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Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). Retrospective case study and review of the literature. Tertiary referral center. Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. Therapeutical. Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences.

Objectives: To assess the validity of the subtotal petrosectomy (STP) technique in problematic cases of cochlear implant (CI) surgery, and review indications, outcomes, and related controversies. Study Design: This is a retrospective review of data from a private quaternary referral center of otology and skull base surgery. Patients and Methods: A review of patients who underwent CI with STP (STP-CI) as the leading approach was performed. Demographics, indications, surgical details, and main outcomes were evaluated. The surgeries performed were usually single-stage procedures encompassing a comprehensive mastoidectomy, blind sac closure of the external auditory canal (EAC), and mastoid obliteration with autologous fat. Results: A total of 107 cases were included. Mean follow-up was 7.1 years (range 1–13 years). The most frequent indication for STP-CI was chronic otitis media with/without cholesteatoma (32.7%), followed by open mastoid cavity (26.1%), and cochlear ossification (17.7%). Other difficult conditions where STP facilitates successful implantation include inner-ear malformations, temporal-bone trauma, unfavorable anatomic conditions, and revision surgery. A planned staged procedure was performed in 3 cases. The rate of major complications was 5.6% (n = 6). Three patients developed postauricular wound dehiscence which eventually resulted in device extrusion. No cases of recurrent/entrapped cholesteatoma, EAC breakdown, or meningitis were encountered. This is the largest single-center series of STP-CI reported in the literature. Conclusions: When CI is intended in technically challenging cases or associated with a high risk of complications, STP is effective and reliable. Safe implantation and excellent long-term outcomes can be achieved provided surgical steps are properly followed. Single-stage procedures can be performed in most cases, even when there is active middle-ear disease.

Objective We present an extremely rare report of congenital cholesteatoma (CC) in identical twins with atypical location. Methods Two consecutive cases of CC in identical twins from diagnosis to treatment and a literature review are presented. Results The twin children presented to our otology and lateral skull base clinic with different clinical picture. Both underwent thorough audiological workup and imaging and early surgical intervention. The 6-year follow-up of these two children has been uneventful with no recurrence of disease and hearing restoration. Conclusion Multiple theories exist to explain this rare pathology. Modified Levenson’s criteria define it as white mass behind intact tympanic membrane. Atypical location of CC with recurrent otitis media is not uncommon; hence, further study is required. To our best knowledge, this is the second reported occurrence in identical twins but first report with differential presentation and atypical location in both cases.

To evaluate incidence, demographics, surgical, and radiological correlates of incomplete and false tract electrode array insertions during cochlear implantation (CI). To evaluate outcomes in patients with incomplete electrode insertion (IEI). Retrospective analysis. Otology and skull base center. Charts of 18 patients (19 ears) with incomplete or false tract insertions of the electrode array were evaluated who underwent CI, with at least 1 year follow-up (from 470 cases). Demographic findings, etiologies, pre-operative radiologic findings, operative records, post-operative plain radiographic assessment for extent of electrode insertion, and switch-on mapping were evaluated. Audiological outcomes were evaluated using maximum and last recorded vowel, word, sentence, and comprehension scores for patients with IEI. Incidence of insertional abnormalities was 4.25% with 17 instances of incomplete and 2 cases of insertion into superior semicircular canal. Mean age and duration of deafness were 55.18 ± 4.62 and 22.12 ± 5.71 years. Etiologies in the IEI group were idiopathic, otosclerosis, meningitis, chronic otitis media (COM), temporal bone fractures, and Neurofibromatosis-2. 29.4% cases had cochlear luminal obstruction. Mean radiological and active electrophysiological length of insertion was 20.49 ± 0.66 and 19.49 ± 0.88 mm, respectively. No significant correlation was observed between audiological outcomes and insertional length except in time to achieve maximum word scores (p = 0.04). Age at implantation had significant correlations with last recorded word and comprehension scores at mean follow-up of 42.9 months, and with time to achieve maximum auditory scores. IEI during cochlear implantation using straight electrodes can occur with or without cochlear luminal obstruction. Age plays an important role in the auditory rehabilitation in this patient subset.

