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An auditory profile of sclerosteosis

Published online by Cambridge University Press:  19 March 2014

J M Potgieter ,
D W Swanepoel ,
B M Heinze ,
L M Hofmeyr ,
A A S Burger  and
H Hamersma
J M Potgieter
Affiliation:
Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa
D W Swanepoel*
Affiliation:
Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa Ear Sciences Centre, School of Surgery, University of Western Australia, Nedlands, Australia Ear ScienceInstitute Australia, Subiaco, Western Australia, Australia
B M Heinze
Affiliation:
Department of Speech-Language Pathology and Audiology, University of Pretoria, South Africa
L M Hofmeyr
Affiliation:
Department of Otorhinolaryngology, Faculty of Health Sciences, University of Pretoria, South Africa
A A S Burger
Affiliation:
Muelmed Mediclinic, Pretoria, South Africa
H Hamersma
Affiliation:
Private Practice, Roodepoort, South Africa
*
Address for correspondence: Prof D Swanepoel, Department of Speech-Language Pathology and Audiology, Communication Pathology Building, University of Pretoria, Pretoria 0002, Republic of South Africa E-mail: dewet.swanepoel@up.ac.za
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Abstract

Objective:

To characterise auditory involvement secondary to excessive craniotubular bone growth in individuals with sclerosteosis in South Africa.

Methods:

This cross-sectional study assessed the auditory profile of 10 participants with sclerosteosis. An auditory test battery was used and results for each ear were recorded using descriptive and comparative analyses.

Results:

All participants presented with bilateral, mixed hearing losses. Of the 20 ears, hearing loss was moderate in 5 per cent (n = 1), severe in 55 per cent (n = 11) and profound in 40 per cent (n = 8). Air–bone gaps were smaller in older participants, although the difference was not statistically significant (p > 0.05). Computed tomography scans indicated pervasive abnormalities of the external auditory canal, tympanic membrane, middle-ear space, ossicles, oval window, round window and internal auditory canal. Narrowed internal auditory canals corresponded to poor speech discrimination, indicative of retrocochlear pathology and absent auditory brainstem response waves.

Conclusion:

Progressive abnormal bone formation in sclerosteosis involves the middle ear, the round and oval windows of the cochlea, and the internal auditory canal. The condition compromises conductive, sensory and neural auditory pathways, which results in moderate to profound, mixed hearing loss.

Keywords

AudiologyBone DysplasiasHearing Loss, ConductiveSclerosteosisSensorineural Hearing Loss
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 128 , Issue 4 , April 2014 , pp. 336 - 344
Copyright
Copyright © JLO (1984) Limited 2014 

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