Early fitting in cochlear implant surgery: preliminary results.

PURPOSE: Cochlear implants are usually activated 3-5 weeks after surgery; to date, no universal protocol exists regarding switch on and fitting of these devices. The aim of the study was to assess safety and functional results of activation and fitting of cochlear implant within 24 h following surgery. METHODS: In this retrospective case-control study, 15 adult patients who underwent cochlear implant surgery, for a total of 20 cochlear implant procedures were analyzed. In particular, clinical safety and feasibility were investigated by examinating patients at activation and at each follow-up. Values of electrodes' impedance and most comfortable loudness (MCL) were analyzed from the time of surgery to 12 months after activation. Free-field pure tone average (PTA) was also recorded. RESULTS: No major or minor complications were reported and all patients could perform the early fitting. Activation modality influenced impedance values only in the short term but the differences were not statistically significant (p > 0.05). Mean MCL values in the early fitting group were lower than MCL of the late fitting in all follow-up sessions, and the difference was statistically significant (p < 0.05). The mean PTA was lower in the early fitting group but the difference was not statistically significant (p < 0.05). CONCLUSIONS: Early fitting of cochlear implants is safe, allows for an early rehabilitation and can have possible beneficial effects on stimulation levels and dynamic range.

Temporal bone computed tomography checklist-TeSLANO: introduction of a standardized preoperative imaging evaluation for middle ear surgery.

PURPOSE: 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. STUDY DESIGN: Single-blinded prospective cohort study. SETTING: Tertiary care university hospital and referral center for otology and skull base surgery. METHODS: 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 < 0.05. RESULTS: Introduction of the checklist improved global identification of critical anatomical structures from 48.75 to 89.17% (P < 0.05). The postimplementation identification percentage improved for both the senior and junior groups (P < 0.05). CONCLUSIONS: The imaging checklist TeSLANO allows a systematic approach to review TBCT scans and significantly improves identification of critical anatomical structures in Otorhinolaryngology residents.

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.

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.

Cochlear implantation in patients with Cogan syndrome: long-term results.

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.

Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

Lipomas of the internal auditory canal and cerebellopontine angle.

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.

Long-term results of external auditory canal closure and mastoid obliteration in cochlear implantation after radical mastoidectomy: a clinical and radiological study.

Cochlear implant candidates with mastoid cavity present a significant challenge to safe cochlear implantation because of possible spread of infection to the inner ear as well as an increased risk of electrode array extrusion. Closure of the external auditory canal is one of the several surgical techniques utilized to block the potential entry routes for infection and to protect the implanted device. The main concern after external auditory canal closure is the risk of developing a cholesteatoma, which can lead to an asymptomatic erosion of the temporal bone and/or cochlear implant failure. In this study we present the results of very long-term (mean 12 years) clinical and radiological follow-up in 12 patients who underwent external auditory canal closure associated with mastoid and Eustachian tube obliteration to facilitate cochlear implantation. To date, with a mean ± SD follow-up of 12 ± 4.7 years (range 5-21 years), the only complication experienced was the breakdown of the EAC closure in one patient, successfully treated by performing a rotation skin flap. The results of this study confirmed that external auditory canal closure is a reliable technique in cochlear implantation after radical mastoidectomy provided that a rigorous surgical technique is performed. A right balance between the need to reduce costs and to avoid unnecessary doses of radiation to patients and the task of a radiological surveillance may be represented by performing computed tomography 12-18 months postoperatively and then, only if clinically warranted.

Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases.

The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.

Skull base chondrosarcomas: surgical treatment and results.

