Subtotal Petrosectomy (SP) in Cochlear Implantation (CI): A Report of 92 Cases.

In most cases, cochlear implantation is a straightforward procedure. Nevertheless, there are clinical situations in which the presence of the middle ear may compromise access and/or the outcome in terms of complications. This article includes a series of patients for whom we eliminated the middle ear to facilitate placement of the electrode array of the implant and/or reduce potential complications. A total of 92 cases in 83 patients, managed by the senior author, are included in this series. Different indications are outlined that justify associating a subtotal petrosectomy technique with cochlear implantation. The steps of the technique are described. We include complications from this series that compare favorably with standard techniques.

International Survey of Operative Practices for Otologists and Neurotologists During the COVID-19 Crisis.

OBJECTIVE: To investigate the influence of the COVID-19 pandemic on operative practices of otology and neurotology providers internationally. STUDY DESIGN: Cross-sectional survey. METHODS: A 78-question survey was distributed to otologists and neurotologists between May 12, 2020 and June 8, 2020 to assess the impact of the pandemic on surgical practices. Sections within the survey delineated time periods: prior to the crisis, onset of the crisis, during the crisis, postcrisis transition. RESULTS: Of 396 survey respondents, 284 participants from 38 countries met inclusion criteria.Respondents were 16.9% female and 82.4% male, with a most common age range of 40 to 49 years (36.3%). 69.8% of participants had been in practice for over 10 years and most respondents worked in an academic medical center (79.2%). The average operative weekly caseload was 5.3 (SD 3.9) per surgeon prior to the crisis, 0.7 (SD 1.2) during the COVID-19 crisis, and 3.5 (SD 3.3) for those who had begun a postcrisis transition at the time of survey administration (p < 0.001). 71.5% of providers did not perform an elective otologic or neurotologic operative procedure during the initial crisis period. 49.8% reported modifying their surgical technique due to the COVID-19 pandemic. Use of powered air-purifying respirators and filtering facepiece 2 or 3 (FFP2/FFP3) respirators were in minimal supply for 66.9% and 62.3% of respondents, respectively. CONCLUSION: The COVID-19 pandemic impacted the otology and neurotology community globally, resulting in significant changes in operative volume and case selection. Modification of surgical technique and shortages of personal protective equipment were frequently reported.

Parálisis facial: guía de práctica clínica de la Sociedad Española de ORL / Facial paralysis: Clinical practice guideline of the Spanish Society of Otolaryngology

La parálisis de Bell es la forma más común de paresia o parálisis facial. Sin embargo, no todos los pacientes con parálisis facial tienen una parálisis de Bell. Otras causas frecuentes incluyen las secuelas del tratamiento del neurinoma del VIII par, el cáncer de cabeza y cuello, la iatrogenia, el zóster ótico y los traumatismos. El abordaje de cada una de estas situaciones es totalmente diferente. El objetivo de esta guía es servir de consejo para el tratamiento y el seguimiento de los pacientes con parálisis facial. Nuestra idea es que la guía sea práctica, haciendo hincapié en recomendaciones efectivas y útiles en el manejo diario de los pacientes. Esta guía ha sido promovida por la Sociedad Española de ORL y escrita por médicos con experiencia en la enfermedad del nervio facial, incluyendo al menos un especialista de cada comunidad autónoma. Redactada en un formato de preguntas y respuestas, incluye 56 cuestiones relevantes relacionadas con el nervio facial

Bell's palsy is the most common diagnosis associated with facial nerve weakness or paralysis. However, not all patients with facial paresis/paralysis have Bell's palsy. Other common causes include treatment of vestibular schwannoma, head and neck tumours, iatrogenic injuries, Herpes zoster, or trauma. The approach to each of these conditions varies widely. The purpose of this guideline is to provide clinicians with guidance on the treatment and monitoring of patients with different causes of facial paralysis. We intend to draft a practical guideline, focusing on operationalised recommendations deemed to be useful in the daily management of patients. This guideline was promoted by the Spanish Society of Otolaryngology and developed by a group of physicians with an interest in facial nerve disorders, including at least one physician from each Autonomous Community. In a question and answer format, it includes 56 relevant topics related to the facial nerve

Facial paralysis: Clinical practice guideline of the Spanish Society of Otolaryngology. / Parálisis facial: guía de práctica clínica de la Sociedad Española de ORL.

