Effect of Frequency Discrimination Training on Tinnitus in Individuals with Flat Sensorineural Hearing loss.

Tinnitus is associated with sensorineural hearing loss irrespective of its severity and configuration. Frequency discrimination training is a contemporary method used for the treatment of tinnitus. However, its efficacy in treating tinnitus associated with flat sensorineural hearing loss is not studied yet. The objectives were to assess (a) treatment effect across sessions on tinnitus percept using subjective questionnaires (b) association in the severity and handicap of tinnitus before and after FDT treatment. A total of 16 participants with mean age of 56 years, who had subjective tinnitus and flat sensorineural hearing loss ranging from mild to moderate were included in the study. However, only 11 participants completed the treatment regime. Each participant was provided FDT in a game format for 15 days. The Quantitative (tinnitus pitch and loudness in each session) and qualitative measurements (THI and TFI) were assessed in each participant. Friedman test revealed a significant reduction in handicap from tinnitus as reflected in THI and reduced functionality impairment from tinnitus as reflected in TFI across sessions. Besides, a significant association was observed in the Chi-square test in severity and handicap of tinnitus before and after therapy. A change in pitch and reduced loudness was noted in eight of 11 participants. Three of them had no tinnitus perception at the end of the treatment regime. The current study findings demonstrate the efficacy of FDT using a game module in treating tinnitus associated with flat sensorineural hearing loss. The perceived severity and handicap of tinnitus reduces as a function of treatment.

Neurologic complications of genetic channelopathies.

This chapter describes what a channelopathy is and how mutations in the genes result in different types of clinical abnormalities. It provides a description of common types of cardiac channelopathies with examples of how there are some areas of overlap with sensory-neuromuscular channelopathies. We describe the cardiac channelopathies of Jervell and Lange-Nielson syndrome, Andersen-Tawil syndrome, Timothy syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, and sinoatrial node dysfunction and deafness. We also discuss sudden unexpected death in epilepsy and how it could relate to some cardiac channelopathies.

Cochlear implantation and mastoid obliteration in a patient with Alström Syndrome.

OBJECTIVES: To describe the outcomes of cochlear implantation with mastoid obliteration in a patient with Alström Syndrome and chronic otorrhea. METHODS: This is a single case discussion of a patient with Alström Syndrome and chronic otorrhea who underwent unilateral cochlear implantation and mastoid obliteration in January 2019. Subsequent contralateral procedure was performed 4 months later. Implantation was pursued due to the progressive sensorineural hearing-loss that is characteristic of Alström Syndrome. Serial Audiograms were obtained before and after procedure. RESULTS: Following implantation, audiological reports improved to near normal thresholds from the previous 60-85 dB sloping hearing loss. The patient's language skills rapidly improved as well as the ability to express her personality. Mastoid obliteration effectively resolved the chronic otorrhea that further complicated this case. CONCLUSION: Cochlear implantation with mastoid obliteration was successful in improving hearing thresholds and resolving chronic otorrhea in a patient with Alström Syndrome.

Síndrome Congénito del ZIKA y su relación con la pérdida auditiva en infantes: Una revisión de la literatura / Congenital ZIKA syndrome and its relationship to hearing loss in infants: A review of literature

Introducción: Los niños con microcefalia secundario al Virus Zika, se agrupan bajo el síndrome congénito del Zika (SZC), el extremo más grave de un espectro de defectos de nacimiento que incluyen además alteraciones en las funciones sensoriales de visión y audición, ya que el virus Zika está incluido dentro de las infecciones virales causadoras de hasta el 40% de las pérdidas auditivas congénitas y adquiridas. Objetivo: identificar en la literatura científica la relación existente entre el Síndrome Congénito del Zika y la pérdida de audición en infantes. Metodología: Revisión sistemática en las principales bases de datos como Science Direct ­ Elsevier, Online Library, Coronavirus Databases, Embase, Springer, entre otras. Resultados: Se encontraron 18 artículos indexados con descriptores de los cuales se incluyeron 8 al cumplir con los criterios de inclusión y exclusión. Discusiones: se evidencia que la pérdida de audición está presente en las diversas poblaciones de infantes evaluadas por medio de pruebas objetivas, las cuales permiten obtener respuestas importantes de la actividad eléctrica a nivel del nervio y la vía auditiva, presentándose fallas notables en los niños con síndrome congénito del Zika. Conclusiones: De acuerdo a los estudios incluidos en la presente investigación es poca la evidencia científica que pueda aportar una asociación significativa y absoluta entre el síndrome congénito del Zika y la pérdida de audición en infantes.

Introduction: Infants with microcephaly secondary to Zika Virus, are grouped under Congenital Zika Syndrome (CZS), the most severe end of a spectrum of birth defects that also include alterations in the sensory functions of vision and hearing, since Zika virus is included within the viral infections causing up to 40% of congenital and acquired hearing loss. Objective: to identify in the scientific literature the relationship between congenital Zika syndrome and hearing loss in infants. Methodology: Systematic review in the main databases such as Science Direct ­ Elsevier, Online Library, Coronavirus Databases, Embase, Springer, among others. Results: 18 articles were found indexed with descriptors of which 8 were included as they met the inclusion and exclusion criteria. Discussions: it is evident that hearing loss is present in the various populations of infants evaluated by means of objective tests, which allow obtaining important responses of the electrical activity at the level of the auditory nerve and pathway, presenting notable failures in children with congenital Zika syndrome. Conclusions: According to the studies included in the present investigation there is little scientific evidence that can provide a significant and absolute association between congenital Zika syndrome and hearing loss in infants.