OBJECTIVES: We describe our experience in the management of patients with skull base chondrosarcoma, an uncommon neoplasm of the skull base. METHODS: Thirteen cases of surgically treated skull base chondrosarcomas were identified. The patients' follow-ups ranged from 7 to 86 months (mean, 47 months). RESULTS: The most common tumor locations were the jugular foramen (5 cases), the petrous apex (3 cases), and the petroclival region (3 cases). An infratemporal fossa type A approach was performed in 2 cases, and 2 patients underwent an infratemporal fossa type B approach. Two patients underwent a transotic approach, 1 patient underwent a petro-occipital transsigmoid approach, and a petro-occipital transsigmoid approach combined with a transotic approach was chosen in 1 case. One patient underwent an infratemporal fossa type C approach combined with a transotic approach, and 2 patients underwent an infratemporal fossa type B approach combined with a transotic approach. One patient underwent an infratemporal fossa type B approach combined with a transzygomatic approach, and the last patient underwent a transmastoid approach. Gross total tumor removal was achieved in all patients. Postoperative radiotherapy was performed in 7 cases. The most common complications were lower cranial nerve deficits. Two patients experienced recurrences, 36 months and 6 years after surgical removal. CONCLUSIONS: We believe that the primary treatment for chondrosarcomas of the skull base is gross total surgical resection. We usually do not recommend radiotherapy as the primary treatment for patients with skull base chondrosarcomas; however, radiotherapy may be considered as an alternative primary treatment in selected cases in which there are serious medical contraindications to surgery, as well as in elderly patients. We reserve postoperative radiotherapy for patients with histologically aggressive tumors (grade II or III), as well as for cases of subtotal resection or recurrent tumors.

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.

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.

Intracranial facial nerve grafting after removal of vestibular schwannoma.

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.

Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation.

OBJECTIVE: We aimed to evaluate and compare the auditory performance of neurofibromatosis type 2 (NF2) patients with bilateral total deafness fitted with cochlear or auditory brainstem implants. PATIENTS AND METHODS: A retrospective case review was performed. Nine patients suffering from NF2 who underwent hearing rehabilitation by means of cochlear (4 patients) or auditory brainstem (5 patients) implantation participated in the study. Postoperative auditory performance was assessed using closed- and open-set tests. RESULTS: In the group of patients fitted with a cochlear implant, 3 subjects achieved open-set speech recognition abilities comparable to those of standard adult postlingual implant patients; the remaining patient scored 0% in all open-set format tests, reporting benefits only in environmental sound detection and lip-reading. Among the 5 patients who underwent auditory brainstem implantation, 1 reached good open-set speech recognition skills, scoring 70% in the common phrases comprehension test, and she was able to communicate on the telephone. Two other patients achieved open-set speech understanding (respectively, 33 and 41% in the common phrases comprehension test), reporting daily use of their device. The remaining 2 patients did not achieve any level of open-set speech perception, obtaining only improved access to environmental sound and lip-reading skills. CONCLUSIONS: Our study confirmed literature data reporting that cochlear implantation may offer open-set speech communication in NF2 patients. In this small cohort, cochlear implant patients performed better than auditory brainstem implant patients, even if variability in auditory performance was observed with both devices. More studies are needed in order to clarify the role and reliability of electrophysiological tests in predicting the residual functionality of the cochlear nerve after tumor removal.

Nasal polyposis in Churg-Strauss syndrome.

OBJECTIVES: Churg-Strauss syndrome (CSS) is a systemic vasculitic disorder of unknown etiology that affects small-to-medium-size blood vessels. Patients affected by CSS frequently show ear, nose, and throat manifestations, which are often present at the time of disease onset. The purpose of this study was to determine the frequency of nasal polyposis in a series of 29 patients with CSS and to correlate the nasal findings to the total health situation of these patients. STUDY DESIGN: Retrospective analysis. SETTING: Department of Otolaryngology and Department of Clinical Medicine, Nephrology and Health Science, University of Parma. METHODS: Twenty-nine patients with CSS were identified. Of the 29 patients, 17 (58.6%) had nasal polyposis and were enrolled in this study. The nasal polyps were graded according to the Lund and Mackay endoscopic and radiological classifications. RESULTS: At diagnosis, endoscopic intranasal evaluation identified nasal polyposis of grade 3 in nine cases (52.9%), grade 2 in six cases (35.2%), and grade 1 in the remaining case (5.8%). After corticosteroid and immunosuppressive therapy, clinical remission was achieved in 14 patients (82.3%), whereas 3 patients experienced a relapse. Posttreatment endoscopic evaluation showed a permanent disappearance (grade 0) of nasal polyps in eight patients (47%). The other nine patients (52.92%) were found to have a small polyp situated in the middle meatus (grade 1). CONCLUSIONS: Nasal polyposis in patients with CSS may represent the initial phase of the syndrome, though patients often have concurrent pulmonary disease. Corticosteroid therapy either alone or combined with immunosuppressive drugs usually yielded improvement or stabilization.

Audiologic Manifestations of Marshall Syndrome.

