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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.

To analyze rate and type of complications in cochlear implant (CI) recipients receiving immunosuppressive therapy following solid organ transplant (SOT). Retrospective case series. English language literature review. Tertiary referral center. Cochlear implantation surgery following solid organ transplantation (SOT) and immunosuppressive therapy. 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. 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). Cochlear implantation is a safe and effective intervention, even during immunosuppressive therapy after organ transplantation.

Aim of the study was to evaluate if the introduction of the imaging checklist TeSLANO would improve the identification of crucial anatomical findings from temporal bone computed tomography (TBCT) scans among a cohort of Otolaryngology residents. Single-blinded prospective cohort study. Tertiary care university hospital and referral center for otology and skull base surgery. The cohort was composed by eight Otorhinolaryngology residents (four junior and four senior) that were individually asked to identify all relevant anatomic findings from preoperative TBCT scans. Supervising surgeon showed to each resident four TBCT scans before and four after the introduction of a systematic checklist evaluation system based on the TeSLANO acronym. Statistical analysis was performed using McNemar’s test and results were considered significant with a P value

Background and aim: Dermoid cysts (DC) of the head and neck are rare congenital anomalies derived from entrapment of ectodermal cells at lines of fusion in the embryo into mesoderm. Methods: We describe a 22-years-old female with an unusual presentation of DC in the subcutaneous tissue of the retro-auricular region. Results: The pathological examination of the surgically removed specimen confirmed the diagnosis of Dermoid Cysts. Conclusions: A DC in this region is rare and may be misdiagnosed as a retro-auricular lymph node. Complete excision of the lesion must be achieved with pathology study to confirm diagnosis. (www.actabiomedica.it)

Noonan syndrome (NS) is a congenital disorder characterized by a wide heterogeneity in clinical and genetic features. Hearing loss can frequently occur in NS, although not always mentioned in its diagnostic criteria. We are reporting on a child with an established NS who underwent bilateral cochlear implantation (CI) in the setting of cochlear nerve deficiency.We present the case of a child-girl affected by NS. Newborn hearing screening and audiological evaluations reveled an asymmetric sensorineural hearing loss (SNHL), profound at left ear and severe at right ear. Hearing aids were fitted at the age of six months. Brain magnetic resonance imaging showed hypoplastic cochlear nerves. Due to progressive worsening of the hearing thresholds and inappropriate speech development, at the age of 2 years she underwent a left-sided cochlear implantation. Four years later, right ear was also implanted. Six years after the first surgery, a partial extrusion of the electrode array was noticed. Explantation and reimplantation of a new device was performed, adopting a subtotal petrosectomy approach. The patient reached a score of 95% in open-set speech perception tests.Hearing loss is a frequent finding in patients with NS; however, its nature and severity are very heterogenous. In consideration of the possible progression of SNHL, audiological follow-up in NS patients must be carefully and periodically performed so as to early detect worsening of hearing threshold. If indicated, cochlear implantation should be considered, taking account of audiological and systemic features of this syndrome.

To report our experience in performing cochlear implantation under local anesthesia in a group of patients who were deemed unfit for general anesthesia. A retrospective chart review was performed to analyze undesirable events and any other discomfort complained by patients during cochlear implantation. Analysis of patient’s satisfaction was performed by means of a survey instrument. We have also compared the duration of surgery and hospitalization time with a control group that was implanted under general anesthesia. Twenty-one cochlear implantation in 20 patients were performed under local anesthesia. Age of patients ranged from 38 to 85 years. All interventions were successfully completed without any conversions to general anesthesia. Discomfort during surgery was reported in five cases: vertigo triggered by electrode insertion in two patients, pain during the round window approach in two patients and distress during the use of drill in one case; no patient experienced agitation. During the postoperative period, no complications or unpleasant experiences were reported. Only two patients stated that they would not perform cochlear implantation again under local anesthesia. Lower duration surgery and hospitalization time were found in the local anesthesia group. Local anesthesia with conscious sedation is a safe and effective alternative for cochlear implant candidates considered unfit for general anesthesia. Fundamental for a successful procedure are preoperative counselling, accurate selection of the patients and constant intraoperative assistance. Unfeasibility of facial nerve monitoring and minor detrimental effect on training are the principal disadvantages in performing cochlear implantation under local anesthesia.

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). Retrospective case series. Tertiary referral university hospital. 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. 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. 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.

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.

Backgrounds: To describe presentation, diagnosis and management of a case of spontaneous temporal bone pneumocele with large intracranial extension and brain compression associated with external auditory canal pneumatocele. Methods: A 34-year-old male patient was referred for a large intracranial epidural air collection compressing the right cerebral hemisphere. The patient was asymptomatic except for persisting fullness of the right ear for which he performed frequent Valsalva maneuvers. CT scan showed luminal enlargement of right temporal bone with thinning of the limiting bone, focal dehiscences at the level of the external auditory canal (EAC) and the medial wall of the mastoid tip and remodeling of the calvarial bone. Audiometry was normal bilaterally. A retroauricular transmastoid approach was performed reconstructing the defect in the EAC using bone paste and fibrin glue. The epidural cavity was sealed off by means of temporalis fascia, abdominal fat graft and fibrin glue. Results: After 17 months, MRI showed complete resolution of the intracranial air collection with expansion of the right cerebral hemisphere. Conclusions: Intracranial extension of a temporal bone pneumocele is a rare condition due to progressive expansion of mastoid pneumatization in the epidural space with bone resorption, enlargement of the aerial spaces and chronic calvarial bone changes. Repeated Valsalva maneuvers may sustain the remodeling process. Although usually asymptomatic, there is high risk of acute mass effect particularly in case of trauma and infections. A correct diagnosis is the necessary premise for appropriate treatment. Keywords: Pneumocele, Pneumatocele, Hyperpneumatization, Temporal bone, Pneumocephalus

