Search results for: intracochlear schwannoma

Authors: Boxiang Zhuang

Abstract:

This study analyzes the clinical manifestations, hearing rehabilitation methods and outcomes of a complex case of neurofibromatosis type II (NF2). Methods: The clinical manifestations, medical history, clinical data, surgical methods and postoperative hearing rehabilitation outcomes of an NF2 patient were analyzed to determine the hearing reconstruction method and postoperative effect for a special type of NF2 acoustic neuroma. Results: The patient had bilateral acoustic neuromas with profound sensorineural hearing loss in both ears. Peripheral blood genetic testing did not reveal pathogenic gene mutations, suggesting mosaicism. The patient had an intracochlear schwannoma in the right ear and severely impaired vision in both eyes. Cochlear implantation with tumor retention was performed in the right ear. After 2 months of family-based auditory and speech rehabilitation, the Categories of Auditory Performance (CAP) score improved from 0 to 5. Conclusion: NF2 has complex clinical manifestations and poor prognosis. For NF2 patients with intracochlear tumors, cochlear implantation with tumor retention can be used to reconstruct hearing.

Keywords: NF2, intracochlear schwannoma, hearing reconstruction, cochlear implantation

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Authors: Jessie Justice

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This is a case report of a vestibular schwannoma presenting with numbness to the left lower lip and tongue and altered taste. The aim of this case is to raise awareness of differential diagnoses for trigeminal nerve paraesthesia and, hence, prompt thorough investigation. A 65-year-old male was referred to the Oral and Maxillofacial department regarding sudden-onset of numbness to his left lower lip and left tongue, with altered taste sensation subsequently developing. The patient was simultaneously being investigated for severe hearing loss in his left ear. On examination, there was altered sensation in the distribution of the left inferior alveolar nerve and left lingual nerve. There was no palpable cervical lymphadenopathy and no intra-oral lesions or dental cause for the symptoms. Due to his hearing loss in the left ear, the patient was sent for magnetic resonance imaging of the internal auditory meatus by the Ear, Nose and Throat (ENT) department, revealing a 2.5cm mass within the left cerebellopontine angle presumed to be a vestibular schwannoma. This led to the diagnosis of trigeminal nerve compression by a medium vestibular schwannoma. Consequently, the patient was followed up by an ENT, who referred him for stereotactic radiosurgery. A literature review regarding vestibular schwannomas presenting with orofacial paraesthesia was then carried out. A review of the literature has shown the incidence of vestibular schwannoma to be 3-5 cases per 100,000. It has been reported that approximately 5% of vestibular schwannoma cases display orofacial dysaesthesia, and about 1-3% of cases exhibit trigeminal neuralgia symptoms. This is a rare case of vestibular schwannoma causing trigeminal nerve paraesthesia. The aim of this study is to raise awareness of alternative causes of trigeminal nerve paraesthesia and the available literature surrounding this.

Keywords: acoustic neuroma, orofacial dysaesthesia, trigeminal nerve paraesthesia, vestibular schwannoma

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Authors: Gabriel Hunduma

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Asymptomatic intra-thoracic neurogenic tumours are rare. Tumours arising from the intercostal nerves of the chest wall are exceedingly rare. This paper reports an incidental discovery of a neurogenic intercostal tumour while being investigated for Coronavirus Disease 2019 (COVID-19). A 54-year-old female underwent a thoracotomy and resection for an intercostal tumour. Pre-operative images showed an intrathoracic mass, and the biopsy revealed a schwannoma. The most common presenting symptom recorded in literature is chest pain; however, our case remained asymptomatic despite the size of the mass and thoracic area it occupied. After an extensive search of the literature, COVID-19 was found to have an influence on the development of certain cells in breast cancer. Hence there is a possibility that COVID-19 played a role in progressing the development of the schwannoma cells.

