Comorbidity and Severity in Childhood Apraxia of Speech: A Retrospective Chart Review.

PURPOSE: The purpose of this study was to investigate comorbidity prevalence and patterns in childhood apraxia of speech (CAS) and their relationship to severity. METHOD: In this retroactive cross-sectional study, medical records for 375 children with CAS (M age = 4;9 [years;months], SD = 2;9) were examined for comorbid conditions. The total number of comorbid conditions and the number of communication-related comorbidities were regressed on CAS severity as rated by speech-language pathologists during diagnosis. The relationship between CAS severity and the presence of four common comorbid conditions was also examined using ordinal or multinomial regressions. RESULTS: Overall, 83 children were classified with mild CAS; 35, with moderate CAS; and 257, with severe CAS. Only one child had no comorbidities. The average number of comorbid conditions was 8.4 (SD = 3.4), and the average number of communication-related comorbidities was 5.6 (SD = 2.2). Over 95% of children had comorbid expressive language impairment. Children with comorbid intellectual disability (78.1%), receptive language impairment (72.5%), and nonspeech apraxia (37.3%; including limb, nonspeech oromotor, and oculomotor apraxia) were significantly more likely to have severe CAS than children without these comorbidities. However, children with comorbid autism spectrum disorder (33.6%) were no more likely to have severe CAS than children without autism. CONCLUSIONS: Comorbidity appears to be the rule, rather than the exception, for children with CAS. Comorbid intellectual disability, receptive language impairment, and nonspeech apraxia confer additional risk for more severe forms of CAS. Findings are limited by being from a convenience sample of participants but inform future models of comorbidity. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.22096622.

Language and Audiological Outcomes Among Infants Implanted Before 9 and 12 Months of Age Versus Older Children: A Continuum of Benefit Associated With Cochlear Implantation at Successively Younger Ages.

OBJECTIVE: To compare language and audiological outcomes among infants (<9 and <12 mo) and older children receiving cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients receiving CI between October 1995 and October 2019. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Most recent language and audiological assessment scores were evaluated by age group. RESULTS: A total of 118 children were studied, including 19 who were implanted <9 months of age, 19 implanted 9 to <12 months of age, and 80 implanted 12 to <36 months of age. The mean duration of follow-up was 7.4 ±â€Š5.0 years. Most recent REEL-3 receptive (88 ±â€Š12 vs. 73 ±â€Š15; p = 0.020) and expressive (95 ±â€Š13 vs. 79 ±â€Š12; p = 0.013) communication scores were significantly higher in the <9 months group compared to the 9 to <12 months group. PLS and OWLS auditory comprehension and oral expression scores were significantly higher in the <12 months group compared to the 12 to <36 months group. The difference in NU-CHIPS scores between <12 and 12 to <36 months was statistically significant (89% ±â€Š6 vs. 83% ±â€Š12; p = 0.009). LNT scores differed significantly between <9 and 9 to <12 months (94% ±â€Š4 vs. 86% ±â€Š10; p = 0.028). CONCLUSIONS: The recent FDA expansion of pediatric CI eligibility criteria to include infants as young as 9 months of age should not serve as a strict clinical cutoff. Rather, CI can be pursued in appropriately selected younger infants to optimize language and audiological outcomes.

Injection Pharyngoplasty With a Hyaluronic Acid and Dextranomer Copolymer to Treat Velopharyngeal Insufficiency in Adults.

OBJECTIVE: To describe the treatment of adult velopharyngeal insufficiency (VPI) with injection of a hyaluronic acid and dextranomer copolymer (Dx/HA). PATIENTS AND METHODS: This was a retrospective case series of 25 consecutively treated adults with VPI who underwent Dx/HA injection pharyngoplasty in a multidisciplinary clinic from January 1, 2011, to December 31, 2014. Data recorded included etiology of VPI, perceptual analysis of resonance, nasalance scores, and estimation of velopharyngeal gap characteristics on video nasendoscopy before and after the intervention. Statistical comparisons were made using a 2-tailed Wilcoxon signed rank test and the Kruskal-Wallis test. RESULTS: Patients had VPI due to a neurologic etiology, due to a benign anatomic etiology, or acquired after treatment for a head and neck malignancy. Injections were performed with local anesthesia, monitored anesthesia care, or general anesthesia. There were statistically significant improvements in speech resonance, nasalance, and velopharyngeal gap size after treatment. Patients with neurologic or benign anatomic etiologies of their VPI had more significant improvement than those with VPI after treatment of malignancy. Nineteen of the 25 patients required only 1 injection to achieve their final result. CONCLUSION: Injection pharyngoplasty with a readily available Dx/HA is an effective treatment for VPI that allows for titration to complete velopharyngeal closure under local anesthesia or light sedation. It is most effective in patients with nonmalignant etiologies of VPI and in those with good lateral wall motion. Complications experienced were postoperative neck pain and occult retropharyngeal fluid collection, highlighting the importance of follow-up.

