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Language ENvironment Analysis (LENA) in Phelan-McDermid Syndrome: Validity and Suggestions for Use in Minimally Verbal Children with Autism Spectrum Disorder

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Abstract

Phelan-McDermid syndrome (PMS) is a single-locus cause of developmental delay, autism spectrum disorder, and minimal verbal abilities. There is an urgent need to identify objective outcome measures of expressive language for use in this and other minimally verbal populations. One potential tool is an automated language processor called Language ENvironment Analysis (LENA). LENA was used to obtain over 542 h of audio in 18 children with PMS. LENA performance was adequate in a subset of children with PMS, specifically younger children and those with fewer stereotypic vocalizations. One LENA-derived language measure, Vocalization Ratio, had improved accuracy in this sample and may represent a novel expressive language measure for use in severely affected populations.

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Acknowledgments

We would like to thank all the participants for taking part in our research. Jacquelin Rankine would like to thank the Icahn School of Medicine and the Patient Oriented Research Training and Leadership (PORTAL) Program for providing a research stipend during the time required to complete this work which was prepared as part of a master’s thesis. We would like to thank the Icahn School of Medicine at Mount Sinai Center for Biostatics for providing statistical support for this work. Dr. Alexander Kolevzon received research support from NIMH (R34 MH100276-01), NINDS (U54 NS092090- 01), the Autism Science Foundation, the Seaver Foundation, Hoffmann-La Roche, and Neuren Pharmaceuticals.

Author Contributions

All authors contributed extensively to the work presented in this paper. JR designed the study, collected and analyzed the data, wrote the initial draft of the paper, and participated in revising the manuscript. EL, SL, HR, and EF helped with data collection and analysis. PS, TW, JB, and AK helped with designing the study. All authors assisted with manuscript development and participated in revising the manuscript.

Funding

Jacquelin Rankine received a research stipend from the Icahn School of Medicine at Mount Sinai Patient Oriented Research Training and Leadership (PORTAL) Program.

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Author notes

  1. Stacey Lurie

    Present address: Ferkauf Graduate School of Psychology, Yeshiva University, Bronx, NY, USA

Authors and Affiliations

  1. Seaver Autism Center for Research and Treatment, Icahn School of Medicine at Mount Sinai, Box 1668, One Gustave L. Levy Place, New York, NY, 10029, USA

    Jacquelin Rankine, Erin Li, Stacey Lurie, Hillary Rieger, Emily Fourie, Paige M. Siper, A. Ting Wang, Joseph D. Buxbaum & Alexander Kolevzon

  2. Department of Psychiatry, Icahn School of Medicine at Mount Sinai, Box 1230, One Gustave L. Levy Place, New York, NY, 10029, USA

    Paige M. Siper, A. Ting Wang, Joseph D. Buxbaum & Alexander Kolevzon

  3. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Alexander Kolevzon

  4. Friedman Brain Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    A. Ting Wang, Joseph D. Buxbaum & Alexander Kolevzon

  5. Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Joseph D. Buxbaum & Alexander Kolevzon

  6. Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    A. Ting Wang & Joseph D. Buxbaum

  7. Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Joseph D. Buxbaum

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  1. Jacquelin Rankine
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  2. Erin Li
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Correspondence to Alexander Kolevzon.

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Conflict of interest

Dr. Joseph Buxbaum, Director of the Seaver Autism Center at Mount Sinai, and the Icahn School of Medicine at Mount Sinai hold a shared patent for the use of Insulin-Like Growth Factor-1 in Phelan-McDermid syndrome. Dr. Alexander Kolevzon received research support from NIMH (R34 MH100276-01), NINDS (U54 NS092090- 01), the Autism Science Foundation, the Seaver Foundation, Hoffmann-La Roche, and Neuren Pharmaceuticals.

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Informed consent was obtained from caregivers of all individual participants included in the study.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Rankine, J., Li, E., Lurie, S. et al. Language ENvironment Analysis (LENA) in Phelan-McDermid Syndrome: Validity and Suggestions for Use in Minimally Verbal Children with Autism Spectrum Disorder. J Autism Dev Disord 47, 1605–1617 (2017). https://doi.org/10.1007/s10803-017-3082-8

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