Objective To present the first case of osteonecrosis of the external auditory canal associated with sorafenib treatment. Patient 58-year-old patient with right-sided otorrhea and otalgia was treated for otitis externa for 1 month without improvement. Otoscopic examination revealed a large defect in the inferior wall of the tympanic bone filled with skin debris and bony fragments. Previous medical history included treatment with sorafenib for metastatic renal cell cancer; he had never been exposed to radiotherapy. Computed tomography of the temporal bone showed a large right external auditory canal bony erosion with involvement of the tympanic bone and bony sequestra extending to the mastoid cells and temporomandibular joint. Histologic examination revealed necrotic bone and inflammatory changes with no signs of malignancy. A diagnosis of osteonecrosis of external auditory canal was made. Intervention Right subtotal petrosectomy with obliteration of surgical cavity with abdominal fat was performed. Results Final histological report revealed avascular necrosis of the bone with perivascular lymphocitic infiltration of the soft tissues. Diagnosis of medication-related external auditory canal osteonecrosis was confirmed. Conclusion Medication-related osteonecrosis of the temporal bone is not a well-known entity among otolaryngologists and could therefore be misclassified as another diagnosis. In patients with othorrea and earache following sorafenib treatment, temporal bone osteonecrosis should be suspected.

The high prevalence of middle ear disease with related hearing loss in Kabuki syndrome requires the diagnostic and treatment expertise of otologists. This case report describes outcomes and changes in the quality of life of a patient affected by Kabuki syndrome with a history of recalcitrant chronic otitis media and mixed hearing loss who had undergone several unsuccessful surgical procedures before solving his problems by means of subtotal petrosectomy and active middle ear implant. (www.actabiomedica.it)

Objective: To report clinical presentation, management and outcomes of a rare complication of cochlear implant surgery. Patient: A 68-year-old man, affected by profound bilateral deafness because of superficial cerebral hemosiderosis, presented to Authors’ Department 8 days after cochlear implant surgery with vomiting, fever, and mental confusion. Brain computed tomographic (CT) scan showed a massive collection of intracranial air from an osteodural defect in the right tegmen mastoideum because of repeated nose blowing in the postoperative period. Intervention: A multilayer reconstruction of the tegmen with obliteration of the mastoid cavity using abdominal subcutaneous adipose tissue was performed, preserving the cochlear implant in place. Main Outcome and Results: Following surgery the patient showed rapid neurological improvement and CT scan performed 2 days later showed complete resolution of the intracranial air collection. He is currently using the cochlear implant with open set performances. Conclusion: Pneumocephalus is a rare complication of cochlear implant surgery. In patients with severe neurological signs following cochlear implantation (CI), pneumocephalus should be suspected. Drilling of mastoid air cells may expose dura mater and positive high pressure events may break meningeal layers and force air into the cranial cavity.

Objective Marshall syndrome is a genetic disorder caused by mutations in the COL11A1 gene. This syndrome is characterized by skeletal, ophthalmologic, craniofacial, and auditory abnormalities. Hearing loss is among the main manifestations reported in this disorder being observed in approximately 80% of affected individuals.The present study aims to describe the audiologic characteristics of three members of a family with Marshall syndrome and also serves as a review of the literature. Study design Family study. Setting Tertiary care otology and skull base center. Patients We report the audiologic findings in a family with Marshall syndrome consisting of a mother and her son and daughter. Intervention(s) The audiologic evaluation included tympanometry, acoustic reflexes testing, auditory brainstem response, transient otoacoustic emissions, pure-tone audiometry, speech audiometry in quiet, and conditioned play audiometry. These methods were applied according to the age of the patients. In addition, we provide a review of the English-language literature in an attempt to clarify the auditory phenotype of this syndrome. Results All 3 affected individuals had heterozygous c.3816+1G>A mutation in the splicing donor site of intron 50 of the COL11A1 gene. All three patients in our study had bilateral sensorineural hearing loss. Hearing impairment ranged from mild to moderate in the daughter, moderate in the son, and from mild to moderate in their mother. Conclusion The majority of individuals with Marshall syndrome present early-onset bilateral sensorineural hearing loss. Hearing impairment is usually detected in early childhood, progresses gradually, and becomes stable in late adulthood, with a severity ranging from mild to severe.

Tonsillar lymphoma usually presents as unilateral or bilateral infiltration of diffuse large B‐cell lymphomas. We report a case of a 79‐year‐old man with near‐complete obstruction of the upper airways due to T‐cell prolymphocytic leukemia cells. Surgical resection was safely performed to reduce burden of disease.

