Unilateral Hearing Loss Due to Cochlear Nerve Involvement as Isolated Symptom of a Primary Medulloblastoma

2019 ◽  
Vol 51 (02) ◽  
pp. 170-172
Author(s):  
Janine Magg ◽  
Thomas Nägele ◽  
Michael Alber ◽  
Annette Weichselbaum ◽  
Martin Ebinger ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Pediatric Population ◽  
Wnt Signaling Pathway ◽  
Neurofibromatosis Type ◽  
Cochlear Nerve ◽  
Vestibular Schwannomas ◽  
Clinical Feature ◽  
Unilateral Hearing Loss ◽  
Common Symptom ◽  
The Central Nervous System

AbstractUnilateral sensorineural hearing loss is a common symptom of vestibular schwannomas in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas and is often the initial clinical feature. While rare cases of sensorineural impairment presenting as vision or hearing loss due to metastatic medulloblastoma are known, hearing loss as an isolated presenting symptom of primary malignant neuroepithelial tumors of the central nervous system has not been reported in the pediatric population so far. We present two adolescents with unilateral hearing loss due to cochlear nerve dysfunction as the only symptom of a primary nonmetastatic medulloblastoma of the WNT signaling pathway family members subgroup.

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

2020 ◽  
Vol 129 (12) ◽  
pp. 1229-1238
Author(s):  
Matthew J. Urban ◽  
Dennis M. Moore ◽  
Keri Kwarta ◽  
John Leonetti ◽  
Rebecca Rajasekhar ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Observation Time ◽  
Vestibular Schwannomas ◽  
Open Set ◽  
Pubmed Database ◽  
Auditory Brainstem Implants ◽  
Hearing Outcomes

Objectives: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed. Methods: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. MESH search in NCBI PubMed database between 1992 and 2019 for reported cases of cochlear implantation with unresected vestibular schwannoma. Results: Seven patients underwent ipsilateral cochlear implantation in the presence of observed or irradiated vestibular schwannomas. Four patients had sporadic tumors with severe-profound contralateral hearing loss caused by presbycusis/hereditary sensorineural hearing loss, and three patients with NF2 lost contralateral hearing after prior surgical resection. Prior to implantation, one VS was observed without growth for a period of 7 years and the others were treated with radiotherapy. Mean post-operative sentence score was 63.9% (range 48-91) at an average of 28 (range 2-84) months follow up. All patients in this cohort obtained open set speech perception. While analysis of the literature is limited by heterogenous data reporting, 85% of implants with observed schwannomas achieved some open set perception, and 67% of patients previously radiated schwannomas. Furthermore, blending literature outcomes for post implantation sentence testing in quiet without lip-reading show 59.0 ± 35% for patients with CI and observed tumors and 55.7 ± 35% for patients with radiated tumors, with both groups ranging 0 to 100%. Conclusion: This retrospective series and literature review highlight that hearing outcomes with CI for VS patients are superior to those achieved with ABI. However, important considerations including imaging, delayed hearing loss, and observation time cannot be ignored in this population.

Bony cochlear nerve canal stenosis and speech discrimination in pediatric unilateral hearing loss

2015 ◽  
Vol 125 (7) ◽  
pp. 1691-1696 ◽  
Author(s):  
Patricia L. Purcell ◽  
Ayaka J. Iwata ◽  
Grace S. Phillips ◽  
Angelisa M. Paladin ◽  
Kathleen C. Y. Sie ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Nerve ◽  
Unilateral Hearing Loss ◽  
Speech Discrimination ◽  
Canal Stenosis

