Cochlear hearing loss in patients with Laron syndrome

Eur Arch Otorhinolaryngol. 2012 Feb;269(2):461-6. doi: 10.1007/s00405-011-1668-x. Epub 2011 Jul 8.

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.

MeSH terms

  • Acoustic Impedance Tests
  • Adolescent
  • Age Determination by Skeleton
  • Audiometry, Pure-Tone
  • Audiometry, Speech
  • Child
  • Child, Preschool
  • Evoked Potentials, Auditory, Brain Stem / drug effects
  • Female
  • Hearing Loss, Sensorineural / diagnosis*
  • Hearing Loss, Sensorineural / drug therapy
  • Humans
  • Hyperacusis / diagnosis
  • Hyperacusis / drug therapy
  • Infant
  • Insulin-Like Growth Factor I / therapeutic use
  • Laron Syndrome / diagnosis*
  • Laron Syndrome / drug therapy
  • Loudness Perception / drug effects
  • Male
  • Otoacoustic Emissions, Spontaneous / drug effects
  • Prospective Studies
  • Reflex, Acoustic / drug effects
  • Secondary Prevention
  • Young Adult

Substances

  • Insulin-Like Growth Factor I