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.

Update of ABI Outcomes in Non-tumor Adults

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P61-P62
Author(s):  
Vittorio Colletti ◽  
Robert V. Shannon ◽  
Marco Carner ◽  
Sheila Veronese ◽  
Liliana Colletti
Keyword(s):  
Cochlear Implant ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Tumor Patients ◽  
Open Set ◽  
Sound Detection ◽  
The Difference ◽  
Auditory Brainstem Implants ◽  
Hearing Outcomes

Objective The traditional indication for auditory brainstem implants (ABIs) is neurofibromatosis type 2 (NF2) in subjects older than 12 years. In our department, the indications for ABIs have been extended to non-tumor children and adults with cochlear or cochlear nerve injuries or malfunctions who would not benefit from a cochlear implant. The results obtained in non-tumor adults are reported in this study. Methods In our department, from April 1997 to February 2008, ABIs have been implanted in a total of 114 patients (83 adults and 31 children) for different tumor and non-tumor diseases. 15 patients out of 114 have elsewhere previously had a CI with no sound detection. The retrosigmoid-transmeatal approach was used in all tumor patients and the retrosigmoid approach was used in all non-tumor patients. Results All patients had a functioning implantation, and reported auditory sensations with activation of various numbers of electrodes. At 1 year after implantations, non-tumor adults scored from 10% to 100% in open set speech perception tests (average 57%), and tumor patients scored from 5% to 30% (average 11%). The difference between these results is statistically significant (p < 0.01). Conclusions Hearing outcomes are better in non-tumor than tumor patients, with a significant number of non-tumor patients that are able to understand speech at a level comparable to that of the most successful cochlear implant users, including conversational telephone use.

Auditory brainstem implants in neurofibromatosis Type 2: is open speech perception feasible?

2014 ◽  
Vol 120 (2) ◽  
pp. 546-558 ◽  
Author(s):  
Cordula Matthies ◽  
Stefan Brill ◽  
Csanad Varallyay ◽  
Laszlo Solymosi ◽  
Goetz Gelbrich ◽  
...  
Keyword(s):  
Speech Perception ◽  
Word Recognition ◽  
Tumor Volume ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Open Set ◽  
Auditory Mode ◽  
Auditory Brainstem Implants

Object Patients with bilateral auditory nerve destruction may perceive some auditory input with auditory brainstem implants (ABIs). Despite technological developments and trials in new stimulation sites, hearing is very variable and of limited quality. The goal of this study was to identify advantageous and critical factors that influence the quality of auditory function, especially speech perception. Methods The authors conducted a prospective study on ABI operations performed with the aid of multimodality neuromonitoring between 2005 and 2009 in 18 patients with neurofibromatosis Type 2. Outcome was evaluated by testing word recognition (monotrochee-polysyllabic word test at auditory-only mode [MTPa]) and open speech perception (Hochmair-Schulz-Moser [HSM] sentence test), both in pure auditory mode. The primary outcome was the HSM score at 24 months. The predictive meaning of general clinical data, tumor volume, number of active electrodes, duration of deafness, and early hearing data was examined. Results In 16 successful ABI activations the average score for MTPa was 89% (SD 13%), and for HSM it was 41% (SD 32%) at 24 months. There were 2 nonresponders, 1 after radiosurgery and the other in an anatomical variant. Direct facial nerve reconstruction during the same surgery was followed by successful nerve recovery in 2 patients, with a simultaneous very good HSM result. Patients' age, tumor extension, and tumor volume were not negative predictors. There was an inverse relationship between HSM scores and deafness duration; 50% or higher HSM scores were found only in patients with ipsilateral deafness duration up to 24 months. The higher the deafness sum of both sides, the less likely that any HSM score will be achieved (p = 0.034). In patients with total deafness duration of less than 240 months, higher numbers of active electrodes were significantly associated with better outcomes. The strongest cross-correlation was identified between early MTPa score at 3 months and 24-month HSM outcome. Conclusions This study documents that open-set speech recognition in pure auditory mode is feasible in patients with ABIs. Large tumor volumes do not prevent good outcome. Positive preconditions are short ipsilateral and short bilateral deafness periods and high number of auditory electrodes. Early ability in pure auditory word recognition tests indicates long-term capability of open speech perception.

Speech Perception in Auditory Brainstem Implants in Neurofibromatosis Type 2

2012 ◽  
Vol 73 (S 02) ◽  
Author(s):  
C. Matthies ◽  
R. Mlynski ◽  
S. Brill ◽  
J. Mueller ◽  
C. Varallyay ◽  
...  
Keyword(s):  
Speech Perception ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Auditory Brainstem Implants

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.