Objective The clinical features and surgical results of “closed type” versus “open type” congenital cholesteatoma were compared in order to analyse the differences between the two forms; whether the morphology of the disease may have a role in the staging systems has been also evaluated. Patients and methods We reviewed retrospectively 95 patients (96 ears) who underwent surgery for congenital cholesteatoma over a 15-year period focusing on the clinical differences between open and closed type congenital cholesteatoma. Results Seventy-one patients (74%) had a closed-type and 25 (26%) an open type congenital cholesteatoma. Our study confirmed the higher prevalence of the closed type, as well as, a younger age at initial diagnosis compared with the open type congenital cholesteatoma. Other differences between the two forms were: modality of diagnosis (pathognomonic otoscopy in 100% of the closed type and in 40% of the open type), positive history for otitis media with effusion (51.4% in closed type vs 20% in open type), involvement of the tympanic membrane quadrants (anterior quadrants were more frequently involved in the closed forms, whereas posterior quadrants were more frequently involved in the open forms), disease extension and aggressiveness. A residual cholesteatoma was found in 6 out of the 71 patients (8.4%) with a closed type congenital cholesteatoma and in 10 out of the 25 patients (40%) with an open type congenital cholesteatoma. After adjusting for potential confounders, open-type congenital cholesteatoma was significantly associated with residual cholesteatoma compared to the closed-type (odds ratio [OR] 7.39, 95% confidence interval [CI] 1.10–49.77, p = 0.03). Conclusion This study confirmed that the open congenital cholesteatoma has global clinical features that are uniquely different from the classical closed form. These differences could reflect a distinct pathogenesis, but there is no proof of this to date. The classification of the congenital cholesteatoma could be further refined by adding the morphologic type of the disease.

Object The authors evaluated the behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas (VSs). Methods The case records of all patients who underwent surgical treatment of VSs were analyzed. All patients in whom an incomplete excision had been performed were analyzed. Incomplete excision was defined as near-total resection (NTR), subtotal resection (STR), and partial resection (PR). Tumors in the NTR and STR categories were followed up with a wait-and-rescan approach, whereas the tumors in the PR category were subjected to a second-stage surgery and were excluded from this series. All patients included in the study underwent baseline MRI at the 3rd and 12th postoperative months, and repeat imaging was subsequently performed every year for 7–10 years postoperatively or as indicated clinically. Preoperative and postoperative facial function was noted. Results Of the 2368 patients who underwent surgery for VS, 111 patients who had incomplete excisions of VSs were included in the study. Of these patients, 73 (65.77%) had undergone NTR and 38 (34.23%) had undergone STR. Of the VSs, 62 (55.86%) were cystic and 44 (70.97%) of these cystic VSs underwent NTR. The residual tumor was left behind on the facial nerve alone in 62 patients (55.86%), on the facial nerve and vessels in 2 patients (1.80%), on the facial nerve and brainstem in 15 patients (13.51%), and on the brainstem alone in 25 patients (22.52%). In the 105 patients with normal preoperative facial nerve function, postoperative facial nerve function was House-Brackmann (HB) Grades I and II in 51 patients (48.57%), HB Grade III in 34 patients (32.38%), and HB Grades IV–VI in 20 patients (19.05%). Seven patients (6.3%) showed evidence of tumor regrowth on follow-up MRI. All 7 patients (100%) who showed evidence of tumor regrowth had undergone STR. No patient in the NTR group exhibited regrowth. The Kaplan-Meier plot demonstrated a 5-year tumor regrowth-free survival of 92%, with a mean disease-free interval of 140 months (95% CI 127–151 months). The follow-up period ranged from 12 to 156 months (mean 45.4 months). Conclusions The authors' report and review of the literature show that there is undoubtedly merit for NTR and STR for preservation of the facial nerve. On the basis of this they propose an algorithm for the management of incomplete VS excisions. Patients who undergo incomplete excisions must be subjected to follow-up MRI for a period of at least 7–10 years. When compared with STR, NTR via an enlarged translabyrinthine approach has shown to have a lower rate of regrowth of residual tumor, while having almost the same result in terms of facial nerve function.

The primary goals of surgery of tympanomastoid paragangliomas (TMPs) are tumor eradication and hearing preservation. Though the surgical management of TMPs has been dealt with widely in the literature, the effects of TMPs themselves on preoperative hearing and the audiological outcomes after surgery have not been analyzed in detail. This article comprehensively evaluates the preoperative hearing and the long-term hearing outcomes after surgery of TMPs. This study is based on a study population of 145 patients which is the largest reported in the literature. The surgical approaches for all patients with TMPs were formulated according to an algorithm developed by the authors. Complete tumor removal with excellent hearing results can be achieved by approaching the tumor classes by the right surgical technique. TMPs could possibly induce sensorineural hearing loss in higher frequencies, and future studies could be directed towards this.

