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

CONCLUSIONS: 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. OBJECTIVES: 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. PATIENTS AND METHODS: 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. RESULTS: 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.

[Vestibular schwannoma (acoustic neurinoma). Natural history and quality of life]. / Schwannome vestibulaire (neurinome de l'acoustique). Histoire naturelle et qualité de vie.

Management of patients with vestibular shwannomas has gradually improved with the development of stereotaxic radiotherapy, our better understanding of these tumours' natural history, and the increasing accent placed on quality of life. The aim of this study was two-fold:--to evaluate the natural history and tumor growth in a series of 386 patients presenting with small- or medium-sized tumors amenable to conservative management; and ii) to compare quality of life in 356 patients undergoing surgery (n = 198), radiosurgery (n = 23) or conservative management (n = 135). The annual tumor growth rate was < 1 mm in 58.6% of patients, 1-3 mm in 29.2%, and > 3 mm in 12.2%. The overall growth rate did not differ significantly between intrameatal and extrameatal tumors (1.02 +/- 1.8 and 1.40 +/- 3.1 mm/y, respectively). The tumor growth rate was not related to sex, age, initial hearing status or initial tumor grade. Late diagnosis was the only factor significantly associated with the tumor growth rate. All three treatment modalities had a negative impact on QOL, but surgery led to a significant deterioration. This study supports a conservative "wait-and-scan" policy for patients with small tumours, most of which are slow-growing. Long-term radiological controls are needed even for non growing tumours. Surgery significantly degrades quality of life.