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.

Intralabyrinthine schwannoma: Surgical management and cochlear lumen preservation.

The present video reports the surgical removal of an intralabyrinthine schwannoma. The video contains patient's medical history, preoperative radiological evaluations and detailed description of surgical steps of the procedure, consisting in labyrinthectomy, cochleostomy and insertion of a dummy electrode in the preserved cochlear lumen within the context of a subtotal petrosectomy.

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.

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.

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.

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.

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.

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.

Bedside Surgical Tracheostomy in the Intensive Care Unit during Covid-19 Pandemic.

OBJECTIVES: To describe Otolaryngologists' perspective in managing COVID-19 patients with acute respiratory distress syndrome (ARDS) requiring tracheostomy in the ICUs during the pandemic peak in a dramatic scenario with limited resources. SETTING: Tertiary referral university hospital, regional hub in northern Italy during SARS CoV 2 pandemic peak (March 9th to April 10th, 2020). METHODS: Technical description of open bedside tracheostomies performed in ICUs on COVID-19 patients during pandemic peak with particular focus on resource allocation and healthcare professionals coordination. A dedicated "airway team" was created in order to avoid transportation of critically ill patients and reduce facility contamination. RESULTS: During the COVID-19 pandemic, bedside minimally invasive tracheostomy in the ICU was selected by the Authors over conventional surgical technique or percutaneous procedures for both technical and operational reasons. Otolaryngologists' experience derived from direct involvement in 24 tracheostomies is reported. CONCLUSIONS: Tracheostomies on COVID-19 patients should be performed in a safe and standardized setting. The limited resources available in the pandemic peak required meticulous organization and optimal allocation of the resources to grant safety of both patients and healthcare workers.

Safety of bedside surgical tracheostomy during COVID-19 pandemic: A retrospective observational study.

Data regarding safety of bedside surgical tracheostomy in novel coronavirus 2019 (COVID-19) mechanically ventilated patients admitted to the intensive care unit (ICU) are lacking. We performed this study to assess the safety of bedside surgical tracheostomy in COVID-19 patients admitted to ICU. This retrospective, single-center, cohort observational study (conducted between February, 23 and April, 30, 2020) was performed in our 45-bed dedicated COVID-19 ICU. Inclusion criteria were: a) age over 18 years; b) confirmed diagnosis of COVID-19 infection (with nasopharyngeal/oropharyngeal swab); c) invasive mechanical ventilation and d) clinical indication for tracheostomy. The objectives of this study were to describe: 1) perioperative complications, 2) perioperative alterations in respiratory gas exchange and 3) occurrence of COVID-19 infection among health-care providers involved into the procedure. A total of 125 COVID-19 patients were admitted to the ICU during the study period. Of those, 66 (53%) underwent tracheostomy. Tracheostomy was performed after a mean of 6.1 (± 2.1) days since ICU admission. Most of tracheostomies (47/66, 71%) were performed by intensivists and the mean time of the procedure was 22 (± 4.4) minutes. No intraprocedural complications was reported. Stoma infection and bleeding were reported in 2 patients and 7 patients, respectively, in the post-procedure period, without significant clinical consequences. The mean PaO2 / FiO2 was significantly lower at the end of tracheostomy (117.6 ± 35.4) then at the beginning (133.4 ± 39.2) or 24 hours before (135.8 ± 51.3) the procedure. However, PaO2/FiO2 progressively increased at 24 hours after tracheostomy (142 ± 50.7). None of the members involved in the tracheotomy procedures developed COVID-19 infection. Bedside surgical tracheostomy appears to be feasible and safe, both for patients and for health care workers, during COVID-19 pandemic in an experienced center.

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.

Bilateral tonsillar infiltration of T-cell prolymphocytic leukemia.

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

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.

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.

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.

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.

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.

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.