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.

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.

An uncommon cause of conductive hearing loss.

The current video presents the surgical management of a middle ear osteoma through a retroauricolar endocanalar approach, under local anesthesia. The video contains patient's medical history, pre-operative radiological evaluation, surgical approach to the lesion and clinical follow up.

Jugular Foramen Metastasis from Lung Cancer: A Case of "A Mass without His Syndrome".

Jugular foramen (JF) metastasis is rare and often presents as JF syndrome. A 73-year-old male complained of left-sided mastoid pain that irradiated to the neck since the past 3 months. Onset of facial nerve (FN) palsy and persistence of the symptomatology despite corticosteroid therapy demanded radiologic evaluation. Computed tomography and magnetic resonance imaging showed a wide osteolytic lesion of the left JF with involvement of the third segment of the FN. The patient underwent transmastoid incisional biopsy. Histopathological examination showed an adenocarcinoma that was suggested to be of respiratory origin. A primary pulmonary lesion and metastasis to other sits were detected. The patient died 1 month after the initiation of the chemotherapy. Persistent mastoid pain and progressive FN palsy must be considered indicative of JF malignant lesions. Despite early diagnosis, secondary lesions of the JF are characterized by a poor prognosis; however, accurate diagnosis may avoid unnecessary aggressive surgery.