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The first reported treatment of Nager syndrome associated hearing loss with bone-anchored hearing aids: case report

  • G P Davies (a1) and I J M Johnson (a2)
Abstract
Objective:

To report the first case of treatment of Nager syndrome associated conductive hearing loss with bone-anchored hearing aids, in a three-year-old boy.

Method:

Clinical case report and current literature review regarding the use of bone-anchored hearing aids in the treatment of conductive hearing loss in children.

Results:

A three year eight month old boy with Nager syndrome was successfully treated for conductive hearing loss using bilateral bone-anchored hearing aids.

Conclusion:

This is the first case report of the use of bone-anchored hearing aids to treat Nager syndrome associated conductive hearing loss. Treatment was safe and successful in this case.

Copyright
Corresponding author
Address for correspondence: Dr Gareth Peter Davies, Tudor Lodge, Brookfield Road, Churchdown, Gloucester GL3 2PF, UK Fax: +44 (0)1452 617 135 E-mail: g.p.davies1@ncl.ac.uk
References
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1Herrmann, BW, Karzon, R, Molter, DW. Otologic and audiologic features of Nager acrofacial dysostosis. Int J Pediatr Otorhinolaryngol 2005;69:1053–9
2Opitz, C, Stoll, C, Ring, P. Nager, syndrome. Problems and possibilities of therapy. J Orofac Orthop 2000;61:226–36
3Zhang, Y, Dai, Y, Liu, Y, Ren, J. Mandibulofacial dysostosis, microtia, and limb anomalies in a newborn: a new form of acrofacial dysostosis syndrome? Clin Genet 2010;78:570–4
4Genetics Home Reference. In: http://ghr.nlm.nih.gov/condition/nager-syndrome [7 September 2011]
5Powell, RH, Burrell, SP, Cooper, HR, Proops, DW. The Birmingham bone anchored hearing aid programme: paediatric experience and results. J Laryngol Otol 1996;21(suppl):21–9
6Peyton's place. In: http://peytonsparents.blogspot.com [4 March 2011]
7The BAHAUsers Support Forum – Parental Chat. http://www.baha-users-support.com/cgi-bin/2bb/2bb.cgi?seq=msg&board=12&msg=1247846844&rn=6 [4 March 2011]
8Snik, A, Leijendeckers, J, Hol, M, Mylanus, E, Cremers, C. The bone-anchored hearing aid for children: recent developments. Int J Audiol 2008;47:554–9
9Lloyd, S, Almeyda, J, Sirimanna, KS, Albert, DM, Bailey, CM. Updated surgical experience with bone-anchored hearing aids in children. J Laryngol Otol 2007;121:826–31
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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