Cholesteatomas in children have a more aggressive growth pattern compared to adults, which leads to a higher incidence of both residual and recurrent disease. A staged canal wall-up or a canal wall-down tympanomastoidectomy (CWUT and CWDT, respectively) is selected depending on the extent of the disease and condition of the middle ear (ME) cleft and mastoid. Endoscopic ear surgery (EES) has been recently introduced as an adjuvant tool for the treatment of this pathology even in the pediatric population. Objectives To analyze long term outcomes of CWUT and CWDT in the pediatric population, focusing on residual and recurrence rates of cholesteatoma and hearing results. A literature review including cases treated with EES were discussed. Material and methods Pediatric patients treated for cholesteatoma involving both the ME and mastoid with a follow-up (FU) of at least 4 years were retrospectively analyzed in a quaternary referral center for otology and lateral skull base surgery. Patients were grouped according to the surgical technique (CWUT versus CWDT). Rates of residual and recurrent cholesteatoma after each surgical technique were reported and compared. Mean Air-Bone Gap (ABG) of 0.5-1-2-4 KHz was measured and reported before the first surgery and at the last post-operative FU. Results Two-hundred and thirty-six cases fulfilled our inclusion criteria. The mean FU was 100.4 ± 44.2 months (median 89 months). One-hundred and five (44.5%) cases underwent a CWUT, whereas 131 (55.5%) a CWDT. A second stage surgery was performed in 73.5% of CWUT and 58.7% of CWDT. Among the CWUT group, residual cholesteatoma occurred in 22 (21%) ears and recurrence in 24 (22.9%). Patients undergoing CWDT showed lower rates of both residual and recurrent cholesteatoma (7.6% and 2.3%, respectively). ABG improvement was noted for both groups, even though CWUT showed better post-operative hearing results. Conclusions The CWDT technique offers a definite surgical therapy, with minimal residual and recurrence rates and audiological results comparable to the CWUT technique. EES must still prove its added benefit or equivalence to pure microscopic approaches.

Objectives To evaluate the long-term surgical outcomes of cochlear implantation (CI) in chronic otitis media (COM) with cholesteatoma and open cavities using subtotal petrosectomy (STP). To review device explantation (DE) patients and reimplantation considerations. Study design Retrospective review. Setting Otology and skull base center. Patients and methods Charts of 35 patients (36 ears) with COM with cholesteatoma, including open cavities, who underwent CI were reviewed for surgical outcomes and DE. Patient demographics, pathologies, previous surgeries, staging of implantation, salient intraoperative findings at the time of implantation and follow-up were evaluated. Details of patients with DE were evaluated for cause, operative findings, and reimplantation considerations. Results Mean age of patients was 65.94 years. Nineteen open cavities, 11 primary cholesteatomas, 3 petrous bone cholesteatomas, and 3 atelectatic middle ears represented the pathologies with 31 patients of CI with concurrent STP and 5 patients where implantation was staged. The mean follow-up was 7.16 years ranging from 2 to 13 years. Four patients (11%) had DE due to extrusion and cavity infection with three reimplanted in same or contralateral ear. All explantations occurred within 24 months of primary implantation. No residual or recurrent cholesteatoma was observed in any of the patients during follow-up. Conclusion CI is feasible in COM with cholesteatoma and open cavities with the use of STP and single-stage implantation can be performed in the absence of purulence. Despite low risk of residual cholesteatoma post meticulous disease removal, risk of DE remains, particularly in open cavity patients, and is higher than standard implantation. Reimplantation is often possible with careful considerations.

Objectives 1) To review the surgical and auditory outcomes and complications of cochlear implantation in cases with cochlear ossification. 2) To evaluate association between the extent and etiology of ossification to outcomes. Study design Retrospective study. Setting Otology and skull base surgery center. Subjects and methods Charts of 40 patients (42 ears) with cochlear ossification undergoing cochlear implantation were reviewed. Demographic features, operative findings, auditory outcomes, and complications were analyzed. Operative findings included extent of cochlear ossification, extent of drilling required to obtain patent cochlear lumen, approach (posterior tympanotomy/subtotal petrosectomy), electrode insertion (partial/complete, scala tympani/vestibuli), and complications. Auditory outcomes were assessed over a 4-year follow-up period using vowel, word, sentence, and comprehension scores. Patients were divided into groups (otosclerotic/non-otosclerotic and round window/basal turn ossification) for comparison of auditory outcomes. Outcomes were compared with 60 randomly identified controls (adults with postlingual deafness) who underwent implantation with no cochlear ossification. Results The median age and duration of deafness of patients was 54.39 and 27.15 years, respectively. Etiology of cochlear ossification was otosclerosis in 23 of 42 ears and mixed in 19 of 42 ears (chronic otitis media, temporal bone fractures, idiopathic, meningitis, Cogan's syndrome) with exclusive round window involvement in 54.7% of cases and the rest having partial or complete basal turn ossification. 59.5% ears underwent subtotal petrosectomy for implantation. Three patients underwent scala vestibuli insertion and five had incomplete electrode insertion. Auditory outcomes were comparable in otosclerotic and non-otosclerotic cases and in round window and basal turn ossification cases. No significant differences were observed in auditory scores when compared with controls with no ossification. Conclusions Cochlear implantation in cochlear ossification is feasible despite surgical challenges and modifications. Auditory outcomes in basal turn ossification appear to be comparable to cases with no ossification with extent of ossification having no significant association with outcomes.