Bell's palsy is the most common diagnosis associated with facial nerve weakness or paralysis. However, not all patients with facial paresis/paralysis have Bell's palsy. Other common causes include treatment of vestibular schwannoma, head and neck tumours, iatrogenic injuries, Herpes zoster, or trauma. The approach to each of these conditions varies widely. The purpose of this guideline is to provide clinicians with guidance on the treatment and monitoring of patients with different causes of facial paralysis. We intend to draft a practical guideline, focusing on operationalised recommendations deemed to be useful in the daily management of patients. This guideline was promoted by the Spanish Society of Otolaryngology and developed by a group of physicians with an interest in facial nerve disorders, including at least one physician from each Autonomous Community. In a question and answer format, it includes 56 relevant topics related to the facial nerve.

Anatomical Variations of the Intrapetrous Portion of the Facial Nerve.

The intrapetrous facial nerve has the second longest intraosseous course of all cranial nerves, after the mandibular nerve. But it is by far the most complex considering the anatomical structures closely related to it. The auditory and vestibular portions of the inner ear, the dura of the middle fossa and posterior fossa, the sigmoid sinus and jugular bulb, and the internal carotid artery are close enough to merit attention. This article includes an anatomical study on 100 temporal bones with anatomical references as seen from the middle fossa and from the transmastoid approaches that may help identifying the facial nerve and protecting surrounding structures. Anatomical variability was present and noteworthy when considering the venous drainage system through the temporal bone and the mastoid pneumatization. The distance from the geniculate ganglion to the hiatus falopii offered the highest variability with a range of 0 to 7.75 mm and a mean of 3.30 mm. The geniculate ganglion was dehiscent in 20.8% of the specimens and the superior semicircular canal was spontaneously blue-lined in 27% of the cases. Through the transmastoid approach, the highest variability was found regarding the distance between the vertical portion of the facial nerve and the jugular bulb (range from 1.5 to 10.0 mm), the sigmoid sinus (range from 0 to 13.25 mm) and the internal carotid artery (range from 6.0 to 15.0 mm). This study highlights the importance of the relative variability of the facial nerve to other surrounding structures within the petrous portion of the temporal bone. Anat Rec, 302:588-598, 2019. © 2018 Wiley Periodicals, Inc.

Round and Oval Window Anatomic Variability: Its Implication for the Vibroplasty Technique.

OBJECTIVE: The objective of this study is to evaluate the anatomical variability of round and oval window regions and its relationship with their closest structures, to determine its implication on the fitting and stabilization of the middle ear implant Vibrant Soundbridge. METHODS: Variations of the anatomy of round and oval window regions were assessed in a total of 85 human dissected temporal bones. Afterward, we evaluated the adaptation and subsequent stabilization of the floating mass transducer (FMT) of the Vibrant Soundbridge in 67 cases in round window (RW) and in 22 cases in oval window (OW), and the influence that the variability of the different anatomical features examined had on this stabilization. We also assessed access and surgeon's view of the RW niche through the facial recess approach. RESULTS: Stabilization of the FMT in the RW was achieved in 53 (79%) of the 67 cases; we found that the less favorable anatomical conditions for stabilization were: membrane smaller than 1.5 mm, presence of a high jugular bulb and a narrow or very narrow RW niche. Frequently, two or more of these conditions happened simultaneously. In seven cases (22%) access to the RW through facial recess approach did not allow positioning the FMT in place. OW stabilization succeeded in 18 (82%) of the 22 cases. CONCLUSION: Round and oval window vibroplasty are difficult surgical techniques. To place the FMT directly on the OW may be easier as we do not have to drill the niche. In both regions there are some anatomical conditions that hinder fitting the FMT and even make it impossible. Once fitted, the main problem is to achieve good stabilization of the device.

Ipsilateral cochlear implantation in patients with sporadic vestibular schwannoma in the only or best hearing ear and in patients with NF2.

