Intralabyrinthine schwannoma: Surgical management and cochlear lumen preservation.

The present video reports the surgical removal of an intralabyrinthine schwannoma. The video contains patient's medical history, preoperative radiological evaluations and detailed description of surgical steps of the procedure, consisting in labyrinthectomy, cochleostomy and insertion of a dummy electrode in the preserved cochlear lumen within the context of a subtotal petrosectomy.

Management of complicated temporal bone fracture.

The current video presents the surgical management of a complicated temporal bone fracture. The video contains patient's medical history, preoperative radiological evaluations, and detailed surgical approach to manage the disease. The current video presents the surgical management of a complicated temporal bone fracture. The video contains patient's medical history, preoperative radiological evaluations, and detailed surgical approach to manage the disease.

Drainage of a petrous apex cholesterol granuloma through an infracochlear approach.

The current video presents the surgical management of a cholesterol granuloma of the anterior petrous apex, through the infracochlear approach. The video contains patient's medical history, pre-operative radiological evaluation, surgical approach to the lesion and radiological follow up. Surgery was conducted by an otology and skull base team in a tertiary referral center. The patient is a 49-years-old male, who was referred to our center for a four months history of right tinnitus and fullness. The clinical evaluation was unremarkable and the audiometric testing showed a right sensorineural hearing loss with normal contralateral hearing. A high-resolution CT-scan of the temporal bone was performed showing a lesion occupying the right petrous apex and eroding the cochlea. In a subsequent MRI scan, the lesion appeared hyperintense in both T1- and T2-weighted images. Those radiological features prompted us to the diagnosis of a cholesterol granuloma, and the selected treatment was a drainage via infracochlear approach. The infracochlear approach, firstly described by Giddings et al. [1] in 1991, represents a direct route to the petrous apex, that can be chosen in selected cases with favorable anatomical conditions and that allows respecting of the hearing mechanism. After surgical drainage of the granuloma, no worsening of the pure tone threshold was confirmed by the audiological evaluation. The hospital stay was uneventful and the patient was discharge one day postoperatively. One-year postoperative MRI scan showed signal reduction of the cholesterol granuloma.

An uncommon cause of conductive hearing loss.

The current video presents the surgical management of a middle ear osteoma through a retroauricolar endocanalar approach, under local anesthesia. The video contains patient's medical history, pre-operative radiological evaluation, surgical approach to the lesion and clinical follow up.

Cochlear implantation after solid organ transplantation: long term results and review of the literature.

OBJECTIVE: To analyze rate and type of complications in cochlear implant (CI) recipients receiving immunosuppressive therapy following solid organ transplant (SOT). STUDY DESIGN: Retrospective case series. English language literature review. SETTING: Tertiary referral center. INTERVENTION: Cochlear implantation surgery following solid organ transplantation (SOT) and immunosuppressive therapy. METHODS: Data of patients who received CI after SOT and with at least one year of follow up were reviewed. Main outcome measures were the rate and type of complications, classified as major (requiring a second surgical procedure) and minor (requiring medical therapy). A search was performed in PubMed database on January 2019 using the keywords: organ transplant; cochlear implant, complications, deafness, solid organ transplant, immunosuppressive therapy. Only studies reporting on patients who have been implanted after the transplant procedure and with a follow up period of at least 1 year were considered. Final analysis was performed on pooled data. RESULTS: Four patients received CI surgery following SOT. Age at treatment ranged from 40 to 47 years (mean 44.25 years). Follow-up after implantation averaged 5.25 years (range 1-10 years), without complications. Review of the available literature on the subject yielded seven papers; a total of 26 procedures in 22 patients satisfied inclusion criteria. Pooled data from the present series and from the literature were analyzed; the global rate of complications was 16.6%, with 10% major (3 of 30 procedures) and 6.6% minor (2 of 30 procedures). The three reported cases of major complications appear unrelated to SOT. Major complications were found in one case over 16 procedures in pediatric patients (6.2%), while in adults the percentage raised to 14.3% (2/14 procedures). CONCLUSIONS: Cochlear implantation is a safe and effective intervention, even during immunosuppressive therapy after organ transplantation.