Keywords: thoracic surgery, intercostal schwannoma, chest wall oncology, COVID-19

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Authors: Jeongyeon Park, Yeo Jun Yoon, Jiyoung Seo, In Seok Moon, Hae Jun Lee, Kiwon Song

Abstract:

Cold Atmospheric Pressure Plasma (CAPP) is defined as a partially ionized gas with electrically charged particles at room temperature and atmospheric pressure. CAPP generates reactive oxygen species (ROS) and reactive nitrogen species (RNS), and has potential as a new apoptosis-promoting cancer therapy. With an annular type dielectric barrier discharge (DBD) CAPP-generating device combined with a helium (He) gas feeding system, we showed that CAPP selectively induced apoptosis in various cancer cells while it promoted proliferation of the adipose tissue-derived stem cell (ASC). The apoptotic effect of CAPP was highly selective toward p53-mutated cancer cells. The intracellular ROS was mainly responsible for apoptotic cell death in CAPP-treated cancer cells. CAPP induced apoptosis even in doxorubicin-resistant cancer cell lines, demonstrating the feasibility of CAPP as a potent cancer therapy. With the same device and exposure conditions to cancer cells, CAPP stimulated proliferation of the ASC, a kind of mesenchymal stem cell that is capable of self-renewing and differentiating into adipocytes, chondrocytes, osteoblasts and neurons. CAPP-treated ASCs expressed the stem cell markers and differentiated into adipocytes as untreated ASCs. The increase of proliferation by CAPP in ASCs was offset by a NO scavenger but was not affected by ROS scavengers, suggesting that NO generated by CAPP is responsible for the activated proliferation in ASCs. Usually, cancer stem cells are reported to be resistant to known cancer therapies. When we applied CAPP of the same device and exposure conditions to cancer cells to liver cancer stem cells (CSCs) that express CD133 and epithelial cell adhesion molecule (EpCAM) cancer stem cell markers, apoptotic cell death was not examined. Apoptotic cell death of liver CSCs was induced by the CAPP generated from a device with an air-based flatten type DBD. An exposure of liver CSCs to CAPP decreased the viability of liver CSCs to a great extent, suggesting plasma be used as a promising anti-cancer treatment. To validate whether CAPP can be a promising anti-cancer treatment or an adjuvant modality to eliminate remnant tumor in cancer surgery of vestibular schwannoma, we applied CAPP to mouse schwannoma cell line SC4 Nf2 ‑/‑ and human schwannoma cell line HEI-193. A CAPP treatment leads to anti-proliferative effect in both cell lines. We are currently studying the molecular mechanisms of differential physiological effect of CAPP; the proliferation of ASCs and apoptosis of various cancer cells and CSCs.

Keywords: cold atmospheric pressure plasma, apoptosis, proliferation, cancer cells, adult stem cells

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Authors: Joseph Mary Ssembatya, Blessing Michael Taremwa

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Background: Multiple schwannomas are typically associated with neurofibromatosis type 1 (NF1), but rare cases occur independently of neurofibromatosis. Schwannomas are benign, slow-growing tumors, primarily affecting the cervical and lumbar spine. When large, they may extend over multiple vertebral levels, posing surgical challenges. Case Presentation: A 13-year-old Ugandan Munyankore female patient, presented with a 6-year history of progressive quadriparesis, particularly in the lower limbs. Clinical examination showed hypertonia and hyperreflexia, with no indicators of neurofibromatosis or prior trauma. MRI revealed two “kissing” schwannomas extending from C2 to T2 in the cervical spine. Decompressive surgery was performed through laminoplasty and partial lesion resection, and histology confirmed schwannoma. Two weeks postoperatively, the patient experienced cerebrospinal fluid (CSF) leakage, neck pain, and headache, which required re-operation and duraplasty. Following these interventions, the patient’s neurological status stabilized, with noted improvement in lower limb strength. Discussion: “Kissing” schwannomas are most frequently documented in the cerebellopontine angle, rarely in the spine, and even more rarely in children. While multiple schwannomas are often associated with NF2, this case had no family history or clinical signs of the disorder. Giant invasive spinal schwannomas (GISS) that span multiple vertebrae demand intricate surgical approaches due to their proximity to neurovascular structures. Conclusion: This is the first reported case of kissing cervical schwannomas in a young patient from a low- to middle-income country. Surgical decompression, though challenging, is critical for neurological recovery in such advanced cases.