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.

Posterior petrous face meningiomas: an algorithm for surgical management.

OBJECTIVE: The objective of the present study was to report our surgical strategy in the management of 81 patients with posterior petrous face meningiomas. STUDY DESIGN: Retrospective study. SETTING: This study was conducted at a quaternary private otology and cranial base center. PATIENTS: Of 139 patients with posterior fossa meningioma, 81 occurred on the posterior petrous face of the temporal bone and were the object of this study. INTERVENTIONS: Thirty-one patients were approached by the enlarged translabyrinthine approach. The enlarged translabyrinthine approach with transapical extension Type II was performedin 29 patients. The combined retrosigmoid-retrolabyrinthine approach was chosen in 8 cases. The modified transcochlear approach Type A with permanent posterior transposition of the facial nerve (FN) was performed in 6 patients. Two patients underwent a retrolabyrinthine subtemporal transapical approach. One patient underwent a transpetrous middle cranial fossa approach. Four patients with intracanalicular meningiomas were operated on through the enlarged middle cranial fossa approach. RESULTS: Total removal of the tumor (Simpson Grades I and II) was achieved in most patients (92.5%). The FN was anatomically preserved in 79 of the 81 (97.5%) patients. Five patients had less than 1 year follow-up, and 2 patients were lost to follow-up and were excluded in evaluation of the final FN outcome. At 1-year follow-up, 46 patients (63%) had Grade I to II, 19 (26%) had Grade III, 4 (5.4%) had Grade IV, 1 (1.3%) had Grade V, and 3 (4.1%) had Grade VI. Hearing-preserving surgery was attempted in 15 patients (18.5%) with preoperative serviceable hearing. Of these 15 patients, 11 had their hearing preserved at the same preoperative level, and 4 experienced postoperative deafness. Postoperatively, a new deficit of 1 or more of the lower cranial nerves was recorded in 3 patients. One patient experienced subcutaneous cerebrospinal fluid collection that required surgical management. CONCLUSION: Total tumor removal (Simpson Grades I-II) remains our treatment of choice and takes priority over hearing preservation. Subtotal resection is indicated for older and debilitated patients with giant lesions to relieve the tumor compression on the cerebellum and brainstem. Subtotal removal is also preferred in the face of the absence of a plane of cleavage between the tumor and the brainstem, in the presence of encasement of vital neurovascular structures, in elderly patients with tumors adherent to preoperatively normal facial or lower cranial nerves.

Ear, nose and throat manifestations of Churg-Strauss syndrome.

CONCLUSION: Ear, nose and throat (ENT) involvement is common in Churg-Strauss syndrome (CSS), usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. Otolaryngologists may play a pivotal role in making an early diagnosis of this disease. OBJECTIVES: CSS is a systemic vasculitic disorder that affects small to medium-sized blood vessels. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by activated eosinophils. Patients affected by CSS frequently have ENT manifestations, which are often present at the time of disease onset and may represent relevant clues for the diagnosis. Thus, our objective was to present the ENT manifestations at the onset, at the diagnosis and at some point during the course of the disease in a series of patients with CSS collected at a single center. MATERIALS AND METHODS: Twenty-eight patients with CSS, as defined according to the 1990 American College of Rheumatology classification criteria, were identified. Twenty-one (75%) of these patients had ENT involvement. We evaluated the clinical course, laboratory data, histologic findings, treatment and outcomes. RESULTS: Of the 21 patients, 13 (61.9%) had ENT involvement at asthma onset and 8 (38%) at diagnosis or during follow-up. The most common ENT manifestations were allergic rhinitis in 9 (42.8%) patients and nasal polyposis in 16 (76.1%). Three (14.2%) patients developed chronic rhinosinusitis without polyps, three (14.2%) had nasal crusting, one (4.7%) serous otitis media, one (4.7%) purulent otitis media, two (9.5%) progressive sensorineural hearing loss, and one (4.7%) unilateral facial palsy. Corticosteroid therapy associated with immunosuppressive drugs usually yielded improvement or stabilization.

Clinical findings and surgical results of middle ear cholesteatoma behind an intact tympanic membrane in adults.

BACKGROUND AND AIM: 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. METHODS: We analyzed retrospectively 27 adult patients (age > 18 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. RESULTS: 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. CONCLUSIONS: 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.