Cochlear Implantation in Extraordinary Cases

Clinical Otolaryngology, 2010

Dear Editor, Cochlear implantation in congenital and acquired deafness is an approved auditory rehabilitation treatment. Malformations are of special interest nowadays, as the indication for cochlear implant surgery is expanding. Inner-ear malformations constitute about 20% of congenital sensorineural hearing loss. 1 In a retrospective evaluation, we found 12% malformations in children and 3% malformations in adults. 2 In 1987, Jackler 1 presented a classification of inner-ear malformations, based on the embryological genesis. Sennaroglu and Saatci 3 suggested an extension, based on the initial classification. The basic classification distinguishes malformations of the cochlea and vestibular labyrinth, and additionally abnormalities of the internal auditory canal (IAC). This classification provides an excellent basis for detecting malformations of the inner ear. Nevertheless, it must be expanded to include malformations due to syndromes, which, in their complexity, are not simple to classify, for example innerear malformations in Mondini dysplasia, CHARGE-syndrome and X-linked deafness syndrome. We used these classifications in 29 patients as a basis for a newly developed flow chart which enabled us to preoperatively calculate adaptation of the surgical procedure according to the extent of the malformations. The description of our flow chart is based on two patients with complex malformations of the labyrinth.

Hearing, Balance and Communication, 2018

Purpose: To successfully analyze the surgical and audiological results of cochlear implantation in patients afflicted with inner ear malformations. This study briefly presents our surgical strategies, the selected implants and the audiological results. Method: Objective hearing threshold was measured using an auditory brainstem response and middle latency response audiometry. Structural imaging of high resolution temporal bone computer tomography and inner ear MR scan was performed in support of all patients. Images revealed bilateral cochlear malformation in seven patients. Three ears with common cavity deformity, two ears with incomplete partition type I and three ears suffering from cochlear hypoplasia were all rehabilitated using cochlear implantation. The type of the electrode and the surgical technique were individually determined reflective of the type of malformation. The success of the surgery and potential postoperative complications were duly noted and fully registered. Postoperative audiological measurements were performed and the results were analyzed. Results: The average audiological follow-up was 40.3 months. The average free field, pure-tone threshold of speech frequencies (0.5-3 kHz) gained postoperatively were within the range of 32.5-41.6 dB. In two of the procedures, a cerebrospinal fluid gusher did occur and was successfully managed intraoperatively, all without any postoperative complications. Conclusion: Temporal bone high resolution CT and inner ear MRI imaging are mandatory to detect and characterize the inner ear malformation. Early implantation of malformed cochlea is crucial towards achieving ideal postoperative audiological results. Notably, an intraoperative gusher is not regarded as a contraindication of the cochlear implantation.

International Journal of Pediatric Otorhinolaryngology, 2008

The objective of this retrospective study is to evaluate the efficiency of hyperosmolar therapy for cerebrum spinal fluid (CSF) leakage in cochlear implant (CI) surgery in children with inner ear malformations. Between 1991 and 2006, 490 cochlear implantations were performed in Armand Trousseau Children's Hospital. Thirty-seven patients (7.5%) had inner ear malformation. They were classified as isolated enlargement of the vestibular aqueduct (EVA) (18 cases), incomplete partition (IP) (11 cases), common cavity (CC) (1 case) and variable canal and vestibular malformations (VSCC) (7 cases). A hyperosmolar protocol was applied during surgery to 13 patients after 2003 (Gp) to be compared to the 24 patients without treatment previously to this date (G0). Mean age at implant CI was 8.1 years (1-20 years), mean follow up was 3.9 years (1 month-15 years). Per operative observations were collected for all patients with an empiric method of evaluation of the leakage. A grading using five steps ranged from Grade 0 (no leak) to Grade 4 (gusher). Grading, complications and perceptive results in closed and open set word (Lafon lists) at respectively preoperatively, at 3 and 24 months were gathered and compared between the two groups. Important per operative leak was observed (Grade 4) in 24.3% cases (9/37) of Grade 4, 88.8% of them in G0 (8/9). In 66.6% cases there was a severe dysplasia (CC or IP) (6/9), to be compared to the 21.4% of cases of severe dysplasia with Grade < 3 (6/28) ( p = 0.02). Grade 4 was seen in 45% cases of IP (5/11); it represented 33.3% of the IP in Gp (1/3), and 50% of the IP in G0 (4/8) ( p > 0.05). Grade 4 was seen in 16.6% cases of EVA (3/18); there were no Grade 4 observed in Gp (0/10), and 37.5% cases of EVA in G0 (3/8) ( p = 0.04). Grade 4 was observed in 100% case in CC in the G0 (1/1). Severe complications were misplacement of the electrode in one case (G0), persistent leakage in one case (G0) and meningitis in one case (Gp). Vertigo was observed in 29.7% of cases (11/37) in this population, 72.7% of them in G0 * Corresponding author at: Service d'Oto-rhino-laryngologie et de chirurgie cervico-faciale, Hôpital d'enfants Armand-Trousseau,