Gain-of-function mutations in DNMT3A in patients with paraganglioma.

PURPOSE: The high percentage of patients carrying germline mutations makes pheochromocytomas/paragangliomas the most heritable of all tumors. However, there are still cases unexplained by mutations in the known genes. We aimed to identify the genetic cause of disease in patients strongly suspected of having hereditary tumors. METHODS: Whole-exome sequencing was applied to the germlines of a parent-proband trio. Genome-wide methylome analysis, RNA-seq, CRISPR/Cas9 gene editing, and targeted sequencing were also performed. RESULTS: We identified a novel de novo germline mutation in DNMT3A, affecting a highly conserved residue located close to the aromatic cage that binds to trimethylated histone H3. DNMT3A-mutated tumors exhibited significant hypermethylation of homeobox-containing genes, suggesting an activating role of the mutation. CRISPR/Cas9-mediated knock-in in HeLa cells led to global changes in methylation, providing evidence of the DNMT3A-altered function. Targeted sequencing revealed subclonal somatic mutations in six additional paragangliomas. Finally, a second germline DNMT3A mutation, also causing global tumor DNA hypermethylation, was found in a patient with a family history of pheochromocytoma. CONCLUSION: Our findings suggest that DNMT3A may be a susceptibility gene for paragangliomas and, if confirmed in future studies, would represent the first example of gain-of-function mutations affecting a DNA methyltransferase gene involved in cancer predisposition.

Cochlear implantation in children with CHARGE syndrome: a report of eight cases.

OBJECTIVE: To report neuroradiologic findings, surgical strategies and clinical and audiological results in a series of children with CHARGE syndrome (CS) who had been evaluated for cochlear implantation (CI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral university hospital. PATIENTS: Eight profoundly deafened children with CS were included. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre- and post-operatively. Neuroradiological and intraoperative findings, surgical planning, and post-operative complications were analyzed. RESULTS: Six children were profoundly deaf from birth and 2 had progressive hearing loss to profound levels. Cochlear nerve deficiency (CND) was noted in 5 out of the 6 patients with congenital sensorineural hearing loss (SNHL). Seven children underwent CI. Surgery was performed using standard transmastoid facial recess approach in 3 ears, subtotal petrosectomy in 3, and transmastoid single-slit labyrinthotomy in one. Temporary facial palsy occurred in one patient. In the group of patients with congenital SNHL, 2 children benefitted from CI and developed spoken language; the remaining 3 children obtained improved access to environmental sounds and used signs and gestures as their main mode of communication. The two patients with progressive SNHL had preoperative verbal language and continued to use verbal language after CI. CONCLUSIONS: The constant presence of temporal bone anomalies in children with CS requires surgical expertise in performing non-standard approaches for safe and effective CI. Patients with progressive SNHL and normal cochlear nerves had satisfactory results with CI. Limited benefits have been observed in presence of CND.

Prevention and management of vascular complications in middle ear and cochlear implant surgery.

The objective of this study is to illustrate prevention strategies and management of vascular complications from the jugular bulb (JB) and internal carotid artery (ICA) during middle ear surgery or cochlear implantation. The study design is retrospective case series. The setting is tertiary referral university hospital. Patients were included if presented pre- or intraoperative evidence of high-risk anatomical anomalies of ICA or JB during middle ear or cochlear implant surgery, intraoperative vascular injury, or revision surgery after the previous iatrogenic vascular lesions. The main outcome measures are surgical outcomes and complications rate. Ten subjects were identified: three underwent cochlear implant surgery and seven underwent middle ear surgery. Among the cochlear implant patients, two presented with anomalies of the JB impeding access to the cochlear lumen and one underwent revision surgery for incorrect positioning of the array in the carotid canal. Subtotal petrosectomy was performed in all cases. Anomalies of the JB were preoperatively identified in two patients with attic and external auditory canal cholesteatoma, respectively. In a patient, a high and dehiscent JB was found during myringoplasty, while another underwent revision surgery after iatrogenic injury of the JB. A dehiscent ICA complicated middle ear effusion in one case, while in another case, a carotid aneurysm determined a cholesterol granuloma. Rupture of a pseudoaneurysm of the ICA occurred in a child during second-stage surgery and required permanent balloon occlusion without neurological complications. Knowledge of normal anatomy and its variants and preoperative imaging are the basis for prevention of vascular complications during middle ear or cochlear implant surgery.

