OBJECTIVES/HYPOTHESIS Compare outcomes among children with inner ear malformations and/or cochlear nerve deficiency (CND) who have received a cochlear implant (CI). STUDY DESIGN Individual retrospective cohort study from 1993 to 2010. METHODS A select cohort of 76 children was identified. Imaging characteristics, operative findings, complications, mapping parameters, and performance were as...

Introduction: Congenital anomalies of the cochlea and labyrinth can be associated with meningitis and varying degrees of hearing loss or deafness. Despite antibiotics, meningitis remains a life threatening complication.   Case Report: We report a case of recurrent meningitis following episodes of otitis media in a cochlear implantee child with bilateral vestibulocochlear malformation, due to f...

Objectives: To determine the frequency and factors associated with inner ear anomalies among cochlear implant candidates. Method: The cross-sectional study was conducted at Department of Otolaryngology, Aga Khan University Hospital, Karachi, from October 2021 to March 2022, comprised patients either gender aged <18 years who received implants. All subjects underwent computed tomography magne...

Isolated congenital malformations of semicircular canals are rare abnormalities. Most inner ear abnormalities occur in syndromes and are associated with hearing loss. Unilateral or bilateral single aplasia of one semicircular canal does not usually result in vertigo, but these become clinically important if there are clinical complaints of vertigo. Computed tomography imaging and high resolutio...

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVA...

CHD7 is a chromodomain gene mutated in CHARGE syndrome, a multiple anomaly condition characterized by ocular coloboma, heart defects, atresia of the choanae, retarded growth and development, genital hypoplasia, and ear defects including deafness and semicircular canal dysgenesis. Mice with heterozygous Chd7 deficiency have circling behavior and semicircular canal defects and are an excellent an...

Proper morphogenesis of inner ear semicircular canals requires precise regulation of cellular proliferation, epithelial-to-mesenchymal transition, and fusion of epithelial plates. Epigenetic regulation of these processes is not well understood, but is likely to involve chromatin remodeling enzymes. CHD7 is a chromodomain-containing, ATP dependent helicase protein that is highly expressed in the...

Vascular anomalies are categorized into vascular tumours or vascular malformations on the basis of clinical features and histopathology. The literature regarding high flow arteriovenous malformations of the auricle is rare. A case of a patient clinically diagnosed with an arteriovenous malformation of the external ear and was managed with preoperative embolization, surgical excision and reconst...

Abstract Purpose New perspectives on rehabilitation options for inner ear malformations have still been studied in the literature. This study investigated cognitive, language, and motor skills of auditory brainstem implant (ABI) users unimodal bimodal groups. Methods The competency participants was assessed with Bruininks–Oseretsky Motor Proficiency Test‐2 Short Form (BOT2 SF). Language perform...