Topodiagnosis of deafness: strategy for treatment of neurofibromatosis type 2

Marangos, N.; Stecker, M.; Laszig, Roland

doi:10.1258/0022215001904770

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…Interestingly, this patient was the only one with a negative response on electrophysiologic testing. Although EPS testing presents some shortcomings, mainly because based on a subjective assessment, we agree with other authors [Friedmann et al, 1998;Marangos et al, 2000] in considering ESP as a useful test in the preoperative evaluation of NF2 patients selected for cochlear implantation. In the same way, when implantation is performed concomitantly with tumor removal, CNAP study can provide information about the residual functionality of the cochlear nerve, assisting in the choice between cochlear and brainstem implant.…”

Section: Discussionsupporting

confidence: 88%

Hearing Rehabilitation in Neurofibromatosis Type 2 Patients: Cochlear versus Auditory Brainstem Implantation

Vincenti¹,

Pasanisi²,

Guida³

et al. 2008

Audiol Neurotol

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Objective: We aimed to evaluate and compare the auditory performance of neurofibromatosis type 2 (NF2) patients with bilateral total deafness fitted with cochlear or auditory brainstem implants. Patients and Methods: A retrospective case review was performed. Nine patients suffering from NF2 who underwent hearing rehabilitation by means of cochlear (4 patients) or auditory brainstem (5 patients) implantation participated in the study. Postoperative auditory performance was assessed using closed- and open-set tests. Results: In the group of patients fitted with a cochlear implant, 3 subjects achieved open-set speech recognition abilities comparable to those of standard adult postlingual implant patients; the remaining patient scored 0% in all open-set format tests, reporting benefits only in environmental sound detection and lip-reading. Among the 5 patients who underwent auditory brainstem implantation, 1 reached good open-set speech recognition skills, scoring 70% in the common phrases comprehension test, and she was able to communicate on the telephone. Two other patients achieved open-set speech understanding (respectively, 33 and 41% in the common phrases comprehension test), reporting daily use of their device. The remaining 2 patients did not achieve any level of open-set speech perception, obtaining only improved access to environmental sound and lip-reading skills. Conclusions: Our study confirmed literature data reporting that cochlear implantation may offer open-set speech communication in NF2 patients. In this small cohort, cochlear implant patients performed better than auditory brainstem implant patients, even if variability in auditory performance was observed with both devices. More studies are needed in order to clarify the role and reliability of electrophysiological tests in predicting the residual functionality of the cochlear nerve after tumor removal.

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Section: Discussionsupporting

confidence: 88%

Hearing Rehabilitation in Neurofibromatosis Type 2 Patients: Cochlear versus Auditory Brainstem Implantation

Vincenti¹,

Pasanisi²,

Guida³

et al. 2008

Audiol Neurotol

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“…It should therefore be repeated after some months when the postoperative swelling and scar formation is completed. Marangos et al [30] reported on 8 (from 16) NF-2 patients with a positive test result upon ECoG. Lambert et al [10] used EABR recordings in patients after AN removal.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation after Acoustic Tumour Resection in Neurofibromatosis Type 2: Impact of Intra- and Postoperative Neural Response Telemetry Monitoring

Nölle¹,

Todt²,

Basta³

et al. 2003

ORL

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The present paper reports about a 16-year-old male with neurofibromatosis type 2 (NF-2) of the Wishart type with bilateral deafness who had undergone cochlear implantation after resection of the acoustic neuroma (AN) of the same side. Neural response telemetry (NRT) recordings are essential in those patients during cochlear implantation where no stapedial reflexes can be electrically elicited due to the resection of the AN. In the present case, amplitude growth function and a type II pattern of the NRT waveforms could be well established. The comparison of the N₁ response intra-operatively and after 2 years showed a decline in latency by 50% and an increase in absolute amplitude by 10 times at the same current level of electrical stimulation. This improved auditory nerve transduction suggested a change to a ‘faster’ encoding strategy to improve speech understanding. The change from SPEAK to ACE 18 months after the operation led to an increase in the open-set sentence recognition test from 52 to 88%. Thus, NRT recordings monitor the intra-operative success of electrode placement and help to assess the integrity of the auditory pathway. Moreover, they can reliably be used in programming the speech processor postoperatively as objective tool. In patients with NF-2, the restoration of hearing can be successfully achieved in several ways. The indications for hearing implants (auditory brain stem and cochlear implants) should be carefully considered with respect to the remaining, functional integrity of the auditory nerve and the technical possibilities to monitor the success of these procedures.

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“…Neurofibromatosis type 2(NF2) [3] is an inherited disease. The main manifestation of this disease is the developmental of symmetric and nonmalignant brain tumor in the region of cranial nerve VIII which is the Auditory-Vestibular Nerve that transmit sensory information from the Inner ear to the brain.…”

mentioning

confidence: 99%

Cochlear Implant (CI) + Auditory Brainstem Implant (ABI) For Neurofibromatosis Type 2(NF2) Patients

Bina¹,

Hourizadeh²

2015

JOENTR

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A cochlear implant (CI) is a surgically implanted electronic device that provides a sense of sound to a person who is profoundly deaf or severely hard of hearing. Cochlear implants may help provide hearing in patients who are deaf because of damage to sensory hair cells in their cochlea. In those patients, the implants often can enable sufficient hearing for better understanding of speech. The quality of sound is different from natural hearing, with less sound information being received and processed by the brain. However, many patients are able to hear and understand speech and environmental sounds. Newer devices and processingstrategies allow recipients to hear better in noise, enjoy music, and even use their implant processors while swimming.

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Topodiagnosis of deafness: strategy for treatment of neurofibromatosis type 2

Cited by 5 publications

References 10 publications

Hearing Rehabilitation in Neurofibromatosis Type 2 Patients: Cochlear versus Auditory Brainstem Implantation

Hearing Rehabilitation in Neurofibromatosis Type 2 Patients: Cochlear versus Auditory Brainstem Implantation

Cochlear Implantation after Acoustic Tumour Resection in Neurofibromatosis Type 2: Impact of Intra- and Postoperative Neural Response Telemetry Monitoring

Cochlear Implant (CI) + Auditory Brainstem Implant (ABI) For Neurofibromatosis Type 2(NF2) Patients

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