Growth Hormone Treatment Does Not Affect Incidences of Middle Ear Disease or Hearing Loss in Infants and Toddlers with Turner Syndrome

Abstract

Context: No randomized, controlled, prospective study has evaluated the effect of growth hormone (GH) on the rates of middle ear (ME) disease and hearing loss in girls with Turner syndrome (TS). Design: A 2-year, prospective, randomized, controlled, open-label, multicenter, clinical trial (‘Toddler Turner Study’; August 1999 to August 2003) was carried out. Setting: The study was conducted at 11 US pediatric endocrine centers. Subjects: Eighty-eight girls with TS, aged 9 months to 4 years, were enrolled. Intervention: The interventions comprised recombinant GH (50 µg/kg/day, n = 45) or no treatment (n = 43) for 2 years. Main Outcome Measures: The outcome measures included occurrence rates of ear-related problems, otitis media (OM) and associated antibiotic treatments, tympanometric assessment of ME function and hearing assessment by audiology. Results: At baseline, 57% of the girls (mean age = 1.98 ± 1.00 years) had a history of recurrent OM, 33% had undergone tympanostomy tube (t-tube) insertion and 27% had abnormal hearing. There was no significant difference between the treatment groups for annual incidence of OM episodes (untreated control: 1.9 ± 1.4; GH-treated: 1.5 ± 1.6, p = 0.17). A quarter of the subjects underwent ear surgeries (mainly t-tube insertions) during the study. Recurrent or persistent abnormality of ME function on tympanometry was present in 28–45% of the girls without t-tubes at the 6 postbaseline visits. Hearing deficits were found in 19–32% of the girls at the annual postbaseline visits. Most of these were conductive deficits, however, 2 girls had findings consistent with sensorineural hearing loss, which was evident before 3 years of age. Conclusions: Ear and hearing problems are common in infants and toddlers with TS and are not significantly influenced by GH treatment. Girls with TS need early, regular and thorough ME monitoring by their primary care provider and/or otolaryngologist, and at least annual hearing evaluations by a pediatric audiologist.

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…Hormone treatment has been hypothesized to influence cranial development and consequently middle ear function. Growth hormone, estradiol, and oxandrolone have not been shown to have a significant influence on hearing ability and middle ear function (Davenport et al, ; Hultcrantz, ; Ostberg, Beckman, Cadge, & Conway, ; Verver et al, ).…”

Turner syndrome and sexual differentiation of the brain: implications for understanding male-biased neurodevelopmental disorders

“…Ear infections are especially common in the first few years of life. For example, in the “Toddler Turner Study”, at the baseline study visit (mean age = 2 years), 26% had tympanostomy tubes present, another 64% had evidence of abnormal middle ear function, and 27% had abnormal hearing in one or both ears (Davenport et al 2010 ). In addition, progressive sensorineural hearing loss with a unique dip in the 1.5–2 kHz region and/or a high frequency loss (above 8 kHz) may present very early and necessitate the use of hearing aids in childhood.…”

Ear health and hearing surveillance in girls and women with Turner's syndrome: recommendations from the Turner's Syndrome Support Society

“…Two large cross‐sectional studies (each more than a hundred subjects; one study in girls and one in women) have shown no association between conductive hearing loss and prior treatment with oestrogen or growth hormone. One randomised controlled trial of 2 years of growth hormone treatment in girls with TS (88 girls aged 9 months to 4 years) showed no difference in the incidence of middle ear disease . There is evidence, therefore, that growth hormone and oestrogen therapy are unhelpful in preventing the development of middle ear disease, but equally there is nothing to suggest they worsen those problems.…”

“…Above the age of 40, 27% of women with TS will have hearing thresholds greater than 20 dB and 27% will be wearing hearing aids . Not all women with TS who have a significant hearing impairment have been identified and given hearing aids; many authors have suggested that regular hearing surveillance in this population would be of benefit although there are no reports of the effectiveness of such a screening programme in the literature to date. A 2007 report from an American expert panel suggested annual hearing screening for those with a history of middle ear disease, and hearing screening every 2–3 years for those with no such history .…”