European Archives of Oto-Rhino-Laryngology

, Volume 269, Issue 2, pp 461–466

Cochlear hearing loss in patients with Laron syndrome

Authors

  • Joseph Attias
    • Department of Communication Sciences & DisordersUniversity of Haifa
    • Institute of Audiology and Clinical NeurophysiologySchneider Children’s Medical Center of Israel
  • Omer Zarchi
    • Institute of Audiology and Clinical NeurophysiologySchneider Children’s Medical Center of Israel
    • The Interdisciplinary Ph.D. Program in NeuroscienceTel Aviv University
  • Ben I. Nageris
    • Department of Otolaryngology, Head and Neck Surgery,Rabin Medical Center
    • Sackler School of MedicineTel Aviv University
    • Endocrinology and Diabetes Research UnitSchneider Children’s Medical Center of Israel
    • Sackler School of MedicineTel Aviv University
Otology

DOI: 10.1007/s00405-011-1668-x

Cite this article as:
Attias, J., Zarchi, O., Nageris, B.I. et al. Eur Arch Otorhinolaryngol (2012) 269: 461. doi:10.1007/s00405-011-1668-x

Abstract

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age <2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

Keywords

Cochlear hearing lossHyperacusisLaron syndromeGrowth hormone insensitivity

Copyright information

© Springer-Verlag 2011