur and rarely give local metástasis. Only 4 pediatric cases have been published. We have completely removed the tumor in our patient, using a conservative surgical treatment in a single stage.