Background The classic infratemporal fossa type A approach (IFTA-A) permits superior and anterior exposure of the jugular foramen. The transcondylar-transtubercular extension facilitates posteroinferior and medial access to the jugular foramen. The purpose of this study was to present the IFTA-A with transcondylar-transtubercular extension and to assess its surgical results. Methods A review of patients with tympanojugular paraganglioma who underwent resection through the IFTA-A with transcondylar-transtubercular extension was performed. Results In all, 39 patients were included in the study. The average follow-up was 23.6 months. Gross total tumor removal was achieved in 87.2% of the cases and there was evidence of recurrence in 5.9% of this group. Conclusion The transcondylar-transtubercular extension of the classic IFTA-A is aimed at making the excision of Fisch type C2 to C4 tympanojugular paragangliomas simpler and safer by drilling out one third of the lateral part of the occipital condyle and removing the jugular process and jugular tubercle. © 2013 Wiley Periodicals, Inc. Head Neck 36: 1581–1588, 2014

Background and aim: As in other syndromes characterized by craniofacial anomalies, middle ear cholesteatoma is known to have a high prevalence in Turner syndrome. The aim of this study was to review a multicenter experience with the surgical management of middle ear cholesteatoma in children with Turner syndrome. Methods: We retrospectively analyzed sixteen girls with Turner syndrome who underwent otologic surgery for middle ear cholesteatoma between January 2000 and December 2012. Surgery was performed in 3 tertiary care otologic centers. Four patients had bilateral disease, resulting in a total of 20 ears treated. The following data were recorded: age, history of ventilation tube insertion, status of the controlateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. Results: Follow-up ranged from 3 to 15 years (mean 7 years). Fourteen ears underwent canal wall down mastoidectomy: no cases of recurrent cholesteatoma were observed in these cases; revision mastoidectomy with cavity obliteration was needed in 2 ears (14.3%) for recurrent otorrhea. In the remaining 6 ears a staged canal wall up mastoidectomy was performed: 1 child showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A postoperative air-bone gap result of 0 to 20 dB was achieved in 6 ears (30%); in 9 ears (45%) postoperative air-bone gap was between 21 and 30 dB, while in 5 (25%) was >30 dB. Bone conduction thresholds remained unaffected in all cases. Conclusions: Cholesteatoma in children with Turner syndrome is a challenging entity for the otologic surgeon. Although not mandatory, canal wall down mastoidectomy should be regarded as the technique of choice to achieve a safe and dry ear in TS children with middle ear cholesteatoma. Intact canal wall mastoidectomy should be adopted only in appropriately selected patients such as those with limited attic cholesteatoma that can be regularly followed-up. (www.actabiomedica.it)

Objective The objectives of this study were to evaluate outcomes from facial nerve (FN) cable grafting in patients who experienced FN transection during vestibular schwannoma removal and to compare the FN outcomes of patients who underwent FN grafting by using fibrin glue with those of patients who underwent FN grafting by using microsuture. Material and methods We retrospectively evaluated a series of 33 patients in whom FN grafting was achieved either by using microsuture (8 cases) or fibrin glue (25 cases). Immediate repair of the FN was performed in all cases at the time of initial resection. The patients FN function was assessed preoperatively, in the immediate postoperative period, and at 3, 6, 9, and 12 months or more postoperatively using the House-Brackmann grading system. All patients had at least 1-year follow-up. Results At 12 months, a House-Brackmann grade III was achieved in 75% of those who underwent cable nerve graft interposition by using microsuture and in 76% of those who underwent cable nerve graft interposition by using fibrin glue. Analysis of final FN function outcomes demonstrated no statistically significant difference in FN outcomes between the 2 groups ( P = .891, Mann-Whitney U test; P = .1, Fisher exact test). Conclusions The functional results after FN cable grafting by using fibrin glue exclusively were equivalent to those obtained with microsuture. However, the technique of FN repair by means of fibrin glue is technically simple, less time-consuming, and imparts less trauma on the nerve than does the traditional suture method.

Objectives Our protocol to manage the intralabyrinthine schwannoma (ILS). Methods Retrospective chart review of 7 consecutive patients managed for ILS. Results Five patients underwent surgical removal of the lesions and none experienced significant complications or recurrent disease. One patient refused surgical treatment and was closely followed by serial MRI scans with no signs of tumor growth. One patient is presently managed conservatively due to a good hearing. Conclusions Diagnosis of ILS is based on high resolution MRI scans and should be included in the differential diagnosis of patients investigated for cochleovestibular symptoms. Treatment modality of ILS is controversial and depends patients’ age, severity of vertigo and hearing loss. In the authors’ experience surgery is indicated in all cases with invalidating vertigo and in the young patients with severe hearing loss. Conservative strategy is advised in older patients and in cases with preserved good hearing and no invalidating vertigo. These patients must be followed up using high resolution MRI because of the risk of tumor growth into the internal auditory canal. While tumors with exclusive involvement of the inner ear may be removed by means of a completely extradural approach, tumor growth with involvement of the internal auditory canal dictates the necessity of opening the dura exposing the patients to the potential risks associated with the procedure.

Objective: Extensive involvement of the internal carotid artery (ICA) by jugular and vagal paragangliomas represents a challenging problem for skull-base surgeons: radical tumor resection and preservation of the vessel often cannot be achieved in these cases. The aim of this article was to report the management of a complex case of right ipsilateral jugular and vagal paragangliomas in a patient with absence of the contralateral ICA due to a previous removal of a left carotid body tumor. Surgical removal of the lesions was performed after stenting of the cervical and intratemporal segment of the ICA. Methods: The charts and all the examinations of the patient were retrospectively reviewed. The patient, a 40-year-old man, was treated at the Gruppo Otologico, a private quaternary referral center for neurotology and skull-base surgery. Results: The tumors were completely resected during surgery and manipulation of the ICA was greatly facilitated by the stent. No complication occurred during or immediately after surgery; after a follow-up period of 24 months there was no sign of recurrence and imaging studies demonstrated patency of the stented vessel. Conclusion: This report suggests that placement of endovascular stents in the cervical and temporal segments of the ICA has the potential of changing the whole therapeutic management in cases of temporal and vagal paragangliomas that surround and invade the vessel, especially in those cases in which permanent balloon occlusion of the ICA is not feasible.

