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Inner-ear malformations as a cause of single-sided deafness

Published online by Cambridge University Press:  08 June 2020

E Tahir ,
M D Bajin ,
S Jafarov ,
M Ö Yıldırım ,
B Ç Çınar ,
G Sennaroğlu  and
L Sennaroğlu
E Tahir*
Affiliation:
Department of Otolaryngology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey
M D Bajin
Affiliation:
Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
S Jafarov
Affiliation:
Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
M Ö Yıldırım
Affiliation:
Department of Audiology, Hacettepe University, Ankara, Turkey
B Ç Çınar
Affiliation:
Department of Audiology, Hacettepe University, Ankara, Turkey
G Sennaroğlu
Affiliation:
Department of Audiology, Hacettepe University, Ankara, Turkey
L Sennaroğlu
Affiliation:
Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
*
Author for correspondence: Dr Emel Tahir, Department of Otolaryngology, Ondokuz Mayıs University School of Medicine, Kurupelit Körfez, Atakum, Samsun, Turkey E-mail: [email protected]
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Abstract

Objective

To determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management.

Methods

A retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears.

Results

Out of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent).

Conclusion

Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

Keywords

Hearing Loss, UnilateralDeafnessEar, InnerCochlear Nerve
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 134 , Issue 6 , June 2020 , pp. 509 - 518
Copyright
Copyright © The Author(s), 2020. Published by Cambridge University Press

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Footnotes

Dr E Tahir takes responsibility for the integrity of the content of the paper

Presented at the 10th International Conference on Cholesteatoma and Middle Ear Surgery (‘CHOLE’ conference), 5–8 June 2016, Edinburgh, Scotland, UK, and published as supplementary material in The Journal of Laryngology & Otology 2016;130(suppl 3):S33.

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