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Cochlear hearing loss in patients with Laron syndrome

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

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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References

  1. Laron Z (2004) Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958–2003. J Clin Endocrinol Metab 89:1031–1044

    Article  PubMed  CAS  Google Scholar 

  2. Shevah O, Laron Z (2006) Genetic analysis of the pedigrees and molecular defects of the GH-receptor gene in the Israeli cohort of patients with Laron syndrome. Pediatr Endocrinol Rev 3(3 Suppl):S489–S497

    Google Scholar 

  3. Laron Z (1983) Deficiencies of growth hormone and somatomedins in man. Spec Top Endocrinol Metab 5:149–199

    PubMed  CAS  Google Scholar 

  4. Laron Z (2002) Growth hormone and insulin like growth factor I: effects on brain. In: Pfaff D, Arnold A, Etgen A et al (eds) Hormones, brain and behavior. Academic Press, Amsterdam, pp 75–96

    Chapter  Google Scholar 

  5. Varela-Nieto I, Morales-Garcia JA, Vigil P, Diaz-Casares A, Gorospe I, Sánchez-Galiano S, Cañon S, Camarero G, Contreras J, Cediel R, Leon Y (2004) Trophic effects of insulin-like growth factor-I (IGF-I) in the inner ear. Hear Res 196:19–25

    Article  PubMed  CAS  Google Scholar 

  6. Walenkamp MJ, Karperien M, Pereira AM, Hilhorst-Hofstee Y, van Doorn J, Chen JW, Mohan S, Denley A, Forbes B, van Duyvenvoorde HA, van Thiel SW, Sluimers CA, Bax JJ, de Laat JA, Breuning MB, Romijn JA (2005) Wit JM (2005) Homozygous and heterozygous expression of a novel insulin-like growth factor-I mutation. J Clin Endocrinol Metab 90:2855–2864

    Article  PubMed  CAS  Google Scholar 

  7. Woods KA, Camacho-Hubner C, Savage MO, Clark AJ (1996) Intrauterine growth retardation and postnatal growth failure associated with deletion of the insulin-like growth factor I gene. N Engl J Med 335:1363–1367

    Article  PubMed  CAS  Google Scholar 

  8. Bonapace G, Concolino D, Formicola S, Strisciuglio P (2003) A novel mutation in a patient with insulin-like growth factor 1 (IGF1) deficiency. J Med Genet 40:913–917

    Article  PubMed  CAS  Google Scholar 

  9. Iwai K, Nakagawa T, Endo T, Matsuoka Y, Kita T, Kim TS, Tabata Y, Ito J (2006) Cochlear protection by local insulin-like growth factor-1 application using biodegradable hydrogel. Laryngoscope 116:529–533

    Article  PubMed  CAS  Google Scholar 

  10. Linseman DA, Phelps RA, Bouchard RJ, Le SS, Laessig TA, McClure ML, Heidenreich KA (2002) Insulin-like growth factor-I blocks Bcl-2 interacting mediator of cell death (Bim) induction and intrinsic death signaling in cerebellar granule neurons. J Neurosci 22:9287–9297

    PubMed  CAS  Google Scholar 

  11. Armstrong CS, Wuarin L, Ishii DN (2000) Uptake of circulating insulin-like growth factor-I into the cerebrospinal fluid of normal and diabetic rats and normalization of IGF-II mRNA content in diabetic rat brain. J Neurosci Res 59:649–660

    Article  PubMed  CAS  Google Scholar 

  12. Camarero G, Villar MA, Contreras J, Fernández-Moreno C, Pichel JG, Avendaño C, Varela-Nieto I (2002) Cochlear abnormalities in insulin-like growth factor-1 mouse mutants. Hear Res 170:2–11

    Article  PubMed  CAS  Google Scholar 

  13. Sanchez-Calderon H, Milo M, Leon Y, Varela-Nieto I (2007) A network of growth and transcription factors controls neuronal differentiation and survival in the developing ear. Int J Dev Biol 51:557–570

