Cochlear Implantation in Inner Ear Malformations — A Review Article (original) (raw)
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Otolaryngology-Head and Neck Surgery, 2017
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.
ORL
Introduction: There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, cerebrospinal fluid (CSF) leaks, erroneous electrode insertion, or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of inner ear malformations (IEMs) on surgical complications and outcomes of cochlear implantation. Methods: In order to assess the impact of IEMs on cochlear implant (CI) outcomes, 2 groups of patients with similar epidemiological parameters were selected from among 863 patients. Both the study group (patients with an IEM) and control group (patients with a normal inner ear) included 25 patients who received a CI and completed at least 1 year of follow-up. Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Types of surgical complicat...
Cochlear implantation in patients afflicted with inner ear malformations – the Pécs experience
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, 2020
This series plays a role as a clinical reference in the rapidly evolving subspecialty of modern otology and neurotology. Written by prominent academic authorities, this series integrates contents from all fi elds of medicine and covers every aspect of the fi eld, including surgical issues in pediatric audiology, neurotology and neurology, genetic testing, oncological study in auditory and vestibular organs, geriatric audiology and neurotology, and new clinical application of bone conduction hearing etc. Historical developments and unsolved problems of each fi eld will also be described in detail to help readers' understanding.The editors and contributors hope that this book series will contribute to medical residents and experts of otolaryngology and related clinical medicines in the evaluation of patients with otological and neurotological disorders.
Management of cochlear implantation in patients with malformations
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.
Cochlear implantation in children with congenital inner ear malformations
The …, 2004
To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. Study Design: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. Methods: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed-and open-set testing of speech perception were analyzed. Results: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. Conclusions: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.
2020
Objective: To evaluate the auditory perception outcomes of cochlear implant (CI) in children with different types of inner ear malformations (IEMs) and to compare them with CI users with the normal cochlea. Design: Retrospective and prospective data collection. Settings: Tertiary referral hospital. Participants: There were 274 CI users with and without IEMs as two groups. Both groups' chronological age at implantation and duration of cochlear implant usage was matched (±8 months). Main outcome measures: All subjects were evaluated preoperatively and postoperatively with Ling's sound test and auditory perception test battery, which includes the Meaningful Auditory Integration Scale (MAIS), close-set Pattern Perception Test (PPT), and openset Sentence Recognition Test (SRT). Also, children with IEMs were assessed for language development. Results: The incidence of IEMs were incomplete partition-II, 40 (29.19%), incomplete partition-I, 36 (26.2%), cochlear hypoplasia, 26 (18.9%), enlarged vestibular aqueduct, 14 (%10.2), incomplete partition-III, 10 (%7.2), common cavity, 8 (5.8%) and dilatation of vestibule, 3 (2.1%) patients. The significant difference was seen in Ling's sound test and auditory perception test battery scores of children with incomplete partition-I, cochlear hypoplasia, and common cavity (p-value < .005). Conclusion: IEMs group showed different progress according to the type of ear anomaly. Although CI users with enlarged vestibular aqueduct (EVA) had the highest scores, users with common cavity had the lowest scores. Taking these results, caused by anatomical differences, in to account is very critical in follow-ups and rehabilitation programs. Each cochlear implant user should be evaluated according to his/her individual needs. Keypoints • Radiological results play a crucial role in detecting different types of IEMs. • It is critical to evaluate the functional hearing and auditory perception skills of children with different types of IEMs in the first year after surgery. • Depending on our clinical experience, children with IP-I, common cavity, and cochlear hypoplasia are developing slowly, and especially this group should be followed intensively in terms of auditory perception and language skills. • Children with IEMs who did not develop as a result of follow-up, instead of the "wait and see" strategy, should be concluded quickly.
Congenital Malformation of the Inner Ear and Pediatric Cochlear Implantation
Otology & 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.
Cochlear implant and inner ear malformation
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,
European Archives of Oto-Rhino-Laryngology, 2015
Inner ear bone malformations are one cause of profound sensorineural hearing loss. This investigation focused on those affecting the posterior labyrinth, especially enlarged vestibular aqueduct syndrome, which is associated with fluctuating and progressive hearing loss. The objectives of this study were to analyze the behavior of the electrical stimulation, auditory functionality and linguistic development in patients with inner ear malformations involving the posterior labyrinth. The study included ten patients undergoing cochlear implantation (cases: five with enlarged vestibular aqueduct, two with vestibular aqueduct stenosis/aplasia, and three with semicircular canal disorders). Post-implantation, data were gathered on the electrical stimulation threshold and maximum comfort levels and on the number of functioning electrodes. Evaluation of Auditory Responses to Speech (EARS) subtests were used to assess auditory functionality and language acquisition at 6, 12, and 24 months post