Otology

European Archives of Oto-Rhino-Laryngology

, Volume 269, Issue 2, pp 461-466

First online:

Cochlear hearing loss in patients with Laron syndrome

  • Joseph AttiasAffiliated withDepartment of Communication Sciences & Disorders, University of HaifaInstitute of Audiology and Clinical Neurophysiology, Schneider Children’s Medical Center of Israel
  • , Omer ZarchiAffiliated withInstitute of Audiology and Clinical Neurophysiology, Schneider Children’s Medical Center of IsraelThe Interdisciplinary Ph.D. Program in Neuroscience, Tel Aviv University
  • , Ben I. NagerisAffiliated withDepartment of Otolaryngology, Head and Neck Surgery,, Rabin Medical CenterSackler School of Medicine, Tel Aviv University
  • , Zvi LaronAffiliated withEndocrinology and Diabetes Research Unit, Schneider Children’s Medical Center of IsraelSackler School of Medicine, Tel Aviv University Email author 

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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.

Keywords

Cochlear hearing loss Hyperacusis Laron syndrome Growth hormone insensitivity