scholarly journals Thyroid function in hearing impaired children with cochlear implant

2015 ◽  
Vol 62 (3) ◽  
pp. 276-279
Author(s):  
Alexandra-Cristina Neagu ◽  
◽  
Monica Gheorghiu ◽  
◽  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Thyroid Function ◽  
Congenital Hypothyroidism ◽  
Serum Levels ◽  
Study Group ◽  
Congenital Hearing Loss ◽  
Pendred Syndrome ◽  
Impaired Children ◽  
Thyroid Abnormalities

Congenital hypothyroidism may be associated with hearing loss. However, the prevalence of thyroid abnormalities in children with severe hearing loss is not clearly evaluated. The authors assessed the thyroid function in 30 children with profound sensorineural bilateral hearing loss, with cochlear implant, to determine if there is any association between congenital hearing loss and hypothyroidism. Serum levels of TSH and free thyroxine were normal in the study group. None had Pendred syndrome. We present a literature review on the association between hearing loss and thyroid abnormalities and the importance of testing hearing in children with congenital hypothyroidism.

scholarly journals Relationship between proximity to a cochlear implant center and early presentation in children with congenital hearing loss

2020 ◽  
Vol 41 (3) ◽  
pp. 314-317
Author(s):  
Yazeed Al-shawi ◽  
Fahad Alrawaf ◽  
Najd Al-Gazlan ◽  
Munahi Al-Qahtani ◽  
Fida Almuhawas
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Congenital Hearing Loss ◽  
Early Presentation

scholarly journals Effects of families’ socioeconomic and education level on auditory skills of cochlear implant users: an Iranian population study

2020 ◽  
Author(s):  
Mohammadsaleh Moosapour ◽  
Mohammad Ajalloueyan ◽  
Farzaneh Zamiri Abdollahi ◽  
Maryam Delphi ◽  
Ali Berri Dizaji
Keyword(s):  
Socioeconomic Status ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Educational Level ◽  
Literacy Skill ◽  
Cross Sectional ◽  
The Family ◽  
Keywords Cochlear Implant ◽  
Best Fitting ◽  
Impaired Children

Background and Aim: Hearing loss can have disabling effects on all aspects of children’s life and demographic factors of families can have significant effects on children’s auditory deve­lopment. The main aim of the study was deter­mining the effects of socioeconomic and educa­tion level on auditory behaviors of hearing-impaired children. Methods: The study was cross sectional descrip­tive-analytic study and was conducted on 207 parents of children under age of four years with native Persian speaking parents with literacy skill. Their hearing impairment was identified before the first month of age. Early occurrence of hearing loss was considered to exclude any effe­cts of early exposure to normal auditory stimuli on the outcome measurements. The children had at least 3-month experience with the cochlear implant (CI) after best fitting and adaptation to their device at the time of the study. Samples were selected by convenience sampling method from available subjects. For determining socioeconomic level, Ghodratnama socioeco­nomic status (SES) questionnaire was used. Infa­nts and Toddlers Meaningful Auditory Integ­ration Scale (IT-MAIS) was selected for the auditory beha­viors study. Results: Socioeconomic and educational level of the family showed no significant effects on audi­tory behaviors. The age of receiving auditory ass­istive device had weak but statistically signifi­cant effect on the outcome. Conclusion: It seems that socioeconomic status and educational level of the family did not con­tribute to the auditory behaviors of children with cochlear implant. The generalizations of these results need further studies. Keywords: Cochlear implant; pediatric; auditory behaviors; socioeconomic status

Magnetic resonance imaging versus computed tomography in pre-operative evaluation of cochlear implant candidates with congenital hearing loss

2002 ◽  
Vol 116 (10) ◽  
pp. 804-810 ◽  
Author(s):  
Levent Sennaroglu ◽  
Isil Saatci ◽  
Ayse Aralasmak ◽  
Bulent Gursel ◽  
Ergin Turan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Congenital Hearing Loss ◽  
Resonance Imaging

Recent reports indicate that the cochlear nerve may be absent in some cases of congenital sensorineural hearing loss. The aim of this prospective study was to determine the incidence of cochlear nerve anomaly in cochlear implant candidates with congenital hearing loss using magnetic resonance imaging (MRI). Twenty-seven patients with congenital profound bilateral sensorineural hearing loss who were being evaluated for the cochlear implant procedure were studied.These patients had high-resolution computerized tomography (CT), through the petrous bone in axial sections. MRI examinations consisted of T1 and turbo spin echo (TSE) T2-weighted 3 mm axial images, and additional 3D Fourier Transform T2-weighted TSE sequences obtained on three different planes (axial, perpendicular and parallel to the internal auditory canal (IAC) i.e. oblique sagittal and coronal, respectively) for the purpose of cochlear nerve demonstration. Results showed that all of the 14 patients with normal CT of the temporal bone, had four distinct nerves in the distal part of the IAC on TSE-MRI. Thirteen patients demonstrated various bony malformations of the cochleovestibular system on CT. MRI revealed the absence of the cochleovestibular nerve in four patients where the IAC was very narrow or completely absent on CT. One patient with severe Mondini malformation who had an enlarged IAC demonstrated an isolated absent cochlear nerve.

