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Published in: European Archives of Oto-Rhino-Laryngology 2/2012

01-02-2012 | Otology

Cochlear hearing loss in patients with Laron syndrome

Authors: Joseph Attias, Omer Zarchi, Ben I. Nageris, Zvi Laron

Published in: European Archives of Oto-Rhino-Laryngology | Issue 2/2012

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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.
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Metadata
Title
Cochlear hearing loss in patients with Laron syndrome
Authors
Joseph Attias
Omer Zarchi
Ben I. Nageris
Zvi Laron
Publication date
01-02-2012
Publisher
Springer-Verlag
Published in
European Archives of Oto-Rhino-Laryngology / Issue 2/2012
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-011-1668-x

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