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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: emeltahir@hotmail.com
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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.

References

Ead, B, Hale, S, DeAlwis, D, Lieu, JE. Pilot study of cognition in children with unilateral hearing loss. Int J Pediatr Otorhinolaryngol 2013;77:1856–60CrossRefGoogle ScholarPubMed
Arndt, S, Prosse, S, Laszig, R, Wesarg, T, Aschendorff, A, Hassepass, F. Cochlear implantation in children with single-sided deafness: does aetiology and duration of deafness matter? Audiol Neurootol 2015;20(suppl 1):21–3010.1159/000380744CrossRefGoogle ScholarPubMed
Hassepass, F, Bulla, S, Aschendorff, A, Maier, W, Traser, L, Steinmetz, C et al. The bonebridge as a transcutaneous bone conduction hearing system: preliminary surgical and audiological results in children and adolescents. Eur Arch Otorhinolaryngol 2015;272:2235–41CrossRefGoogle ScholarPubMed
Mertens, G, De Bodt, M, Van de Heyning, P. Cochlear implantation as a long-term treatment for ipsilateral incapacitating tinnitus in subjects with unilateral hearing loss up to 10 years. Hear Res 2016;331:1–6CrossRefGoogle ScholarPubMed
Sennaroğlu, L, Bajin, MD. Classification and current management of inner ear malformations. Balkan Med J 2017;34:397–411CrossRefGoogle ScholarPubMed
Friedmann, DR, Ahmed, OH, McMenomey, SO, Shapiro, WH, Waltzman, SB, Roland, JT Jr. Single-sided deafness cochlear implantation: candidacy, evaluation, and outcomes in children and adults. Otol Neurotol 2016;37:154–6010.1097/MAO.0000000000000951CrossRefGoogle ScholarPubMed
Bess, FH, Klee, T, Culbertson, JL. Identification, assessment, and management of children with unilateral sensorineural hearing loss. Ear Hear 1986;7:43–51CrossRefGoogle ScholarPubMed
Welsh, LW, Welsh, JJ, Rosen, LF, Dragonette, JE. Functional impairments due to unilateral deafness. Ann Otol Rhinol Laryngol 2004;113:987–93CrossRefGoogle ScholarPubMed
Song, JJ, Choi, HG, Oh, SH, Chang, SO, Kim, CS, Lee, JH. Unilateral sensorineural hearing loss in children: the importance of temporal bone computed tomography and audiometric follow-up. Otol Neurotol 2009;30:604–8CrossRefGoogle ScholarPubMed
Propst, EJ, Greinwald, JH, Schmithorst, V. Neuroanatomic differences in children with unilateral sensorineural hearing loss detected using functional magnetic resonance imaging. Arch Otolaryngol Head Neck Surg 2010;1361:22–610.1001/archoto.2009.208CrossRefGoogle Scholar
Simons, JP, Mandell, DL, Arjmand, EM. Computed tomography and magnetic resonance imaging in pediatric unilateral and asymmetric sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2006;132:186–9210.1001/archotol.132.2.186CrossRefGoogle ScholarPubMed
Bamiou, DE, Savy, L, O'Mahoney, C, Phelps, P, Sirimanna, T. Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management. Int J Pediatr Otorhinolaryngol 1995;51:91–910.1016/S0165-5876(99)00261-XCrossRefGoogle Scholar
McClay, JE, Booth, TN, Parry, DA, Johnson, R, Roland, P. Evaluation of pediatric sensorineural hearing loss with magnetic resonance imaging. Arch Otolaryngol Head Neck Surg 2008;134:945–5210.1001/archotol.134.9.945CrossRefGoogle ScholarPubMed
Usami, SI, Kitoh, R, Moteki, H, Nishio, SY, Kitano, T, Kobayashi, M et al. Etiology of single-sided deafness and asymmetrical hearing loss. Acta Otolaryngol 2017;137(suppl 565):2–7CrossRefGoogle ScholarPubMed
Fatterpekar, GM, Mukherji, SK, Alley, J, Lin, Y, Castillo, M. Hypoplasia of the bony canal for the cochlear nerve in patients with congenital sensorineural hearing loss: initial observations. Radiology 2000;2151:243–610.1148/radiology.215.1.r00ap36243CrossRefGoogle Scholar
Masuda, S, Usui, S, Matsunaga, T. High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol 2013;77:228–32CrossRefGoogle ScholarPubMed
Yi, JS, Lim, HW, Kang, BC, Park, SY, Park, HJ, Lee, KS. Proportion of bony cochlear nerve canal anomalies in unilateral sensorineural hearing loss in children. Int J Pediatr Otorhinolaryngol 2013;77:530–3CrossRefGoogle ScholarPubMed
Nakano, A, Arimoto, Y, Matsunaga, T. Cochlear nerve deficiency and associated clinical features in patients with bilateral and unilateral hearing loss. Otol Neurotol 2013;34:554–8CrossRefGoogle ScholarPubMed
Purcell, PL, Shinn, JR, Coggeshall, SS, Phillips, G, Paladin, A, Sie, KCY et al. Progression of unilateral hearing loss in children with and without ipsilateral cochlear nerve canal stenosis: a hazard analysis. Otol Neurotol 2017;38:138–44CrossRefGoogle ScholarPubMed
van Beeck Calkoen, EA, Sanchez Aliaga, E, Merkus, P, Smit, CF, van de Kamp, JM, Mulder, MF et al. High prevalence of abnormalities on CT and MR imaging in children with unilateral sensorineural hearing loss irrespective of age or degree of hearing loss. Int J Pediatr Otorhinolaryngol 2017;97:185–91CrossRefGoogle ScholarPubMed
Haffey, T, Fowler, N, Anne, S. Evaluation of unilateral sensorineural hearing loss in the pediatric patient. Int J Pediatr Otorhinolaryngol 2013;77:955–8CrossRefGoogle ScholarPubMed
Clemmens, CS, Guidi, J, Caroff, A, Cohn, SJ, Brant, JA, Laury, AM et al. Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg 2013;149:318–25CrossRefGoogle ScholarPubMed
Huang, BY, Roche, JP, Buchman, CA, Castillo, M. Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency. Am J Neuroradiol 2010;31:1972–9CrossRefGoogle ScholarPubMed
Birdane, L, İncesulu, A, Özüdoğru, E, Cingi, C, Caklı, H, Gürbüz, MK et al. Evaluation of the vestibular system and etiology in children with unilateral sensorineural hearing loss. J Int Adv Otol 2016;12:161–5CrossRefGoogle ScholarPubMed
Dumper, J, Hodgetts, B, Liu, R, Brandner, N. Indications for bone-anchored hearing aids: a functional outcomes study. J Otolaryngol Head Neck Surg 2009;38:96–105Google ScholarPubMed
Vermeire, K, Van de Heyning, P. Binaural hearing after cochlear implantation in subjects with unilateral sensorineural deafness and tinnitus. Audiol Neurotol 2009;143:163–71CrossRefGoogle Scholar
Alemi, AS, Chan, DK. Progressive hearing loss and head trauma in enlarged vestibular aqueduct: a systematic review and meta-analysis. Otolaryngol Head Neck Surg 2015;153:512–17CrossRefGoogle ScholarPubMed