Objectives To describe long-term clinical outcomes of cochlear implantation in deaf children with symptomatic epilepsy. Materials and methods A retrospective data analysis review of patients implanted at the Cochlear Implant Center of the University of Parma, Italy, was performed, searching for implanted children with a confirmed diagnosis of symptomatic epilepsy. Clinical data, imaging findings, pre- and post-operative epilepsy pattern and EEG traces were analyzed; communicative skills were assessed using the Profile of Actual Linguistic Skills. Results Search retrieved two patients affected by profound bilateral sensorineural hearing loss and symptomatic epilepsy (associated respectively with methylmalonic acidemia and cerebral palsy). After careful parental counselling both patients were offered and underwent cochlear implantation. Activation and use of cochlear implant did not determine substantial changes of pre-existing seizure pattern and EEG traces. Both patients showed substantial development of their communicative abilities. Conclusions Cochlear implantation in children with symptomatic epilepsy did not determine variations in seizure pattern or EEG traces. Both patients experienced substantial benefit from cochlear implantation.

Cholesteatoma usually arises in the middle ear by the formation of a retraction pocket or a tympanic membrane perforation. In some cases, cholesteatoma presents behind an intact tympanic membrane (ITMC) and the underlying mechanism of its development is controversial. The aim of this study was to describe clinical features, pathogenesis and surgical results in a series of adult patients affected by ITMC.We analyzed retrospectively 27 adult patients (age18 years) diagnosed with ITMC who underwent surgery between 1994 and 2013. We investigated the demographic data, presenting symptoms, otoscopic findings, disease location, surgical technique, postoperative complications and outcomes.Diagnosis was made on the basis of a white mass seen through the tympanic membrane in 24 cases and on explorative surgery in the remaining 3 patients. In 16 cases cholesteatoma was related to an acquired cause, while in 11 ears a congenital origin was supposed. Hearing loss was the chief complaint and it was present in 19 (70.3%) subjects. Cholesteatoma was managed by purely endaural or retroauricular transcanal tympanoplasty in 12 cases, by planned staged canal wall up mastoidectomy in 10 cases (37%), by canal wall down mastoidectomy in 3 cases, and by modified Bondy technique in 2 cases. A recurrent cholesteatoma was observed in one ear; one patient experienced a postoperative profound sensorineural hearing loss.ITMC in adults may have both congenital and acquired origin. It may grow silently over many years and develops into a massive size before being detected. Each patient's management should be tailored to clinical findings.

The objective was to evaluate the long-term clinical outcome of the bony outer attic wall reconstruction (scutumplasty) by using autogenous bone paté in a series of patients who underwent canal wall up (CWU) mastoidectomy for middle ear cholesteatoma. In addition, an histological analysis of bone paté graft specimens taken during the second-look procedure 12 months after the first surgery, was carried out. A series of 134 ears from 128 patients with cholesteatoma who underwent staged CWU mastoidectomy and attic reconstruction with bone paté, between 1995 and 2000 inclusive, was retrospectively analyzed. In ten randomly selected ears, biopsies from the newly built outer attic wall were taken during the second-look operation and were microscopically analyzed. At the second stage operation, the bone paté graft was found normal in 64.9% of cases. In 39 (29.1%) patients there was a remarkable hypertrophy of the bone paté which was subsequently thinned out by using a diamond burr. A partial resorption of the bone paté was observed in 4.4% of ears and a total resorption in 1.4%. Residual cholesteatoma was found in 13.4% of ears. At the last follow-up visit, recurrent cholesteatoma was observed in 5.2% of ears. Self cleaning attic retraction pocket occurred in 4.4% of ears. Twelve months after implantation, histologic examination on the newly built bone showed a well-structured bony tissue. In particular, osteoblasts lined bone trabeculae with signs of an active tissue remodeling. In conclusion, the reconstruction of the outer attic wall should be considered a fundamental step when performing a CWU procedure in order to prevent attic retractions and recurrences of cholesteatoma. In our experience, autogenous bone paté graft has demonstrated good long-term results in outer attic wall reconstruction being adaptable, well tolerated, stable and of low cost.

Many treatment modalities of ingrown toenail are reported in the literature, often associated with unacceptably high recurrence rate. The authors present their technique, which aims at reducing the convexity of the nail fold. After complete removal of the nail plate and accurate debridement of the granulomatous tissue, a wedge-shaped ellipsis of skin and subcutaneous tissue, lateral to the affected nail fold, is removed. Approximation of the margins of the resulting defect determines eversion of the nail fold. One hundred twenty ingrown toenails were treated with the wedge excision of the nail fold at the outpatient clinic of the department of plastic surgery, Campus Bio-Medico University, Rome, Italy, between January 1998 and January 2002. Six recurrences were observed. In addition to the high cure rate, short postoperative pain duration, and morbidity as well as low risk of postoperative infection, the remarkable esthetic results achievable with this method are indicated.