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.

Are the current treatment strategies for facial nerve schwannoma appropriate also for complex cases?

OBJECTIVES: To describe the decision-making strategies for complex facial nerve schwannomas (FNSs). MATERIALS AND METHODS: Charts belonging to 103 consecutive patients with facial nerve tumors managed between 1990 and 2011 were examined retrospectively to identify complex FNSs. To be classified as complex, at least one of the following criteria had to be met: (1) FNS with large intraparotid tumor component and preoperative good facial nerve function (3 cases); (2) multiple-segment FNSs with extension to both the cerebellopontine angle and the middle cranial fossa in patients with preoperative good hearing (5 cases); (3) fast-growing FNS with preoperative good facial nerve function (4 cases), and (4) large FNS compressing the temporal lobe with preoperative normal facial nerve function (1 case). RESULTS: Thirteen patients were classified as complex; 12 patients had total tumor removal with sural nerve grafting and 1 patient had partial tumor removal. Two patients with intratemporal-intraparotid FNS underwent a transmastoid-transparotid approach. One patient with a tumor extending from the geniculate ganglion to the parotid portion of the facial nerve underwent a combined middle fossa transmastoid-transparotid approach. A transcochlear approach with temporal craniotomy was performed in all the patients with multiple-segment FNS as well as in patients with fast-growing tumors extending both in the cerebellopontine angle and middle cranial fossa. A partial tumor removal through the middle fossa approach was performed in 1 patient with a large tumor compressing the temporal lobe. CONCLUSIONS: Therapeutic options for patients with FNS include surgical intervention, observation and radiotherapy. Nowadays, surgical resection with facial nerve repair is usually the standard management for patients with poor facial function (House-Brackmann grade III or worse). In patients presenting with normal or near-normal facial nerve function, initial observation with periodic examination and imaging is usually recommended. However, on rare occasions surgeons can be faced with a situation in which the management decision-making process is particularly challenging. In these complex cases treatment should be individualized. We recommend early surgical intervention regardless of the preoperative facial and hearing functions in the following cases: intratemporal FNSs extending with a large tumor component into the parotid, multiple-segment FNSs extending in both the cerebellopontine angle and the middle cranial fossa, fast-growing FNSs, and large FNSs with temporal lobe compression.

Congenital Retrosigmoid Cholesteatoma: Case Series and Literature Review.

BACKGROUND: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity. METHODS: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier. RESULTS: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases. CONCLUSION: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Revision cochlear implant surgery for clinical reasons.

Objective: To report the authors' experience in a series of patients treated with cochlear implant (CI) revision surgery due to medical problems. Methods: Revision CI surgeries performed in a tertiary referral centre for medical reasons not related to skin conditions were reviewed; patients were included if device removal was required. Results: 17 cochlear implant patients were reviewed. The main reasons requiring revision surgery with device removal were: retraction pocket/iatrogenic cholesteatoma (6/17), chronic otitis (3/17), extrusion in previous canal wall down procedures (2/17) or in previous subtotal petrosectomy (2/17), misplacement/partial array insertion (2/17) and residual petrous bone cholesteatoma (2/17). In all cases surgery was performed through a subtotal petrosectomy. Cochlear fibrosis/ossification of the basal turn was found in 5 cases and uncovered mastoid portion of the facial nerve in 3 patients. The only complication was an abdominal seroma. A positive difference was observed between the number of active electrodes and comfort levels before and after revision surgery. Conclusions: In CI revision surgeries performed for medical reasons, subtotal petrosectomy offers invaluable advantages and should be considered as first choice during surgical planning.

