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Bilateral Cochlear Implants: Maximizing Expected Outcomes

Wallis, Kate, E., MD, MPH; Blum, Nathan, J., MD; Waryasz, Stephanie, A., Au D; Augustyn, Marilyn, MD

Journal of Developmental & Behavioral Pediatrics: February/March 2018 - Volume 39 - Issue 2 - p 177–179
doi: 10.1097/DBP.0000000000000547
Challenging Case

CASE: 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?

Disclosure: The authors declare no conflict of interest.

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