Clinical, pathologic, and radiologic response in patients with neurofibromatosis type 2 (NF2) undergoing bevacizumab treatment.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e13049-e13049
Author(s):  
Niamh Coleman ◽  
Megan Greally ◽  
Lynda O' Riordan ◽  
Teresa Doyle ◽  
Oscar S. Breathnach ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Energy Levels ◽  
Neurofibromatosis Type ◽  
Vestibular Schwannomas ◽  
Response To Treatment ◽  
Benign Tumors ◽  
Subjective Response ◽  
Mri Imaging ◽  
Profound Hearing Loss ◽  
Bevacizumab Treatment

e13049 Background: NF2 is a rare inherited genetic condition characterized by multiple "benign" tumors in the peripheral and central nervous system. Profound hearing loss as a result of bilateral vestibular schwannomas is a major debilitating complication with a profound effect on quality of life. Vascular endothelial growth factor (VEGF) has been shown to be produced by vestibular schwannoma tumor cells. Bevacizumab, a humanized monoclonal antibody against VEGF, has been proposed as a treatment for patients with advanced NF2, who are poor candidates for surgery and radiation therapy. The aim of this study was to investigate and assess the benefit of treatment with bevacizumab in the treatment of patients with advanced NF2. Methods: Five patients with inoperable NF2 were assessed prospectively from 03/12-01/13. Pathological assessment of tissue for vascularity was performed in each of the patients’ tissue. Objective assessments, including audiogram and MRI brain, were performed pre- and during therapy. Qualitative assessments were undertaken to assess subjective response to treatment. Results: We identified 5 patients with advanced NF2 [n=5], 3 female 2 male with a median age 40 years [range 28 – 52] as outlined in the Table. Tumor-volume reduction of >20% was noted on MRI imaging in the vestibular schwannomas of 2 patients. 2 patients showed objective improvement on audiogram with subjective hearing improvement also noted. Other subjective responses noted include increased energy levels, less headaches, better concentration. Conclusions: Bevacizumab treatment in patients with advanced NF2 resulted in some symptomatic improvement, tumour shrinkage, improvement in disability. Patients with vestibular schwannomas appeared to receive the most benefit, and so in terms of palliation, the benefits may be most significant in patients with specific disabilities, such as hearing loss. [Table: see text]

scholarly journals A Complete Constellation of Nervous System Lesions of NF2: Imaging Evaluation

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Kiran Gangadhar ◽  
Sandeep Kumar ◽  
Lovekesh Bhatia ◽  
Arjit Agarwal
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Neurofibromatosis Type ◽  
Radiological Findings ◽  
Case Patient ◽  
Cutaneous Lesions ◽  
Imaging Evaluation ◽  
The Central Nervous System

The radiological findings fulfilling the criteria of neurofibromatosis type 2 (NF2) were reviewed. NF2 is a rare disease with few cutaneous but frequent, typical radiological findings in the central nervous system. The presenting symptom is most commonly hearing loss due to acoustic schwannomas, although symptoms emanating from other intracranial or tumors are not uncommon. The discovery of multiple spinal neurofibromas or multiple meningiomas without cutaneous lesions should initiate a search for acoustic schwannomas even when the patient has normal hearing as in our case patient who actually presented for weakness of all four limbs.

scholarly journals Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas

Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2355
Author(s):  
Isabel Gugel ◽  
Florian Grimm ◽  
Julian Zipfel ◽  
Christian Teuber ◽  
Ulrike Ernemann ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Course ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Speech Discrimination ◽  
Predictive Values ◽  
Presenting Symptoms

The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. In this retrospective single-center study, we addressed this issue by reviewing 106 patients with 194 VS. Presenting symptoms attributable to VS commonly occur in 87% of adults and 31% of children. Age at onset significantly correlates with tumor volumes at presentation (p = 0.034). In addition, age at onset significantly correlates with pure-tone average (p = 0.0001), speech discrimination scores (p = 0.001), age at beginning of hearing loss (p = 0.0001), age at deafness (p = 0.0001), and age at first surgery (p = 0.0001). Patients presenting with VS related symptoms had significantly (p < 0.05) worse hearing values at presentation and after surgery. These patients also exhibited higher growth rates and tumor volumes compared to patients with non-VS related presenting symptoms, but this difference did not reach the significance level of p < 0.05. Due to the late appearance of these symptoms, the time of beginning hearing loss, surgery and deafness is significantly delayed (p < 0.05) compared to patients not presenting with VS. In summary, age at onset and type of presenting symptom provide excellent prognostic parameters for predicting VS- and hearing-related clinical course.

