Cochlear hearing loss in patients with Laron syndrome

Attias, Joseph; Zarchi, Omer; Nageris, Ben I.; Laron, Zvi

doi:10.1007/s00405-011-1668-x

Cited by 34 publications

(30 citation statements)

References 19 publications

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“…Neurosensorial hearing loss is a quite common finding in children with perinatal problems and also in children with alterations in GH secretion or its signaling pathways [87,88]. We treated with GH and specific auditory stimulation a child with cerebral palsy, The patient at admission.…”

Section: Ghd and Nervous System Functioningmentioning

confidence: 99%

Growth Hormone Deficiency: Is It Just a Problem of Growth Impairment? Part I

Devesa¹

2020

Growth Disorders and Acromegaly

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The concept that the growth hormone (GH) is a merely metabolic pituitary hormone with effects on the longitudinal growth of the organism until the end of puberty has been questioned in recent years. We know today that the expression of GH also occurs in virtually all organs and tissues where it performs very important autocrine/paracrine and even intracrine functions. GH acts on all organs and tissues, being particularly important in the development of the brain during the fetal period. In addition, the hormone, after interacting with its membrane receptor, is internalized together with its receptor, allowing it to reach the cell nucleus where it acts as a transcription factor. In the first part of this review, we will analyze the effects of GH on the brain, the cardiovascular system, and the gonadal system, as well as the adverse effects that occur in the GH deficiency not treated in children and adults. GH is absolutely necessary for a normal brain development and also for repairing the nervous system after an injury. Moreover, GH plays a very important role in the cardiovascular system, as well as in normal gonadal functioning.

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Section: Ghd and Nervous System Functioningmentioning

confidence: 99%

Growth Hormone Deficiency: Is It Just a Problem of Growth Impairment? Part I

Devesa¹

2020

Growth Disorders and Acromegaly

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“…In humans, patients with Laron syndrome treated preventively with IGF-1 develop normal hearing [89]. It has been demonstrated that topical application of IGF-1 in the round window, prior tympanostomy, of patients with sudden hearing loss is a safe and effective therapy with minor side effects for those patients in whom initial treatment Fig.…”

Section: Igf-1mentioning

confidence: 99%

Hormone Therapy: Challenges for Treating Hearing Impairments

Guerra¹,

Devesa²

2019

SN Compr. Clin. Med.

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The purpose of this review is to summarize the advances and discoveries in the potential treatment with hormone analogs and some challenges still pending, beyond the classic treatment with corticosteroids. We conduct a review of the functions of hormones on hearing, in human and animal models, the presence of their most relevant receptors, and the desirable and undesirable effects for their therapeutic uses. Different hormones play a regulatory role in the development and maintenance of hearing. Hormone receptors in the ear have been identified over the years, which can be a support to use them as new therapeutic targets Moreover, their mediators, that include cells, neurotrophic factors, or other hormones, could be also useful for treating various hearing impairments The use of synthetic analogs could exert a therapeutic effect on hearing. Hormone therapy, in fact, can contribute positively to the treatment and prevention of various auditory pathologies, through the regeneration or protection. Considering that an optimal result has to be a garantee, it is a must to know their unwanted effects and contraindications. The use of hormones may protect, regenerate, and modulate multiple pathological conditions of the ear, but it would be necessary to standardize drug dosages, find alternative routes, and conduct prospective studies in humans.

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“…In human beings, SNHL occurs in patients with mutations in Igf1 gene (Woods et al, 1996; Bonapace et al, 2003; Walenkamp et al, 2005), primary IGF-1 deficiency (Attias et al, 2012), or low serum IGF-1 levels due to other genetic defects (Barrenas et al, 2000; Johnson et al, 2007), indicating the importance of IGF-1 in hearing. Replacement therapy using recombinant IGF-1 rescues patients from the hearing loss in Laron syndrome (Attias et al, 2012). The SNHL in Laron syndrome patients is attributed to cochlear dysfunction, based on otoacoustic emission test results (Attias et al, 2012).…”

Section: Igf-1 and The Inner Earmentioning

confidence: 99%

“…Replacement therapy using recombinant IGF-1 rescues patients from the hearing loss in Laron syndrome (Attias et al, 2012). The SNHL in Laron syndrome patients is attributed to cochlear dysfunction, based on otoacoustic emission test results (Attias et al, 2012). However, in a mouse model of IGF-1 deficiency, that is in IGF-1 knockout mice (Camarero et al, 2001, 2002; Cediel et al, 2006; Riquelme et al, 2010), the causes of SNHL were reported as the loss of spiral ganglion neurons, abnormal myelination of the cochlear nerve, and degeneration of the stria vascularis, based on the results of morphological (Camarero et al, 2001, 2002) and auditory brain stem response studies (Cediel et al, 2006; Riquelme et al, 2010).…”

Section: Igf-1 and The Inner Earmentioning

confidence: 99%

Application of insulin-like growth factor-1 in the treatment of inner ear disorders

2014

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Sensorineural hearing loss (SNHL) is considered an intractable disease, given that hair and supporting cells (HCs and SCs) of the postnatal mammalian cochlea are unable to regenerate. However, with progress in regenerative medicine in the 21st century, several innovative approaches for achieving regeneration of inner ear HCs and SCs have become available. These methods include stem cell transplantation, overexpression of specific genes, and treatment with growth factors. Insulin-like growth factor-1 (IGF-1) is one of the growth factors that are involved in the development of the inner ear. Treatment with IGF-1 maintains HC numbers in the postnatal mammalian cochlea after various types of HC injuries, with activation of two major pathways downstream of IGF-1 signaling. In the aminoglycoside-treated neonatal mouse cochlear explant culture, promotion of the cell-cycle in SCs as well as inhibition of HC apoptosis was observed in the IGF-1-treated group. Activation of downstream molecules was observed in SCs and, in turn, SCs contribute to the maintenance of HC numbers. Using comprehensive analysis of the gene expression, the candidate effector molecules of the IGF-1 signaling pathway in the protection of HCs were identified as Netrin1 and Gap43. Based on these studies, a clinical trial has sought to investigate the effects of IGF-1 on SNHL. Sudden SNHL (SSHL) that was refractory to systemic steroids was treated with IGF-1 in a gelatin hydrogel and the outcome was compared with a historical control of hyperbaric oxygen therapy. The proportion of patients showing hearing improvement was significantly higher in the IGF-1-treatment group at 24 weeks after treatment than in the control group. A randomized clinical trial is ongoing to compare the effect of IGF-1 treatment with that of intra-tympanic steroids for SSHL that is refractory to systemic steroids.

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Cochlear hearing loss in patients with Laron syndrome

Cited by 34 publications

References 19 publications

Growth Hormone Deficiency: Is It Just a Problem of Growth Impairment? Part I

Growth Hormone Deficiency: Is It Just a Problem of Growth Impairment? Part I

Hormone Therapy: Challenges for Treating Hearing Impairments

Application of insulin-like growth factor-1 in the treatment of inner ear disorders

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