Avgeri Caroline, Sideris Giorgos, Moriki Dafni, Douros Konstantinos, Delides Alexander, Nikolopoulos Thomas
National & Kapodistrian University of Athens School of Medicine, 2nd Otolaryngology Department, "Attikon" University Hospital, Athens, Greece.
National & Kapodistrian University of Athens School of Medicine, 3rd Department of Pediatrics, Pediatric Allergy and Respiratory Unit, "Attikon" University Hospital, Athens, Greece.
J Int Adv Otol. 2025 Mar 25;21(2):1-4. doi: 10.5152/iao.2025.241872.
Primary ciliary dyskinesia (PCD) is a rare genetic disorder that affects the respiratory and auditory systems. This study aims to assess the prevalence, type, and severity of bilateral hearing loss (HL) in PCD and Kartagener syndrome (KS) patients, examining age-related differences and chronic impacts of otologic pathologies.
A total of 19 patients (38 ears), including 6 children and 13 adults, were evaluated from June to September 2021. Comprehensive clinical examinations included otoscopy, tympanometry, and pure tone audiometry (PTA) for air and bone conduction. Tympanometry findings were compared with otoscopic results. Statistical analyses were conducted using SPSS v16.0 (SPSS Inc.; Chicago, IL, USA), with a significance threshold of P ≤ .05.
Hearing loss was identified in 42.1% of patients, with conductive HL predominant in children (3 out of 4), while mixed HL was more common in adults (3 out of 4). Tympanometry results showed 57.9% type A and 42.1% type B findings, correlating with otoscopic observations. Chronic otitis media with effusion (OME) and tympanosclerosis (TS) were the primary pathologies contributing to middle ear damage. Age was significantly correlated with HL severity (P= .005). Mild HL was most common (62.5%), followed by moderate HL (25%) and moderately severe HL (12.5%).
This study identifies distinct age-related patterns in the type and severity of HL among PCD patients, with sensorineural components observed in adults due to progressive middle ear damage. Audiological evaluations are essential for identifying these complications. Further research is needed to optimize treatment approaches and understand the progression of HL in PCD/KS patients.
原发性纤毛运动障碍（PCD）是一种影响呼吸和听觉系统的罕见遗传性疾病。本研究旨在评估PCD和卡塔格内综合征（KS）患者双侧听力损失（HL）的患病率、类型和严重程度，研究年龄相关差异以及耳科病理的慢性影响。
2021年6月至9月对总共19例患者（38耳）进行了评估，其中包括6名儿童和13名成人。全面的临床检查包括耳镜检查、鼓室导抗图检查以及气导和骨导纯音听力测定（PTA）。将鼓室导抗图检查结果与耳镜检查结果进行比较。使用SPSS v16.0（SPSS公司；美国伊利诺伊州芝加哥）进行统计分析，显著性阈值为P≤0.05。
42.1%的患者存在听力损失，儿童中以传导性HL为主（4例中有3例），而混合性HL在成人中更为常见（4例中有3例）。鼓室导抗图检查结果显示，A型为57.9%，B型为42.1%，与耳镜检查结果相关。伴有积液的慢性中耳炎（OME）和鼓室硬化（TS）是导致中耳损伤的主要病理情况。年龄与HL严重程度显著相关（P = 0.005）。轻度HL最为常见（62.5%），其次是中度HL（25%）和中度重度HL（12.5%）。
本研究确定了PCD患者HL类型和严重程度中与年龄相关的不同模式，由于中耳渐进性损伤，在成人中观察到感音神经性成分。听力评估对于识别这些并发症至关重要。需要进一步研究以优化治疗方法并了解PCD/KS患者HL的进展情况。