From 1996 through 2001, the authors performed 26 vulvo-perineal reconstructions after vulvar cancer resection. A unilateral or bilateral medial thigh septo-fascio-cutaneous island flap was used in all cases. The vascular supply to this flap is guaranteed by the underlying fascial plexus that arises from the convergence of three longitudinal rows of vessels (anterior, medial, and posterior). The flap is fashioned in a triangle shape and incorporates the underlying muscular fascia, sparing the intermuscular septal perforators. It is advanced in a V-Y fashion and is easily adapted and secured to the recipient site. Competent urethral and anal meati are reconstructed with multiple intercalated flaps. Scarring at the donor site is limited and acceptable. The nerve supply guarantees acceptable sensation at the reconstructed area. The medial thigh septo-fascio-cutaneous flap allows a valid one-stage vulvo-perineal reconstruction with a simple surgical execution process after superficial soft tissue resections.

The objective of this study was to evaluate the long-term outcomes of patients with Cogan syndrome (CS) who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of CS. Measures included word and sentence recognition scores. The speech recognition performance was rated before cochlear implantation and at 1 and 5 years after implantation. The speech materials were presented in quiet only condition. The mean 12-month post-operative word and sentence recognition scores were 91.4 and 93.1 %, respectively. Five years after implantation, the group means for word and sentence recognition tests were 94 and 96.3 %, respectively. No patients in this series experienced flap complication or other local or systemic complications. This long-term study on 12 subjects with CS over 5 years of cochlear implant use reveals that cochlear implantation is safe in the long term and provides excellent and stable hearing results.

The authors evaluated the behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas (VSs).The case records of all patients who underwent surgical treatment of VSs were analyzed. All patients in whom an incomplete excision had been performed were analyzed. Incomplete excision was defined as near-total resection (NTR), subtotal resection (STR), and partial resection (PR). Tumors in the NTR and STR categories were followed up with a wait-and-rescan approach, whereas the tumors in the PR category were subjected to a second-stage surgery and were excluded from this series. All patients included in the study underwent baseline MRI at the 3rd and 12th postoperative months, and repeat imaging was subsequently performed every year for 7-10 years postoperatively or as indicated clinically. Preoperative and postoperative facial function was noted.Of the 2368 patients who underwent surgery for VS, 111 patients who had incomplete excisions of VSs were included in the study. Of these patients, 73 (65.77%) had undergone NTR and 38 (34.23%) had undergone STR. Of the VSs, 62 (55.86%) were cystic and 44 (70.97%) of these cystic VSs underwent NTR. The residual tumor was left behind on the facial nerve alone in 62 patients (55.86%), on the facial nerve and vessels in 2 patients (1.80%), on the facial nerve and brainstem in 15 patients (13.51%), and on the brainstem alone in 25 patients (22.52%). In the 105 patients with normal preoperative facial nerve function, postoperative facial nerve function was House-Brackmann (HB) Grades I and II in 51 patients (48.57%), HB Grade III in 34 patients (32.38%), and HB Grades IV-VI in 20 patients (19.05%). Seven patients (6.3%) showed evidence of tumor regrowth on follow-up MRI. All 7 patients (100%) who showed evidence of tumor regrowth had undergone STR. No patient in the NTR group exhibited regrowth. The Kaplan-Meier plot demonstrated a 5-year tumor regrowth-free survival of 92%, with a mean disease-free interval of 140 months (95% CI 127-151 months). The follow-up period ranged from 12 to 156 months (mean 45.4 months).The authors' report and review of the literature show that there is undoubtedly merit for NTR and STR for preservation of the facial nerve. On the basis of this they propose an algorithm for the management of incomplete VS excisions. Patients who undergo incomplete excisions must be subjected to follow-up MRI for a period of at least 7-10 years. When compared with STR, NTR via an enlarged translabyrinthine approach has shown to have a lower rate of regrowth of residual tumor, while having almost the same result in terms of facial nerve function.

Objective: This study aimed to report our experience in the management of patients with intraoperatively diagnosed intracranial facial nerve schwannomas (FNSs) and propose a decision-making strategy. Methods: Twenty-three patients with FNS of the internal auditory canal and/or cerebellopontine angle operated on between 1992 and 2012 were identified. Results: Preoperatively, all cases have been radiographically diagnosed as vestibular schwannomas. Operative procedures consisted of total tumor resection with grafting in 43.4% of patients, near-total resection leaving behind the tumor capsule overlying the facial nerve in 21.7%, total tumor resection with preservation of anatomic continuity of the facial nerve in 13%, and subtotal resection in 4.3%. Four patients (17.4%) underwent bony decompression with no tumor removal. Conclusion: Management of FNS diagnosed at surgery represents a significant clinical challenge. We considered total tumor resection with grafting when patients presented with preoperative facial nerve palsy (≥ grade III). Both subtotal and near-total tumor removal can be performed in patients with preoperative good facial function and/or large tumors with brainstem compression. Patients with small tumors who were selected for hearing preservation surgery can be considered for bony decompression. Fascicle preservation surgery may be an option when a clear cleavage plane between the tumor and the facial nerve is found.

