Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes

Daneshi, Ahmad; Farhadi, Mohammad; Ajalloueyan, Mohammad; Rajati, Mohsen; Hashemi, Seyyed Alireza; Ghasemi, M; Emamdjomeh, Hesamaldin; Asghari, Alimohamad; Mohseni, Mohammad; Mohebbi, Saleh; Hosseinzadeh, Farideh; Mirsalehi, Marjan

doi:10.1016/j.ijporl.2020.109901

Cited by 31 publications

(25 citation statements)

References 30 publications

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“…Sixty-four percent to 100% of the electrodes were responsive, except in one patient in whom the implant was ascertained to have no contact to the inner wall of the cavity and had only 27% of electrodes that were active. The hearing outcomes for cochlear implants in common cavity cases are generally poor, with the worst outcomes among those with cochlear deformations after long-term follow-up [3,[11][12][13].…”

Section: Discussionmentioning

confidence: 99%

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Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Bae

Choi

2022

Clin Exp Otorhinolaryngol

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Objectives. Common cavity deformity is a rare congenital bony labyrinth malformation associated with profound hearing loss. Cochlear implants are widely used for hearing rehabilitation for common cavity deformities; however, the reported prognosis is poor. Due to the deformed anatomical structure, it is important to consider the position of the electrodes to maximize the performance of the cochlear implant. The present study discusses the impact of electrode placement on hearing outcomes. Methods.A retrospective medical chart review of eight common cavity deformity patients (10 cochlear implants) who received cochlear implants was performed at a single university hospital. In all eight patients, implant surgery was performed using single-slit labyrinthotomy. Electrodes wer e manually bent before insertion to prevent misplacement and to reduce physical damage to the neuroepithelium.Results. Four of the 10 electrodes were misplaced, with their tips placed in the anterior semicircular canal or internal auditory canal. However, after implant surgery, all patients-including those with misplaced electrodes-gained auditory perception and improved hearing function. One patient who had electrodes that did not contact the inner wall of the cavity showed limited activity of the electrodes (27%) compared to others (64%-100%).Conclusions. Proper contact of the electrode with the inner wall was more likely to be important for cochlear implant success in cases of common cavity deformity than appropriate placement of the electrode tip.

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Section: Discussionmentioning

confidence: 99%

“…The hearing outcomes for cochlear implants in common cavity cases are generally poor, with the worst outcomes among those with cochlear deformations after long-term follow-up [ 3 , 11 - 13 ]. Our study also identified a poor prognosis, especially in language performance.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Bae

Choi

2022

Clin Exp Otorhinolaryngol

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“…Several authors have [27,28] reported that malformations of inner ear do not preclude cochlear implantation and favorable functional results can be gained but a promising conclusion cannot be made as the included studies had smaller sample sizes and heterogeneous outcome scales. In a recent multicentre study [29] with the largest study population of 107 patients with cochleovestibular anomalies; twenty-one patients had enlarged vestibular aqueduct (19.62%), all of which showed benefit from cochlear implantation. A median CAP score of 5 at two years follow up and a median SIR score of 3 at two years follow up were achieved.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Acoustic and Linguistic Performances Following Cochlear Implantation in Large Vestibular Aqueduct Syndrome (LVAS)

Shakrawal

Sonkhya

Agarwal

et al. 2021

Indian J Otolaryngol Head Neck Surg

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Aim: This study aims to collate the outcomes in acoustic and linguistic performances after cochlear implantation in LVAS. Methods: In a hospital-based prospective interventional study, seven prelingual children with bilateral profound sensorineural hearing loss (SNHL) with LVAS were recruited. They underwent unilateral cochlear implantation between December 2013 and December 2015 in the Department of Otorhinolaryngology and Head Neck Surgery at a tertiary care center. Outcomes of auditory and speech performances were assessed in the form of revised categories of auditory perception (CAP), infant toddler meaningful auditory integration scale (IT-MAIS) and speech intelligibility rating (SIR) scores preoperatively, at one and two years follow up. Results: The mean age of implantation was 4 years. The median revised CAP, IT-MAIS, and SIR scores after one and two years of follow-up were 6, 27, 3, and 11, 36, 5 respectively. The mean follow-up duration was 21.3 months. Conclusion: Pre op median revised CAP, IT-MAIS, and SIR scores of 0, 1, 1 showed improvement to 11, 36, 5 at two years follow up which was statistically significant. Hence, we conclude that good functional outcomes post-operatively advocate the significance of cochlear implantation in LVAS.

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“…Presently, more than 5% of the world's population (~466 million people) suffer from the HI; according to the World Health Organization, this rate is expected to increase to more than 900 million by 2050 4 . The effective therapeutic procedure for HI is still based on hearing amplification and cochlear implantation, though they cannot restore the natural hearing power 5,6 . Understanding the molecular mechanisms of the hearing process not only does shed light on the intracellular mechanisms but also makes it possible to manipulate them for HI treatment 7 …”

Section: Introductionmentioning

confidence: 99%

How Transmembrane Inner Ear (TMIE) plays role in the auditory system: A mystery to us

Farhadi

Razmara

Balali

et al. 2021

J Cellular Molecular Medi

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Different cellular mechanisms contribute to the hearing sense, so it is obvious that any disruption in such processes leads to hearing impairment that greatly influences the global economy and quality of life of the patients and their relatives. In the past two decades, transmembrane inner ear (TMIE) protein has received a great deal of research interest because its impairments cause hereditary deafness in humans. This evolutionarily conserved membrane protein contributes to a fundamental complex that plays role in the maintenance and function of the sensory hair cells. Although the critical roles of the TMIE in mechanoelectrical transduction or hearing procedures have been discussed, there are little to no review papers summarizing the roles of the TMIE in the auditory system. In order to fill this gap, herein, we discuss the important roles of this protein in the auditory system including its role in mechanotransduction, olivocochlear synapse, morphology and different signalling pathways; we also review the genotype‐phenotype correlation that can per se show the possible roles of this protein in the auditory system.

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Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes

Cited by 31 publications

References 30 publications

Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Outcomes of Acoustic and Linguistic Performances Following Cochlear Implantation in Large Vestibular Aqueduct Syndrome (LVAS)

How Transmembrane Inner Ear (TMIE) plays role in the auditory system: A mystery to us

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