    Article  PubMed  CAS  Google Scholar 

  14. Cediel R, Riquelme R, Contreras J, Díaz A, Varela-Nieto I (2006) Sensorineural hearing loss in insulin-like growth factor I-null mice: a new model of human deafness. Eur J Neurosci 23:587–590

    Article  PubMed  CAS  Google Scholar 

  15. Chernausek SD, Backeljauw PF, Frane J, Kuntze J, Underwood LE, GH Insensitivity Syndrome Collaborative Group (2007) Long-term treatment with recombinant insulin-like growth factor (IGF)-I in children with severe IGF-I deficiency due to growth hormone insensitivity. J Clin Endocrinol Metab 92:902–910

    Article  PubMed  CAS  Google Scholar 

  16. Greulich WW, Pyle SJ (1959) Radiographic atlas of skeletal development of the hand and wrist, 2nd edn. Stanford University Press, Stanford, p 256

    Google Scholar 

  17. Attias J, Furst M, Furman V, Reshef I, Horowitz G, Bresloff I (1995) Noise-induced otoacoustic emission loss with or without hearing loss. Ear Hear 16:612–618

    Article  PubMed  CAS  Google Scholar 

  18. Attias J, Raveh E, Ben-Naftali NF, Zarchi O, Gothelf D (2008) Hyperactive auditory efferent system and lack of acoustic reflexes in Williams syndrome. J Basic Clin Physiol Pharmacol 19:193–207

    Article  PubMed  Google Scholar 

  19. Gothelf D, Farber N, Raveh E, Apter A, Attias J (2006) Hyperacusis in Williams syndrome: characteristics and associated neuroaudiologic abnormalities. Neurology 66:390–395

    Article  PubMed  CAS  Google Scholar 

  20. Laron Z (2008) Insulin-like growth factor-I treatment of children with Laron syndrome (primary growth hormone insensitivity). Pediatr Endocrinol Rev 5:766–771

    PubMed  Google Scholar 

  21. Katzenell U, Segal S (2001) Hyperacusis: review and clinical guidelines. Otol Neurotol 22:321–327

    Article  PubMed  CAS  Google Scholar 

  22. Kornreich L, Horev G, Schwarz M, Karmazyn B, Laron Z (2002) Craniofacial and brain abnormalities in Laron syndrome (primary growth hormone insensitivity). Eur J Endocrinol 146(4):499–503

    Article  PubMed  CAS  Google Scholar 

  23. Edwards L, Crocker S (2007) Psychological processes in deaf children with complex needs: an evidence-based practical guide. Jessica Kingsley Publishers, London

    Google Scholar 

  24. Olusanya BO, Newton VE (2007) Global burden of childhood hearing impairment and disease control priorities for developing countries. Lancet 369:1314–1317

    Article  PubMed  Google Scholar 

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Acknowledgments

The authors thank Gloria Ginzach for her help in the preparation of the manuscript.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

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

    Joseph Attias

  2. Institute of Audiology and Clinical Neurophysiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel

    Joseph Attias & Omer Zarchi

  3. Endocrinology and Diabetes Research Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, Petach Tikva, Israel

    Zvi Laron

  4. Department of Otolaryngology, Head and Neck Surgery,, Rabin Medical Center, Petach Tikva, Israel

    Ben I. Nageris

  5. The Interdisciplinary Ph.D. Program in Neuroscience, Tel Aviv University, Tel Aviv, Israel

    Omer Zarchi

  6. Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel

    Ben I. Nageris & Zvi Laron

Authors
  1. Joseph Attias
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  2. Omer Zarchi
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  3. Ben I. Nageris
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  4. Zvi Laron
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Corresponding author

Correspondence to Zvi Laron.

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Attias, J., Zarchi, O., Nageris, B.I. et al. Cochlear hearing loss in patients with Laron syndrome. Eur Arch Otorhinolaryngol 269, 461–466 (2012). https://doi.org/10.1007/s00405-011-1668-x

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  • DOI: https://doi.org/10.1007/s00405-011-1668-x

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