Department of Pediatrics, Hematology-Oncology Unit, Ain Shams University, Cairo, Egypt.
Department of Radiodiagnosis, Ain Shams University, Cairo, Egypt.
Hemoglobin. 2020 Nov;44(6):411-417. doi: 10.1080/03630269.2020.1837863. Epub 2020 Nov 8.
Hearing impairment is a reported complication of sickle cell disease, yet inner ear pathology is not fully understood. The study purpose was to examine the patterns of inner ear involvement in patients with sickle cell disease by magnetic resonance imaging (MRI) and to assess its association with auditory functions. A cross-sectional study included 22 children with sickle cell disease examined for inner ear pathology by audiogram, MRI inner ear and transcranial Doppler (TCD) with revision of their hospital records for transfusion, chelation and hydroxyurea (HU) therapy. Abnormal MRI in the form of intrinsic T1 hyperintensity within the lumen of inner ear structures and cochlear neuropathy was found in five (22.7%) patients; left middle cerebral artery (MCA) flow velocity was higher in patients with abnormal MRI (83.4 ± 5.3 cm/sec) compared to normal MRI (68.2 ± 11.1 cm/sec) ( = 0.015), however, none of the patients had TCD of >170 cm/sec. There was no significant difference between patients with normal and abnormal MRI as regards hearing level and speech audiometry. Sensorineural hearing loss (SNHL) was present in two (9.1%) and conductive hearing loss (CHL) in two (9.1%) patients. There was a significant negative correlation between right ear mean hearing level and right MCA flow velocity and significant negative correlation between left ear mean hearing level and basilar artery (BA) flow velocity. We concluded that inner ear pathology is not uncommon in asymptomatic patients with sickle cell anemia, yet it did not correlate with hearing impairment and may occur with normal TCD results.
听力损伤是镰状细胞病的一种报道并发症，但内耳病理学尚未完全了解。本研究的目的是通过磁共振成像（MRI）检查镰状细胞病患者的内耳受累模式，并评估其与听觉功能的关系。一项横断面研究纳入了 22 例镰状细胞病患儿，通过听力图、MRI 内耳和经颅多普勒（TCD）检查评估内耳病理学，并查阅其病历以评估输血、螯合和羟基脲（HU）治疗情况。5 例（22.7%）患儿存在内耳结构管腔内固有 T1 高信号的异常 MRI，表现为耳蜗神经病变；异常 MRI 患儿的左侧大脑中动脉（MCA）血流速度高于正常 MRI 患儿（83.4 ± 5.3 cm/sec 比 68.2 ± 11.1 cm/sec）（ = 0.015），但无一例 TCD ＞170 cm/sec。MRI 正常和异常患儿的听力水平和言语测听均无显著差异。2 例（9.1%）患儿存在感音神经性听力损失（SNHL），2 例（9.1%）患儿存在传导性听力损失（CHL）。右耳平均听力水平与右侧 MCA 血流速度呈显著负相关，左耳平均听力水平与基底动脉（BA）血流速度呈显著负相关。我们得出结论，无症状镰状细胞贫血患者的内耳病理学并不罕见，但与听力损伤无关，并且可能在 TCD 结果正常的情况下发生。
