Forli Francesca, Capobianco Silvia, Berrettini Stefano, Lazzerini Francesco, Malesci Rita, Fetoni Anna Rita, Salomè Serena, Brotto Davide, Trevisi Patrizia, Franz Leonardo, Genovese Elisabetta, Ciorba Andrea, Palma Silvia
Otolaryngology, Audiology, and Phoniatrics Unit, University of Pisa, 56124 Pisa, Italy.
Hearing Implant Section, Karolinska Institutet, 14186 Stockholm, Sweden.
Children (Basel). 2025 Jul 10;12(7):908. doi: 10.3390/children12070908.
: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL) in children. In cases of severe-to-profound SNHL, cochlear implantation (CI) is a widely used intervention, but outcomes remain variable due to possible neurodevelopmental comorbidities. This study aimed to evaluate the long-term auditory and language outcomes in children with cCMV after CI and to explore clinical and radiological predictors of post-CI performance. : Fifty-three children with cCMV and bilateral severe-to-profound SNHL who underwent CI at five tertiary referral centers in Italy were included in the study. Auditory and language outcomes were assessed pre- and post-implantation using the Categories of Auditory Performance II (CAP-II) scale, the Nottingham 3-Level Classification, and the Bates Language Development Scale. Brain MRI abnormalities were classified according to the Alarcón classification. Correlations were explored between outcome scores and symptomatic status at birth, MRI findings, and neurodevelopmental comorbidities. : At birth, 40 children (75.5%) were symptomatic and 13 (24.5%) asymptomatic. Neurodevelopmental comorbidities were present in 19 children (35.8%). MRI was normal in 15 (28.3%), mildly abnormal in 26 (49%), and moderately to severely abnormal in 12 (22.6%). Auditory and language outcomes improved significantly post-CI ( < 0.001), though the outcomes varied widely. Twenty-five children (47%) reached CAP level ≥ 6, and thirteen (23%) reached Bates Level 6. Symptomatic status at birth correlated weakly with worse CAP (ρ = -0.291, = 0.038) and Bates (ρ = -0.310, = 0.028) scores. Higher Alarcón scores were significantly associated with neurodevelopmental comorbidities, though not directly with post-CI auditory and language outcomes. Finally, the presence of neurodevelopmental disabilities was generally associated with lower results, even if without statistical significance. : CI provides substantial auditory and language benefit in children with cCMV, even in cases of severe neurodevelopmental comorbidities. MRI and developmental assessments, as well as perinatal history for clinical signs and symptoms, are helpful in guiding expectations and personalizing post-implantation support.
先天性巨细胞病毒（cCMV）感染是儿童感音神经性听力损失（SNHL）最常见的非遗传病因。在重度至极重度SNHL病例中，人工耳蜗植入（CI）是一种广泛应用的干预措施，但由于可能存在神经发育合并症，其效果仍存在差异。本研究旨在评估CI术后cCMV感染儿童的长期听觉和语言效果，并探索CI术后表现的临床和影像学预测因素。
本研究纳入了意大利五个三级转诊中心53例患有cCMV且双侧重度至极重度SNHL并接受CI的儿童。使用听觉表现类别II（CAP-II）量表、诺丁汉3级分类法和贝茨语言发展量表在植入前后评估听觉和语言效果。脑MRI异常根据阿拉孔分类法进行分类。探讨了结果评分与出生时症状状态、MRI结果和神经发育合并症之间的相关性。
出生时，40例儿童（75.5%）有症状，13例（24.5%）无症状。19例儿童（35.8%）存在神经发育合并症。15例（28.3%）MRI正常；26例（49%）轻度异常；12例（22.6%）中度至重度异常。CI术后听觉和语言效果显著改善（<0.001），尽管效果差异很大。25例儿童（47%）达到CAP水平≥6，13例（23%）达到贝茨水平6。出生时的症状状态与较差的CAP（ρ=-0.291，=0.038）和贝茨（ρ=-0.310，=0.028）评分弱相关。较高的阿拉孔评分与神经发育合并症显著相关，但与CI术后听觉和语言效果无直接关联。最后，神经发育障碍的存在通常与较低的结果相关，即使无统计学意义。
CI为cCMV感染儿童带来显著的听觉和语言益处，即使在存在严重神经发育合并症的情况下。MRI和发育评估，以及围产期临床症状和体征史，有助于指导预期并使植入后支持个性化。