Current trends on subtotal petrosectomy with cochlear implantation in recalcitrant chronic middle ear disorders.

Objective: To establish the safety and effectiveness of subtotal petrosectomy with cochlear implantation in patients affected by chronic middle ear disorders to refractory to previous surgical treatments. Methods: A multicentre, retrospective study was conducted on patients affected by recalcitrant chronic middle ear disorders who underwent cochlear implantation in combination with subtotal petrosectomy. Patients' details were collected from databases of 11 Italian tertiary referral centres. Additionally, a review of the most updated literature was carried out. Results: 55 patients were included with a mean follow-up time of 44 months. Cholesteatoma was the most common middle ear recurrent pathology and 50.9% of patients had an open cavity. 80% of patients underwent a single stage surgery. One case of explantation for device failure was reported among the 7 patients with post-operative complications. Conclusions: Subtotal petrosectomy with cochlear implantation is a benchmark for management of patients with recalcitrant chronic middle ear disorders. A single stage procedure is the most recommended strategy. Optimal follow-up is still debated. Further studies are required to investigate the role of this surgery in paediatric patients.

Vertebral artery involvement by tympanojugular paragangliomas: management and outcomes with a proposed addition to the fisch classification.

OBJECTIVES: To present the management and outcomes of cases of tympanojugular paraganglioma (TJP) with vertebral artery (VA) involvement, and to propose the addition of the new 'V' category to the Fisch classification. MATERIALS AND METHODS: Retrospective analysis of data from a quaternary neuro-otologic and skull base referral center. We studied 230 TJP patients to identify the cases with VA involvement and present their management, surgical findings and outcome. RESULTS: Out of 230 patients, 8 patients were found to have VA involvement by TJP. The extradural artery was involved in 1 patient and the intradural VA was involved in 6 patients. The intradural and extradural VA were simultaneously involved in 1 patient. Seven of the 8 patients underwent surgery. In 2 patients, preoperative occlusion was performed (1 with balloon, 1 with coils). In 5 of these 7 patients, the tumor was successfully separated from the VA by microdissection with or without endovascular intervention. In another patient, the tumor together with the involved VA was removed. Of 7 patients who had surgery, 1 patient did not undergo endovascular intervention and had subtotal tumor removal from the VA. There were no serious complications in removing the tumor from around the VA. CONCLUSION: Although uncommon, the intradural or extradural VA can be involved by TJP. To avoid vascular accidents, the VA should be thoroughly evaluated at the planning stage. We advocate that the addition of a 'V' category would help to identify patients who need individualized VA management as part of a complete surgical evaluation.

Effects of early commercial air travel on graft healing rates after tympanoplasty.

OBJECTIVES: We sought to determine whether commercial air travel soon after tympanoplasty significantly affects graft healing rates. METHODS: We performed a retrospective analysis of 169 patients who underwent tympanoplasty from 1993 to 2009, comparing two groups of patients: 69 patients who flew 1 day after surgery and 100 who did not. The confounding factors analyzed were side of surgery, size of perforation, surgical approach, graft material, and grafting technique. The primary outcome measure analyzed was successful closure of the perforation at the first follow-up visit, at 4 weeks, evidenced by direct otoendoscopic examination. RESULTS: There was no significant difference in the confounding variables between the two groups. There was no significant difference in the primary outcome measure of graft healing rates between the two groups (p = 0.494). Additionally, the overall graft healing rates compared favorably with previously published data from other authors. CONCLUSIONS: Early commercial air travel after tympanoplasty does not significantly affect graft healing rates and should be considered a relatively safe option.

Congenital mastoid cholesteatoma: case series, definition, surgical key points, and literature review.

