J Dev Behav Pediatr. 2018 Feb/Mar;39(2):177-179. doi: 10.1097/DBP.0000000000000547.
Sonia is a 4 years 1 month-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 years 7 months years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate. She could follow 1-step directions in English but had more difficulty after 2-step directions.Sonia was born in Puerto Rico at 40 weeks gestation after an uncomplicated pregnancy. She failed her newborn hearing test and was given hearing aids that did not seem to help.At age 2 years, Sonia, her mother, and younger sister moved to the United States where she was diagnosed with bilateral severe-to-profound hearing loss. Genetic testing led to a diagnosis of Waardenburg syndrome (group of genetic conditions that can cause hearing loss and changes in coloring [pigmentation] of the hair, skin, and eyes). She received bilateral cochlear implants 6 months later.Sonia's mother is primarily Spanish-speaking and mostly communicates with her in Spanish or with gestures but has recently begun to learn American Sign Language (ASL). In a preschool program at a specialized school for the deaf, Sonia is learning both English and ASL. Sonia seems to prefer to use ASL to communicate.Sonia receives speech and language therapy (SLT) 3 times per week (90 minutes total) individually in school and once per week within a group. She is also receiving outpatient SLT once per week. Therapy sessions are completed in English, with the aid of an ASL interpreter. Sonia's language scores remain low, with her receptive skills in the first percentile, and her expressive skills in the fifth percentile.During her evaluation in Developmental and Behavioral Pediatrics, an ASL interpreter was present, and the examiner is a fluent Spanish speaker. Testing was completed through a combination of English, Spanish, and ASL. Sonia seemed to prefer ASL to communicate, although she used some English words with errors of pronunciation. On the Beery Visual-Motor Integration Test, she obtained a standard score of 95. Parent and teacher rating scales were not significant for symptoms of attention-deficit/hyperactivity disorder.What factors are contributing to her slow language acquisition and how would you modify her treatment plan?
索尼娅是一名 4 岁 1 个月大的女孩，患有瓦登伯格综合征和双侧感音神经性听力损失，在 2 岁 7 个月大时接受了双侧人工耳蜗植入。她因语言技能的发展不如预期而被言语/语言病理学家转介到发育行为儿科学，当时她使用大约 20 个手语和 1 个口语词（妈妈）进行交流。在评估时（植入后 18 个月），她已经掌握了大约 70 个英语和西班牙语单词，并使用无数的手语进行交流。她可以用英语完成 1 步指令，但在 2 步指令后会遇到更多困难。索尼娅在波多黎各怀孕 40 周时足月出生，孕期无并发症。她在新生儿听力测试中未通过，并被给予助听器，但似乎没有帮助。2 岁时，索尼娅、她的母亲和妹妹搬到了美国，她被诊断出患有双侧重度至极重度听力损失。基因检测导致瓦登伯格综合征（一组可能导致听力损失和头发、皮肤和眼睛颜色[色素沉着]改变的遗传疾病）的诊断。6 个月后，她接受了双侧人工耳蜗植入。索尼娅的母亲主要说西班牙语，大多用西班牙语与她交流或打手势，但最近开始学习美国手语（ASL）。在一所聋人特殊学校的学前班，索尼娅正在学习英语和 ASL。索尼娅似乎更喜欢用 ASL 进行交流。索尼娅每周接受 3 次（每次 90 分钟）个人言语语言治疗（SLT），每周在一个小组中接受 1 次，每周还接受 1 次门诊 SLT。治疗课程全部用英语进行，有 ASL 翻译协助。索尼娅的语言分数仍然很低，接受能力在第一百分位，表达能力在第五百分位。在发育行为儿科学评估期间，有一名 ASL 翻译在场，检查者是一名流利的西班牙语使用者。测试是通过英语、西班牙语和 ASL 的组合完成的。索尼娅似乎更喜欢用 ASL 进行交流，尽管她在使用英语单词时发音有误。在比耶视觉运动整合测试中，她的标准得分为 95。父母和老师的评分量表对注意力缺陷/多动障碍的症状没有显著意义。是什么因素导致她语言习得缓慢，你将如何修改她的治疗计划？
