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

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.

Twenty years of experience in revision cochlear implant surgery: signs that indicate the need for revision surgery to audiologists.

OBJECTIVE: To report device failures, audiological signs and other reasons for revision cochlear implant surgery, and discuss indications for revision surgery. METHODS: Revision procedures between November 1997 and August 2017 were retrospectively analysed. Over 20 years, 2181 cochlear implant operations were performed, and 114 patients underwent 127 revision operations. RESULTS: The revision rate was 4.67 per cent. The full insertion rate for revision cochlear implant surgery was 88.2 per cent. The most frequent reasons for revision surgery were: device failure (59 per cent), wound breakdown (9.4 per cent) and electrode malposition (8.7 per cent). The device failure rate was: 2.78 per cent for Advanced Bionics, 1.82 per cent for Cochlear and 5.25 per cent for Med-El systems. The number of active electrodes was significantly increased only for Med-El devices after revision surgery. The most common complaints among 61 patients were: gradually decreased auditory performance, sudden internal device shutdown and headaches. CONCLUSION: The most common reason for revision surgery was device failure. Patients should be evaluated for device failure in cases of: no hearing despite appropriate follow up, side effects such as facial nerve stimulation, and rejection of speech processor use in paediatrics. After revision surgery, most patients have successful outcomes.