The aim of this study was to evaluate the cochlear implant (CI) performances in neurofibromatosis type 2 (NF2) patients with bilateral vestibular schwannoma (VS) and in patients with sporadic VS in the only or better hearing ear. All patients with bilateral VS or sporadic VS in the only or better hearing ear who underwent cochlear implantation, either simultaneous to VS surgery or staged after treatment for VS, in the tumor side were chosen for the study. Postimplantation audiometric scores (sound detection, closed-set and open-set discrimination scores) and device use patterns were the main outcome measures. 15 patients were implanted. Eight patients (53 %) were NF2 and seven patients had VS in the only or better hearing ear. One patient was explanted for cerebrospinal fluid leak. In the CI-only condition, the other 14 patients obtained sound detection, 64 % of them achieving open-set discrimination (mean 70 ± 38 %) and 85 % achieving closed-set discrimination (mean 41 ± 33 %). At the last follow-up 10 patients (67 %) were using the CI. Cochlear implantation provides hearing in particular cases of patients with bilateral VS or VS in the only or better hearing ear. As long as anatomic preservation of the cochlear nerve is achieved, cochlear implantation may offer improvement in communication skills for most patients.

Subtotal Petrosectomy for Cochlear Implantation: Lessons Learned After 110 Cases.

OBJECTIVES: The purpose of this study was to review the indications for subtotal petrosectomy for cochlear implantation, report our management of complications, as well as review those technical factors that are critical for successful implantation. METHODS: Patients (n = 104) that underwent subtotal petrosectomy with closure of the external auditory canal and obliteration of the cavity with abdominal fat in combination with cochlear implantation were analyzed. RESULTS: The most frequent indication for subtotal petrosectomy was the existence of a previous canal wall down technique. Postoperative complications occurred in 13 patients (11.83%). Extrusion of the device took place in 5 cases (4.55%). CONCLUSIONS: Subtotal petrosectomy in cochlear implantation permits obtaining a cavity isolated from the external environment, and when needed, it improves the access and visibility during the surgical procedure. Subtotal petrosectomy is a safe technique, with a low rate of complications.

Cochlear implantation versus auditory brainstem implantation in bilateral total deafness after head trauma: personal experience and review of the literature.

OBJECTIVE: To determine the effectiveness of cochlear implant (CI) in hearing restoration after temporal bone (TB) fractures and investigate the adequacy of auditory brainstem implant (ABI) indication for TB fractures. STUDY DESIGN: Retrospective clinical study; a systematic review of the literature in PubMed was also performed to identify all published cases of bilateral TB fractures or bilateral deafness after head trauma treated by means of CI or ABI. SETTINGS: Quaternary otology and skull base surgery referral center. PATIENTS: Eleven consecutive patients presented with bilateral severe-to-profound sensorineural hearing loss after head trauma. INTERVENTIONS: CI as primary intervention or following a previous treatment. MAIN OUTCOME MEASURES: CI performances were evaluated in the auditory-only condition in both closed-set and open-set formats. RESULTS: Fourteen CI were placed, 11 as primary treatment and 3 after ABI failure. At the last follow-up, all patients gained useful open-set speech perception. In secondary CI, all patients obtained better auditory results with the CI if compared with ABI. CI performance did not decrease with time in any case. CONCLUSION: Cochlear implantation after TB fractures has proved to have excellent audiometric results. The aim of the initial evaluation of a patient with bilateral anacoustic ears from head trauma should always be to rehabilitate their hearing with a CI. The incidence of labyrinthitis ossificans, negative electrophysiologic testing, the risk of postoperative meningitis or facial nerve stimulation should not be the determinant factors that favor ABI placement.

Hyaluronic acid gel weight: a nonsurgical option for the management of paralytic lagophthalmos.

OBJECTIVES/HYPOTHESIS: Management of lagophthalmos should be a priority in the treatment of patients with facial palsy. The aim of the study was to evaluate the safety and efficacy of injecting hyaluronic acid gel into the upper eyelid as a nonsurgical alternative for patients with temporary facial palsy. METHOD/STUDY DESIGN: Retrospective study of 26 patients treated with hyaluronic acid gel injected into the pretarsal region of the upper eyelid. Measurements taken before and after treatment were standardized and compared using digitized photographs. Patients were followed up for 1 year, and overall outcomes were assessed. RESULTS: All patients initially demonstrated improvement in lagophthalmos, which decreased to 0.0 mm. After 1 month, a significant increase in lagophthalmos was observed in two patients (initial fissure of 8 and 9 mm), and a platinum weight was implanted to control keratopathy. The remaining patients (initial lagophthalmos below 6.5 mm) maintained the improvement until facial restoration. Only three patients had recurrent lagophthalmos (2 mm) due to resorption, which was resolved by injecting an additional 0.3 cc. The mean improvement in lagophthalmos was 4.6 mm (range, 3.5-6.5 mm). Complications included transient ecchymosis and minimal blepharoptosis due to nonreabsorption in five patients. These patients were successfully treated with hyaluronidase. CONCLUSIONS: Hyaluronic acid gel has proven effective in reducing paralytic lagophthalmos and controlling keratopathy in patients with temporary facial palsy, especially those with palpebral fissure with attempted closure no greater than 6.5 mm. Injection of hyaluronic acid gel is safe, quick, and easily performed. In addition, it is more cost-effective than surgery.

