Cochlear implantation in children with congenital inner ear malformations (original) (raw)
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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.
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.
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.
Cochlear Implantation in Inner Ear Malformations — A Review Article
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.
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.
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.
Otology & Neurotology, 2020
Objective: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear. Intervention(s): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear. Main Outcome Measures: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed. Results: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome. Conclusions: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.
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.
2015
Introduction: The cochlear implantation in patients with inner ear malformation has always been a challenge even to the most experienced clinicians. We present the case of a child of 8 years old with profound bilateral sensorineural hearing loss and the absence of language development with indication of cochlear implantation as single solution for hearing and speech rehabilitation. Methods: The audiological assessment indicates the cochlear implantation. Preoperative CT scan revealed a bilateral inner ear malformation (cochlear common cavity deformity on the right side, cochlear aplasia on the left ear and bilateral vestibular malformation). Brain magnetic resonance showed the presence of auditory nerve only to the right side. The girl was implanted on the right ear in the common cavity with an Advanced Bionics device, HiRes90K with HiFocus1j electrode. Results: The insertion of the portelectrode inside the malformed inner ear was confirmed by X-ray. Audiological evaluation had show...
Cochlear Implants in Forty-Eight Children with Cochlear and/or Vestibular Abnormality
Audiology and Neurotology, 2011
CT and MRI scans for 48 children with cochlear and/or vestibular abnormality were classified in decreasing severity; common cavity, Mondini plus enlarged vestibular aqueduct, Mondini dysplasia alone and enlarged vestibular aqueduct alone. No significant relationship between degree of cochlea abnormality and surgical issues (cerebrospinal fluid gusher, depth of insertion, number of electrodes) or speech perception/language outcomes was found. A significant relationship was observed between cerebrospinal fluid gusher and partial electrode insertion, fewer active electrodes and poorer sentence understanding. Optimum language outcomes were associated with younger age at implant.