Objectives: Lipomas of the internal auditory canal (IAC) and cerebellopontine angle (CPA) are exceedingly rare lesions. The purpose of this report was to describe our experience with lipomas of the IAC and CPA and perform a review of the literature. Methods: We report 8 cases of lipomas involving the IAC and/or the CPA that were managed at Gruppo Otologico between April 1987 and October 2012. Results: Four cases of entirely intracanalicular lipomas were radiologically misinterpreted as vestibular schwannomas and underwent tumor removal by a translabyrinthine approach. Two of these patients experienced postoperative facial nerve palsy. Lipomas were suspected in 4 patients on the basis of imaging findings and were managed conservatively. Of these 4 cases, 3 did not show any growth after an average period of 28 months, and 1 case demonstrated tumor growth on follow-up imaging. Conclusions: Neuroimaging represents an extremely important tool for this diagnosis. Attempts to achieve complete resection may result in severe neurologic sequelae, especially in large lesions. Observation with repeated imaging in order to detect growth of the lesion is usually recommended. Debulking of the tumor, mainly aimed at brain stem and cranial nerve decompression, should be considered in cases of disabling and uncontrolled neurologic symptoms and signs.

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.

Gynecomastia is a benign enlargement of the male breast due to a physiological or pathological factor that interferes with the balance between estrogens and androgens in the serum. Gynecomastia itself requires no treatment unless the persistent enlargement of the male breast is a source of embarrassment and/or distress for the adolescent or adult man. The indications for the surgical treatment of gynecomastia are founded on two main objectives: (1) the restoration of male chest shape and (2) diagnostic evaluation of suspected breast lesions. The diagnostic evaluation begins with an adequate history and a thorough breast examination helped by laboratory tests and instrumental research. Several approaches for surgical treatment have been described in the literature. Some problems arise in patients who have significant enlargement and ptosis of the breast that will require skin reduction and in some patients requiring nipple-areola complex reduction. The authors believe that the complete circumareolar technique with purse-string suture creates the best aesthetic results, with fewer complications, in patients with moderate and severe ptotic glandular breast enlargements that have skin redundancy combined with areolar enlargement. From 1995 through 1999, a total of 10 male patients with moderate to severe gynecomastia were treated surgically using a complete circumareolar approach. All patients achieved a good aesthetic contour of the chest. Only two patients required a revision of the circumareolar scar to correct postoperative enlargement.

Objectives: To investigate the long-term results of preoperative stenting of the internal carotid artery (ICA) in complex head and neck paragangliomas (HNP) as well as to report on indications and technical details of the procedure. Method: A comprehensive retrospective review of patients affected by HNP, consecutively operated on and preoperatively treated with stenting of the ICA in a quaternary referral skull base center, was performed. Results: Nineteen patients affected by complex HNP were identified, on whom 21 preoperative stenting procedures were performed. The mean follow-up period after stent insertion was 53.8 months; the patients' age ranged from 33 to 56 years. Fourteen patients were affected by tympanojugular paragangliomas, 4 by vagal paragangliomas and 1 by bilateral carotid body tumors. Five patients presented with recurrent tumors, while 7 presented with multiple or bilateral HNP. There were no complications associated with endovascular procedures. Total tumor removal was accomplished in 52.4% of the cases with 1 recurrence. An advanced stage was the main factor conditioning total removal. Clinical control was obtained in 80% of the patients with residual disease. Total tumor removal from and around the ICA was obtained in 95.2% of the cases. Long-term stent patency was evident in 20 of 21 cases. Conclusions: Preoperative stenting of the ICA represents a safe and effective procedure in selected cases, obviating the need for balloon occlusion or bypass procedures and reducing the risk of intraoperative vascular injury.

Head and neck paragangliomas, rare neoplasms of the paraganglia composed of nests of neurosecretory and glial cells embedded in vascular stroma, provide a remarkable example of organoid tumor architecture. To identify genes and pathways commonly deregulated in head and neck paraganglioma, we integrated high-density genome-wide copy number variation (CNV) analysis with microRNA and immunomorphological studies. Gene-centric CNV analysis of 24 cases identified a list of 104 genes most significantly targeted by tumor-associated alterations. The “NOTCH signaling pathway” was the most significantly enriched term in the list (P = 0.002 after Bonferroni or Benjamini correction). Expression of the relevant NOTCH pathway proteins in sustentacular (glial), chief (neuroendocrine) and endothelial cells was confirmed by immunohistochemistry in 47 head and neck paraganglioma cases. There were no relationships between level and pattern of NOTCH1/JAG2 protein expression and germline mutation status in the SDH genes, implicated in paraganglioma predisposition, or the presence/absence of immunostaining for SDHB, a surrogate marker of SDH mutations. Interestingly, NOTCH upregulation was observed also in cases with no evidence of CNVs at NOTCH signaling genes, suggesting altered epigenetic modulation of this pathway. To address this issue we performed microarray-based microRNA expression analyses. Notably 5 microRNAs (miR-200a,b,c and miR-34b,c), including those most downregulated in the tumors, correlated to NOTCH signaling and directly targeted NOTCH1 in in vitro experiments using SH-SY5Y neuroblastoma cells. Furthermore, lentiviral transduction of miR-200s and miR-34s in patient-derived primary tympano-jugular paraganglioma cell cultures was associated with NOTCH1 downregulation and increased levels of markers of cell toxicity and cell death. Taken together, our results provide an integrated view of common molecular alterations associated with head and neck paraganglioma and reveal an essential role of NOTCH pathway deregulation in this tumor type. Electronic supplementary material The online version of this article (doi:10.1007/s00401-013-1165-y) contains supplementary material, which is available to authorized users.

