Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings

2017 ◽  
Vol 156 (5) ◽  
pp. 783-793 ◽  
Author(s):  
Zachary Farhood ◽  
Shaun A. Nguyen ◽  
Stephen C. Miller ◽  
Meredith A. Holcomb ◽  
Ted A. Meyer ◽  
...  
Keyword(s):  
Speech Perception ◽  
Facial Nerve ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Classification Systems ◽  
Closed Set ◽  
Inner Ear Malformations ◽  
Open Set ◽  
Ear Malformations ◽  
The Impact

Objective (1) To analyze reported speech perception outcomes in patients with inner ear malformations who undergo cochlear implantation, (2) to review the surgical complications and findings, and (3) to compare the 2 classification systems of Jackler and Sennaroglu. Data Sources PubMed, Scopus (including Embase), Medline, and CINAHL Plus. Review Methods Fifty-nine articles were included that contained speech perception and/or intraoperative data. Cases were differentiated depending on whether the Jackler or Sennaroglu malformation classification was used. A meta-analysis of proportions examined incidences of complete insertion, gusher, and facial nerve aberrancy. For speech perception data, weighted means and standard deviations were calculated for all malformations for short-, medium-, and long-term follow-up. Speech tests were grouped into 3 categories—closed-set words, open-set words, and open-set sentences—and then compared through a comparison-of-means t test. Results Complete insertion was seen in 81.8% of all inner ear malformations (95% CI: 72.6-89.5); gusher was reported in 39.1% of cases (95% CI: 30.3-48.2); and facial nerve anomalies were encountered in 34.4% (95% CI: 20.1-50.3). Significant improvements in average performance were seen for closed- and open-set tests across all malformation types at 12 months postoperatively. Conclusions Cochlear implantation outcomes are favorable for those with inner ear malformations from a surgical and speech outcome standpoint. Accurate classification of anatomic malformations, as well as standardization of postimplantation speech outcomes, is necessary to improve understanding of the impact of implantation in this difficult patient population.

Cochlear Implantation in Children with Inner Ear Malformations

1998 ◽  
Vol 107 (6) ◽  
pp. 492-500 ◽  
Author(s):  
Audie L. Woolley ◽  
Rodney P. Lusk ◽  
Virginia Jenison ◽  
Robert S. Bahadori ◽  
Barbara S. Stroer ◽  
...  
Keyword(s):  
Speech Perception ◽  
Cochlear Implant ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Csf Leak ◽  
Closed Set ◽  
Inner Ear Malformations ◽  
Open Set ◽  
Ear Malformations ◽  
Csf Leaks

We performed a case study and intervention study, with follow-up of 1 to 5 years, in 4 children with inner ear malformations who underwent implantation of a multichannel cochlear implant (Nucleus, Cochlear Corporation) at ages 3 to 12 years. Malformations included a common cavity deformity, 2 incomplete partitions, and 1 case of isolated bilateral vestibular aqueduct enlargement. One child had a single-channel implant placed at 3 years of age, and this was exchanged for a 22-channel implant at age 9. One child had her implant placed at age 4.5 years, but due to complications from a cerebrospinal fluid (CSF) leak had the initial implant removed and replaced at age 5 years during repair of the CSF leak. Intraoperative findings included a CSF leak at the time of surgery in 3 patients. One patient contracted bacterial meningitis 7 months postimplantation that was thought to be secondary to acute otitis media in the unoperated ear. Bilateral CSF leaks were noted in the middle ear by a lumbar puncture radionuclide and fluorescein dye study. Successful repair of the CSF leaks and reimplantation of the cochlear implant was carried out in this patient. Mapping and programing of the implant was found to be challenging in each of these patients. All patients demonstrated improved performance after implantation. Two patients demonstrated some open-set speech perception. One patient demonstrates improved use of temporal cues in a structured closed set. One patient has achieved no significant speech recognition at this time, but does have improved sound detection and awareness. Cochlear implantation in children with congenital inner ear abnormalities can be a successful method of rehabilitation. It should be recognized that the postoperative speech perception results may be highly variable among patients, and that intraoperative complications may occur.

Speech perception and production in children with inner ear malformations after cochlear implantation

2012 ◽  
Vol 76 (9) ◽  
pp. 1370-1374 ◽  
Author(s):  
Dimitrios Rachovitsas ◽  
George Psillas ◽  
Vasiliki Chatzigiannakidou ◽  
Stefanos Triaridis ◽  
Jiannis Constantinidis ◽  
...  
Keyword(s):  
Speech Perception ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Speech Perception And Production

Sequential Bilateral Cochlear Implantation in a Child with Severe External, Middle, and Inner Ear Malformations: Surgical Considerations and Practical Aspects

ORL ◽  
2021 ◽  
pp. 1-7
Author(s):  
Dejun Zhang ◽  
Yongyi Yuan ◽  
Yu Su ◽  
Guojian Wang ◽  
Chang Guo ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Inner Ear ◽  
Middle Ear ◽  
Cochlear Implantation ◽  
Intraoperative Imaging ◽  
Preoperative Imaging ◽  
Anatomical Landmarks ◽  
Inner Ear Malformations ◽  
Facial Nerve Monitoring ◽  
Ear Malformations

Cochlear implantation (CI) is a safe and beneficial surgery for children with congenital inner ear malformations, with the exception of cochlear nerve aplasia. The combination of microtia with middle and inner ear abnormalities is extremely uncommon and sufficiently severe to make a surgical approach to the cochlea difficult. We report herein the case of a 2-year-old girl who presented with profound bilateral sensorineural hearing loss, congenital aural atresia, microtia, and inner ear malformations. High-resolution computed tomography revealed poor development of the bilateral middle ear spaces, absence of the incus and stapes, aberrant courses of facial nerves, aplastic lateral semicircular canals, and covered round windows. With intraoperative imaging assistance, sequential bilateral CI was performed using a transmastoid approach with no complication. We propose that CI is feasible in patients with severe external and middle ear malformations. However, major malformations increase the risk of complications. As the facial nerve and cochlea are difficult to locate due to the lack of important anatomical landmarks, detailed planning and adequate preparation, including review of the preoperative imaging data, and the use of facial nerve monitoring and intraoperative imaging are very important. In addition, experienced surgeons should perform CI to ensure the success of the operation.

