To determine audiological and clinical results of cochlear implantation (CI) comparing two populations with single-sided deafness (SSD): post-lingually deaf children between 6 and 12 years of age, and post-lingually deaf adults, in order to evaluate the effect of CI in different age groups. Retrospective case review. Tertiary clinic. Twenty-three children and twenty-one adult patients that were candidates for CI with single-side deafness were included. In all cases we evaluate: Speech perception thresholds; disyllabic words test (65 dB SPL) were performed in the modalities S0-SCI-SNH and Auditory Lateralization Test. The Speech, Spatial, and Qualities of Hearing Scale (SSQ) questionnaire was also used. All results were obtained after 12 months of CI activation. In children, the most common etiology was idiopathic sensory-neural hearing loss. They showed positive results in the Auditory Lateralization Test. In the Speech Test, word recognition in noise improved from 2% preoperatively to 61.1% at a mean follow-up of 1 year (S0 condition) in children [test with signal in CI side 60% and signal normal hearing side (plugged) 31%]. The processor was used for >12 h in all cases. With respect to the SSQ questionnaire, parents were more satisfied within the postoperative period than within the preoperative period. For adults, the most common etiology was idiopathic sudden sensorineural hearing loss (SNHL). Positive results in the Auditory Lateralization Test were found. With respect to the Speech Test in quiet conditions: Word recognition in noise improved from 5.7% preoperatively to 71.8% at a mean follow-up of 1 year [test with signal in CI side 68% and signal normal hearing side (plugged) 41%]. The processor was used for >12 h. In the SSQ questionnaire, the post-operative results showed a beneficial effect of the CI. No adverse events were reported during the study period. No differences were found between children and adults in all tests in this study. Cochlear implantation in post-lingually deaf adults and children with SSD can achieve a speech perception outcome comparable with CI in conventional candidates. Improvements in spatial hearing were also observed. Careful patient selection and counseling regarding potential benefits are important to optimize outcomes.
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http://dx.doi.org/10.3389/fneur.2021.760831 | DOI Listing |
Worldwide, congenital deafness and pigmentation disorders impact millions with their diverse manifestations, and among these genetic conditions, mutations in the Microphthalmia-associated transcription factor (MITF: OMIM#156845) gene are notable for their profound effects on melanocyte development and auditory functions. This study reports a novel porcupine model exhibiting spontaneous deafness and pigmentation abnormalities reminiscent of human Waardenburg Syndrome Type 2 (WS2: OMIM#193510). Through phenotypic characterization, including coat color, skin, eye morphology, and auditory brainstem response (ABR) assessments, we identified hypopigmentation and complete deafness in mutant porcupines.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
December 2024
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Bochkov Research Centre for Medical Genetics, Moscow, Russia.
A fifth world case of autosomal recessive Siddiqi syndrome (SIDDIS) related to ene is presented. In a consanguineous Lezgin (a Dagestan ethnicity) family, there were two affected brothers aged 28 yrs (proband, personally examined) and 32 yrs. Whole-exome sequencing followed by familial Sanger sequencing detected a novel missence variant c.
View Article and Find Full Text PDFZh Nevrol Psikhiatr Im S S Korsakova
December 2024
Federal Center of Brain Research and Neurotechnologies, Moscow, Russia.
According to the literature, a number of anti-epileptic drugs (AEDs) have an ototoxic effect. The mechanism of hearing dysfunction due to the use of AEDs is not well known. The main clinical manifestations of the cochleotoxic effect of the drugs are: tinnitus, sensorineural hearing loss, impaired pitch perception, hyperacusis.
View Article and Find Full Text PDFBMC Musculoskelet Disord
December 2024
Physical medicine & rehabilitation research center, School of medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: Pompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation.
Aims: This study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease.
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