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.

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.

Surgical anatomy of posterior tympanotomy: influence of the retrotympanum on round window exposure.

OBJECTIVES: To describe how the retrotympanic structures could influence the visibility of the round window niche and the round window membrane during cochlear implant surgery, and to investigate if a round window approach is possible even in cases with unfavourable anatomy. METHODS: Video recordings from 37 patients who underwent cochlear implantation were reviewed. The visibility of the round window niche and round window membrane at different timepoints was assessed according to a modified version of the Saint Thomas Hospital classification. The structures that concealed the round window niche and round window membrane were evaluated. RESULTS: After posterior tympanotomy, 54 per cent of cases had limited exposure (classes IIa, IIb and III) of the round window niche. After remodelling the retrotympanum, round window niche visibility significantly increased, with 100 per cent class I and IIa cases. Following remodelling of the round window niche, visibility of more than 50 per cent of the round window membrane surface was achieved in 100 per cent of cases. CONCLUSION: Remodelling the retrotympanum and the round window niche significantly increased exposure of the round window niche and round window membrane respectively, allowing round window insertion in all cases.

Features and management of a schwannoma of the chorda tympani and review of the literature.

Intrinsic facial nerve tumors are rare lesions. Among the different histology types, schwannomas is the most frequently reported in literature. Other histological types of facial nerve tumors are hemangiomas, meningiomas, and neurofibromas. Chorda tympani schwannomas (CTSs) are extremely rare entities and are considered as an independent subgroup of facial nerve schwannomas because of their clinical characteristics. The aim of this report is to present the clinical and radiological features and the management of a CTS in a 27-year-old male presenting with conductive hearing loss.

Fusion computed tomography-magnetic resonance imaging scans for pre-operative staging of congenital middle-ear cholesteatoma.

OBJECTIVE: To evaluate if fusion computed tomography-diffusion-weighted magnetic resonance imaging may have a role in the pre-operative assessment of congenital middle-ear cholesteatoma. METHODS: A retrospective chart review of surgically treated congenital middle-ear cholesteatoma patients over a 2-year timespan was conducted. Pre-operative staging was performed on computed tomography and fusion computed tomography-diffusion-weighted magnetic resonance imaging based on extension of the disease according to the ChOLE classification system and the Potsic classification system. Intra-operative staging was compared to imaging findings to evaluate accuracy of the two imaging modalities in predicting congenital middle-ear cholesteatoma extent. RESULTS: Computed tomography was able to correctly predict congenital middle-ear cholesteatoma extent in three out of six cases according to the ChOLE classification system, all of which were staged as Ch1a and Ch1b on pre-operative computed tomography. Cases in which computed tomography was not able correctly to determine congenital middle-ear cholesteatoma extent were staged as Ch3 on pre-operative computed tomography. Fusion scans correctly determined congenital middle-ear cholesteatoma extent in all cases according to the ChOLE classification. CONCLUSIONS: Fusion computed tomography-diffusion-weighted magnetic resonance imaging may be helpful in cases of congenital middle-ear cholesteatoma where pre-operative computed tomography shows mastoid and antrum opacification, in which computed tomography alone may overestimate cholesteatoma extension beyond the level of the lateral semi-circular canal.

The Tensor Tympani Tendon: A Hypothetical Site of Origin of Congenital Cholesteatoma.

Multiple theories have been discussed about the etiopathogenesis of congenital middle ear cholesteatoma (CMEC) and its specific site of origin. The intraoperative identification of the precise location of the keratinous mass is important to guarantee its complete removal, in order to reduce the risk of recurrence. This study proposes the tensor tympani tendon (TTT) as a possible site of origin of CMEC. All CMECs treated between 2013 and 2019 were reviewed. Only Potsic stage I lesions were included. Preoperative radiologic images were compared to intraoperative findings. Three removed TTT were sent for histologic evaluation. Seven patients were included (M:F = 3:4). Preoperative CT images were classified as type A in 2 cases (28.6%) and type B in 5 cases (71.4%). At intraoperative evaluation all CMEC sacs were found pedunculated on the TTT. The histologic examinations confirmed the connection between the cholesteatomatous sac and the TTT. According to the correlation of imaging, intraoperative findings and histology, we proposed that the TTT could be the primary site from which CMEC originates.

Dermoid Cyst of the retroauricular region: a rare clinicopathological entity.

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

Newborn hearing screening programme based on an integrated hospital and community care system. Results of the first 4 years of activity.

Since January 2012, babies born in the province of Modena, Italy, have routinely undergone hearing testing as part of a two-stage screening programme. Newborn hearing screening (NHS) has been based on an integrated hospital and community care system and this study aims to assess screening coverage, referral rates, the prevalence, type and extent of hearing loss several years into the programme. Data were collected from January 1, 2012 to December 31, 2015. Coverage was over 99% in all five facilities of the province. The ratio of "fails of the screening" to the total number of infants tested varied over the period from 1.2% to 0.9% in the third level facility, and from 0.8% to 0.4% in the other four. Although hearing loss was mainly associated with dysmorphic\syndromic diseases or a family history of hearing loss, some 23% of cases were identified with no known risk factors. We highlight the importance of the NHSP and the need for strong support from healthcare administrators to ensure high coverage. This is especially true since although the prevalence of hearing loss was higher among infants with audiological risk factors, several cases of hearing loss were found in newborns with no known risk factors.

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.

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.

Presbiphonya.

AIM: This article attempts to describe the aging process of the vocal folds and the main features of the aged voice. BACKGROUND: In the world ageing population era, aging diseases and aging disorders are crucial. Voice disorders (presbyphonia) are common in the elderly and have a significant impact on communication and quality of life. Some of these disorders depend on the vocal folds, which consist of an extracellular matrix (ECM), fibrous proteins, interstitial proteins, and glycosaminoglycans. The density and spatial arrangement of these elements are important, as changes in their deposition can alter the biomechanical properties and vibratory function of the vocal folds. DISCUSSION: The aging voice process is analyzed in detail from mechanical factors like pulmonary bellows alteration, to hormonal factors and life style. CONCLUSIONS: The elderly people undergoe mechanical, anatomical and functional changes: alterations of the pulmonary bellows, systemic changes like hormonal disregulation, and laryngeal changes, that resulting in hoarseness, which is difficult to treat.

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.