Cochlear Implants International, 2004

Expanding selection criteria and increasing numbers of cochlear implantations have revealed several challenging cases with regard to surgery and medical point of view. Cochlear implantation of congenitally deaf children with inner-ear malformations may involve difficulties in preoperative evaluation, surgical approach and postoperative follow-up. Cochlear nerve aplasia (as evidence of only one existing nerve in three dimensional magnetic resonance imaging) and hypoplastic cochleas are among the most difficult cases and sometimes children are considered unsuitable for cochlear implantation. However, there is always the possibility that hypoplastic cochleas may include ganglion cells and the only nerve in the internal auditory canal (facial nerve) may contain auditory fibres as well. A positive history of auditory experience and evidence of electrical auditory brainstem response or response to the promontory or round window electrical stimulation may imply that a functioning auditory system is in place. Malformations like common cavity deformity, isolated bilateral vestibular aqueduct enlargement and Mondini dysplasia are usually less challenging, although cerebrospinal fluid leak and postoperative meningitis may be a possibility. In cases of post-meningitic deaf patients, ossification (or obliteration) may be a serious problem even if computed tomography and MRI scan show a patent cochlea or minimal changes. Acoustic neuromas and neurofibromatosis type 2 are other challenging cases, as there is always the possibility that the sensorineural hearing loss is secondary to cochlear damage by interference of the tumour to cochlear blood supply. In conclusion, challenging cases are becoming more common as we are expanding selection criteria, and we should be prepared for alternative cochlear implant devices (straight electrode arrays, dual arrays, compressed arrays, etc.), increased rate of possible complications at, and following, surgery, and functional outcome that may vary considerably among implantees.

Otology &amp; Neurotology, 2004

Objectives: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. Study Design: Clinical and audiometric evaluation in 13 patients. Methods: Patient data concerning surgery, postoperative follow-up, and pre-and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. Setting: Tertiary referral center. Patients: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). Results: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. Conclusions: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.

2009

Our study was designed to compare two surgical approaches that are currently employed in cochlear implantation.

2012

UNLABELLED Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss. OBJECTIVE To describe the surgical complications of cochlear implantation. MATERIALS AND METHODS Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach. RESULTS Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 ± 6.7 years for prelingual loss and 12.1 ± 11.6 years for postlingual. Mean follow-up was 3.9 ± 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membr...

The Journal of Laryngology &amp; Otology, 2008

Objective:To describe problems and complications associated with cochlear implantation, and their management, in a Danish patient population comprising both paediatric and adult patients.Design:Retrospective chart review.Setting:Tertiary referral centre.Subjects:Three hundred and thirteen consecutive cochlear implantations were studied. The median age of the study population was 10 years. Sixty per cent of patients were children and 40 per cent were adult; 52 per cent were female and 48 per cent were male.Intervention:Two hundred and ninety-four patients received a Cochlear Nucleus®implant. The remaining 19 received an Advanced Bionics implant.Main outcome measure:Presence of problems and complications after cochlear implantation.Results:Post-operative complications were found in 15.7 per cent of patients. The majority of these complications (11.2 per cent) were minor; 4.5 per cent were major. The major complications included one patient with meningitis, one patient with multiple an...

Cochlear Implants International, 2010

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 metaanalysis 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 categoriesclosed-set words, open-set words, and open-set sentencesand 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.

Journal of Neurological Surgery Part B: Skull Base, 2018

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better ...