Hearing Status in Adult Individuals with Lifetime, Untreated Isolated Growth Hormone Deficiency

Prado-Barreto, Valéria Maria; Salvatori, Roberto; Júnior, Ronaldo Carvalho Santos; Brandão-Martins, Mariane B.; Correa, Eric A.; Garcez, Flávia Barreto; Valença, Eugênia H O; Souza, Anita H. O.; Pereira, Rossana M. C.; Nunes, Marco Antônio Prado; D’Ávila, Jeferson Sampaio; Aguiar‐Oliveira, Manuel H.

doi:10.1177/0194599813517987

Cited by 21 publications

(15 citation statements)

References 30 publications

(96 reference statements)

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“…They generally do not complain of tiredness, even at advanced age (38). Compared with controls from the same area, these untreated IGHD subjects report dizziness more frequently, and have mild high-tones sensorineural hearing loss (39). This sensorineural hearing loss is less severe than the profound sensorineural deafness found in a patient with deletion of the IGF-I gene (36).…”

Section: Overall Homozygous Phenotypementioning

confidence: 82%

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation

Aguiar‐Oliveira

Souza

Oliveira

et al. 2017

European Journal of Endocrinology

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Twenty years ago, we described kindred of 105 individuals with isolated GH deficiency (IGHD) in Itabaianinha County, in northeast Brazil, carrying a homozygous mutation in the GH-releasing hormone receptor gene. These subjects exhibit markedly reduced GH responsiveness to stimulatory tests, and anterior pituitary hypoplasia. Serum concentrations of IGF-I, IGF binding protein type 3 and the acid-labile subunit are markedly reduced, with a lesser reduction of IGF-II. The most striking physical findings of these IGHD individuals are the proportionate short stature, doll facies, high-pitched voice and visceral obesity with reduced fat-free mass. There is neither microphallus, nor neonatal hypoglycemia. Puberty is delayed, menopause anticipated, but fertility is preserved in both genders. The reduction in bone sizes is not even, with mean standard deviation scores for height of −7.2, total maxillary length of −6.5, total facial height of −4.3 and cephalic perimeter of −2.7. In addition, the non-osseous growth is not uniform, preserving some organs, like pancreas, liver, kidney, brain and eyes, and compromising others such as thyroid, heart, uterus and spleen. These subjects present higher prevalence of dizziness, mild high-tones sensorineural hearing loss, reduction of vascular retinal branching points, increase of optic disk, genu valgum and increased systolic blood pressure. Biochemically, they have high low density lipoprotein cholesterol and C-reactive protein levels, but maintain increased insulin sensitivity, and do not show premature atherosclerosis. Finally, they have normal immune function, and normal longevity. This review details the findings and summarizes 20 years of clinical research carried out in this unique population.

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Section: Overall Homozygous Phenotypementioning

confidence: 82%

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation

Aguiar‐Oliveira

Souza

Oliveira

et al. 2017

European Journal of Endocrinology

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“…In addition, in this subgroup we found a reduction in the duration of N3 in IGHD compared to controls, suggesting an additional effect of aging to GHRH receptor deficiency in this model. Interestingly, sleep, as skin ovary, and hearing seems to get older faster in IGHD than controls 51,52 , in contrast to bones, voice and blood vessels [53][54][55] .…”

Section: Discussionmentioning

confidence: 99%

Altered sleep patterns in patients with non-functional GHRH receptor

Oliveira

Salvatori

Marcondes³

et al. 2017

European Journal of Endocrinology

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“…For example, GH stimulates the growth of many body tissues and is heavily involved with cochlear development in the early stages of the embryo (Murray, Dattani, & Clayton, ; Rodríguez‐de la Rosa et al, ). Mutations in GH and GH receptors can cause an endocrine disorder known as GH deficiency (GHD), which leads to delayed bone development, pituitary dwarfism, and hearing loss (Brisset et al, ; Giordano et al, ; Prado‐Barreto et al, ). Muus and colleagues () performed the largest audiological evaluation on young subjects with untreated GHD and found that 83% of the patients suffered from bilateral hearing loss.…”

Section: Additional Hormones and Their Influence On Auditory Functionmentioning

confidence: 99%

Understanding hormone and hormone therapies' impact on the auditory system

Williamson

Liu

Pineros

et al. 2020

J of Neuroscience Research

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Hormones such as estrogen, progesterone, and aldosterone all demonstrate vital roles in sustaining auditory function through either the maintenance of cochlear neurons, up/down regulation of critical molecules (i.e., IGF‐1, BDNF, etc.), or generation of the endocochlear potential. With disease and/or age, hormone expression begins to decline drastically, which ultimately affects cochlear structures and the integrity of cochlear cells. The following review explores the latest findings as well as realistic outcomes for hormone therapy treatment in the auditory system. This information could serve as a potential guide for patients considering hormone therapy as a medicinal choice to alleviate the signs of onset of presbycusis—age‐related hearing loss. Additional scientific investigations could also be carried out to further enhance recent findings.

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Hearing Status in Adult Individuals with Lifetime, Untreated Isolated Growth Hormone Deficiency

Abstract: Compared with controls of the same area, subjects with untreated, congenital lifetime IGHD report more misophonia and dizziness, have predominance of mild high-tones sensorineural hearing loss, and have an absence of stapedial reflex and TEOAEs.

Cited by 21 publications

References 30 publications

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation

Altered sleep patterns in patients with non-functional GHRH receptor

Understanding hormone and hormone therapies' impact on the auditory system

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