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

Tahir, E; Bajin, M D; Jafarov, S; Yıldırım, M Ö; Çınar, B Ç; Sennaroğlu, G; Sennaroğlu, L

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.

TURKEY; RISK of deafness; ACOUSTIC nerve; COCHLEA; COMPUTED tomography; DEAFNESS; EAR canal; INNER ear; MAGNETIC resonance imaging; RETROSPECTIVE studies; DESCRIPTIVE statistics

Journal of Laryngology & Otology, 2020, Vol 134, Issue 6, p509

0022-2151

Academic Journal

10.1017/S0022215120001036