Long-Term Hearing Outcome of Cochlear Implantation in Cases with Simultaneous Intracochlear Schwannoma Resection.

OBJECTIVES: The aim was to analyze the long-term hearing results after simultaneous microsurgical extirpation via enlarged cochleostomy and cochlear implantation in intracochlear schwannoma as compared with non-tumor single-side deafness patients. METHODS: Microsurgical extirpation via enlarged cochleostomy with simultaneous cochlear implantation was performed in 15 cases of intracochlear schwannoma between 2014 and 2021. Speech recognition tests in German language and impedance performances were collected over 36 months of observation and compared with an internal cohort of 52 age matched non-tumor single-side deafness patients. Retrospective cohort study in a tertiary referral center. RESULTS: The surgery proved feasible and uneventful in all cases. In the case of intracochlear schwannoma, the hearing rehabilitation results were highly satisfactory and comparable to those of the non-tumor single-side deafness cohort. The speech recognition performance improved steadily in the first 12 months; afterward, it remained stable, providing indirect evidence against tumor recurrence during the follow-up. One patient required implant revision surgery related to device failure, but no recurrence was registered in the 36 months of observation. CONCLUSIONS: Cochlear implantation is the strategy of choice for hearing rehabilitation in case of intracochlear schwannomas in the long term. In particular, the combination of tumor extirpation via cochleostomy with a cochlear implantation in the same surgical time offers a viable therapy for intracochlear schwannoma, granting a sufficient degree of radicality without compromising the cochlear integrity. This technique allows for revision surgery if required. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1854-1860, 2024.

Intracochlear schwannoma: Imaging diagnosis.

Intralabyrinthine schwannomas (ILS) are rare benign tumors, often responsible for hearing loss. MRI is important in establishing the diagnosis. We present the example of a 48-year-old lady who reported a 3-years history of right-sided sensorineural deafness. MRI demonstrated a loss of the normal hypersignal of the second turn of the right cochlea compatible with intracochlear schwannoma.

Surgical Outcomes of Simultaneous Cochlear Implantation and Intracochlear Schwannoma Removal.

OBJECTIVE: Intracochlear schwannoma is very rare, and complete loss of hearing is inevitable after the removal of this tumor. Here, we discuss cochlear implantation (CI) performed simultaneously with the removal of an intracochlear schwannoma. STUDY DESIGN: Retrospective single-center study. SETTING: Tertiary medical institute. METHODS: Simultaneous CI and intracochlear schwannoma removal were performed in 4 subjects. After subtotal cochleostomy, the tumors were removed meticulously, with preservation of the modiolus. A new slim modiolar electrode (Nucleus CI632) was placed in a manner that hugged the modiolus. The surgical outcomes of functional gain, word recognition score (WRS), sound localization, and hearing in noise and speech intelligibility tests were investigated. RESULTS: Intracochlear schwannomas were removed successfully from the 4 patients, with no remnant tumor. The mean aided hearing threshold 6 months after surgery was 25.0 ± 1.8 dB, and the mean-aided WRS with a 60 dB stimulus was 36.0 ± 18.8% (range 16%-60%). The Categorical Auditory Performance (CAP) score of the 3 single-sided deafness patients under contralateral ear masking was 7. The CAP score of the patient with bilateral sensorineural hearing loss was 6, which improved from a preoperative score of 0. CONCLUSION: When an intracochlear schwannoma does not completely invade the modiolus, CI with simultaneous tumor removal can be performed successfully, resulting in good hearing performance. A slim modiolar electrode can be placed stably at the modiolus after schwannoma removal.

Cochlear Implantation in a Patient with Neurofibromatosis Type 2 and an Intracochlear Schwannoma: A Case Report and Literature Review.

A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.

Cochlear Schwannoma: A Usual Pathology in an Unusual Site.

Intra-labyrinthine schwannomas are rare. We present a case of a 50-year-old male with non-serviceable unilateral sensorineural hearing loss and tinnitus. CE-MRI revealed an enhancing signal in the basal turn of left cochlea suggestive of a schwannoma. A trans-mastoid standard facial recess approach was used for tumor excision. At one year follow up, patient is disease free.

Bilateral intracochlear schwannomas in a patient with no genetic or clinical features of neurofibromatosis type 2.

Schwannomas of the eighth cranial nerve are benign tumours commonly found in the internal auditory meatus or in the cerebellopontine angle. In most cases, they arise from the inferior or vestibular portion of the vestibular nerve. Rarely, these tumours present in the inner ear and are then called intralabyrinthine schwannomas. Bilateral schwannomas are known in neurofibromatosis type 2 (NF2). Bilateral and ipsilateral, multilocular sporadic schwannomas of the eighth cranial nerve have been described as extremely rare findings. This report describes the first case of bilateral sporadic intracochlear schwannomas in a patient with no genetic or clinical features of NF2.