Audiological outcome of stapes surgery for far advanced cochlear otosclerosis

2017 ◽  
Vol 131 (11) ◽  
pp. 961-964 ◽  
Author(s):  
C Heining ◽  
R Banga ◽  
R Irving ◽  
C Coulson ◽  
P Monksfield
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Revision Surgery ◽  
Cochlear Implantation ◽  
Stapes Surgery ◽  
Hearing Thresholds ◽  
Suitable Treatment ◽  
Conductive Component ◽  
Clinically Significant ◽  
Hearing Outcomes

AbstractBackground:Patients with advanced otosclerosis can present with hearing thresholds eligible for cochlear implantation. This study sought to address whether stapes surgery in this patient group provides a clinically significant audiological benefit.Objectives:To assess pre- and post-operative hearing outcomes of patients with advanced otosclerosis, and to determine what proportion of these patients required further surgery including cochlear implantation.Methods:Between 2002 and 2015, 252 patients underwent primary stapes surgery at our institution. Twenty-eight ears in 25 patients were deemed to have advanced otosclerosis, as defined by pure audiometry thresholds over 80 dB. The patients’ records were analysed to determine audiological improvement following stapes surgery, and assess whether any further surgery was required.Results:The audiological outcome for most patients who underwent primary stapes surgery was good. A minority of patients (7 per cent) required revision surgery. Patients who underwent cochlear implantation after stapes surgery (10 per cent) also demonstrated a good audiological outcome.Conclusion:Stapes surgery is a suitable treatment option for patients with advanced otosclerosis, and should be considered mandatory, before offering cochlear implantation, for those with a demonstrable conductive component to their hearing loss. A small group of patients get little benefit from surgery and subsequently a cochlear implant should be considered.

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]

Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2

2018 ◽  
Vol 128 (9) ◽  
pp. 2163-2169 ◽  
Author(s):  
Kevin A. Peng ◽  
Mark B. Lorenz ◽  
Steven R. Otto ◽  
Derald E. Brackmann ◽  
Eric P. Wilkinson
Keyword(s):  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2

The link between Brain and Hearing system

2020 ◽  
Vol 12 (4) ◽  
pp. 114-117
Author(s):  
Alireza Bina
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory System ◽  
Auditory Nerve ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Brain Disorder ◽  
Hearing System

There are some studies which confirmed that dysfunction in Central Nervous System(CNS) may cause a malfunction in the Peripheral Auditory system (Cochlea_ Auditory Nerve, Auditory Neuropathy), but the question is could Brain Disorder without any lesion in the Cochlea and/or Auditory nerve cause Sensorineural Hearing Loss? It means that the Audiogram shows that the patient suffers from sensorineural hearing loss but the site of the lesion is neither Sensory nor Neural while Brain may be involved in charge of this. And if the answer is yes then could we hear with our Brain and without Cochlea and /or Auditory nerve? We deal with this subject in this paper by: Otosclerosis and Meniere’s disease and The Brain Involvement. Hearing Loss following dysfunction in the Central Auditory and/or central non auditory system. Auditory Brainstem Implant in Patients who suffer from Neurofibromatosis Type two compare to Non Tumor cases, Mondini Syndrome, Michel aplasia. Possible role of Utricle and Saccule in Auditory (Hearing) System We propose a new Hypothesis that the External Ear Canal is not the only input of Auditory Signals, Sounds could transfer by our eyes and skin to the Cerebral Cortex and approach to the Cochlea (Backward Auditory input pathway of Sounds).

scholarly journals Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants

2011 ◽  
Vol 115 (4) ◽  
pp. 827-834 ◽  
Author(s):  
Pamela C. Roehm ◽  
Jon Mallen-St. Clair ◽  
Daniel Jethanamest ◽  
John G. Golfinos ◽  
William Shapiro ◽  
...  
Keyword(s):  
Cochlear Implants ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Speech Understanding ◽  
Speech Discrimination ◽  
Auditory Rehabilitation ◽  
Open Set ◽  
Cochlear Ossification

Object The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation. Methods Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise. Results Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15–120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants. Conclusions Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.

Cochlear Implantation in Sudden Bilateral Sensorineural Hearing Loss

1998 ◽  
Vol 77 (4) ◽  
pp. 300-303 ◽  
Author(s):  
Eric W. Sargent ◽  
Douglas L. Beck
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Bilateral Hearing Loss ◽  
Significant Recovery ◽  
Open Set ◽  
Bilateral Sensorineural Hearing Loss

Although 1–4% of all cases of sudden sensorineural hearing loss (SSHL) are bilateral, all such patients reported to date have experienced significant recovery of hearing in at least one ear. We report a case of profound, bilateral idiopathic SSHL without recovery which was treated with cochlear implantation; the first such report to our knowledge. The patient achieved open-set spondee recognition. Individuals with sudden bilateral hearing loss in whom treatable causes have been eliminated may benefit from cochlear implantation.

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