Objectives 1) To review the surgical and auditory outcomes in patients of cochlear implantation in otosclerosis. 2) To review complications and postimplantation facial nerve stimulation (FNS). 3) To compare the auditory outcomes between patients displaying cochlear ossification to the nonossified ones. Study design Retrospective study. Setting Quaternary Otology and Skull base surgery center. Subjects and methods Charts of 36 patients (38 ears) with otosclerosis undergoing cochlear implantation were reviewed from the cochlear implant database. Demographic features, operative findings, auditory outcomes, and postimplantation FNS were analyzed. Operative findings included extent of cochlear ossification, approach (posterior tympantomy/subtotal petrosectomy), electrode insertion (partial/complete, scala tympani/vestibuli), and complications. All the patients underwent implantation using straight electrodes. Auditory outcomes were assessed over a 4-year follow-up period using vowel, word, sentence, and comprehension scores. Patients were divided into two groups (with and without cochlear ossification) for comparison of auditory outcomes. Results The mean age and duration of deafness of patients was 59.72 and 28.9 years respectively. Twenty-three of 38 ears had cochlear ossification, with exclusive round window involvement in 60% of the patients, with the rest having partial or complete basal turn ossification. 36.8% ears underwent subtotal petrosectomy for cochlear ossification. One patient underwent scala vestibuli insertion and two had incomplete electrode insertion. Patients with no ossification had no intra or postoperative complications. One patient had bilateral FNS managed by alterations in programming strategy. Auditory outcomes in patients without any ossification were better than in patients with ossification, though statistically insignificant in most parameters. Conclusion Cochlear implantation in otosclerosis provides good auditory outcomes, despite high incidence of cochlear ossification. Patients of FNS can be managed by alterations in programming strategy, without affecting auditory outcomes.

Objectives/Hypothesis To describe the technique of subtotal petrosectomy (STP), to analyze the outcomes, and to review the literature Study Design A retrospective review. Methods Four hundred sixty cases of STP performed for various indications were included in the study, which was conducted at a quaternary referral center for otology and skull base surgery. Surgical and audiological parameters, and complications were evaluated. Our results were compared with the existing literature on the subject. Results Two hundred ninety-seven (64.6%) patients had been subjected to multiple surgeries before an STP was performed. The most common indication for STP was recurrent chronic otitis with or without cholesteatoma, with 165 (35.9%) patients. Difficult cases of cochlear implantation, temporal bone fractures, and class B3 tympanomastoid paragangliomas were the next most common indications, with 91 (19.8%), 43 (9.4%), and 38 (8.3%) cases, respectively. The median follow-up of the patient pool was 36 ± 19 months. Recidivism and postauricular wound fistula were the most common complications, seen in five (1.1%) patients each. This series of STP is the largest reported in the literature Conclusions STP is a very useful and safe surgical tool in the management of a variety of problematic situations in otology, as it offers the possibility of a definitive cure by offering radical clearance. This procedure can be combined safely with hearing implantation procedures. Level of Evidence 4. Laryngoscope, 127:2833–2842, 2017

To investigate the surgical and audiological outcomes of an installation of a bone-anchored hearing system (BAHS) procedure without tissue reduction using Ponto implants and abutments. Retrospective consecutive case series. Forty-nine patients, 18 years or older and eligible for treatment with a bone-anchored hearing system with tissue preservation surgery, were included in the study. Following a systematic scheme for medical outcomes, we collected the data regarding surgical intervention, quality of life (GBI), skin and soft tissue reactions (Holgers grading system), pain and numbness (VAS). Hearing performance (aided thresholds and speech recognition in noise) was recorded in 20 patients. No implants were lost, skin, and soft tissue reactions were mild in 96 % of the all visits. Quality of life (GBI) generally improved in the aided condition compared to prior to implantation. Audiologically, 100 % of the 20 patients examined showed improvement of speech reception and sound field thresholds comparing aided to unaided. An average improvement of 33 dB on PTA was recorded. The study, presenting data on a large population, treated with tissue preservation and modern titanium implants, shows that this treatment is a viable solution that results in fewer complications, high degree of predictability and good audiological results.

