Early-Onset Sensorineural Hearing Loss in a Child with Turner Syndrome

Roush, Jackson; Davenport, ML; Carlson-Smith, C.

doi:10.1055/s-0042-1748132

Cited by 17 publications

(11 citation statements)

References 27 publications

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“…The main reason for CHL in TS is recurrent persistent otitis media with effusion, chronic middle ear infection, ossicular degeneration following chronic otitis media and cholesteatoma in the middle ear. 25 These conditions result from malfunction of the Eustachian tube and anatomic malformation of the skull base, that causes poor drainage and inadequate ventilation of the middle ear and facilitates nasopharyngeal organisms to reach the middle ear. 10 , 25 , 26 Palatal dysfunction in these patients may be exacerbated by the removal of adenoids.…”

Section: Conductive Hearing Lossmentioning

confidence: 99%

“… 25 These conditions result from malfunction of the Eustachian tube and anatomic malformation of the skull base, that causes poor drainage and inadequate ventilation of the middle ear and facilitates nasopharyngeal organisms to reach the middle ear. 10 , 25 , 26 Palatal dysfunction in these patients may be exacerbated by the removal of adenoids. 5 …”

Section: Conductive Hearing Lossmentioning

confidence: 99%

“…On the other hand, other investigators define it as exceeding 20 dB. 25 , 26 Other possible explanation for the variable results is that the progressive high-frequency hearing loss hides the dip in the middle frequencies. 26 Finally, some studies may have included children so young in whom the dip may have not yet developed.…”

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

“… 26 Finally, some studies may have included children so young in whom the dip may have not yet developed. 25 …”

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

“…Abnormal BAEP (Brainstem Auditory Evoked Potentials) in TS suggests impaired cochlear function which may be caused by a neuropathic disturbance or cochlear ganglion defects. 13 , 25 In fact, “Turner mice” have a loss of auditory brainstem response with increased age. 40 …”

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

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

Alves

Oliveira

2014

Brazilian Journal of Otorhinolaryngology

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Recurrent otitis media, dysfunction of the Eustachian tube, conductive hearing loss during infancy and sensorineural hearing loss in adolescence are the audiologic disorders more common in ST. The karyotype appears to be important in the hearing loss, with studies demonstrating an increased prevalence in patients with monosomy 45,X or isochromosome 46,i(Xq). Morphologic studies of the cochlea are necessary to help out in the clarifying the etiology of the sensorineural hearing loss.

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Section: Conductive Hearing Lossmentioning

confidence: 99%

Section: Conductive Hearing Lossmentioning

confidence: 99%

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

“… 26 Finally, some studies may have included children so young in whom the dip may have not yet developed. 25 …”

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

Section: Sensorineural Hearing Lossmentioning

confidence: 99%

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

Alves

Oliveira

2014

Brazilian Journal of Otorhinolaryngology

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Clinical update on sensorineural hearing loss in Turner syndrome and the X‐chromosome

Bonnard

Bark

Hederstierna

2019

American J of Med Genetics Pt C

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Hearing loss is one of the major medical concerns in girls and women with Turner syndrome (TS) and has a negative effect on well‐being and quality of everyday life. Sensorineural hearing loss is the most common type of hearing loss, affecting more than half of adults with TS. Karyotypes with a loss of the short p‐arm on the X‐chromosome are more prone to ear and hearing problems. The importance of detecting, investigating, and treating hearing loss with hearing aids cannot be emphasized enough. The pathophysiology of the sensorineural hearing loss in TS is not known, but theories regarding estrogen deficiency, the cell cycle delay hypothesis, IGF‐1 deficiency and the possible role of the KDM6A gene are discussed. Due to the diversity of symptoms and conditions within the same karyotype, a combination of genetic factors altered by epigenetic and/or hormonal effects is probable. Further research is needed regarding the pathophysiology of ear and hearing problems in TS to develop new treatment methods.

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Characteristics and progression of hearing loss in children with turner's syndrome

et al. 2019

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Objectives/Hypothesis: Evaluate patterns of hearing impairment in children with Turner's syndrome (TS) and determine factors influencing severity and progression.Study Design: Retrospective database review. Methods: Demographic, audiological, and medical data for children with TS were extracted from the Audiological and Genetic Database to analyze patterns of hearing loss with comorbidities, demographics, and interventions.Results: Two hundred seventy-two children with TS were identified; 213 had audiological data. Of these children, 72.3% (N = 154) had hearing loss in which 84.4% (N = 130) was bilateral. In individual ears, conductive hearing loss was most common (28.7%, n = 73), followed by mixed (22.0%, n = 93) and sensorineural (7.9%, n = 20). Otitis media (odds ratio [OR] = 2.7, 95% confidence interval [CI]: 1.2-6.5), eustachian tube dysfunction (OR = 9.5, 95% CI: 3.2-35.2), and aortic valve anomalies were also associated with higher rates of hearing loss (OR = 3.6, 95% CI: 1.3-11.5). Of ears with quantifiable severity, 16.3% (n = 40) had moderate or worse hearing loss. Aortic coarctation (36.3 vs. 21.3 dB, P < .001), seizures (40.6 vs. 21.3 dB, P = .006), facial anomalies (32.5 vs. 21.3 dB, P = .029), and hypertension (36.3 vs. 21.3 dB, P = .015) portended more severe loss.Conclusions: Children with TS have high rates of hearing loss. High rates of sensorineural loss were unexpected. Nonotologic comorbidities, including seizures, hypertension, and anomalies of the kidney and aorta were associated with greater prevalence, severity, and progression of loss. Children with TS, particularly with the above comorbidities, are at risk for poor hearing outcomes, and should be offered audiological intervention to efficiently direct patient care.

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Early-Onset Sensorineural Hearing Loss in a Child with Turner Syndrome

Cited by 17 publications

References 27 publications

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

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

Clinical update on sensorineural hearing loss in Turner syndrome and the X‐chromosome

Characteristics and progression of hearing loss in children with turner's syndrome

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