Cochlear hearing loss in patients with Laron syndrome.

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.

• Authors: Joseph Attias, Omer Zarchi, Ben I Nageris, Zvi Laron
• Journal: European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery (Eur Arch Otorhinolaryngol) Vol. 269 Issue 2 Pg. 461-6 (Feb 2012) ISSN: 1434-4726 [Electronic] Germany
• PMID: 21735352 (Publication Type: Journal Article)

Chemical References

• Insulin-Like Growth Factor I

Topics

• 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, drug therapy)
• Humans
• Hyperacusis (diagnosis, drug therapy)
• Infant
• Insulin-Like Growth Factor I (therapeutic use)
• Laron Syndrome (diagnosis, drug therapy)
• Loudness Perception (drug effects)
• Male
• Otoacoustic Emissions, Spontaneous (drug effects)
• Prospective Studies
• Reflex, Acoustic (drug effects)
• Secondary Prevention
• Young Adult