From labyrinthine aplasia to otocyst deformity (original) (raw)
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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.
Clinical Course of Pediatric Congenital Inner Ear Malformations
The Laryngoscope, 2000
To determine any factors that could improve the early detection and management of congenital inner ear malformations. Study Design: A retrospective review was performed of all patients with a diagnosis of inner ear malformation at Loyola University Medical Center (LUMC) and the Hospital for Sick Children (HSC) between 1987 and 1995. Clinical records and audiometric data were accumulated. One neuroradiologist reviewed every temporal bone computed tomography (CT) scan. Methods: Forty-six pediatric patients with congenital inner ear anomalies evaluated at two tertiary care hospitals. Results: The average patient age at initial assessment was 25.7 months. A family history of hearing loss was noted in only five patients (12.8%). A major nonotological deformity was seen in 41% of patients. The average hearing threshold was 88 dB. All three patients with sudden hearing loss had vestibular aqueduct enlargement. Two of the three patients with common cavity anomalies had a history of recurrent meningitis. Twenty-seven patients had a vestibular aqueduct deformity, the most frequent radiographic abnormality in the series. Conclusions: Because inner ear malformation was diagnosed after 24 months of age in a significant percentage of patients, we recommend increased parental education and vigilance by primary care practitioners. Universal newborn screening may be the key to earlier detection of these infants. For children with idiopathic sensorineural hearing loss, we recommend a temporal bone CT scan. Patients with vestibular aqueduct enlargement must be counseled about the risk of progressive sensorineural hearing loss, meningitis, and the need to avoid contact sports. Patients with common cavity abnormalities should be considered for exploratory tympanotomy and also educated about the risk for meningitis.
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.
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,
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.
Congenital malformations of the ear
The Indian Journal of Pediatrics, 1992
Tile external ear develops from I and II branchial arches commencing on 38th day of fetal life. The middle ear is formed from the ends of Ist pharyngeal puch and the surrounding mesenchyme, which also is part of the I and II branchial arches. The congenital defects of the external and middle ear usually occurs in combination, and many times with congenital defects of other systems. The cochlear functions i.e. bone conduction is normal in 50% of these cases, thus rehabilitation of these patients with congenital anomalies of external and middle ear is possible. The coexistence of congenital aural atresia with varying degrees of malformation of inner ear may be more frequent than generally assumed. Moderate and severe forms of congenital aural atresia area encountered in about 1 in 10,000 to 20,000 individualJ Tile more severe forms of congenital auricular malformation are always associated with meatal atresia, whereas meatal atresia may, in a few cases be seen in patients with a normal pinna. Atresia of the meatus may be membranous or osseus, membranous atresia is much less common and is characterised by rudimentary cartilagenous canal sep~ated from the middle ear by a dense structure of con
Pakistan Journal of Medical Sciences
Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and l...