Seeliger Tabea, Bönig Lena, Witte Torsten, Thiele Thea, Lesinski-Schiedat Anke, Stangel Martin, Lenarz Thomas, Prenzler Nils Christian, Skripuletz Thomas
Department of Neurology, Hannover Medical School, Hannover, Germany.
Department of Clinical Immunology & Rheumatology, Hannover Medical School, Hannover, Germany.
Ann Transl Med. 2020 Sep;8(17):1069. doi: 10.21037/atm-20-1856.
Sjögren's syndrome is an immunologically mediated disease with salivary and lacrimal gland destruction characterised by typical sicca symptoms of dry mouth and eyes. Awareness of extraglandular neurological manifestations such as polyneuropathy and affection of cranial nerves is rising. Hearing loss as consequence of involvement of the vestibulocochlear nerve presents a severe disability. The exact prevalence and nature of hearing dysfunction in patients with Neuro-Sjögren has been insufficiently evaluated to date.
Thirty patients with Sjögren's syndrome (ACR-EULAR classification criteria) and polyneuropathy were included in the study in the time period between 11/2016 and 03/2018. The median age was 59 years and 57% were females. Auditory function was investigated by pure tone audiometry, Freiburg speech comprehension audiometry, transient evoked otoacoustic emissions and brainstem evoked response audiometry.
Pure tone audiometry revealed hearing loss in 10/30 patients (33%) with severity ranging from mild in most patients (60%) to severe in 10%. In addition, pathological audiometric test findings showed retrocochlear auditory dysfunction in 14 further patients. In total, 24/30 patients (80%) showed pathological test results on audiometric testing suggesting hearing dysfunction.
In conclusion, our results show that hearing dysfunction as a possible consequence of cranial neuropathy in patients with Neuro-Sjögren has been underestimated in previous studies.
干燥综合征是一种免疫介导的疾病，以唾液腺和泪腺破坏为特征，表现为典型的口干和眼干症状。人们对多神经病等腺外神经表现以及颅神经受累的认识正在提高。前庭蜗神经受累导致的听力损失会造成严重残疾。迄今为止，神经干燥综合征患者听力功能障碍的确切患病率和性质尚未得到充分评估。
2016年11月至2018年3月期间，30例符合美国风湿病学会（ACR）-欧洲抗风湿病联盟（EULAR）分类标准且患有多神经病的干燥综合征患者纳入本研究。中位年龄为59岁，57%为女性。通过纯音听力测定、弗莱堡言语理解听力测定、瞬态诱发耳声发射和脑干诱发反应听力测定来研究听觉功能。
纯音听力测定显示，30例患者中有10例（33%）存在听力损失，大多数患者（60%）为轻度，10%为重度。此外，听力测定检查结果异常显示，另有14例患者存在蜗后听觉功能障碍。总计，30例患者中有24例（80%）听力测定检查结果异常，提示存在听力功能障碍。
总之，我们的结果表明，先前的研究低估了神经干燥综合征患者因颅神经病可能导致的听力功能障碍。
