Unlabelled: Cochlear implantation of congenitally deaf children with inner ear malformations is gaining special interest. Although the number of the reported cases is increasing, the decision for implantation needs thorough investigation. Preoperative evaluation, surgical approach and postoperative follow-up can be challenging.
Study Design: A retrospective analysis of two cases with inner ear malformations.
Patients: One patient was a 3-year-old-girl who had cochlear and cochleovestibular nerve aplasia on the left side and incomplete partition on the other side. The other patient was a 5-year-old-boy who had hypoplastic cochlea on both sides. Both of them also had vestibular anomalies. Cases were implanted by using multichannel cochlear implant.
Results: No complications were encountered. Both patients responded to acoustic stimuli, and their speech perception skills were improved. After 10 months of cochlear implant use, their results seem encouraging.
Conclusion: Except cochlear or cochleovestibular nerve agenesis, inner ear malformations cannot be accepted as a contraindication for cochlear implantation. Although there can be difficulties during the surgery or in the postoperative period, patients with inner ear malformations can also benefit from cochlear implantation. It is essential that all possible complications and postoperative performance should be discussed with the parents.
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http://dx.doi.org/10.1016/s0165-5876(02)00152-0 | DOI Listing |
Auris Nasus Larynx
January 2025
Department of Otorhinolaryngology, Head and Neck surgery, Aichi Medical University School of Medicine, 1-1, Yazakokarimata, Nagakute, Aichi 480-1195, Japan.
We present a case of a perilymphatic fistula (PLF) caused by Eustachian tube air inflation (ETAI) that was diagnosed using cochlin-tomoprotein (CTP) testing and successfully treated using transcanal endoscopic ear surgery to seal the inner ear window. A 77-year-old woman developed hearing loss and dizziness after undergoing ETAI at a local ear, nose, and throat clinic. Despite initial bed rest and steroid pulse therapy, the hearing did not improve, and transcanal endoscopic ear surgery was performed to repair the PLF.
View Article and Find Full Text PDFNoise Health
January 2025
Department of Neurology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey.
Background: Patients with multiple sclerosis (MS) experience difficulties in understanding speech in noise despite having normal hearing.
Aim: This study aimed to determine the relationship between speech discrimination in noise (SDN) and medial olivocochlear reflex levels and to compare MS patients with a control group.
Material And Methods: Sixty participants with normal hearing, comprising 30 MS patients and 30 healthy controls, were included.
Hum Genet
January 2025
John P. Hussman Institute for Human Genomics, University of Miami Miller School of Medicine, Miami, FL, USA.
Developmental anomalies of the hearing organ, the cochlea, are diagnosed in approximately one-fourth of individuals with congenital. The majority of patients with cochlear malformations remain etiologically undiagnosed due to insufficient knowledge about underlying genes or the inability to make conclusive interpretations of identified genetic variants. We used exome sequencing for the genetic evaluation of hearing loss associated with cochlear malformations in three probands from unrelated families deafness.
View Article and Find Full Text PDFFASEB J
January 2025
Department of Otolaryngology-Head and Neck Surgery, Xiangya Hospital, Central South University, Changsha, Hunan, China.
Meniere's disease (MD) is an inner ear disease characterized by endolymphatic hydrops (EH). Maintaining a regular daily routine is crucial for MD patients. However, the relationship between circadian rhythms and MD remains unclear.
View Article and Find Full Text PDFSci Rep
January 2025
Marine Biotechnology Fish Nutrition and Health Division, ICAR-Central Marine Fisheries Research Institute, Ernakulam North P O, Kochi, Kerala, 682018, India.
The Indian scad, Decapterus russelli is one of the most exploited pelagic resources of India. Population genetic analyses using mitochondrial and nuclear markers indicated a lack of genetic structuring among populations from Indian waters. As this species is highly migratory, it is also important to establish the environmental influence on its population structure.
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