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

Tahir E., Bajin M. D. , Jafarov S., Yildirim M. O. , Cinar B. C. , Sennaroglu G., ...More

JOURNAL OF LARYNGOLOGY AND OTOLOGY, vol.134, no.6, pp.509-518, 2020 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 134 Issue: 6
  • Publication Date: 2020
  • Doi Number: 10.1017/s0022215120001036
  • Page Numbers: pp.509-518


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.