[Bilateral intracochlear schwannomas in a patient with no genetic or clinical features of neurofibromatosis type 2. German version]. / Bilaterale intracochleäre Schwannome bei einer Patientin ohne genetische oder klinische Hinweise auf Neurofibromatose Typ 2.

Schwannomas of the eighth cranial nerve are benign tumours commonly found in the internal auditory meatus or in the cerebellopontine angle. In most cases, they arise from the inferior or vestibular portion of the vestibular nerve. Rarely, these tumours present in the inner ear and are then called intralabyrinthine schwannomas. Bilateral schwannomas are known in neurofibromatosis type 2 (NF2). Bilateral and ipsilateral, multilocular sporadic schwannomas of the eighth cranial nerve have been described as extremely rare findings. This report describes the first case of bilateral sporadic intracochlear schwannomas in a patient with no genetic or clinical features of NF2.

Intralabyrinthine Schwannomas: Disease Presentation, Tumor Management, and Hearing Rehabilitation.

Intralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

Acoustic schwannoma with intracochlear extension and primary intracochlear schwannoma: removal through translabyrinthine approach with facial bridge cochleostomy and transcanal approach.

Intracochlear schwannomas can occur either as an extension of a larger tumor from the internal auditory canal, or as a solitary labyrinthine tumor. They are currently removed via a translabyrinthine approach extended to the basal turn, adding a transotic approach for tumors lying beyond the basal turn. Facial bridge cochleostomy may be associated with the translabyrinthine approach to enable the whole cochlea to be approached without sacrificing the external auditory canal and tympanum. We describe seven cases, five of which underwent cochlear schwannoma resection with facial bridge cochleostomy, one case with the same procedure for a suspect tumor and one, previously subjected to radical tympanomastoidectomy, who underwent schwannoma resection via a transotic approach. Facial bridge cochleostomy involved removing the bone between the labyrinthine and tympanic portions of the fallopian canal, and exposing the cochlea from the basal to the apical turn. Patients' recovery was uneventful, and long-term magnetic resonance imaging showed no residual tumor. Facial bridge cochleostomy can be a flexible extension of the translabyrinthine approach for tumors extending from the internal auditory canal to the cochlea. The transcanal approach is suitable for the primary exclusive intralabyrinthine tumor. The indications for the different approaches are discussed.

Minimally Invasive Surgery for Intracochlear Schwannoma Removal and Simultaneous Cochlear Implantation

Abstract Introduction Hearing preservation has not yet been reported in patients undergoing resection of intracochlear schwannomas. This study describes a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation that resulted in good hearing. Objective This study aims to describe a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation. Data Synthesis The technique described in this study was developed for a 55-year-old male with a 20-year history of bilateral progressive hearing loss and tinnitus that had a mass in the left apical turn of the cochleameasuring 0.3 cm. Surgery accessed the apical turn of the cochlea. We performed mastoidectomy and posterior tympanotomy and removed incus and tensor tympani muscle to expose the cochlear apex. The tumor was identified and completely resected. After the cochleawas anatomically preserved, it was implanted with a straight electrode via round window insertion. The histopathological examination confirmed intracochlear schwannoma. Speech perception test revealed 100% speech recognition with closed sentences and the average audiometric threshold (500 to 2000 Hz) was 23 dB. Conclusion Our technique led to rehabilitation of the patient and improved hearing without damaging the intracochlear structure, making it possible to perform CI in the same procedure with good results.

Minimally Invasive Surgery for Intracochlear Schwannoma Removal and Simultaneous Cochlear Implantation.

INTRODUCTION: Hearing preservation has not yet been reported in patients undergoing resection of intracochlear schwannomas. This study describes a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation that resulted in good hearing. OBJECTIVE: This study aims to describe a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation. DATA SYNTHESIS: The technique described in this study was developed for a 55-year-old male with a 20-year history of bilateral progressive hearing loss and tinnitus that had a mass in the left apical turn of the cochlea measuring 0.3 cm. Surgery accessed the apical turn of the cochlea. We performed mastoidectomy and posterior tympanotomy and removed incus and tensor tympani muscle to expose the cochlear apex. The tumor was identified and completely resected. After the cochlea was anatomically preserved, it was implanted with a straight electrode via round window insertion. The histopathological examination confirmed intracochlear schwannoma. Speech perception test revealed 100% speech recognition with closed sentences and the average audiometric threshold (500 to 2000 Hz) was 23 dB. CONCLUSION: Our technique led to rehabilitation of the patient and improved hearing without damaging the intracochlear structure, making it possible to perform CI in the same procedure with good results.

Meta-analysis on the clinical outcomes in patients with intralabyrinthine schwannomas: conservative management vs. microsurgery.

