Biomaterial Database

Cochlear hearing loss in patients with Laron syndrome. 2012

Joseph Attias, and Omer Zarchi, and Ben I Nageris, and Zvi Laron

Department of Communication Sciences & Disorders, University of Haifa, Mt. Carmel, Haifa, Israel.

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.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007334	Insulin-Like Growth Factor I	A well-characterized basic peptide believed to be secreted by the liver and to circulate in the blood. It has growth-regulating, insulin-like, and mitogenic activities. This growth factor has a major, but not absolute, dependence on GROWTH HORMONE. It is believed to be mainly active in adults in contrast to INSULIN-LIKE GROWTH FACTOR II, which is a major fetal growth factor.	IGF-I,Somatomedin C,IGF-1,IGF-I-SmC,Insulin Like Growth Factor I,Insulin-Like Somatomedin Peptide I,Insulin Like Somatomedin Peptide I
D008144	Loudness Perception	The perceived attribute of a sound which corresponds to the physical attribute of intensity.	Loudness Perceptions,Perception, Loudness,Perceptions, Loudness
D008297	Male		Males
D011446	Prospective Studies	Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.	Prospective Study,Studies, Prospective,Study, Prospective
D012001	Hyperacusis	An abnormally disproportionate increase in the sensation of loudness in response to auditory stimuli of normal volume. COCHLEAR DISEASES; VESTIBULOCOCHLEAR NERVE DISEASES; FACIAL NERVE DISEASES; STAPES SURGERY; and other disorders may be associated with this condition.	Auditory Hyperesthesia,Loudness Recruitment,Recruitment, Loudness,Hyperacusia,Loudness Perception Disturbances,Phonophobia,Auditory Hyperesthesias,Disturbance, Loudness Perception,Disturbances, Loudness Perception,Hyperacuses,Hyperacusias,Hyperesthesia, Auditory,Hyperesthesias, Auditory,Loudness Perception Disturbance,Loudness Recruitments,Perception Disturbance, Loudness,Perception Disturbances, Loudness,Phonophobias
D012022	Reflex, Acoustic	Intra-aural contraction of tensor tympani and stapedius in response to sound.	Acoustic Reflex
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females