Hearing loss among patients with Turner's syndrome: literature review

Alves, Crésio; Oliveira, Conceição Silva

doi:10.1016/j.bjorl.2013.08.002

Cited by 19 publications

(11 citation statements)

References 48 publications

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“…If estrogen replacement begins after 13 years of age, or if women are deficient in estrogen for more than 2 years, the risk of SNHL is also increased. These data, however, are not independent of age [37]. As we can see, results about sex hormones and hearing appear to be contradictory.…”

Section: Hearing and Sex Hormonescontrasting

confidence: 56%

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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Section: Hearing and Sex Hormonescontrasting

confidence: 56%

Hormone Therapy: Challenges for Treating Hearing Impairments

Guerra¹,

Devesa²

2019

SN Compr. Clin. Med.

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“…This is attributable to loss of Xp carrying the SHOX gene (expressed within pharyngeal arches developing into structures including middle ear ossicles and muscles coordinating Eustachian tube opening and soft palate function) . Additionally, SHOX gene deficiency could cause a delayed cell cycle and fewer cochlea sensory cells at birth, resulting in cochlear dysfunction and therefore SNHL . Notably, the severest type of middle ear disease, cholesteatoma (prevalence 4.9%), was confined to these karyotypes.…”

Section: Discussionmentioning

confidence: 99%

“…4 Proposed mechanisms for otological disease in TS include: loss of genes on the short arm of chromosome X (Xp) 5 with resultant skeletal dysplasia causing altered middle ear anatomy and/or abnormal ossicles; high-arched palate causing a horizontal Eustachian tube with increased risk of otitis media; and impaired lymphatic drainage. [5][6][7] Inner ear problems are also influenced by Xp loss. 7 It is also postulated that there are fewer sensory cells at birth in TS, which decline more rapidly with age.…”

mentioning

confidence: 99%

Prevalence, risk factors and management strategies for otological problems in girls with Turner syndrome

et al. 2019

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Aim To determine the prevalence and risk factors of ear disease in Turner syndrome (TS), propose an algorithm for future surveillance and recommend preventative strategies. Methods Review of TS patients seen in the West of Scotland between 1989 and 2015, with questionnaire follow‐up in 2015. Results Of 168 girls, median age 27.3 (3.8‐47.2) years, ear problems occurred more frequently with 45,X and 45,X/46,XiXq than other karyotypes: 71/103 (69%) versus 23/65 (35%). Recurrent acute otitis media (AOM) first developed at 0‐5 years in 23 (40%) girls, persisting in 16 (10%) at 5‐10 years; and first developing at 5‐10 years in 11 (7%). Persistent otitis media with effusion (OME) first developed at 0‐5 and 5‐10 years in 23 (40%) and 14 (8%) girls. Recurrent AOM was significantly linked with cholesteatoma in 8 (4.9%) girls (7 aged >10 years). Permanent hearing loss was documented in 28 girls (16.7%), with 16 (9.5%) receiving hearing aids (bone‐anchored in 3). Conclusion Acute otitis media and OME occur commonly in preschool TS girls and may persist or newly develop in later childhood. Recurrent AOM predisposes to cholesteatoma. Strategies to reduce otological morbidity include: intensive patient education, annual audiology, vaccinations and a randomised trial of antibiotic prophylaxis in high‐risk groups.

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“…Estrogen deficiency-associated poor mineralization of the cochlear capsule and its role in hearing impairment has not been well studied. [37]…”

Section: Gonadal Disorders and Hearing Lossmentioning

confidence: 99%

Endocrine glands and hearing: Auditory manifestations of various endocrine and metabolic conditions

Cherian

Kapoor

Mathews

et al. 2017

Indian J Endocr Metab

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The aetiology of hearing loss in humans is multifactorial. Besides genetic, environmental and infectious causes, several endocrine and metabolic abnormalities are associated with varying degrees of hearing impairment. The pattern of hearing loss may be conductive, sensori-neural or mixed. The neurophysiology of hearing as well as the anatomical structure of the auditory system may be influenced by changes in the hormonal and metabolic milieu. Optimal management of these conditions requires the integrated efforts of the otolaryngologist and the endocrinologist. The presence of hearing loss especially in the young age group should prompt the clinician to explore the possibility of an associated endocrine or metabolic disorder for timely referral and early initiation of treatment.

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Hearing loss among patients with Turner's syndrome: literature review

Cited by 19 publications

References 48 publications

Hormone Therapy: Challenges for Treating Hearing Impairments

Hormone Therapy: Challenges for Treating Hearing Impairments

Prevalence, risk factors and management strategies for otological problems in girls with Turner syndrome

Endocrine glands and hearing: Auditory manifestations of various endocrine and metabolic conditions

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