A Case of Neurofibromatosis Type 2 in Whom the Tumor was Preserved During a Cochlear Implant Surgery

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  • 腫瘍を温存したまま人工内耳手術を施行した神経線維腫症II型例
  • シュヨウ オ オンゾン シタ ママ ジンコウ ナイジ シュジュツ オ シコウ シタ シンケイ センイ シュショウ Ⅱ カタレイ

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Abstract

<p>Neurofibromatosis type 2 (NF2) is a rare autosomal-dominant hereditary disease characterized by the presence of bilateral acoustic tumors. Some patients with NF2 suffer from bilateral profound hearing loss. An auditory brainstem implant (ABI) directly stimulating the cochlear nucleus is a traditional option for hearing rehabilitation in NF2 patients with profound hearing loss. Recently, cases of NF2 in whom hearing was successfully restored by cochlear implants (CIs) have been reported.</p><p>We report the case of a 61-year-old female patient with NF2, who presented with bilateral hearing loss, in whom excellent hearing function was successfully restored by CI surgery. When the patient was 34 years old, she underwent resection of an acoustic tumor on the right side, which resulted in complete hearing loss on the right side. At the age of 55, an acoustic tumor was detected on the left side, however, since it was small, it was managed conservatively and showed no increase in size during the course of follow-up. Six years later, while the tumor size on the left side remained stable, the hearing loss on the same side had progressed to become severe. As a promontory stimulation test showed remaining neural function in the cochlear nerve, we suspected that the cochlear nerve function may be intact. Therefore, CI surgery was performed on the left side, with excellent recovery of the hearing function for daily communications; the hearing ability remains intact at present, 2 years since the CI surgery. The promontory stimulation test may be useful for predicting the effectiveness of CIs in NF2 patients with bilateral profound hearing loss. CIs may be a useful for some cases of NF2 with bilateral profound hearing loss.</p>

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