Background: Intracochlear schwannomas (ICSs) are a subtype of intralabyrinthine schwannomas, completely located in the cochlear lumen. ICSs are particularly rare in the pediatric population. Putative diagnosis is made on the basis of magnetic resonance findings with signal characteristics that should remain the same at follow-up imaging. Methods: A retrospective review was performed searching for pediatric patients affected by ICS treated at the Otolaryngology Department, Ospedale Ramazzini, Carpi (Italy), and Otolaryngology and Otoneurosurgery Department, Azienda Ospedaliero-Universitaria di Parma, (Italy). A scoping literature review of the period January 2000 - June 2024 was performed. Results: Two cases of ICS in pediatric patients are described. Neither family history nor genetic signs of neurofibromatosis type II were found. A single report was identified in the literature review. Data analysis resumes the pooled data of the latter case and the authors’ patients. The most common symptom at presentation was progressive sensorineural hearing loss (66%). Mean pure tone average at diagnosis was 74.2 dB. Intracochlear location was in the basal turn in 2 cases and in the apical and middle turns in the third patient. All cases initially underwent a “wait and scan” strategy. The mean follow-up time was 23.3 months. Conclusion: Management planning of pediatric ICSs should be accurate as surgical removal may require partial or total cochlear demolition, resulting in vestibular dysfunction and precluding future positioning of a cochlear implant. Close clinical and radiological follow-up with serial MRI scans allows to evaluate both symptom progression and rate of growth, in order to provide patients with the best therapeutic option.
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.
A case of mastoid dermoid cyst (DC) was presented, and differences with cases of other temporal bone DCs were analyzed. The mastoid DC was also compared with mastoid congenital cholesteatoma. We reported a case of a patient with mastoid DC, evaluating her clinical, radiological, and surgical findings. A review of the literature was performed to compare our findings with those reported. The preoperative radiological evaluation prompted us to plan a surgical approach to the lesion, suspecting the presence of a mastoid congenital cholesteatoma. The surgical findings were in line with the presence of a mastoid DC. Only two cases reported in the literature presented features that fulfilled the criteria of a true mastoid DC. A DC confined to the mastoid region is an extremely rare clinical entity, with asymptomatic and slow growth. Preoperative radiological differentiation between congenital cholesteatoma and DCs with atypical features can be difficult. However, surgical excision is the treatment of choice in both cases. Diagnosis is confirmed by the histological evaluation.
Brain abscesses (BAs) are focal infections of the central nervous system (CNS) that start as a localised area of weakening of the brain parenchyma (cerebritis) and develops into a collection of pus surrounded by a capsule. Pyogenic (bacterial) BAs represent the majority of all BAs; in some cases, the diagnostic and therapeutic management can be challenging. Imaging has a primary role in differentiating BAs from other lesions. Conventional magnetic resonance imaging (cMRI) is essential for the identification of the lesion, its localisation and its morphological features. However, cMRI does not allow to reliably differentiate BAs from other intracranial mass lesions such as necrotic tumours. Advanced sequences, such as diffusion-weighted imaging (DWI), perfusion-weighted imaging (PWI) and proton MR spectroscopy (1H-MRS) are very useful in the differential diagnosis from other brain lesions, such as non-pyogenic abscesses or necrotic tumours, and provide essential information on structural, vascular and metabolic characteristics allowing greater neuroradiological confidence. The aim of this pictorial review is to provide a practical approach showing the added value of more advanced MRI techniques in their diagnostic management.
Backgrounds: To describe presentation, diagnosis and management of a case of spontaneous temporal bone pneumocele with large intracranial extension and brain compression associated with external auditory canal pneumatocele. Methods: A 34-year-old male patient was referred for a large intracranial epidural air collection compressing the right cerebral hemisphere. The patient was asymptomatic except for persisting fullness of the right ear for which he performed frequent Valsalva maneuvers. CT scan showed luminal enlargement of right temporal bone with thinning of the limiting bone, focal dehiscences at the level of the external auditory canal (EAC) and the medial wall of the mastoid tip and remodeling of the calvarial bone. Audiometry was normal bilaterally. A retroauricular transmastoid approach was performed reconstructing the defect in the EAC using bone paste and fibrin glue. The epidural cavity was sealed off by means of temporalis fascia, abdominal fat graft and fibrin glue. Results: After 17 months, MRI showed complete resolution of the intracranial air collection with expansion of the right cerebral hemisphere. Conclusions: Intracranial extension of a temporal bone pneumocele is a rare condition due to progressive expansion of mastoid pneumatization in the epidural space with bone resorption, enlargement of the aerial spaces and chronic calvarial bone changes. Repeated Valsalva maneuvers may sustain the remodeling process. Although usually asymptomatic, there is high risk of acute mass effect particularly in case of trauma and infections. A correct diagnosis is the necessary premise for appropriate treatment. Keywords: Pneumocele, Pneumatocele, Hyperpneumatization, Temporal bone, Pneumocephalus
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.
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.
In the multidisciplinary management of patients with inner ear malformations (IEMs), the correct diagnosis makes the differences in terms of clinical and surgical treatment. The complex anatomical landscape of the inner ear, comprising several small structures, makes imaging of this region particularly challenging for general radiologists. Imaging techniques are important for identifying the presence and defining the type of IEM and the cochlear nerve condition. High-resolution magnetic resonance imaging (MRI) sequences and high-resolution computed tomography (HRCT) are the mainstay imaging techniques in this area. Dedicated MRI and HRCT protocols play an important role in the diagnosis and treatment of patients with inner ear disease. The most suitable technique should be selected depending on the clinical setting. However, in cases of congenital malformation of the inner ear, these techniques should be considered complementary. Since prompt intervention has a positive impact on the treatment outcomes, early diagnosis of IEMs is very important in the management of deaf patients. This article reviews the key concepts of IEMs for clinical radiologists by focusing on recent literature updates, discusses the principal imaging findings and clinical implications for every IEM subgroup, thus providing a practical diagnostic approach.