Cognitive dysfunctions are a common clinical feature of schizophrenia and represent important indicators of outcome among patients who are affected. Therefore, a randomized, controlled, monocentric, single-blind trial was carried out to compare two different rehabilitation strategies adopted for the restoration and recovery of cognitive functioning of residential patients with schizophrenia. A sample of 110 residential patients were selected and, during the experimental period, a group of 55 patients was treated with sets of domain-specific exercises (SRT+CRT), whereas an equal control group was treated with sets of non-domain-specific exercises (SRT+PBO) belonging to the Cogpack® software. The effects on the scores (between T0 and T1) of the variables treatment and time and of the interaction time X treatment were analyzed: for the total BACS, the main effect of the between-factors variable treatment is statistically significant (F=201.562 P=0.000), as well as the effect of the within-factors variable “time” (F=496.68 P=0.000).The interaction of these two factors is also statistically significant (F=299.594 P=0.000). The addition of cognitive remediation therapy (CRT) to a standard treatment of metacognitive training (MCT) resulted in a significant improvement in global neurocognitive functioning and has reported positive effects with regard to the strengthening of verbal and working memory, selective and sustained attention at T1. A relevant result is the statistically significance of “time X treatment” for all the tests administered: we can assume that the domain-specific cognitive training amplifies the effects of SRT, as the primary and secondary goals of the present study were achieved.

Objective 1. To analyze the surgical outcomes in the management of petrous apex cholesterol granulomas (PACG) with a brief literature review.2. To evaluate the importance of wait-and-scan management option. Study design Retrospective review. Setting Quaternary referral center for otology and skull base surgery. Subjects and methods Charts of 55 patients with at least 12 months of follow-up were analyzed for demographic, clinical, audiometric, and radiological features. Patients were divided into surgical group (SG) (n = 31) and wait-and-scan (n = 24) (WS) group. Surgical approach was chosen as per hearing status and PACG extension and relation to nearby neurovascular structures and included either drainage by transmastoid-infralabyrinthine approach (TM-IL)/transcanal-infracochlear approach (TC-IC) or resection by infratemporal fossa type B approach (ITF-B). The combination of ITF-B with trans-otic (TO) approach or TO approach solely was used in unserviceable hearing cases. Postoperative outcomes and complications were evaluated in SG. Results Postoperative symptom relief was observed in 24 patients (77.4%). Diplopia and paresthesia recovered in each case and improvement in headache, dizziness, tinnitus, and hearing loss was observed in 87.1% cases. Serviceable hearing was preserved in 24 of 26 cases. Postoperative complication rate was 32.2% including incidences of profound hearing loss, facial nerve paresis, carotid artery injury and intraoperative CSF leaks. Revision surgery was required in 3 (9.6%) cases, after TM-IL approach. Conclusion Surgical drainage is preferable to more aggressive resection procedures, with the latter reserved for recurrent lesions or lesions with severe hearing loss/involvement of critical neurovascular structures. ITF-B approach provides adequate cyst and neurovascular control for resection, while avoiding brain retraction. An initial wait-and-scan approach can be used in most patients where symptoms and imaging justify so.

