年龄≤60岁复发性多软骨炎患者的全耳受累情况分析

Whole ear involvement in non-elder patients with relapsing polychondritis

目的探讨年龄≤60岁复发性多软骨炎(RP)患者全耳受累的临床特征。方法连续录入符合Michet诊断标准且年龄≤60岁的RP患者,分组研究耳各部位损害的临床特点、听力学特点及病情评价。满足正态分布计量资料的2组间比较采用t检验;不满足正态分布计量资料采用Mann-Whitney U检验;计数资料采用χ^2检验。结果2007年12月1日至2017年8月31日期间共174例耳部资料齐全且年龄≤60岁的RP患者入选,男性90例,女性84例。发病年龄8~60岁,平均(39±13)岁;病程1~480个月,中位病程12个月;RP疾病活动指数10~77,中位数为38,各发病类型之间相近;RP器官损伤指数0.1~56,中位数为2.4,各发病类型之间差异较大:前3位的发病类型为耳廓软骨炎32.8%(57/174)、眼睛受累24.7%(43/174)及气道软骨炎21.3%(37/174);耳的各个部分(外中内)均可受累:内耳受累最多见95.4%(166/174),耳蜗及前庭受累概率相近,隐匿性突出;外耳受累55.2%(96/174)以耳廓为主;中耳受累29.9%(52/174)以鼓膜及咽鼓管受累为主,常被忽略;听力损失的主动检出率高达71.8%(125/174);符合WHO-2006听力损失者为14.3%(25/174),其中52.0%(13/25)为残疾性耳聋;儿童RP的非感音神经性耳聋占40.0%(4/10);21岁以后RP以感音神经性耳聋为主;所有患者均具有外中内耳2个部位以上的异常,耳各部位受累交叉重叠现象明显。结论在年龄≤60岁RP人群可见耳各个部分的受累,并具有年龄特点;中内耳受累表现隐匿;主动检查可以早期检出RP患者的听力损失;RP器官损伤指数的潜在应用价值值得进一步研究。

Objective To investigate the clinical and audiological characteristics of non-elder patients with relapsing polychondritis (RP).Methods Clinical and audiological data of patients with RP under 60 years old were collected consecutively and analyzed. The t-test and Mann-Whitney U test were used for statistical analysis between the two groups in quantitative data in normal distribution and non-normal distribution respectively, while Chi-square test was use for qualitative data analysis.Results One hundred and seventy four patients with complete data who fulfilled the Michet criteria were enrolled with a M∶F=1∶1.1. The mean age of disease onset was (39±13) (8-60) years;the median time of disease duration was 12(1-480) months;the median relapsing polychondritis disease activity index (RPDAI) was 38(10-77) and the median RPODI was 2.4(0.1-56). Auricular chondritis (32.8%, 57/174), ocular involvement (24.7%, 43/174) and airway chondritis (21.3%, 37/174) were the top three onset-pattern. All parts of external, middle and inner ear were involved in RP. Inner-ear damage was the most common (95.4%, 166/174) with insidious cochlea and vestibule equally distributed. Auricular chondritis was predominant in external ear involvement (55.2%, 96/174);ET dysfunction was included in eardrum abnormalities of neglected middle-ear involvement (29.9%, 52/174). Positive HL by active detection was 71.8%(125/174) with 14.3(25/174) HL fulfilled world health organization (WHO)-2006 criteria, including 52.0%(13/25) disabling HL. Sensorineural hearing loss (SNHL) was predominant in RP after 21 years old while 60.0%(6/10) was seen in childhood RP with non-SNHL. At least two parts of ear involvement were seen in almost all patients with heavy overlap.ConclusionAll parts of ear are involved in non-elder RP with age related clinical characteristics. Active detection is a key to find insidious middle-and inner-ear involvement for early RP recognition. RPODI is a potential marker for RP evaluation.