Identification of somatic VHL gene mutations in sporadic head and neck paragangliomas in association with activation of the HIF-1α/miR-210 signaling pathway.

CONTEXT: Head and neck paragangliomas (HNPGLs) arise from parasympathetic paraganglias and 35% to 45% are hereditary caused by mutations in succinate dehydrogenase (SDH) genes. The connection between SDH and tumor development is unclear. The most accepted hypothesis proposes a central role for the pseudohypoxic (pHx) pathway activated by hypoxia-inducible factor (HIF). Paradoxically, we showed that activation of HIF in HNPGLs is restricted to a subset of HNPGLs lacking SDH mutations. These tumors overexpress HIF-1α protein and target genes and the HIF-inducible microRNA miR-210 (pHx-HNPGLs). OBJECTIVE: The present study aimed at unraveling the SDH-independent mechanisms involved in the activation of HIF in HNPGLs. DESIGN: The VHL gene was analyzed in 53 tumors by gene sequencing, multiplex-ligation-dependent probe amplification, and quantitative PCR. The miR-210, HIF-1α, and CA9 levels were used as markers of the pHx gene signature. Meta-analysis of the transcriptome of pHx-HNPGLs was performed using the Oncomine platform. Assays in cells lacking functional pVHL and HIF-1α were performed to analyze the role of pVHL/HIF-1α on miR-210 expression. RESULTS: We identified, for the first time, somatic VHL mutations in HNPGLs. These were found in 2 of 4 pHx-HNPGLs with concomitant loss of heterozygosity in one of them; but not in non-pHx-HNPGLs. Meta-analysis of the transcriptome of pHx-HNPGLs revealed that these tumors are highly related to clear cell renal cell carcinoma. Cell-based assays showed that loss of pVHL lead to upregulation of miR-210 mainly via HIF-1α activation. CONCLUSIONS: VHL, involved in tumorigenesis of PGLs and clear cell renal cell carcinomas, may be an important player in the pathogenesis of sporadic HNPGLs via activation of an HIF-1α/miR-210 pHx pathway.

Identification of a signaling axis HIF-1α/microRNA-210/ISCU independent of SDH mutation that defines a subgroup of head and neck paragangliomas.

BACKGROUND: Head and neck paragangliomas (HNPGLs) are rare tumors associated with the parasympathetic nervous system. Most are sporadic, but about one third result from germline mutations in succinate dehydrogenase (SDH) genes (SDHB, SDHC, SDHD, SDHA, or SDHAF2). Although a molecular connection between SDH dysfunction and tumor development is still unclear, the most accepted hypothesis proposes a central role of the pseudohypoxic pathway. SDH dysfunction induces abnormal stabilization of the hypoxia-inducible factors (HIFs) that regulate target genes involved in proliferation, apoptosis, angiogenesis, and metabolism. The involvement of these pathways in the development of sporadic HNPGLs is presently unknown. OBJECTIVE: To get some insights into the hypoxic/pseudohypoxic molecular basis of HNPGLs, we attempted to define the gene, microRNA (miRNA), and HIF-1α expression patterns that distinguish tumors from normal paraganglia tissue. DESIGN: Genome microarray and TaqMan low-density arrays were used to analyze gene and miRNA expression, respectively, in 17 HNPGL tumor tissues and three normal human carotid bodies. Twelve HNPGLs were used for validation of data. HIF-1α, SDHB, and iron-sulfur cluster scaffold protein (ISCU) protein expression was analyzed by immunohistochemistry. RESULTS: We found activation of a canonical HIF-1α-related gene expression signaling only in a subset of HNPGLs from patients that did not harbor germline or somatic SDH mutations. The pseudohypoxic signature consisted in the overexpression of both HIF-1α-target genes and the HIF-1α-inducible miRNA, miR-210, and down-regulation of the miR-210 target gene, ISCU1/2. A decreased level of the iron-sulfur-containing protein SDHB was found by immunohistochemical analysis performed in two of these tumors. CONCLUSIONS: Collectively, this study unveiled a putative signaling axis of HIF-1α/miRNA-210/ISCU in a subset of HNPGLs that could have an impact on SDHB protein stability by a mechanism independent of SDH mutations, thus providing a foundation to better understand the functional interplay between HIF, miR-210, and mitochondria and its relevance in the pathogenesis of HNPGLs.

