Frequency of congenital inner ear malformations among cochlear implant candidates at a tertiary care hospital Pakistan

Abstract

Objectives: To determine the frequency and factors associated with inner ear anomalies among cochlear implant candidates. Method: The cross-sectional study was conducted at the Department of Otolaryngology, Aga Khan University Hospital, Karachi, from October 2021 to March 2022, and comprised patients of either gender aged <18 years who received cochlear implants. All subjects underwent computed tomography and magnetic resonance imaging of the temporal bone to identify any inner ear anomaly. The data gathered included implantation age, gender, computed tomography and magnetic resonance imaging findings, and history of congenital anomaly, neurodevelopmental disorder, family hearing loss, consanguinity and brain-evoked response auditory result. Data was analysed using SPSS 23. Results: Of the 120 patients, 66(55%) were females and 54(45%) were males. The median age of the patients was 3 years (interquartile range: 2-5 years). Of the total, 7(5.8%) patients presented with inner ear malformations; 3(2.5%) each with enlarged vestibular aqueduct, Mondini malformation type II, and 1(0.8%) with semi-circular canal dysplasia. Conclusion: The frequency of inner ear anomalies amongst cochlear implant candidates was 5.8%, and the most common anomalies were enlarged vestibular aqueduct and Mondini malformation type II. Key Words: Cochlear implant, Inner ear anomalies, Inner ear malformations, Hearing loss, Frequency, Consanguinity, loss, Temporal bone, Tomography, Magnetic resonance, Neurodevelopmental disorders, Otolaryngology.

Clinical and genetic attributes of congenital anomalies ascertained at a tertiary care hospital in Rawalpindi, Pakistan

“…The results are comparable to other studies conducted in Pakistan and other countries which showed highest prevalence of CNS defects. 11,12 In this study, the most frequent neurological anomalies were hydrocephaly and spina bifida followed by Down syndrome, while most The reason of the high prevalence of neural tube defects, i.e., spina bifida and associated hydrocephaly could be a lack of maternal folic acid use during the period of conception and early development. 16 Down syndrome is caused by trisomy of chromosome 21 and associated with the advance age of mother.…”

“…The authors remarked that CNS were the most frequently affected system and CA were not uncommon in our society. 11 Another descriptive cross-sectional study was conducted at Mardan Medical Complex, Mardan, and infants with CA born between May 2016-April 2017 were enrolled. A total of 1.23% of newborns exhibited CA.…”