Department of Otolaryngology - Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas.
Otol Neurotol. 2019 Apr;40(4):e381-e385. doi: 10.1097/MAO.0000000000002165.
To describe cochlear implantation (CI) outcomes in patients with neurofibromatosis type 2 (NF2).
Retrospective case series.
A multidisciplinary NF2 clinic at a university hospital.
PATIENTS/INTERVENTIONS: Patients with NF2 who underwent CI.
Pre- and postimplantation audiometric data, including pure-tone average (PTA) and AzBio Sentence scores.
Eight patients with NF2 underwent CI. The mean age at implantation was 28.6 years (range: 17-63 yr) and six were female. The average length of deafness before CI was 3.5 years (range: 0.3-10 yr). Two patients underwent previous microsurgical resection via middle fossa craniotomy and one patient was treated with stereotactic radiotherapy before CI. Two tumors were growing at the time of CI, five tumors were not growing for an average period of 3.8 years (range: 1-6 yr), and one tumor had undergone previous gross total resection. Mean preoperative unaided PTA was 103.1 dB (range: 81.3-115 dB) and all preoperative AzBio scores were 0% in the ear to be implanted. These improved to a mean postoperative PTA of 30.9 dB (range: 12.5-43.8 dB) and mean postoperative AzBio score of 20% (range: 0-82%) with an average follow-up length of 16.6 months (range: 1.4-27.6 mo). Data logging demonstrated that six patients were daily CI users and two were nonusers, one of whom had normal hearing in the contralateral ear.
CI is an effective option for rehabilitating hearing loss in patients with NF2; however, patients with normal contralateral hearing or poor follow-up do not perform as well.
描述神经纤维瘤病 2 型（NF2）患者的耳蜗植入（CI）结果。
回顾性病例系列。
大学医院的多学科 NF2 诊所。
患者/干预措施：接受 NF2 并接受 CI 的患者。
植入前和植入后的听力数据，包括纯音平均（PTA）和 AzBio 句子得分。
8 例 NF2 患者接受了 CI。植入时的平均年龄为 28.6 岁（范围：17-63 岁），其中 6 例为女性。植入前耳聋的平均时间为 3.5 年（范围：0.3-10 年）。2 例患者接受过经中颅窝颅底切开术的先前微创手术切除，1 例患者在 CI 前接受过立体定向放射治疗。CI 时，有 2 个肿瘤在生长，5 个肿瘤在平均 3.8 年（范围：1-6 年）内未生长，1 个肿瘤已接受过大体全切除。术前未辅助 PTA 的平均为 103.1dB（范围：81.3-115dB），所有待植入耳的术前 AzBio 评分均为 0%。这些改善到术后平均 PTA 为 30.9dB（范围：12.5-43.8dB）和术后平均 AzBio 评分为 20%（范围：0-82%），平均随访时间为 16.6 个月（范围：1.4-27.6 个月）。数据记录表明，6 例患者为每日 CI 用户，2 例为非用户，其中 1 例对侧耳听力正常。
CI 是 NF2 患者听力损失康复的有效选择；然而，对侧听力正常或随访不佳的患者表现不佳。
