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Hearing loss and fluctuating hearing levels in X-linked hypophosphataemic osteomalacia

Published online by Cambridge University Press:  18 February 2008

G Pantel ,
R Probst ,
M Podvinec  and
N Gürtler
G Pantel
Affiliation:
Department of Otorhinolaryngology, Kantonsspital Aarau AG, Switzerland Department of Otorhinolaryngology, University Hospital Basel, Switzerland
R Probst
Affiliation:
Department of Otorhinolaryngology, University Hospital Basel, Switzerland
M Podvinec
Affiliation:
Department of Otorhinolaryngology, Kantonsspital Aarau AG, Switzerland
N Gürtler*
Affiliation:
Department of Otorhinolaryngology, Kantonsspital Aarau AG, Switzerland Department of Otorhinolaryngology, University Hospital Basel, Switzerland
*
Address for correspondence: Dr Nicolas Gürtler, HNO Klinik, Kantonsspital AG 5001 Aarau, Switzerland. Fax: +41 62 838 51 09 E-mail: nicolas.guertler@ksa.ch
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Abstract

Background and objective:

X-linked hypophosphataemic osteomalacia is the most common of the genetically determined forms of osteomalacia. The occurrence of hearing loss in X-linked hypophosphataemic osteomalacia has been known since 1984. However, observations on the progression of such hearing loss, and suggestions regarding possible therapy, have not previously been published.

Methods:

Case report of a patient with X-linked hypophosphataemic osteomalacia and hearing loss, with three years' audiological follow up, description of empirical therapy and literature review.

Results:

The patient presented with fluctuating hearing. An audiogram showed mild to severe sensorineural hearing loss mainly in the low and high frequencies. A temporary improvement of 20–40 dB after steroid therapy was observed. Four weeks later, hearing had deteriorated again, mainly in the low frequencies. After one year of fluctuating hearing levels, stabilisation occurred.

Conclusions:

In X-linked hypophosphataemic osteomalacia, hearing loss occurs predominantly in the low and high frequencies. The hearing loss type and progression pattern point to an endolymphatic hydrops as the pathogenetic mechanism. Steroid therapy may be of some benefit.

Keywords

X-linked Dominant Hypophosphataemic RicketsOsteomalaciaSensorineural Hearing Loss
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 1 , January 2009 , pp. 136 - 140
Copyright
Copyright © JLO (1984) Limited 2008

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References

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