Modest Mara C, Carlson Matthew L, Wanna George B, Driscoll Colin L
*Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota; and †Vanderbilt University, Nashville, Tennessee, U.S.A.
Otol Neurotol. 2015 Aug;36(7):1191-6. doi: 10.1097/MAO.0000000000000792.
To date, there have been less than 30 cases of cochlear implantation (CI) in patients with superficial siderosis (SS) reported in the literature. The primary objective of the current study is to evaluate CI outcomes in six additional patients (seven ears) with SS and sensorineural hearing loss (SNHL) and to perform a systematic review of the literature.
Case series and systematic review of the literature.
Two tertiary academic CI centers.
All patients with SS who underwent CI between 2007 and 2014.
INTERVENTION(S): Cochlear implantation.
Pre- and post-implantation speech perception scores and durability of benefit.
A total of seven ears (four males; median age 52 yr) with SS and SNHL met inclusion criteria. All patients developed progressive bilateral SNHL that was no longer amenable to conventional hearing aids. Additional presenting symptoms included vestibulopathy (n = 4), cerebellar ataxia (n = 3), mild dementia (n = 1), and myelopathy (n = 1). All patients underwent uncomplicated CI, and intraoperative device telemetry revealed normal responses in all electrodes. The median postoperative auditory threshold average was 32.5 dB HL (range 16-36 dB) and the median postoperative CNC word score was 51% (range 46-64%). The median duration of follow-up was 15.5 months (range 3-64 mo). All patients demonstrated initial improvement in speech perception testing. Two patients had performance decline and worsening dementia resulting from progressive SS.
Cochlear implantation is a viable strategy for auditory rehabilitation in patients with SS and associated SNHL. Most individuals enjoy benefit from CI; however, patients should be counseled regarding the risks of performance decline with progressive SS.
迄今为止，文献报道的患有表层铁沉积症（SS）的患者接受人工耳蜗植入（CI）的病例不足30例。本研究的主要目的是评估另外6例（7耳）患有SS和感音神经性听力损失（SNHL）的患者的CI效果，并对文献进行系统回顾。
病例系列研究及文献系统回顾。
两个三级学术性CI中心。
2007年至2014年间接受CI的所有SS患者。
人工耳蜗植入。
植入前后的言语感知评分及获益的持续性。
共有7耳（4名男性；中位年龄52岁）患有SS和SNHL的患者符合纳入标准。所有患者均出现进行性双侧SNHL，常规助听器已无法改善听力。其他出现的症状包括前庭病（n = 4）、小脑共济失调（n = 3）、轻度痴呆（n = 1）和脊髓病（n = 1）。所有患者均顺利接受CI，术中设备遥测显示所有电极反应正常。术后中位听阈平均值为32.5 dB HL（范围16 - 36 dB），术后中位CNC单词得分率为51%（范围46 - 64%）。中位随访时间为15.5个月（范围3 - 64个月）。所有患者在言语感知测试中均有初始改善。2例患者因进行性SS导致表现下降和痴呆加重。
人工耳蜗植入是患有SS及相关SNHL患者听觉康复的可行策略。大多数患者能从CI中获益；然而，应向患者告知进行性SS导致表现下降的风险。