Cochlear implantation in patients afflicted with inner ear malformations – the Pécs experience

2018 ◽  
Vol 16 (4) ◽  
pp. 232-237
Author(s):  
Peter Bako ◽  
Adrienn Nemeth ◽  
Krisztian Molnar ◽  
Tamas Toth ◽  
Kinga Harmat ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

B111 Cochlear implantation in congenital middle and inner ear malformations in children

2011 ◽  
Vol 75 ◽  
pp. 30
Author(s):  
H. Skarzynski ◽  
M. Porowski ◽  
M. Mrówka ◽  
P. Mlotkowska-Klimek ◽  
A. Lorens ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

Role of Electrically Evoked Auditory Brainstem Response in Cochlear Implantation of Children With Inner Ear Malformations

2008 ◽  
Vol 29 (5) ◽  
pp. 626-634 ◽  
Author(s):  
Ana H. Kim ◽  
Paul R. Kileny ◽  
H. Alexander Arts ◽  
Hussam K. El-Kashlan ◽  
Steven A. Telian ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Brainstem Response

The role of preoperative imaging for auditory implants in children

2020 ◽  
Vol 74 (6) ◽  
pp. 1-5
Author(s):  
Małgorzata Burzyńska-Makuch ◽  
Józef Mierzwiński ◽  
Karolina Haber
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Preoperative Imaging ◽  
Surgical Approaches ◽  
Clinical Decisions ◽  
Imaging Studies ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Cochlear Ossification ◽  
Temporal Bones

<b>Introduction:</b> Preoperative imaging, besides audiological evaluation, plays a major role in evaluation of candidacy for auditory implants, and in particular cochlear implants. It is essential to assess whether the basic criteria necessary for implantation are met. Diagnostic imaging is crucial not only in determining candidacy, but also determining the feasibility of cochlear implantation as it allow to anticipate surgical difficulties which could preclude or complicate the implantation of the device. The aim of the study is to present the protocol for the evaluation of preoperative imaging studies with particular focus on the factors potentially affecting clinical decisions in children qualified for cochlear implantation. <br><b>Material and method:</b> Preoperative imaging studies of 111 children performed prior to cochlear implantation were analyzed: high-resolution computed tomography (HRCT) of temporal bones and MRI. The assessment was made according to the presented protocol. <br><b>Results:</b> Pathologies and anomalies identified during the assessment of preoperative imaging studies significantly altered clinical decisions in 30% of patients. In the study group, in 17% of patients inner ear malformations were identified. 2.7% of children were disqualified from a cochlear implantation due to severe congenital inner ear malformations. 9% of the patients have had bacterial meningitis. In 50% of them difficulties related to complete or progressive cochlear ossification occurred. In 4.5% of patients less common surgical approaches other than mastoidectomy with a posterior tympanotomy were applied. <br><b>Discussion:</b> Preoperative imaging allow for the identification of significant pathologies and anomalies affecting qualification decisions and further treatment. HRCT and MRI are complementary to each other for preoperative imaging. The two modalities in combination allow accurate and optimal evaluation of the anatomical structures prior to implantation. Inner ear malformations and cochlear ossification following meningitis are relatively frequently encountered in children qualified for a cochlear implant.

Cochlear Implantation in Children with Congenital Inner Ear Malformations

2004 ◽  
Vol 114 (2) ◽  
pp. 309-316 ◽  
Author(s):  
Craig A. Buchman ◽  
Benjamin J. Copeland ◽  
Kathy K. Yu ◽  
Carolyn J. Brown ◽  
Vincent N. Carrasco ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

scholarly journals Severe and persistent facial nerve stimulation after cochlear implantation in a patient with cochlear–facial dehiscence: a case report

2021 ◽  
Vol 49 (11) ◽  
pp. 030006052110578
Author(s):  
Jingyuan Chen ◽  
Biao Chen ◽  
Lifang Zhang ◽  
Yongxin Li
Keyword(s):  
Computed Tomography ◽  
Case Report ◽  
Facial Nerve ◽  
Inner Ear ◽  
Cochlear Implants ◽  
Nerve Stimulation ◽  
Cochlear Implantation ◽  
Computed Tomography Images ◽  
Ear Malformations ◽  
Facial Nerve Stimulation

Generally, cochlear implants (CIs) are effective in helping patients improve their hearing performance; however, some patients have poor hearing performance owing to facial nerve stimulation (FNS), which is often associated with cochlear anomalies. We report a case with a normal cochlea and severe and persistent FNS owing to cochlear–facial dehiscence (CFD) that affected the CI outcomes. Preoperatively, a careful review of the computed tomography images before CI surgery is necessary not only for patients with otosclerosis and inner ear malformations but also for patients with normal cochlear structures because facial nerve anomalies could be present.

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