Background Safe and effective insulin therapy for diabetes mellitus requires initial dose titration and regular adjustments based on blood glucose (BG) monitoring. Our objective was to explore the use of BG measurement in phase-III clinical studies of insulin analogs. These studies provide safety and efficacy information for regulatory authorities and are the basis for insulin analog regulatory approval. Methods A systematic review of phase-III studies of rapid-acting insulin analogs (insulin lispro, insulin aspart and insulin glulisine) and pre-mixed insulin analogs (biphasic insulin aspart and insulin lispro mix) was conducted. Studies were identified using manufacturers’ databases. Search for reports was performed in Medline and registry of clinical trials (clinicaltrials.gov). The European Medicines Agency was contacted to provide Clinical Study Reports. Results Forty-five studies were included. Regular BG measurements were reported in 100 % of the studies and were performed by either self-monitoring of blood glucose (SMBG) alone in 84 %, laboratory alone in 7 %, and both SMBG and laboratory in 9 % of studies. In total, 93 % of the studies reported SMBG. Most studies (91 %) reported insulin therapy adjustments based on BG measurements. Conclusions The findings suggest that BG monitoring and specifically SMBG are co-dependent technologies with insulin analogs. BG measurement is used in most phase-III registration studies for establishing safe and efficacious insulin administration and is recommended in the insulin labels. The indispensable role of SMBG in treatment of insulin-dependent patients should receive attention from health care payers to assess and reimburse SMBG along with insulin to avoid adverse events from inappropriate insulin administration and associated costs.

Purpose The objective of this study was to assess the usefulness of low-dose intratympanic gentamicin administration in patients with Meniere's disease (MD). Materials and Methods We carried out a retrospective review of the charts of 55 patients with definite MD in accordance with the AAO-HNS (American Academy of Otolaryngology–Head and Neck Surgery) Diagnostic Scale who were treated at Gruppo Otologico (Piacenza, Rome, Italy). The treatment protocol consisted of 3 intratympanic injections of gentamicin at a 12-hour interval between each administration. Results Results were resumed in accordance with AAO-HNS criteria and showed complete control of vertigo spells in 60% (class A), good control in 16% (class B), moderate control in 4% (class C), poor control in 16% (class D), and no control in 4% (class E) of the patients. Audiological assessment showed unchanged thresholds in 61%, improvement in 22%, and worsening in 17% of the cases. Conclusion Administration of gentamicin is a good alternative to medical and surgical procedures in the treatment of intractable MD. One-day treatment allows for adequate control of vertigo attacks without compromising hearing and quality of life and with reduced costs.

Glomus tympanicum tumour is one of the commonest neoplasms of the middle ear. It is more common in females. The most common presenting symptom is pulsatile tinnitus. A retrotympanic mass is found in the middle ear in all the cases. Computed tomography (CT) scan is the investigation of choice, however in difficult cases magnetic resonance imaging (MRI) can be helpful. Surgery is regarded as the gold standard of treatment in spite of the fact that radiation therapy has also been advocated in the literature. In this series 17 cases of glomus tympanicum treated at Gruppo Otologico Piacenza – Rome (Italy) were analysed retrospectively. A simple and safe technique has been described. All the cases were female and treated by surgery. The tumour was removed completely in all the cases and the ossicular chain kept intact. Recurrence was encountered in only one case after nine years. Surgical removal of the tumour is recommended as the treatment of choice with the following advantages: complete removal, a low complication rate, a low recurrence rate, acceptable hearing level and minimum morbidity.

Prospective Study of Glomus Jugulare and Tympanicum Tumors: Clinical Findings, Surgical Approach and Outcomes, Short-Term Follow-Up: A Clinical Perspective Facial canal paragangliomas are rare and by definition they arise from the paraganglia along the vertical portion of facial canal. We report a case of a facial canal paragangliomas with extension into the internal auditory canal. This report describes for the first time this avenue of extension of facial canal paragangliomas.

To report our personal experience in the surgical treatment of cholesteatoma in children with Down syndrome.Retrospective study.Tertiary care otology and skull base centers.Nine patients with Down syndrome were surgically treated for cholesteatoma. Two patients had bilateral disease, resulting in a total of 11 ears surgically treated.A canal-wall-up mastoidectomy was performed in two ears; in eight of the ears, a canal-wall-down mastoidectomy was carried out and a modified Bondy procedure was performed in one ear.Residual cholesteatoma was found in one ear after the canal-wall-up mastoidectomy and recurrent cholesteatoma developed in another ear, also after canal-wall-up mastoidectomy. The recurrence required conversion to canal-wall-down mastoidectomy. One patient developed a perforation of the neotympanic membrane that had to be revised.Cholesteatoma in children with Down syndrome is a challenging entity for the otologic surgeon. Otolaryngologists should always suspect a cholesteatoma in each child with Down syndrome presenting warning symptoms such as otorrhea and hearing loss. If there is any doubt on inspection, further imaging studies (high-resolution computed tomography) are necessary. To the best of our knowledge, the current study is the first report to document the surgical treatment of cholesteatoma in subjects with Down syndrome.