Keywords: kissing schwannoma, cervical spine, low resource, young, uganda

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Authors: Sona Babu Rathinam, Lavanya Dharmendran, Therraddi Mutthu

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Objectives: The purpose of this paper is to provides an overview of the neuroendocrine tumors that arise in the oral cavity. Material and Methods: An overview of the relevant papers on neuroendocrine tumors of the oral cavity by various authors was studied and summarized. Results: On the basis of the relevant studies, this paper provides an overview of the classification and histological differentiation of the neuroendocrine tumors that arise in the oral cavity. Conclusions: The basis of classification of neuroendocrine tumors is largely determined by their histologic differentiation. Though they reveal biologic heterogeneity, there should be an awareness of the occurrence of such lesions in the oral cavity to enable them to be detected and treated early.

Keywords: malignant peripheral nerve sheath tumor, olfactory neuroblastoma, paraganglioma, schwannoma

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Authors: Yusuf Bulale, Mark Prince, Geoff Tansley, Peter Brett

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Cochlear Implantation (CI) which became a routine procedure for the last decades is an electronic device that provides a sense of sound for patients who are severely and profoundly deaf. Today, cochlear implantation technology uses electrode array (EA) implanted manually into the cochlea. The optimal success of this implantation depends on the electrode technology and deep insertion techniques. However, this manual insertion procedure may cause mechanical trauma which can lead to a severe destruction of the delicate intracochlear structure. Accordingly, future improvement of the cochlear electrode implant insertion needs reduction of the excessive force application during the cochlear implantation which causes tissue damage and trauma. This study is examined tool-tissue interaction of large prototype scale digit embedded with distributive tactile sensor based upon cochlear electrode and large prototype scale cochlea phantom for simulating the human cochlear which could lead to small-scale digit requirements. The digit, distributive tactile sensors embedded with silicon-substrate was inserted into the cochlea phantom to measure any digit/phantom interaction and position of the digit in order to minimize tissue and trauma damage during the electrode cochlear insertion. The digit has provided tactile information from the digit-phantom insertion interaction such as contact status, tip penetration, obstacles, relative shape and location, contact orientation and multiple contacts. The tests demonstrated that even devices of such a relative simple design with low cost have a potential to improve cochlear implant surgery and other lumen mapping applications by providing tactile sensory feedback information and thus controlling the insertion through sensing and control of the tip of the implant during the insertion. In that approach, the surgeon could minimize the tissue damage and potential damage to the delicate structures within the cochlear caused by current manual electrode insertion of the cochlear implantation. This approach also can be applied to other minimally invasive surgery applications as well as diagnosis and path navigation procedures.

Keywords: cochlear electrode insertion, distributive tactile sensory feedback information, flexible digit, minimally invasive surgery, tool/tissue interaction

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Authors: Daniela Proca, Yuan Rong, Salvatore Luceno, Jalil Nasibli

Abstract:

Introduction: Solitary Fibrous Tumors (SFT) have many histologic mimickers and the only way to diagnose it, particularly in an unusual location, such as peripheral nerve trunks, is to use a comprehensive immunohistochemical staining panel. Monoclonal STAT6 immunostain is highly sensitive and specific for SFTs and particularly useful in the diagnosis of difficult SFT cases. Methods: We describe a solitary fibrous tumor (SFT) involving the right branchial plexus in a 66 yo female with 4-year history of slowly growing chest wall mass with recent dysesthesias in fingers 4th and 5th. MRI showed a well-circumscribed heterogenous mass measuring 5.4 x 3.8 x 4.0 cm and encircling peripheral nerves of the branchial plexus; no involvement of the bone or muscle was noted. A biopsy showed a bland spindled and epithelioid proliferation with no significant mitotic activity, no necrosis, and no atypia; peripheral nerve fascicles were encircled by the lesion. The main clinical and pathologic differential diagnosis included peripheral nerve sheath tumor, particularly schwannoma; HE microscopy didn’t show the classic Antoni A and B areas but showed focal subtle nuclear palisading, as well as prominent vessels with hyalinization. Immunohistochemical stains showed focal, weak cytoplasmic S100 positivity in the lesion; CD 34 and Vimentin were strongly and diffusely positive; the neoplastic cells were negative with AE1/AE3, EMA, CD31, SMA, Desmin, Calretinin, HMB-45, Melan A, PAX-8, NSE. The immunohistochemical and histologic pattern was not typical of peripheral nerve sheath tumor. On additional stains, the tumor was positive with STAT-6 and bcl-2 and focally positive with CD99. Given this profile, the final diagnosis was that of a solitary fibrous tumor. Results: NA Conclusion: Very few SFTs involving peripheral nerves and mimicking a peripheral nerve sheath tumor are described in the literature. Although histologically benign on this biopsy, long-term follow-up is required because of the risk of recurrence of these tumors and their uncertain biological behavior.