Cerebral salt wasting syndrome: postoperative complication in tumours of the cerebellopontine angle.

Cerebral salt wasting (CSW) is a rare complication in posterior fossa tumour surgery. We present two patients with cerebellopontine angle (CPA) tumours who developed cerebral salt wasting postoperatively. Both patients deteriorated in spite of intensive fluid and salt replacement. On CT scan the patients presented mild to moderate ventricular dilation, which was treated with an external ventricular drainage. After the resolution of hydrocephalus, fluid balance rapidly returned to normal in both patients and the clinical status improved. Identification and treatment of secondary obstructive hydrocephalus may contribute to the management of CSW associated to posterior fossa tumour surgery.

Cerebral salt wasting syndrome: postoperative complication in tumours of the cerebellopontine angle

Cerebral salt wasting (CSW) is a rare complication in posterior fossa tumour surgery. We present two patients with cerebellopontine angle (CPA) tumours who developed cerebral salt wasting postoperatively. Both patients deteriorated in spite of intensive fluid and salt replacement. On CT scan the patients presented mild to moderate ventricular dilation, which was treated with an external ventricular drainage. After the resolution of hydrocephalus, fluid balance rapidly returned to normal in both patients and the clinical status improved. Identification and treatment of secondary obstructive hydrocephalus may contribute to the management of CSW associated to posterior fossa tumour surgery (AU)

El síndrome pierde sal cerebral (CSW, en sus siglas en inglés) es una complicación rara en la cirugía de los tumores de la fosa posterior. Presentamos a 2 pacientes con tumores del ángulo pontocerebeloso que desarrollaron un CSW posquirúrgico. Ambos pacientes tuvieron un empeoramiento pese a la fluidoterapia y la reposición de sal intensivas. La tomografía computarizada (TC) mostraba una dilatación ventricular leve a moderada que fue tratada mediante un drenaje ventricular externo. Tras la resolución de la hidrocefalia el balance hidroelectrolí-tico se normalizó rápidamente en ambos pacientes y su situación clínica mejoró. La identificación y el tratamiento precoz de la hidrocefalia obstructiva pueden contribuir al tratamiento del síndrome pierde sal asociado a la cirugía de tumores de la fosa posterior (AU)

Simultaneous cochlear implantation and translabyrinthine removal of vestibular schwannoma in an only hearing ear: report of two cases (neurofibromatosis type 2 and unilateral vestibular schwannoma).

OBJECTIVE: The goal is to confirm the effectiveness of cochlear implantation performed at the time of surgery for tumor removal, using a translabyrinthine approach. STUDY DESIGN: This is a retrospective case review of two cases of vestibular schwannoma (VS) in the only hearing ear in which a cochlear implant (CI) was placed simultaneous to removal of VS through a modified enlarged translabyrinthine approach. The aim of the study is to confirm the effectiveness in terms of hearing rehabilitation. SETTING: The study involved a tertiary referral center in a hospital setting. PATIENTS: The study includes a report of two cases: a case of a Neurofibromatosis Type 2 (NF2) patient who received a MEDEL COMBI 40+ CI after a translabyrinthine surgery for removal of a large (4.0 cm) cystic VS; and a patient with a 1.2 cm VS in the only hearing ear who was submitted to the same strategy and operation, using a Nucleus 24 Contour CI. INTERVENTIONS: The whole strategy used in the two mentioned cases is outlined, including decision making, surgical interventions, and hearing rehabilitation programs. MAIN OUTCOME MEASURES: Surgical and audiometric outcome of two patients who underwent VS resection through a translabyrinthine approach and simultaneous cochlear implantation are shown. RESULTS: Postoperative results demonstrate that a CI could be successful after a translabyrinthine surgery for VS, with hearing performances similar to the best postlingual implanted deaf adults of other origin. CONCLUSION: The present cases demonstrate that cochlear implantation can be successful after a translabyrinthine approach for VS, regardless of the tumor size, the kind of patient (NF2, unilateral VS), and the type of implant. The results also are suggestive that cochlear implantation is more successful if done concurrent with surgery for tumor removal and before hearing is completely lost.