This six-volume series has been compiled to introduce current, up-to-date concepts concerning the diagnosis, evaluation, and management rationale of the various conditions that otorhinolaryngologists encounter in their clinics, hospitals, and operating rooms. It has been our goal to keep this information complete yet concise. -- Milind V. Kirtane and Chris E. de Souza, Series Editors Head and Neck Surgery is a comprehensive textbook that provides detailed information on the latest epidemiology, diagnostics, nutritional support, and emerging technology and therapeutics used to treat head and neck cancer. It will serve as a reference guide for physicians and non-physicians who diagnose, treat, and rehabilitate patients with head and neck tumors. Key Features: * Multidisciplinary care of patients with tumors of the head and neck region* Intraoperative navigation and robotic surgery for head and neck neoplasms* Endoscopic approaches to the anterior skull base* Image guidance for sinus tumors* Minimally invasive techniques in head and neck surgery The contributors to this volume are internationally recognized experts in head and neck surgery, plastic surgery, and oral and maxillofacial surgery, informing the book with a wealth of knowledge in these disciplines.

Objective: Neurofibromatosis Type 2 (NF2) patients have multiple central nervous system tumors and, specifically, bilateral vestibular schwannomas (VSs) causing bilateral deafness. If the cochlear nerve is not preserved during tumor removal, the only hearing rehabilitation in these patients could be via an auditory brainstem implant (ABI). Study Design: Retrospective case study and literature review. Setting: Tertiary referral cranial base center. Patients: In 24 NF2 patients, 25 ABIs were placed in the lateral recess of the fourth ventricle after VS surgery via a translabyrinthine approach. Results: In this series, a large range of results are observed: from open speech and use of the telephone to no ABI use, because of the poor sound identification ability. Of the 24 patients, 19 use their ABI on a daily basis, 4 are nonusers, and 1 died of NF2 progression. A multivariate analysis did not reveal a good predictor for ABI outcome. In literature, the results of ABI in NF2 are difficult to compare, and the overall outcome was poor compared with cochlear implantation results. Conclusion: Auditory brainstem implantation in NF2 patients directly after tumor removal is a safe procedure and the best means of hearing rehabilitation if the cochlear nerve is not preserved. The results in NF2 cases in the literature and these series are poor compared with cochlear implantation. If a cochlear implant is possible, it has the preference over an ABI, also in NF2. Nevertheless, the majority of the patients have benefit of the ABI during daily life particularly in combination with lip reading. Key Words: BrainstemVHearing rehabilitationV ImplantationVNeurofibromatosisVReviewVVestibular schwannoma.

Data obtained from a cohort of 10 post-lingually deaf adult patients indicated that use of a higher stimulation rate, in the setting of the main peak interleaved sampling (MPIS) strategy coupled with the Neurelec-MXM Digisonic SP cochlear implant system, gives a significant advantage in terms of speech perception if compared with a lower rate, especially in a noisy environment.To evaluate speech recognition performances in post-lingually deaf adult cochlear implant recipients using two different stimulation rates (260 pps/e and 600 pps/e) in the setting of the MPIS strategy combined with the MXM-Neurelec Digisonic SP cochlear implant system.Ten post-lingually deaf adults who consecutively received a Neurelec-MXM Digisonic SP device at the CI Center of the University of Parma participated in the study. The study was conducted prospectively as a within-subject repeated measures (ABA protocol) between January 2007 and January 2008. Each subject was exposed to and sequentially tested with two different rates of stimulation (260 pps/e and 600 pps/e), in quiet and in a noisy environment. Statistical analysis was performed on the data obtained.Subjects using the MPIS strategy with a stimulation rate of 600 pps/e performed significantly better in words and phrases recognition tests in both a noisy and a quiet environment.

Tumors other than paragangliomas in the jugular foramen are uncommon. Of these, schwannomas and meningiomas predominate. Little clinical data are available in the literature on these tumors at this site. The purpose of this article is to review our experience at the Gruppo Otologico of the management of these tumors. A retrospective series is presented of 32 consecutive patients affected by jugular foramen schwannomas and meningiomas in which their clinical and radiological signs, together with surgical techniques and outcomes, were reviewed. A single-stage resection was possible for the majority of patients when the petro-occipital trans-sigmoid (POTS) approach was used. This allowed resection of both intra- and extradural components of the tumor with hearing preservation and avoidance of facial nerve transposition. No deaths occurred. Lower cranial nerve palsies constituted the major cause of morbidity, but none of the patients required an adjunctive procedure such as vocal cord medialization, tracheostomy, or percutaneous gastrostomy.

Objectives The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy. Methods This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. Results At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf person's speech, and one child had connected speech intelligible to all listeners. Conclusions Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.