The objective of this review is to compare the symptomatological evolution following conservative management (CM) or microsurgery (MS) in patients with intralabyrinthine schwannomas (ILS). A thorough systematic review of the English and French literature from 1948 to February 2014 was performed using Ovid Medline. An ancestor search was also completed. The major inclusion criterion consisted of a diagnosis of ILS with magnetic resonance imaging. Patients with a classic vestibular schwannoma, cases of incidentaloma during surgery or an autopsy were the main exclusion criteria. Thirty-one studies met our selective criteria. Descriptive data were collected from the articles. Clinical outcomes regarding the hearing loss, tinnitus, vertigo, dizziness and aural fullness were stated as improved, unchanged or worse at the last follow-up. All data were then separated into two different groups according to the management option: CM and MS. The data were analyzed using a Pearson χ (2) test and Fisher's exact test. This meta-analysis suggests that MS has a statistically significant favorable outcome regarding symptom relief compared to CM in patients with ILS suffering from tinnitus, vertigo and dizziness. Hearing level was not compared between treatment groups, as MS leads to anacusis. An indicative bias was the main limitation of this study, as patients suffering from intractable vertigo with moderate-to-severe hearing loss were referred to MS. Therefore, in the presence of a serviceable hearing, we suggest that CM should be the treatment of choice.

Minimally invasive surgical removal of an intracochlear schwannoma causing an intractable paroxysmal positional vertigo.

Intracochlear schwannomas are rare tumours. Diagnosis is based on high-resolution MRI, which should be used for accurate determination of the location of tumours. We report a case of a cochlear schwannoma that presented with profound hearing loss and intractable paroxysmal positional vertigo, which was diagnosed with gadolinium-enhanced MRI and removed using a transcanal minimally-invasive transotic approach.

Intracochlear Schwannoma: Diagnosis and Management

Introduction: Schwannomas of the eighth cranial nerve are benign tumors that usually occur in the internal auditory canal or the cerebellopontine angle cistern. Rarely, these tumors may originate from the neural elements within the vestibule, cochlea, or semicircular canals and are called intralabyrinthine schwannomas. Intracochlear schwannomas (ICSs) represent a small percentage of these tumors, and their diagnosis is based on high-resolution magnetic resonance imaging (MRI). Objectives: To report the clinical and radiologic features and audiometric testing results of an ICS in a 48-year-old man after a 22-month follow-up period. Resumed Report A patient with an 8-year history of persistent tinnitus in his right ear, combined with ipsilateral progressive hearing loss and aural fullness. Audiometry revealed normal hearing in the left ear and a moderate to severe sensorineural hearing loss in the right ear, with decreased speech reception threshold and word recognition score, compared with the exam performed 5 years previously. MRI showed a small intracochlear nodular lesion in the modiolus, isointense on T1 with a high contrast enhancement on T1 postgadolinium images. During the follow-up period, there were no radiologic changes on imaging studies. Thus, a wait-and-scan policy was chosen as the lesion remained stable with no considerable growth and the patient still presents with residual hearing. Conclusions: Once diagnosed, not all ICS patients require surgery. Treatment options for ICS include stereotactic radiotherapy and rescanning policy, depending on the tumor's size, evidence of the tumor's growth, degree of hearing loss, intractable vestibular symptoms, concern about the pathologic diagnosis, and the patient's other medical conditions...

Intracochlear schwannoma: diagnosis and management.

Introduction Schwannomas of the eighth cranial nerve are benign tumors that usually occur in the internal auditory canal or the cerebellopontine angle cistern. Rarely, these tumors may originate from the neural elements within the vestibule, cochlea, or semicircular canals and are called intralabyrinthine schwannomas. Intracochlear schwannomas (ICSs) represent a small percentage of these tumors, and their diagnosis is based on high-resolution magnetic resonance imaging (MRI). Objectives To report the clinical and radiologic features and audiometric testing results of an ICS in a 48-year-old man after a 22-month follow-up period. Resumed Report A patient with an 8-year history of persistent tinnitus in his right ear, combined with ipsilateral progressive hearing loss and aural fullness. Audiometry revealed normal hearing in the left ear and a moderate to severe sensorineural hearing loss in the right ear, with decreased speech reception threshold and word recognition score, compared with the exam performed 5 years previously. MRI showed a small intracochlear nodular lesion in the modiolus, isointense on T1 with a high contrast enhancement on T1 postgadolinium images. During the follow-up period, there were no radiologic changes on imaging studies. Thus, a wait-and-scan policy was chosen as the lesion remained stable with no considerable growth and the patient still presents with residual hearing. Conclusions Once diagnosed, not all ICS patients require surgery. Treatment options for ICS include stereotactic radiotherapy and rescanning policy, depending on the tumor's size, evidence of the tumor's growth, degree of hearing loss, intractable vestibular symptoms, concern about the pathologic diagnosis, and the patient's other medical conditions.