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International Bulletin of Otorhinolaryngology

Hearing Loss Etiology in Patients Submitted to Cochlear Implant

Thais Pontes, Clarisse Loures, Oswaldo Laércio Mendonça Cruz


Introduction: Hearing impairment is the most common sensorial disorder. Deafness has several etiologies. It is important to investigate them, not only because some may lead to lower performance with a cochlear implant, but also to define preventive strategies. Objective: To determine deafness etiology among patients submitted to cochlear implantation.
Methods: Retrospective sampling of patients submitted to cochlear implantation between 2006 and 2017 in a Brazilian referral hearing impairment center. Patients were grouped into post-lingual or pre-lin-gual deafness and an etiological profile was outlined for each group. Results: 159 patients were evaluated, 74 post-lingual and 85 pre-lingual. In post-lingual group, the most common cause of hearing impairment was meningitis (n = 16/21,62%). Other etiologies were: non-syndromic genetic hearing loss (n = 12/16,22%), otosclerosis (n = 7/9,46%), autoimmune (n = 5/6,76%), viral infections – mumps and measles (n = 3/4,05%), Alport syndrome (n = 2/2,7%), Meniиreґs disease (n = 2/2,7%), ototoxicity (n = 2/2,7%), enlarged vestibular aqueduct (n = 2/2,7%), otitis media complications (n = 2/2,7%), trauma (n = 2/2,7%), lues (n = 1/1,35%), vestibular schwannoma (n = 1/1,35%), stroke (n = 1/1,35%), auditory neuropathy (n = 1/1,35%) and 15 patients (20,27%) had undefined etiology. In pre-lingual group, non-syndromic genetic hearing loss was the most prevalent cause (n = 22/25,88%), followed by perinatal complications (n = 20/23,53%), congenital infections – cytomegalovirus, rubella and mumps (n = 8/9,41%), genetic syndromes such as Waardenburg (n = 5/5,88%), meningitis (n = 5/5,88%), malformation – mostly incomplete partition type II (n = 3/3,53%), auditory neuropathy (n = 3/3,53%), ototoxicity (n = 2/2,35%) and 17 patients had undefined causes (20%).
Conclusion: In our population, the most frequent etiology for post-lingual deafness was meningitis and for pre-lingual deafness was non-syndromic genetic causes.


Deafness, Etiology, Cochlear Implantation

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Mielczarek M, Zakrzewska A, Olszewski J. GJB2 sequencing in deaf and profound sensorineural hearing loss children. Otolaryngol Pol. 2016; 70: 21–5.

Calháu CMDF, Lima LRP, Reis AMCS, Capistrano AKB, Lima DVSP, Calháu ACDF, et al. Etiology profile of the patients implanted in the cochlear implant program. Braz J Otorhinolaryngol. 2011; 77: 13–8.

Leal AF. Screening of patients for cochlear implant through a questionnaire online. Group profile of patients pre and peri lingual not summoned. Int Arch Otorhinolaryngol. 2010; 14: 184–91.

Martins MBB, Lima FVF, Júnior RCS, Santos ACG, Barreto VMP, De Jesus EPF. Cochlear implants: our experience and literature review. Int Arch Otorhinolaryngol. 2012; 16: 476–81.

Petersen NK, Jørgensen AW, Ovesen T. Prevalence of various etiologies of hearing loss among cochlear implant recipients: systematic review and meta-analysis. Int. J. Audiol. 2015; 52: 924–32.

Pedrett MDS, Moreira SC. Profile of cochlear implant users of the city of Manaus. Int Arch Otorhinolaryngol. 2012; 16: 452–9.

Chen MM, Oghalai JS. Diagnosis and management of congenital sensorineural hearing loss. Curr Treat Options Pediatr. 2016; 2: 256–65.

Xiong Y, Zhong M, Chen J, Yan YL, Lin XF, Li X. Effect of GJB2 235delC and 30-35delG genetic polymorphisms on risk of congenital deafness in a Chinese population. Genet Mol Res. 2017; 16.

Inscoe JR, Bones C, Inscoe JR, Bones C. Additional difficulties associated with aetiologies of deafness : outcomes from a parent questionnaire of 540 children using cochlear implants. Cochlear Implants Int. 2016; 17: 21–30.

Abdurehim Y, Lehmann A, Zeitouni AG. Predictive value of GJB2 mutation status for hearing outcomes of pediatric cochlear implantation. Otolaryngol Head Neck Surg. 2017; 157: 16–24.

Noguchi Y, Fukuda S, Fukushima K, Gyo K. A nationwide study on enlargement of the vestibular aqueduct in Japan. Auris Nasus Larynx. 2017; 44: 33–39.

Aldhafeeri AM, Alsanosi AA. Prevalence of inner ear anomalies among cochlear implant candidates. Saudi Med J. 2016; 37: 1096–100.

Bayrak F, Catli T, Atsal G, Tokat T, Olgun L. Waardenburg syndrome: an unusual indication of cochlear implantation experienced in 11 patients. J Int Adv Otol. 2017; 13: 230–2.

Nichani J, Bruce I, Mawman D, Khwaja S, Ramsden R, Green K. Cochlear implantation in patients deafened by ototoxic drugs. Cochlear Implant Int. 2013; 14: 207–12.

West N, Brand M, Foghsgaard S, Cayé-thomasen P. Surgical results and complications of cochlear implantation in far-advanced otosclerosis. J Int Adv Otol. 2017; 13: 304-7.

Prenzler NK, Bültmann E, Giourgas A, Steffens M, Salcher RB, Stolle S, et al. Cochlear implantation in patients with definite Meniere’s disease. Eur Arch Otorhinolaryngol. 2017; 274: 751–6.

Kaga K. Auditory nerve disease and auditory neuropathy spectrum disorders. Auris Nasus Larynx. 2016; 43: 10–20.



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