Bengal Journal of Otolaryngology and Head Neck Surgery (Feb 2021)

Communication Profile of de Lange Syndrome

  • Susmi Pani,
  • Palash Dutta,
  • Vidushi Saxena,
  • Asif Ekbal Ansari

DOI
https://doi.org/10.47210/bjohns.2020.v28i3.285
Journal volume & issue
Vol. 28, no. 3

Abstract

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Introduction de Lange Syndrome is an autosomal dominant disorder which was initially described by Vrolik (1849) in a child with severe oligodactayly. Case Report A case of 2y2m/Female child came for speech and hearing evaluation. Audiological examination (BOA, DPOAE, ABR /BAER) and Speech-Language Evaluation, Psychological Examination were done by expertise clinicians. Previously, the child was diagnosed with de Lange Syndrome. Audiological evaluation diagnosed the child to have moderately severe hearing loss. After speech and language evaluation the child was detected with speech language disorder. After psychological evaluation the child was diagnosed with severe developmental delay and having low IQ range. Discussion de Lange syndrome is an autosomal dominant developmental disorder characterized by the sample number of characteristics which requires more rehabilitation options have to be planned. Unfortunately, there is dearth of literature that addresses the characteristics, assessment and intervention of individuals with de Lange syndrome.

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