Keywords: solitary fibrous tumor, pathology, diagnosis, immunohistochemistry

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Authors: Magdy I. A. Alshourbagi

Abstract:

Background: The National Institute for Deafness and Communication Disorders defines idiopathic sudden sensorineural hearing loss as the idiopathic loss of hearing of at least 30 dB across 3 contiguous frequencies occurring within 3 days.The most common clinical presentation involves an individual experiencing a sudden unilateral hearing loss, tinnitus, a sensation of aural fullness and vertigo. The etiologies and pathologies of ISSNHL remain unclear. Several pathophysiological mechanisms have been described including: vascular occlusion, viral infections, labyrinthine membrane breaks, immune associated disease, abnormal cochlear stress response, trauma, abnormal tissue growth, toxins, ototoxic drugs and cochlear membrane damage. The rationale for the use of hyperbaric oxygen to treat ISSHL is supported by an understanding of the high metabolism and paucity of vascularity to the cochlea. The cochlea and the structures within it require a high oxygen supply. The direct vascular supply, particularly to the organ of Corti, is minimal. Tissue oxygenation to the structures within the cochlea occurs via oxygen diffusion from cochlear capillary networks into the perilymph and the cortilymph. . The perilymph is the primary oxygen source for these intracochlear structures. Unfortunately, perilymph oxygen tension is decreased significantly in patients with ISSHL. To achieve a consistent rise of perilymph oxygen content, the arterial-perilymphatic oxygen concentration difference must be extremely high. This can be restored with hyperbaric oxygen therapy. Subject and Methods: A 37 year old man was presented at the clinic with a five days history of muffled hearing and tinnitus of the right ear. Symptoms were sudden onset, with no associated pain, dizziness or otorrhea and no past history of hearing problems or medical illness. Family history was negative. Physical examination was normal. Otologic examination revealed normal tympanic membranes bilaterally, with no evidence of cerumen or middle ear effusion. Tuning fork examination showed positive Rinne test bilaterally but with lateralization of Weber test to the left side, indicating right ear sensorineural hearing loss. Audiometric analysis confirmed sensorineural hearing loss across all frequencies of about 70- dB in the right ear. Routine lab work were all within normal limits. Clinical diagnosis of idiopathic sudden sensorineural hearing loss of the right ear was made and the patient began a medical treatment (corticosteroid, vasodilator and HBO therapy). The recommended treatment profile consists of 100% O2 at 2.5 atmospheres absolute for 60 minutes daily (six days per week) for 40 treatments .The optimal number of HBOT treatments will vary, depending on the severity and duration of symptomatology and the response to treatment. Results: As HBOT is not yet a standard for idiopathic sudden sensorineural hearing loss, it was introduced to this patient as an adjuvant therapy. The HBOT program was scheduled for 40 sessions, we used a 12-seat multi place chamber for the HBOT, which was started at day seven after the hearing loss onset. After the tenth session of HBOT, improvement of both hearing (by audiogram) and tinnitus was obtained in the affected ear (right). Conclusions: In conclusion, HBOT may be used for idiopathic sudden sensorineural hearing loss as an adjuvant therapy. It may promote oxygenation to the inner ear apparatus and revive hearing ability. Patients who fail to respond to oral and intratympanic steroids may benefit from this treatment. Further investigation is warranted, including animal studies to understand the molecular and histopathological aspects of HBOT and randomized control clinical studies.

Keywords: idiopathic sudden sensorineural hearing loss (issnhl), hyperbaric oxygen therapy (hbot), the decibel (db), oxygen (o2)

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