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Objective: To evaluate the auditory functions and progress of speech development in children with and without cochlear anomalies who underwent cochlear implantation due to prelingual profound sensorineural hearing loss (SNHL).
Methods: This study was conducted at Gaziantep University Faculty of Medicine Ear-Nose-Throat Department, between October 2006 and December 2007. A total of 69 children (aged 6 to 24 months) diagnosed with profound SNHL were included. Patients were divided into two groups with respect to the presence of inner ear anomalies: Group-1 consisted of 41 children without inner ear anomaly, whereas Group-2 was composed of 28 patients with inner ear anomalies. The auditory performance was assessed using Listening Progress Profile Test (LPPT) and Monosyllabic Trochee Polysyllabic Test (MTP), the subsections of Evaluation of Auditory Responses to Speech (EARS) test battery.
Results: Preoperative LPPT scores were 5 (12%) in both groups. Mean LPPT values after fitting in Group-1 and Group-2 on 1, 3 and 6 months were 18.5 (44.1%) and 19 (45.6%); 27 (64.2%) and 28 (67.3%); 31 (75%) and 34 (83%), respectively. Postoperatively, MTP scores in Group-1 and Group-2 were 7.5 (62%) and 7.7 (64%) for 3-words set; 10.4 (58%) and 10.6 (59%) for 6-words set; 14.3 (60%) and 14 (59%) for 12-words set, respectively. The rate of stimulation for electrodes was 1345 q/u (quick/unit) in Group-1 and 1310 q/u in Group-2. No statistically significant difference was detected between groups for variables under investigation.
Conclusion: Cochlear implantation is an effective treatment in children with prelingual profound SNHL. Auditory performance and advancement of speech are similar for children with and without inner ear anomalies.
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http://dx.doi.org/10.12669/pjms.342.14066 | DOI Listing |
PLoS Biol
September 2025
Otolaryngology-Head & Neck Surgery, Stanford University School of Medicine, Stanford, California, United States of America.
Morphogens cooperate to guide development of the inner ear cochlea, but how do compartments communicate? A recent study in PLOS Biology reveals how planar cell polarity of individual cells is integrated across distinct regional compartments to ensure proper organ morphogenesis.
View Article and Find Full Text PDFPLoS Biol
September 2025
National Centre for Biological Sciences, Tata Institute of Fundamental Research, Bangalore, India.
Morphogenetic information arises from a combination of genetically encoded cellular properties and emergent cellular behaviors. The spatio-temporal implementation of this information is critical to ensure robust, reproducible tissue shapes, yet the principles underlying its organization remain unknown. We investigated this principle using the mouse auditory epithelium, the organ of Corti (OC).
View Article and Find Full Text PDFHum Genet
September 2025
College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Beijing, 100853, China.
Recessive variants in TWNK cause syndromes arising from mitochondrial DNA (mtDNA) depletion. Hearing loss is the most prevalent manifestation in individuals with these disorders. However, the clinical and pathophysiological features have not been fully elucidated.
View Article and Find Full Text PDFOtolaryngol Head Neck Surg
September 2025
Departament de Cirurgia i Especialitats Medicoquirúrgiques, Facultat de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain.
Objective: To conduct a systematic review and meta-analysis to assess the association between type 2 diabetes and hearing loss.
Data Sources: Search conducted in PubMed and Scopus databases for articles published between January 2019 and April 2024.
Review Methods: Quality assessment and risk of bias analysis were conducted using the Newcastle-Ottawa scale, and meta-analyses of pooled data were performed with Cochrane's Review Manager.
J Assoc Res Otolaryngol
September 2025
Biological Sciences Platform, Hurvitz Brain Sciences Program, Sunnybrook Research Institute, Sunnybrook Health Sciences Centre, 2075 Bayview Ave., Room M1 102, Toronto, ON, M4N 3M5, Canada.
Purpose: Delivery of therapeutics to the inner ear is complicated by their inaccessible location and the presence of the blood-labyrinth barrier that restricts most blood-borne compounds from entering the inner ear. This study addresses the challenge of optimal delivery in treating inner ear disease, focusing on magnetic targeting gene therapy using adeno-associated virus (AAV).
Methods: The investigation explores three AAV serotypes (AAV2 Quad Mut, AAV2 pANC80L65, and AAV9 PHP.