Cochlear Implantation in the Neurofibromatosis Type 2 Patient: Long‐Term Follow‐up

Neff, Brian A.; Wiet, R. Mark; Lasak, John M.; Cohen, Noel L.; Pillsbury, Harold C.; Ramsden, R. T.; Welling, D. Bradley

doi:10.1097/mlg.0b013e31804b1ae7

Cited by 121 publications

(109 citation statements)

References 15 publications

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“…In Type I Neurofibromatosis, 90% of the patients develop at least one-sided VS. Ten times less frequent is the neurofibromatosis type 2 which is associated with bilateral VSs in 90% of the affected patients 2,9 . Considering the large number of the affected patients, early diagnosis and treatment of the disease is mandatory in order to achieve favourable results.…”

Section: Introductionmentioning

confidence: 99%

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Cochlear implantation improves hearing and vertigo in patients after removal of vestibular schwannoma

Roemer¹,

Lenarz²,

Lesinski‐Schiedat³

2017

The International Tinnitus Journal

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Objective: With rising quality of speech perception in cochlear implant users, the indication widens. Nowadays, cochlear implantation is reasonable even in vestibular schwannoma patients. Speech perception with a cochlear implant is in these patients as promising as in patients with sensorineural hearing loss. However, the impact of cochlear implantation on vertigo and tinnitus after removal of vestibular schwannoma has not been investigated yet. Methods: In a retrospective study, we analysed 12 patients treated with a cochlear implant after removal of vestibular schwannoma. Results: In addition to a promising hearing perception -all patients reported improvement of vertigo. This improvement was also demonstrated by postural analysis. Improvement of tinnitus was achieved in 50% of the patients. Conclusion: Cochlear implantation seems a promising treatment for hearing loss, vertigo and even tinnitus in patients after removal of vestibular schwannoma. However, for successful cochlear implantation with adequate speech perception and improvement of vertigo and tinnitus, functional hearing nerve and intact inner ear anatomy is necessary.

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Section: Introductionmentioning

confidence: 99%

“…Therefore, more and more CIs are an option for the treatment of hearing loss for these patients. Several studies suggest a very good hearing rehabilitation of acoustic neuroma patients after CI 9,17,18 . However, nearly all studies are limited due to small patient numbers 19,20 -reporting case files or groups of up to 3 patients.…”

Section: Introductionmentioning

confidence: 99%

Cochlear implantation improves hearing and vertigo in patients after removal of vestibular schwannoma

Roemer¹,

Lenarz²,

Lesinski‐Schiedat³

2017

The International Tinnitus Journal

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“…1,2,4,5 Reports of cochlear implant use in patients with neurofibromatosis type 2 have shown that they are superior to brainstem implants for increasing auditory abilities and for sustaining these results in the long term. 2,5 Therefore, cochlear implants have become an option in such circumstances. The indispensable condition for this approach is that the anatomy and function of the cochlear nerve be preserved.…”

Section: Discussionmentioning

confidence: 99%

“…The possibility of preserving the cochlear nerve in neurofibromatosis cases makes it feasible to fit cochlear implants for improved auditory rehabilitation. 2,4,5 We present the results attained in a patients with neurofibromatosis in whom the neoplasm was removed and a cochlear implant was placed simultaneously. Based on a review of the literature, this is the first documented case of this procedure in Brazil.…”

Section: Introductionmentioning

confidence: 99%

Cochlear implant in type 2 neurofibromatosis: an option for better hearing rehabilitation

Cruz

Vellutini²

2011

Braz. j. otorhinolaryngol. (Impr.)

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“…Functional results show great variability; from noise perception only to useful speech discrimination in audiovisual mode in a few patients so far. Since the early trials by Hitselberger and House in 1979, as reported by Edgerton et al 13 and Hit sel berger et al, 21 profound improvements in implant array, technology, and speech processing strategies 65 have brought about useful improvement in functional outcome, 4,8,9,17,23,29,31,32,37,42,43,45,46,48,58,59 but still results remain less favorable than in cochlear implants or auditory brainstem implants (ABIs) in patients without NF2. 8 Visionindependent speech perception is not generally achieved.…”

mentioning

confidence: 99%

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

Matthies¹,

Brill²,

Várallyay

et al. 2014

JNS

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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.

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Cochlear Implantation in the Neurofibromatosis Type 2 Patient: Long‐Term Follow‐up

Cited by 121 publications

References 15 publications

Cochlear implantation improves hearing and vertigo in patients after removal of vestibular schwannoma

Cochlear implantation improves hearing and vertigo in patients after removal of vestibular schwannoma

Cochlear implant in type 2 neurofibromatosis: an option for better hearing rehabilitation

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

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