Cochlear implantation under local anesthesia and conscious sedation: an Italian experience.

PURPOSE: To report our experience in performing cochlear implantation under local anesthesia in a group of patients who were deemed unfit for general anesthesia. METHODS: 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. RESULT: 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. CONCLUSION: 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.

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.

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

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

A Multicenter Clinical Evaluation of Data Logging in Cochlear Implant Recipients Using Automated Scene Classification Technologies.

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed.

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.

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.

Do Different Types of Microphones Affect Listening Effort in Cochlear Implant Recipients? A Pupillometry Study.

BACKGROUND: It is known that subjects with a cochlear implant (CI) need to exert more listening effort to achieve adequate speech recognition compared to normal hearing subjects. One tool for assessing listening effort is pupillometry. The aim of this study is to evaluate the effectiveness of adaptive directional microphones in reducing listening effort for CI recipients. METHODS: We evaluated listening in noise and listening effort degree (by pupillometry) in eight bimodal subjects with three types of CI microphones and in three sound configurations. RESULTS: We found a correlation only between sound configurations and listening in noise score (p-value 0.0095). The evaluation of the microphone types shows worse scores in listening in noise with Opti Omni (+3.15 dB SNR) microphone than with Split Dir (+1.89 dB SNR) and Speech Omni (+1.43 dB SNR). No correlation was found between microphones and sound configurations and within the pupillometric data. CONCLUSIONS: Different types of microphones have different effects on the listening of CI patients. The difference in the orientation of the sound source is a factor that has an impact on the listening effort results. However, the pupillometry measurements do not significantly correlate with the different microphone types.

A Non-Surgical Wearable Option for Bone Conduction Hearing Implants: A Comparative Study with Conventional Bone Conduction Hearing Aids Mounted on Eyeglasses.

OBJECTIVES: This study aimed to compare the audiological benefits of a non-implantable wearable option for a bone conduction (BC) implant mounted on an arch (SoundArc) to those of traditional BC hearing aids (HAs) mounted on eyeglasses (BCHAs) in patients with moderate to severe conductive or mixed hearing loss. METHODS: A preliminary cross-sectional observational prospective cohort study was conducted in the Tertiary Audiological Department, University Hospital. Fourteen adults with conductive or mixed hearing loss (PTA at 0.5-1-2-4 KHz = 67 ± 15 dB HL) who had been wearing conventional BCHAs mounted on eyeglasses for at least 3 years and had declined surgical implantation of a bone conduction hearing implant (BCHI) were included in the study. Unaided and aided pure-tone air conduction (AC) and bone conduction (BC) thresholds, as well as speech tests in quiet and noise, were recorded at baseline and in two different settings: with a BCHI mounted on SoundArc® and with their own BCHAs mounted on eyeglasses using two couplers. Participants completed questionnaires in both conditions, including the International Inventory for Hearing Aids (IOI-HA), the Hearing Handicap Inventory for Adults/Elderly (HHIA/E), the Speech, Spatial, and Qualities of Hearing Scale (SSQ), a 10-point visual analog scale (VAS), and the Fatigue Impact Scale (FIS). RESULTS: A significant functional gain was observed in both settings (p = 0.0001). Better speech perception in quiet and noise was observed with SoundArc compared to conventional BCHAs on eyeglasses (improvements in word repetition scores in noise: +19.3 at SNR +10 dB, p = 0.002; +12.1 at SNR 0 dB, p = 0.006; and +11.4 at SNR -10 dB, p = 0.002). No significant differences were found in IOI-HA, FIS, and HHIA/E scores. However, significantly better SSQ scores were reported for SoundArc in all domains (p = 0.0038). CONCLUSIONS: Although patients were accustomed to using BCHAs mounted on eyeglasses, the bone conduction wearable option of the BCHI (SoundArc) proved to be a viable alternative for adult patients with conductive or mixed hearing loss who are unable or unwilling to undergo BCHI surgery.

Noonan syndrome: cochlear implantation in the setting of cochlear nerve deficiency.

BACKGROUND AND AIM: 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. CASE PRESENTATION: 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. CONCLUSIONS: 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.

Bulging of the Oval Window in Common Cavity Deformity: A Possible Predictor of Meningitis.

OBJECTIVE: To investigate the prevalence-rate of oval window bulging in the common cavity and its association with bacterial meningitis. PATIENTS: CT and clinical files of 29 children with preliminary diagnosis of common cavity deformity were collected from 13 Italian centers. INTERVENTION: A retrospective case review study was conducted with a centralized evaluation of the temporal bone CT imaging was performed at Azienda Ospedale - Università Padova, Padova, Italy. MAIN OUTCOME MEASURE: Diagnosis of common cavity was reviewed; in addition, a fluid protrusion into the middle-ear cavity through the oval window at CT imaging was considered as oval window bulging. Its association with the history of bacterial meningitis was investigated. RESULTS: Common cavity deformity was confirmed in 14/29 children (mean-age 11.4 ±â€Š3.8; age-range 5-20; nine females) referred with this diagnosis. In 7/14 patients, the common cavity deformity was bilateral (i.e., 21 common cavities). Oval window bulging was found in 3/19 common cavities (concomitant middle-ear effusive otitis hampered the evaluation in two cases), while the internal acoustic meatus fundus was defective in 10/21 cases. History of bacterial meningitis was found in three children (21%) and two of them had oval window bulging at CT. In the case unrelated to oval window bulging, meningitis occurred late at the age of 12 during acute otitis contralateral to common cavity deformity (ipsilaterally to incomplete partition type 1). CONCLUSION: Patients harboring common cavity deformity have a high risk of meningitis in their first years of life. Oval window bulging seems to be associated with a higher risk of meningitis. This information might be important for appropriate surgical planning.

Bonebridge implantation for mixed hearing loss in a patient with Kabuki syndrome.

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.

Osteonecrosis of the External Auditory Canal Associated With Oral Sorafenib Therapy: Sorafenib and Temporal Bone Osteonecrosis.

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.

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.

Surgical management of middle ear cholesteatoma in children with Turner syndrome: a multicenter experience.

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.

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.

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.

Long-term clinical outcomes of cochlear implantation in children with symptomatic epilepsy.

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.

Massive Tension Pneumocephalus Following Cochlear Implant Surgery.

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.