Augmentation pharyngoplasty for treatment of velopharyngeal insufficiency in children: Results with injectable dextranomer and hyaluronic acid copolymer.

OBJECTIVES/HYPOTHESIS: To evaluate the effectiveness, durability, and safety of a tissue filler (dextranomer and hyaluronic acid copolymer) when injected submucosally in the nasopharynx to treat velopharyngeal insufficiency (VPI) in pediatric patients. STUDY DESIGN: Retrospective case series. METHODS: Charts were reviewed for all patients treated with injectable filler at a tertiary children's center for VPI from April 2010 through September 2013. Main outcome measures included perceptual speech assessments before and after the procedure, velopharyngeal gap size on nasendoscopy, and nasalance. Length of follow-up, length of surgery, hospital admission, and complications were also identified. RESULTS: After injection augmentation pharyngoplasty, 93% of patients had no or mild hypernasality, 83% had resolution of nasal grimace, and 96% had resolution of audible nasal emission. Complications were minor and included mucosal infection, neck pain, snoring, and obstructive sleep apnea. CONCLUSIONS: Use of injectable filler to augment the nasopharynx is an effective, minimally invasive therapy for small or moderate-sized velopharyngeal gaps, with shorter procedural times, easier recovery, and acceptable durability. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:S5-S13, 2016.

A motor speech assessment for children with severe speech disorders: reliability and validity evidence.

PURPOSE: In this article, the authors report reliability and validity evidence for the Dynamic Evaluation of Motor Speech Skill (DEMSS), a new test that uses dynamic assessment to aid in the differential diagnosis of childhood apraxia of speech (CAS). METHOD: Participants were 81 children between 36 and 79 months of age who were referred to the Mayo Clinic for diagnosis of speech sound disorders. Children were given the DEMSS and a standard speech and language test battery as part of routine evaluations. Subsequently, intrajudge, interjudge, and test-retest reliability were evaluated for a subset of participants. Construct validity was explored for all 81 participants through the use of agglomerative cluster analysis, sensitivity measures, and likelihood ratios. RESULTS: The mean percentage of agreement for 171 judgments was 89% for test-retest reliability, 89% for intrajudge reliability, and 91% for interjudge reliability. Agglomerative hierarchical cluster analysis showed that total DEMSS scores largely differentiated clusters of children with CAS vs. mild CAS vs. other speech disorders. Positive and negative likelihood ratios and measures of sensitivity and specificity suggested that the DEMSS does not overdiagnose CAS but sometimes fails to identify children with CAS. CONCLUSIONS: The value of the DEMSS in differential diagnosis of severe speech impairments was supported on the basis of evidence of reliability and validity.

Childhood Apraxia of Speech (CAS) in two patients with 16p11.2 microdeletion syndrome.

We report clinical findings that extend the phenotype of the ~550 kb 16p11.2 microdeletion syndrome to include a rare, severe, and persistent pediatric speech sound disorder termed Childhood Apraxia of Speech (CAS). CAS is the speech disorder identified in a multigenerational pedigree ('KE') in which half of the members have a mutation in FOXP2 that co-segregates with CAS, oromotor apraxia, and low scores on a nonword repetition task. Each of the two patients in the current report completed a 2-h assessment protocol that provided information on their cognitive, language, speech, oral mechanism, motor, and developmental histories and performance. Their histories and standard scores on perceptual and acoustic speech tasks met clinical and research criteria for CAS. Array comparative genomic hybridization analyses identified deletions at chromosome 16p11.2 in each patient. These are the first reported cases with well-characterized CAS in the 16p11.2 syndrome literature and the first report of this microdeletion in CAS genetics research. We discuss implications of findings for issues in both literatures.