Christina Hederstierna1, Malou Hultcrantz, Ulf Rosenhall. 1. Division of Otorhinolaryngology and Hearing, Department of Clinical Neuroscience, Karolinska Institute, Stockholm, Sweden. christina.hederstierna@karolinska.se
Abstract
CONCLUSION: Young and middle-aged women with Turner syndrome (TS) have a progressive type of hearing impairment, deteriorating rapidly in adult age. The hearing decline seems to consist of two patterns: a mid-frequency dip, likely of a genetic origin, and a high-frequency loss resembling age-related hearing impairment -- possibly influenced by estrogen deficiency. OBJECTIVES: This was a longitudinal study of hearing thresholds in a group of women with TS that also aimed to determine whether the factors initial age, initial hearing level, karyotype, and presence/absence of a mid-frequency dip influence the rate of decline and could serve as prognostic markers. SUBJECTS AND METHODS: Audiograms of air conduction thresholds in 69 women with TS (aged 28-62 years) were performed twice with an average interval of 10 years. RESULTS: The rate of hearing decline is much higher in women with TS than in age-matched women from the general population. The decline rate is comparable to that seen in 70-90-year-old women, regardless of initial age, hearing level, karyotype, or presence of a mid-frequency dip. The rate of decline is especially high in the high-frequency region, 0.8-2.2 dB per year. The presence of a mid-frequency dip is an especially strong predictor for a future high rate of hearing decline with subsequent social consequences.
CONCLUSION: Young and middle-aged women with Turner syndrome (TS) have a progressive type of hearing impairment, deteriorating rapidly in adult age. The hearing decline seems to consist of two patterns: a mid-frequency dip, likely of a genetic origin, and a high-frequency loss resembling age-related hearing impairment -- possibly influenced by estrogen deficiency. OBJECTIVES: This was a longitudinal study of hearing thresholds in a group of women with TS that also aimed to determine whether the factors initial age, initial hearing level, karyotype, and presence/absence of a mid-frequency dip influence the rate of decline and could serve as prognostic markers. SUBJECTS AND METHODS: Audiograms of air conduction thresholds in 69 women with TS (aged 28-62 years) were performed twice with an average interval of 10 years. RESULTS: The rate of hearing decline is much higher in women with TS than in age-matched women from the general population. The decline rate is comparable to that seen in 70-90-year-old women, regardless of initial age, hearing level, karyotype, or presence of a mid-frequency dip. The rate of decline is especially high in the high-frequency region, 0.8-2.2 dB per year. The presence of a mid-frequency dip is an especially strong predictor for a future high rate of hearing decline with subsequent social consequences.