Management of Cochlear Nerve Hypoplasia and Aplasia.

Management of Cochlear Nerve Hypoplasia and Aplasia. Adv Otorhinolaryngol. 2018;81:81-92 Authors: Freeman SR, Sennaroglu L Abstract Approximately 2% of congenital profound deafness cases are due to cochlear nerve (CN) deficiency. MRI is essential to confirm if the nerve is deficient, but because of limitations with resolution, especially when the internal auditory canal is narrowed, it is often unable to distinguish between hypoplasia and aplasia. A full audiometric test battery should always be performed, even if the MRI suggests CN aplasia, as there will sometimes be evidence of audition. Electrically evoked auditory brainstem response testing can be carried out transtympanically via the round window or using an intracochlear test electrode to help determine the status of the CN. If any test suggests the presence of a CN, then cochlear implantation (CI) should be considered. Children should be followed up closely with audiometric, electrophysiological and language assessments to determine the benefits. They may initially show benefit but fail to progress. CI results are variable and often result in poor outcomes with Categories of Auditory Perception scores of
Source: Advances in Oto-Rhino-Laryngology - Category: ENT & OMF Tags: Adv Otorhinolaryngol Source Type: research

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Abhiruchi Galhotra, Preeti SahuIndian Journal of Community Medicine 2019 44(4):299-302 Hearing is the key to learning spoken language, performing academically, and engaging socially for children. Degree of hearing loss quantifies the hearing ability from mild to profound, based on the audiometric findings for an individual across certain frequencies or pitches. Early identification and appropriate intervention is the prime need. A probable strategy is to ensure that every newborn is screened for possible hearing loss at the birth in the hospital. In India, hearing screening facility is mostly available to newborns brought...
Source: Indian Journal of Community Medicine - Category: International Medicine & Public Health Authors: Source Type: research
Conclusions: In the early postoperative period, complete HP was possible in a majority of patients from both groups, but slightly better HP outcomes were achieved by Flex20. In the long term, the length of the electrodes does not affect the degree of HP or speech understanding.
Source: Otology and Neurotology - Category: ENT & OMF Tags: COCHLEAR IMPLANTS Source Type: research
Abstract The tuning fork tests have been under attack since their first use in clinical examination. However, the tuning fork is small and fits into every white coat, and tuning fork tests for hearing are easy, accurate and inexpensive. They should be used in patients with an acute unilateral hearing loss if an electric audiometer is not available. After more than 100 years, the tuning fork is not obsolete; tuning fork tests are a very useful if used correctly and for the appropriate indication. PMID: 31444233 [PubMed - as supplied by publisher]
Source: Practical Neurology - Category: Neurology Authors: Tags: Pract Neurol Source Type: research
In conclusion, for the radiological assessment of TB fractures, the entire VA should be regarded as a part of the otic capsule, and delayed inner ear sequelae should be anticipated for retrolabyrinthine fracture lines that course into or through the VA. When considering treatment options in cases similar to the present, our findings suggest that surgical interventions targeting the eES (ES shunting/decompression procedures (31–33), which are used in MD with the intention to drain the hydropic endolymphatic fluid space, or to improve the fluid resorptive functions of the eES, respectively, most likely cannot work as p...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Conclusion: Our findings indicate that the genetic test based on WES can be useful in diagnosing SYM1 patients, with particular advantages in preventing the fetus from contacting harmful X-ray through the traditional radiography. The novel pathogenic mutation identified would further expand our understanding of the mutation spectrum of NOG in association with SYM1 disease and provide a guidance on how to determine whether the fetus is affected by SYM1 through the prenatal diagnosis. Introduction Proximal symphalangism (MIM#185800, SYM1) is a rare autosomal dominant bone disorder with principal features of variable ...
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
Conclusions: We have shown that severity of MWS symptoms gradually increased with age toward distinct generation-specific phenotypes. A uniform trajectory of disease evolution has encouraged us to postpone institution of IL-1 blockade in affected oligosymptomatic children. This report illustrates importance of close interdisciplinary collaboration. Introduction Cryopyrinopathies (Cryopyrin Associated Periodic Syndromes, CAPS) belong to autoinflammatory disorders with autosomal dominant inheritance caused by the gain-of-function point mutation of NLRP3 (NACHT, LRR, and PYD domains-containing protein 3) gene which en...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Publication date: Available online 11 April 2019Source: NeurocomputingAuthor(s): Pei-Zhen Li, Ling Huang, Chang-Dong Wang, Chuan Li, Jian-Huang LaiAbstractTinnitus is the phantom perception of sound and can have negative effect on the quality of life. Sudden deafness is generally defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies and within a three-day period, which is usually unilateral and can be associated with tinnitus. The exact pathogenesis of such auditory diseases is still unknown, and alterations in the functional connectivity of the brain network are s...
Source: Neurocomputing - Category: Neuroscience Source Type: research
Authors: Chen F, Ni W, Li W, Li H Abstract Cochlear implant (CI) is currently the only medical treatment available to partially restore hearing to patients with profound-to-severe hearing loss. CI is fundamentally distinct from hearing aid (HA) use, as implants are surgically placed under the skin behind the ear where they bypass the normal sound-conducting mechanism, convert sound signals into electrical stimulation, and directly stimulate the residual auditory nerves. In recent years, CI has evolved into one of the most profound advances in modern medicine and provided hearing to more than 320,000 deaf patients. ...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Abstract To investigate the application of inner ear 3-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL) accompanied by inner ear hemorrhage. A total of 1252 SSNHL patients who were admitted from January 2010 to April 2018 were included in the study. The patients' clinical features, complete blood counts, coagulation profiles, audiometry data, and MRI scans were retrospectively reviewed. Twenty-four patients had high labyrinth signals on inner ear 3D-FLAIR MRI (24/1252, 1.9%) that were diagnosed as inner ear hemorr...
Source: Ear, Nose and Throat Journal - Category: ENT & OMF Authors: Tags: Ear Nose Throat J Source Type: research
Conclusions: CI is an effective option for rehabilitating hearing loss in patients with NF2; however, patients with normal contralateral hearing or poor follow-up do not perform as well.
Source: Otology and Neurotology - Category: ENT & OMF Tags: COCHLEAR IMPLANTS Source Type: research
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