OBJECTIVES: We evaluate 3 new cases of congenital cholesteatoma confined to the mastoid process, and compare them with cases presented in the literature in order to better define this rare lesion. METHODS: We performed a retrospective chart analysis of all congenital cholesteatomas treated surgically in a tertiary referral and skull base center. We performed a complete analysis (history, radiologic, and surgical) of all patients with congenital cholesteatoma confined to the mastoid process; we then performed a literature review and compared our findings with the presented cases. RESULTS: The results of preoperative imaging were in line with the surgical findings. The most important surgical issue in this type of lesion was the management of the sigmoid sinus and the jugular bulb. Half of the cases previously reported in the literature appeared not to fulfill the definition criteria of a congenital cholesteatoma of the mastoid process. CONCLUSIONS: Congenital cholesteatoma confined to the mastoid process is a rare lesion, and is even more exceptional upon critical review of the literature. Symptoms are often lacking or nonspecific, and although cases have a congenital origin, the diagnosis often is not made until adulthood. A combined congenital cholesteatoma group with middle ear and mastoid features seems to fill in the gap in the definition. Management of the sigmoid sinus and the jugular bulb is the most demanding surgical key point.

Management of cochlear fistula and the role of hearing: a five-case report.

OBJECTIVE: Cochlear fistula (CF) is a rare finding, usually associated with extensive middle ear cholesteatoma. There is agreement on the fact that removing the cholesteatoma matrix on a CF exposes the ear to a high risk of sensorineural hearing loss or dead ear. The aim of the study is to describe the presentation, possible treatment strategies and related outcomes for patients with CF in chronic otitis media (COM). METHODS: The study considers a retrospective case series of patients with CF diagnosis supported by CT-scan and intraoperative/otoscopic evidence. RESULTS: Five cases of CF were identified, 4 associated with cholesteatoma and 1 associated with non-cholesteatomatous COM. Two patients presenting with anacusis underwent a subtotal petrosectomy. Two patients with useful hearing underwent a radical mastoidectomy in order to preserve the cholesteatoma matrix on the promontorium. One patient with good hearing and COM was treated conservatively. CONCLUSIONS: Conservative management should be considered for rare cases of CF in COM with residual hearing. Matrix preservation through radical/modified radical mastoidectomy is strongly advised in the presence of useful preoperative bone conduction. Subtotal petrosectomy should be considered the preferred option in presence of cholesteatoma with preoperative profound hearing loss.

Capillary Venous Malformation With Secondary Aneurysmal Bone Cyst of Temporal Bone.

Aneurysmal bone cysts (ABCs) arising from vascular malformation are extremely rare, and none have been reported in the literature in English till now. We report a very rare case of secondary ABC of left temporal bone in a 5-year-old Caucasian boy who presented with a left sudden facial palsy associated with a painless non-tender mass of the left temporo-parietal region. The computed tomography (CT) and magnetic resonance imaging (MRI) features were suggestive of ABC secondary to a capillary venous malformation, with concurrent involvement of the squamous, mastoid, and petrous portions of the temporal bone. Surgical resection was performed. On follow-up, the patient was found to be doing well.

Bedside Surgical Tracheostomy in the Intensive Care Unit during Covid-19 Pandemic.

OBJECTIVES: To describe Otolaryngologists' perspective in managing COVID-19 patients with acute respiratory distress syndrome (ARDS) requiring tracheostomy in the ICUs during the pandemic peak in a dramatic scenario with limited resources. SETTING: Tertiary referral university hospital, regional hub in northern Italy during SARS CoV 2 pandemic peak (March 9th to April 10th, 2020). METHODS: Technical description of open bedside tracheostomies performed in ICUs on COVID-19 patients during pandemic peak with particular focus on resource allocation and healthcare professionals coordination. A dedicated "airway team" was created in order to avoid transportation of critically ill patients and reduce facility contamination. RESULTS: During the COVID-19 pandemic, bedside minimally invasive tracheostomy in the ICU was selected by the Authors over conventional surgical technique or percutaneous procedures for both technical and operational reasons. Otolaryngologists' experience derived from direct involvement in 24 tracheostomies is reported. CONCLUSIONS: Tracheostomies on COVID-19 patients should be performed in a safe and standardized setting. The limited resources available in the pandemic peak required meticulous organization and optimal allocation of the resources to grant safety of both patients and healthcare workers.