Etiological Factors and Audiological Evaluation of Children with Congenital Unilateral Hearing Loss

Year 2021, , 39 - 51, 30.04.2021

İlkem Kara Mine Baydan , Bilgehan Böke Gamze Atay Burçe Özgen Mocan , Gülsüm Genç

https://doi.org/10.21020/husbfd.750228

Abstract

Objectives: The aim of the present study is to evaluate the audiological, radiological, and etiological in terms of clinical findings relating to babies and children with congenital unilateral hearing loss. Materials and Methods: Audiometric tests, tympanometric and acoustic reflex measurements, otoacoustic emission tests, and auditory brainstem response assessments were conducted. Twenty-nine babies and children (13 F ;16 M) diagnosed with congenital unilateral hearing loss, between the ages of 3–87 months, were included in this study. Results: Of these patients, 65.5% (n:19) were diagnosed with sensorineural hearing loss and 34.5% (n:10) with conductive hearing loss. Of the subjects with sensorineural hearing loss, 57.9% were diagnosed with profound hearing loss. Of the subjects with conductive hearing loss, 7 of the 10 (70%) had been diagnosed with microtia. Of the patients with conductive hearing loss, all those who had received CT scans were diagnosed with ossicular chain malformations. The most frequent risk factor for sensorineural hearing loss is intermarriage (26.3%), whereas the most frequent risk factor for conductive hearing loss is craniofacial anomalies (30%). Conclusion: The interdisciplinary work in otology, audiology, and radiology is essential for early diagnosis and effective treatment of congenital unilateral hearing loss cases.

Keywords

unilateral hearing loss, congenital, audiologic findings

References

• 1.Gordon K, Papsin B. Special edition on unilateral deafness and hearing loss: An introduction and overview. Hearing research. 2019;372:1. 2.Laury AM, Casey S, McKay S, Germiller JA. Etiology of unilateral neural hearing loss in children. International journal of pediatric otorhinolaryngology. 2009;73(3):417-27. 3.Fitzpatrick EM, Gaboury I, Durieux-Smith A, Coyle D, Whittingham J, Nassrallah F. Auditory and language outcomes in children with unilateral hearing loss. Hearing research. 2019;372:42-51. 4.van Wieringen A, Boudewyns A, Sangen A, Wouters J, Desloovere C. Unilateral congenital hearing loss in children: Challenges and potentials. Hearing research. 2019;372:29-41. 5.Newton VE. Paediatric audiological medicine: John Wiley & Sons; 2008. 6.Vartiainen E, Karjalainen S. Prevalence and etiology of unilateral sensorineural hearing impairment in a Finnish childhood population. International journal of pediatric otorhinolaryngology. 1998;43(3):253-9. 7.Genç GA, Konukseven Ö, Muluk NB, Kirkim G, Başar FS, Tuncer Ü, et al. Features of unilateral hearing loss detected by newborn hearing screening programme in different regions of Turkey. Auris Nasus Larynx. 2013;40(3):251-9. 8.Jakubíková J, Kabátová Z, Pavlovčinová G, Profant M. Newborn hearing screening and strategy for early detection of hearing loss in infants. International journal of pediatric otorhinolaryngology. 2009;73(4):607-12. 9.Brookhouser PE, Worthington DW, Kelly WJ. Unilateral hearing loss in children. The Laryngoscope. 1991;101(12):1264-72. 10.2010 Summary of Laterality, Type and Severiy of Identified Hearing Losses: By Ear. 2010 statistics at glance. 2010. 11.Yelverton JC, Dominguez LM, Chapman DA, Wang S, Pandya A, Dodson KM. Risk factors associated with unilateral hearing loss. JAMA Otolaryngology–Head & Neck Surgery. 2013;139(1):59-63. 12.Dodson KM, Georgolios A, Barr N, Nguyen B, Sismanis A, Arnos KS, et al. Etiology of unilateral hearing loss in a national hereditary deafness repository. American journal of otolaryngology. 2012;33(5):590-4. 13.Forrester MB, Merz RD. Descriptive epidemiology of anotia and microtia, Hawaii, 1986–2002. Congenital anomalies. 2005;45(4):119-24. 14.Cama E, Inches I, Muzzi E, Sadushi O, Santarelli R, De Colle W, et al. Temporal bone high-resolution computed tomography in non-syndromic unilateral hearing loss in children. ORL. 2012;74(2):70-7. 15.Yi JS LH, 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(4):530-3. 16.Bartel-Friedrich S, Wulke C. Classification and diagnosis of ear malformations. GMS current topics in otorhinolaryngology, head and neck surgery. 2007;6. 17. Clemmens CS, Guidi J, Caroff A, Cohn SJ, Brant JA, Laury AM, et al. Unilateral cochlear nerve deficiency in children. Otolaryngology--Head and Neck Surgery. 2013;149(2):318-25. 18.Nicholas BD, Kesser BW. Unilateral Aural Atresia: Current Management Issues and Results. Current Otorhinolaryngology Reports. 2013;1(2):92-8. 19.Masuda S, Usui S, Matsunaga T. High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss. International journal of pediatric otorhinolaryngology. 2013;77(2):228-32. 20.Song J-J, 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. Otology & Neurotology. 2009;30(5):604-8. 21.Radyasyon Güvenlik Kılavuzu. (2013)2013.