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Voice problems due to virilization in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Authors

  • Ulrika Nygren,

    Corresponding author
    1. Division of Speech-Language Pathology, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden
    2. Department of Speech and Language Pathology, Karolinska University Hospital, Stockholm, Sweden
    • Correspondence: Ulrika Nygren, Division of Speech-Language Pathology B69, Karolinska University Hospital, SE-141 86 Stockholm, Sweden. Tel.: +46 8 517 72564; Fax: +46 8 585 81505; E-mail: ulrika.nygren@ki.se

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  • Helena Filipsson Nyström,

    1. Department of Endocrinology, Sahlgrenska University Hospital, Göteborg, Sweden
    2. Department of Endocrinology, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
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  • Henrik Falhammar,

    1. Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
    2. Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden
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  • Kerstin Hagenfeldt,

    1. Department of Women's and Children's Health and Center of Molecular Medicine, Karolinska Institutet, Stockholm, Sweden
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  • Agneta Nordenskjöld,

    1. Department of Women's and Children's Health and Center of Molecular Medicine, Karolinska Institutet, Stockholm, Sweden
    2. Department of Paediatric Surgery, Astrid Lindgren Children Hospital, Karolinska University Hospital, Stockholm, Sweden
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  • Maria Södersten

    1. Division of Speech-Language Pathology, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden
    2. Department of Speech and Language Pathology, Karolinska University Hospital, Stockholm, Sweden
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Summary

Objective

Congenital adrenal hyperplasia (CAH) is an autosomal recessive inherited disorder in which the lack of 21-hydroxylase results in cortisol and aldosterone insufficiency and an overproduction of adrenal androgens. High levels of androgens in women may cause virilization of the larynx and a masculine voice. The purpose of the present study was to investigate subjective voice problems due to virilization in women with CAH.

Design/Patients

Participants were 42 women with CAH between 25 and 71 years of age, and 43 age-matched female healthy control subjects. All patients, but two, were in good disease control.

Measurements

A validated Swedish version of the Voice Handicap Index (VHI) and questions related to voice virilization were used. Endocrine data were obtained from medical files.

Results

Patients scored significantly higher on VHI when the results were divided into no/mild, moderate and severe voice handicap as compared with the control subjects. They rated significantly higher for ‘dark voice’ and for ‘being perceived as a man on the phone’ compared with controls. Seven per cent of the women with CAH had voice problems clearly related to voice virilization. High ratings of dark voice were significantly associated with long periods of under-treatment with glucocorticoids and higher bone mineral density but not with severity of mutation.

Conclusion

Subjective voice problems due to voice virilization may occur in women with CAH. This further emphasizes the importance of avoiding long periods of increased androgen levels to prevent irreversible voice changes. For these patients, we recommend referral to voice assessment and treatment.

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