Hearing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis Type 2

2001 ◽  
Vol 95 (6) ◽  
pp. 1028-1033 ◽  
Author(s):  
Michel Kalamarides ◽  
Alexis Bozorg Grayeli ◽  
Didier Bouccara ◽  
Emmanuelle Ambert Dahan ◽  
Wolf Peter Sollmann ◽  
...  
Keyword(s):  
Radiation Treatment ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Sound Recognition ◽  
Tumor Removal ◽  
Environmental Sound ◽  
Content Type ◽  
Auditory Brainstem Implants

✓ The auditory brainstem implant (ABI) is designed to restore useful auditory sensations in patients with neurofibromatosis Type 2 (NF2). The implantation is usually performed at the time of tumor removal in patients who do not undergo radiation treatment. The authors evaluated the performance of ABIs in three patients with NF2 in whom vestibular schwannoma continued to grow after radiation treatment. These three patients with NF2 received a 21-channel ABI; a translabyrinthine approach was used for both the tumor removal and the ABI placement. The interval between radiosurgery and the tumor removal plus device implantation ranged from 2 to 11 years. In all cases, the tumor was growing and the patients presented with total deafness. The mean number of active electrodes in these three patients was equivalent to the average results reported in other patients who received ABIs. The patients in this study used the ABI regularly for everyday life and obtained useful levels of environmental sound recognition. It is concluded that hearing function can be rehabilitated using ABIs in patients with NF2, even if radiosurgery fails to control the tumor growth.

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

scholarly journals Cochlear Implantation in Patients with Neurofibromatosis Type 2 and Patients with Vestibular Schwannoma in the Only Hearing Ear

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Erika Celis-Aguilar ◽  
Luis Lassaletta ◽  
Javier Gavilán
Keyword(s):  
Cochlear Implants ◽  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Environmental Sound ◽  
Auditory Brainstem Implant ◽  
Surgical Option

Cochlear implants are a new surgical option in the hearing rehabilitation of patients with neurofibromatosis type 2 (NF2) and patients with vestibular schwannoma (VS) in the only hearing ear. Auditory brainstem implant (ABI) has been the standard surgical treatment for these patients. We performed a literature review of patients with NF2 and patients with VS in the only hearing ear. Cochlear implantation (CI) provided some auditory benefit in all patients. Preservation of cochlear nerve integrity is crucial after VS resection. Results ranged from environmental sound awareness to excellent benefit with telephone use. Promontory stimulation is recommended although not crucial. MRI can be performed safely in cochlear implanted patients.

scholarly journals Auditory Brainstem Implants in Neurofibromatosis Type 2: Early and Long-term Results

2017 ◽  
Vol 78 (S 01) ◽  
pp. S1-S156
Author(s):  
Cordula Matthies ◽  
Goetz Gelbrich ◽  
Robert Mlynski ◽  
Rudolf Hagen ◽  
Wafaa Shehata-Dieler
Keyword(s):  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Long Term Results ◽  
Auditory Brainstem Implants

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.

Early and Long-Term Outcome in Auditory Brainstem Implants in Neurofibromatosis Type 2

2016 ◽  
Vol 77 (S 02) ◽  
Author(s):  
Cordula Matthies ◽  
Stefan Brill ◽  
Wilma Großmann ◽  
Maria Hummel ◽  
Goetz Gelbrich ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Auditory Brainstem Implants

scholarly journals Magnetic Resonance Imaging in a Neurofibromatosis Type 2 Patient with a Novel MRI-Compatible Auditory Brainstem Implant

2017 ◽  
Vol 78 (01) ◽  
pp. e12-e14 ◽  
Author(s):  
Matthew Shew ◽  
Judson Bertsch ◽  
Paul Camarata ◽  
Hinrich Staecker
Keyword(s):  
Neurofibromatosis Type ◽  
The United States ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
The Body ◽  
Receiver Coil ◽  
Speech Processor ◽  
Auditory Brainstem Implants ◽  
The Magnetic Field

AbstractAuditory brainstem implantation has become a key technique for the rehabilitation of hearing in patients with neurofibromatosis type 2. The nature of this devastating genetic disease requires ongoing MRI for the patient's lifespan. Today, most auditory brainstem implants require removal of the magnet that connects the internal device to the external speech processor to undergo imaging as their disease progresses. Patients have the option of having a short procedure to have the magnet taken out and replaced each time, or alternately using a headband to secure the processor over the receiver coil of the internal device. Novel magnet technology has led to the development of a freely rotating magnet that can be used inside the magnetic field of an MRI scanner without losing magnet strength and without being displaced from the body of the device. We report one of the first patients implanted with a Med-El Synchrony ABI in the United States who subsequently underwent successful imaging with MRI 1.5 tesla to follow for other existing schwannomas.

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