A Case of Vestibular Schwannoma with Deafness Showing Remarkable Hearing Recovery Following Hearing-Preserving Surgery.

Yusuke Sakaki, Makoto Hosoya, Takanori Nishiyama, Takeshi Wakabayashi, Marie N Shimanuki, Hiroyuki Ozawa, Naoki Oishi
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Abstract

Surgery for vestibular schwannoma can be divided into hearing-preserving and nonhearing-preserving surgeries. Hearing-preserving surgery is usually not considered in patients with deafness due to vestibular schwannoma, because hearing is unlikely to improve, and surgery aims to maximize the tumor resection at the expense of hearing. We report an extremely rare case of a 46-year-old man with unilateral profound hearing loss due to a vestibular schwannoma with marked cystic degeneration in the left cistern, which significantly recovered to near-normal hearing levels after hearing-preserving surgery. Hearing loss gradually worsened, and preoperative pure-tone evaluation showed complete hearing loss in the left ear. However, the response to the distortion product otoacoustic emission was preserved, and hearing loss was considered to be retrocochlear. Tumor resection was performed using the retrolabyrinthine approach with continuous monitoring using dorsal cochlear nucleus action potential, auditory brainstem response, and facial nerve function muscle action potential. The cistern portion of the tumor was almost completely resected along with the wall. Postoperatively, the pure-tone threshold on the left side markedly improved. The present case clearly demonstrates the possibility of hearing recovery in patients with retrocochlear hearing loss. We should consider expanding the indications for hearing-preserving surgery.

一例前庭神经鞘瘤伴耳聋患者在保听手术后表现出显著的听力恢复。
前庭神经鞘瘤的手术可分为听力保留手术和非听力保留手术。前庭神经鞘瘤耳聋患者通常不考虑听力保留手术,因为听力不太可能改善,而手术旨在以牺牲听力为代价最大限度地切除肿瘤。我们报告了一例极为罕见的病例,一名46岁的男子因前庭神经鞘瘤导致单侧深度听力损失,左池有明显的囊性变性,在听力保留手术后,该患者的听力水平显著恢复到接近正常水平。听力损失逐渐恶化,术前纯音评估显示左耳完全听力损失。然而,对畸变产物耳声发射的反应得以保留,听力损失被认为是耳蜗后的。肿瘤切除采用迷路后入路,并通过耳蜗背侧核动作电位、听性脑干反应和面神经功能肌肉动作电位进行连续监测。肿瘤的池部几乎与壁一起被完全切除。术后左侧纯音阈值明显改善。本病例清楚地证明了耳蜗后听力损失患者听力恢复的可能性。我们应该考虑扩大听力保留手术的适应症。
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