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Acta Otolaryngol. 2009 Sep;129(9):971-5. doi: 10.1080/00016480802510202.

Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2.

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  • 1Department of Otorhinolaryngology-Head and Neck Surgery, AP-HP, Hôpital Lariboisière, Service ORL, Université Paris 7, Paris, France.



New technological developments will most probably improve the efficiency of auditory brainstem implantation (ABI). Meanwhile, cochlear implantation in patients who have undergone prior reductive surgery, and who have maintained a positive electric stimulation, is an excellent alternative for rehabilitating complete and bilateral hearing loss in patients with neurofibromatosis type 2 (NF2). Auditory results are far better than those reported after ABI. Long-term follow-up will be necessary to demonstrate the validity of this strategy.


ABIs restore some degree of auditory perception in NF2 patients with bilateral and complete hearing loss, but results are often inadequate for maintaining social and professional activities. The aim of this study was to report the results of auditory rehabilitation by cochlear implantation in three cases of NF2.


This was a retrospective study undertaken in a tertiary referral center. The first patient had undergone previous surgery for a left grade III vestibular schwannoma (VS) and then underwent irradiation for a right grade I VS. Two years after irradiation, he suddenly lost his remaining hearing. Electric promontory stimulation was positive and cochlear implantation was performed. The second patient had undergone surgery for a left grade III VS and followed for a right grade II VS. She suddenly lost her remaining hearing. A cytoreductive surgery was performed and the cochlear nerve was preserved. Postoperative electric stimulation was positive. She was then implanted with a cochlear implant. The third patient presented with a right stage III and a left stage I VS. She first underwent a subtotal removal of the left VS with immediate cochlear implantation. She then underwent removal of the right VS stage III with no possible preservation of the cochlear nerve.


All three patients had excellent postoperative speech performance and were back to work 3 months after implantation. Imaging follow-ups at 4, 2, and 1 year, respectively, do not show any evolution of the tumor.

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