Auditory Brainstem Implants in Children with Inner Ear Anomalies: An Indian Perspective

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

Download Full-text

MIDDLE AND INNER EAR ANOMALIES IN A CONJOINED TWIN

The Laryngoscope ◽

10.1288/00005537-197407000-00014 ◽

1974 ◽

Vol 84 (7) ◽

pp. 1188-1201 ◽

Cited By ~ 6

Author(s):

Makoto Igarashi ◽

Donald B. Singer ◽

Bobby R. Alford ◽

Ted A. Cook

Keyword(s):

Inner Ear ◽

Ear Anomalies

Download Full-text

Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽

10.1093/qjmed/hcab106.007 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Alaa Nasser Hussain Zaher ◽

Tougan Taha Abd El Aziz ◽

Ahmed Samy Abdelrahman

Keyword(s):

Magnetic Resonance Imaging ◽

Computed Tomography ◽

Hearing Loss ◽

Cochlear Implant ◽

Inner Ear ◽

Resonance Imaging ◽

High Resolution Computed Tomography ◽

Vestibular Aqueduct ◽

Incomplete Partition ◽

Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

Download Full-text

Speech production accuracy of children with auditory brainstem implants: A comparison with peers with cochlear implants and typical hearing using Levenshtein Distance

First Language ◽

10.1177/01427237211042216 ◽

2021 ◽

pp. 014272372110422

Author(s):

Jolien Faes ◽

Joris Gillis ◽

Steven Gillis

Keyword(s):

Cochlear Implants ◽

Spontaneous Speech ◽

Auditory Brainstem ◽

Speech Development ◽

Levenshtein Distance ◽

Limited Information ◽

Sensorineural Hearing Impairment ◽

Auditory Brainstem Implants ◽

Considerable Individual Variation ◽

Hearing Children

Auditory brainstem implantation (ABI) is a recent innovation in pediatric hearing restoration in children with a sensorineural hearing impairment. Only limited information is available on the spontaneous speech development of severe-to-profound congenitally hearing-impaired children who received an ABI. The purpose of this study was to investigate longitudinally the accuracy of ABI children’s word productions in spontaneous speech in comparison to the accuracy of children who received a cochlear implant and children with normal hearing. The data of this study consist of recordings of the spontaneous speech of the first three Dutch-speaking children living in Belgium who received an ABI. The children’s utterances were phonemically transcribed and for each word, the distance between the child’s production and the standard adult phonemic transcription was computed using the Levenshtein Distance as a metric. The same procedure was applied to the longitudinal data of the children with CI and the normally hearing children. The main result was that the Levenshtein Distance decreased in the three children with ABI but it remained significantly higher than that of children with typical hearing and cochlear implants matched on chronological age, hearing age, and lexicon size. In other words, the phonemic accuracy increased in the children with ABI but stayed well below that of children without hearing loss and children with cochlear implants. Moreover, the analyses revealed considerable individual variation between the children with ABI.

Download Full-text