Abstract
Inner ear malformations (IEM) represent approximately 20% of congenital hearing loss cases based on radiology [1, 2]. Majority of these patients have bilateral severe to profound hearing loss and are candidates for cochlear implantation. Those cases with severe malformations may require modifications during the surgical approach for implant placement. Decision-making between cochlear implantation (CI) and auditory brainstem implantation (ABI) may also be challenging in some cases of IEMs.
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References
Sennaroglu L. Cochlear implantation in inner ear malformations–a review article. Cochlear Implants Int. 2010;11(1):4–41.
Lemmerling MM, et al. Normal modiolus: CT appearance in patients with a large vestibular aqueduct. Radiology. 1997;204(1):213–9.
Sennaroglu L, et al. Auditory brainstem implantation in children and non-neurofibromatosis type 2 patients: a consensus statement. Otol Neurotol. 2011;32(2):187–91.
Sennaroglu L. Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int. 2016;17(1):3–20.
Sennaroglu L, Ba** MD. Classification and current management of inner ear malformations. Balkan Med J. 2017;34(5):397–411.
Ozgen B, et al. Complete labyrinthine aplasia: clinical and radiologic findings with review of the literature. AJNR Am J Neuroradiol. 2009;30(4):774–80.
Sennaroglu L. Cochlear implantation in inner ear malformations–a review article. Cochlear Implants Int. 2009;
McElveen JT Jr, et al. Cochlear implantation in common cavity malformations using a transmastoid labyrinthotomy approach. Laryngoscope. 1997;107(8):1032–6.
Beltrame MA, et al. Double posterior labyrinthotomy technique: results in three Med-El patients with common cavity. Otol Neurotol. 2005;26(2):177–82.
Sennaroglu L, et al. Long-term results of ABI in children with severe inner ear malformations. Otol Neurotol. 2016;37(7):865–72.
Pamuk G, et al. Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection. J Laryngol Otol. 2021:1–7.
Sennaroglu L, et al. Cochlear hypoplasia type four with anteriorly displaced facial nerve canal. Otol Neurotol. 2016;37(10):e407–9.
Sennaroglu L, Tahir E. A novel classification: anomalous routes of the facial nerve in relation to inner ear malformations. Laryngoscope. 2020;
Sennaroglu L, Atay G, Ba** MD. A new cochlear implant electrode with a “cork”-type stopper for inner ear malformations. Auris Nasus Larynx. 2014;41(4):331–6.
Sennaroglu L, et al. Simultaneous cochlear and auditory brainstem implantation in children with severe inner ear malformations: initial surgical and audiological results. Otol Neurotol. 2020;41(5):625–30.
Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002;112(12):2230–41.
Sennaroglu L, Saatci I. Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol. 2004;25(4):520–9. discussion 529.
Phelps PD, King A, Michaels L. Cochlear dysplasia and meningitis. Am J Otol. 1994;15(4):551–7.
Sennaroglu L, Ba** MD. Management of stapes footplate fistula in inner ear malformations. Int J Pediatr Otorhinolaryngol. 2021;140:110525.
Lo WW. What is a ‘Mondini’ and what difference does a name make? Am J Neuroradiol. 1999;20(8):1442–4.
Sennaroglu L. Another evidence for pressure transfer mechanism in incomplete partition two anomaly via enlarged vestibular aqueduct. Cochlear Implants Int. 2018;19(6):355–7.
Ba** MD, et al. The association between modiolar base anomalies and intraoperative cerebrospinal fluid leakage in patients with incomplete partition type-II anomaly: a classification system and presentation of 73 cases. Otol Neurotol. 2018;39(7):e538–42.
Nance WE, et al. X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher. Birth Defects Orig Artic Ser. 1971;7(4):64–9.
Phelps PD, et al. X-linked deafness, stapes gushers and a distinctive defect of the inner ear. Neuroradiology. 1991;33(4):326–30.
Sennaroglu L, Sarac S, Ergin T. Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol. 2006;27(5):615–23.
Talbot JM, Wilson DF. Computed tomographic diagnosis of X-linked congenital mixed deafness, fixation of the stapedial footplate, and perilymphatic gusher. Am J Otol. 1994;15(2):177–82.
Sennaroglu L. Special article: incomplete partition type III. In: Naito Y, editor. Pediatric ear diseases diagnostic imaging atlas and case reports. Karger; 2013. p. 106–8.
Wilkins A, et al. Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss. Arch Otolaryngol–Head Neck Surg. 2012;138(4):383–8.
Sennaroglu L, et al. Consensus statement: long-term results of ABI in children with complex inner ear malformations and decision making between CI and ABI. Cochlear Implants Int. 2016;17(4):163–71.
Ozkan HB, et al. Audiological performance in children with inner ear malformations before and after cochlear implantation: a cohort study of 274 patients. Clin Otolaryngol. 2021;46(1):154–60.
Sennaroglu L, Atay G. Auditory brainstem implantation in children. Curr Otorhinolaryngol Rep. 2013;1:80–91.
Batuk MO, et al. Bimodal stimulation in children with inner ear malformation: one side cochlear implant and contralateral auditory brainstem implant. Clin Otolaryngol. 2020;45(2):231–8.
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Sennaroglu, L. (2022). Classification and Management of Inner Ear Malformations. In: DeSaSouza, S. (eds) Cochlear Implants. Springer, Singapore. https://doi.org/10.1007/978-981-19-0452-3_11
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