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Review
. 2016 Jul;57(4):817-23.
doi: 10.3349/ymj.2016.57.4.817.

Hearing Restoration in Neurofibromatosis Type II Patients

Jeon Mi Lee  1 , Jin Woo Chang  2 , Jae Young Choi  1 , Won Seok Chang  3 , In Seok Moon  4
Affiliations
Review

Hearing Restoration in Neurofibromatosis Type II Patients

Jeon Mi Lee et al. Yonsei Med J. 2016 Jul.

Abstract

Patients with neurofibromatosis type II will eventually succumb to bilateral deafness. For patients with hearing loss, modern medical science technology can provide efficient hearing restoration through a number of various methods. In this article, several hearing restoration methods for patients with neurofibromatosis type II are introduced.

Keywords: Neurofibromatosis type II; auditory brain stem implantation; cochlear implantation; hearing aids; vestibular schwannoma.

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Conflict of interest statement

The authors have no financial conflicts of interest.

Figures

Fig. 1
Fig. 1. Mechanisms of a bone-conduction hearing implant. Vibrations generated from the device are transferred to the contralateral cochlea and recognized on the contralateral side.
Fig. 2
Fig. 2. Categorization of bone-conduction hearing implants. (A) Ponto®. (B) Baha® BP100. (C) Bonebridge™. (D) Softband Baha®. (E) Baha® Attract. (F) Sophono®. Photos provided courtesy of Oticon Medical (A), Cochlear Bone Anchored Solutions AB (B, D, and E), MED-EL (C), and Medtronic (F).
Fig. 3
Fig. 3. Concurrent tumor removal and auditory brainstem implant via the translabyrinthine approach. (A) Implantable internal device. (B) Diagram of auditory brainstem implant via translabyrinthine approach. The tumor, which originated from the cochleovestibular nerve, was resected with the nerve. A flat electrode was inserted, which stimulated the dorsal cochlear nucleus. Photos provided courtesy of Cochlear Headquarters (A). The figure was created by Dong-Su Jang, medical illustrator (B).
Fig. 4
Fig. 4. Comparison of the retrosigmoid approach and the translabyrinthine approach for tumor removal. (A) Severe cerebellar retraction is needed to expose the tumor; however, tumors in the internal auditory canal are not readily removable via the retrosigmoid approach. (B) In the translabyrinthine approach, tumors in the internal auditory canal are well exposed without cerebellar retraction. Arrows indicate the direction of visual field.
See this image and copyright information in PMC

References

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