scholarly journals Progression of Unilateral Hearing Loss in Children With and Without Ipsilateral Cochlear Nerve Canal Stenosis

2017 ◽  
Vol 38 (6) ◽  
pp. e138-e144 ◽  
Author(s):  
Patricia L. Purcell ◽  
Justin R. Shinn ◽  
Scott S. Coggeshall ◽  
Grace Phillips ◽  
Angelisa Paladin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Nerve ◽  
Unilateral Hearing Loss ◽  
Canal Stenosis

scholarly journals Binaural Integration of Periodically Alternating Speech following Cochlear Implantation in Subjects with Profound Sensorineural Unilateral Hearing Loss

2015 ◽  
Vol 20 (Suppl. 1) ◽  
pp. 73-78 ◽  
Author(s):  
Thomas Wesarg ◽  
Nicole Richter ◽  
Horst Hessel ◽  
Stefanie Günther ◽  
Susan Arndt ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Auditory System ◽  
Unilateral Hearing Loss ◽  
Central Auditory System ◽  
Cortical Level ◽  
Study Results ◽  
Recognition Ability ◽  
The Central Nervous System ◽  
Speech Segments

In cochlear implant (CI) recipients with unilateral hearing loss (UHL) and normal hearing (NH) in the contralateral ear, the central auditory system receives signals of different auditory modalities, i.e. electrically via the CI ear as well as acoustically via the NH ear. The present study investigates binaural integration of bimodal stimulation in the central auditory system of 10 CI subjects with UHL by applying a modified version of the Rapidly Alternating Speech Perception (RASP) test to characterise speech recognition ability under monotic and dichotic listening arrangements. Subsequently, the results for each monotic and dichotic test condition were compared to quantify the binaural benefit from CI usage. The study results demonstrate significantly improved speech recognition under dichotic compared to monotic listening conditions, providing evidence that there is binaural integration of acoustically and electrically transmitted speech segments in the central nervous system at brainstem and cortical levels. In contrast to more commonly used tests of binaural integration, such as localisation, the RASP test provides the clinical option to investigate binaural integration involving structures at the cortical level.

scholarly journals Simultaneous cochlear implantation as a therapeutic option in vestibular schwannoma surgery: case report

2018 ◽  
Vol 44 (3) ◽  
pp. E9 ◽  
Author(s):  
Pedro Helo dos Santos Neto ◽  
Johnni Oswaldo Zamponi ◽  
Rogério Hamerschmidt ◽  
Gislaine Richter Minhoto Wiemes ◽  
Marcio S. Rassi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Therapeutic Option ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Common Symptom ◽  
Profound Hearing Loss ◽  
Microsurgical Resection ◽  
Speech Recognition In Noise

Hearing loss is the most common symptom of vestibular schwannomas (VSs). The management of these lesions includes observation, radiosurgery, and microsurgical resection. Hearing preservation and rehabilitation are the major challenges after the tumor treatment. A 43-year-old man with previous left-sided profound hearing loss and tinnitus presented with a 2-mm left-sided intracanalicular VS. The decision was made to perform a simultaneous cochlear implantation (CI) and microsurgical resection of the tumor. The patient did well postoperatively, with significant improvement of tinnitus, sound localization, and speech recognition in noise. Previous reports of simultaneous CI and VS resection in patients with neurofibromatosis type 2 and sporadic VS in the only hearing ear have been described. The role of CI in patients with VS and normal contralateral hearing has been recently described, showing positive outcomes due to the binaural benefits. Tinnitus also can be treated by the implantation of the cochlear device. The simultaneous microsurgical removal of VS and implantation of a cochlear device is a feasible approach in patients with unilateral hearing loss and severe tinnitus.

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