scholarly journals Syndromic Hearing Loss: A Brief Review of Common Presentations and Genetics

2018 ◽  
Vol 07 (01) ◽  
pp. 001-008 ◽  
Author(s):  
John Gettelfinger ◽  
John Dahl
Keyword(s):  
Hearing Loss ◽  
Birth Defects ◽  
Alport Syndrome ◽  
Neurofibromatosis Type ◽  
Treacher Collins Syndrome ◽  
Congenital Hearing Loss ◽  
Pendred Syndrome ◽  
Wide Range ◽  
Syndromic Hearing Loss

AbstractCongenital hearing loss is one of the most common birth defects worldwide, with around 1 in 500 people experiencing some form of severe hearing loss. While over 400 different syndromes involving hearing loss have been described, it is important to be familiar with a wide range of syndromes involving hearing loss so an early diagnosis can be made and early intervention can be pursued to maximize functional hearing and speech-language development in the setting of verbal communication. This review aims to describe the presentation and genetics for some of the most frequently occurring syndromes involving hearing loss, including neurofibromatosis type 2, branchio-oto-renal syndrome, Treacher Collins syndrome, Stickler syndrome, Waardenburg syndrome, Pendred syndrome, Jervell and Lange-Nielsen syndrome, Usher syndromes, Refsum disease, Alport syndrome, MELAS, and MERRF.

Surgical Treatment and Rehabilitation of Prelingually and Perilingually Deafened Children and Adults with the Nucleus Multichannel Cochlear Implant

1994 ◽  
Vol 73 (3) ◽  
pp. 169-175 ◽  
Author(s):  
Juan Manuel García ◽  
Clemencia Barón de Otero ◽  
Jorge García ◽  
Augusto Peñaranda ◽  
Claudia Niño ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Surgical Complication ◽  
Rehabilitation Program ◽  
Speech Discrimination ◽  
Congenital Hearing Loss ◽  
Open Set ◽  
Speech Reception ◽  
Discrimination Testing ◽  
Deaf Adults

We began our program in September 1992, using the Nucleus 22 Channel Cochlear Implant. To date, we have operated on four patients, one child with congenital hearing loss, two prelinguistically deaf adults and one perilingually deaf adult. Our results have shown a significant increase in auditory and speech reception and perception skills in the child. The perilingually deaf adult is able to understand speech in open set speech discrimination testing and, although we do not expect open set speech discrimination in the prelinguistically deaf adults, to date their results have been satisfactory. The two prelingually deaf adults are in an audiological rehabilitation program. Their response in prosodic aspects of speech and lipreading ability with sound have improved significantly. The only surgical complication was an infection of the flap in the child, but it was treated satisfactorily with I.V. penicillin.

scholarly journals A case of congenital hypothyroidism combined with sensorineural hearing loss (Pendred syndrome) caused by a TPO gene defect

2017 ◽  
Vol 63 (2) ◽  
pp. 110-113
Author(s):  
Nina A. Makretskaya ◽  
Olga B. Bezlepkina ◽  
Olga A. Chikulaeva ◽  
Evgeny V. Vasilyev ◽  
Vasiliy M. Petrov ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Congenital Hypothyroidism ◽  
Molecular Genetic ◽  
Clinical Manifestations ◽  
Molecular Genetic Analysis ◽  
Sensorineural Hearing ◽  
Gene Defect ◽  
Pendred Syndrome ◽  
Disease Pattern

Congenital hypothyroidism is a genetically heterogeneous group of diseases caused by two mechanisms: gland dysgenesis and dyshormonogenesis. The disease pattern includes a number of syndromic forms, one of which is a combination of congenital hypothyroidism and sensorineural hearing loss (Pendred syndrome) initially associated with SLC26A4 gene defects. The article describes a patient with clinical manifestations of Pendred syndrome who was diagnosed with a TPO gene defect during a molecular genetic analysis using next generation sequencing (NGS). Therefore, a combination of congenital hypothyroidism and sensorineural hearing loss can have a different molecular basis. Our findings illustrate the value of NGS for genetic verification of the diagnosis.

scholarly journals A novel case of concurrent occurrence of demyelinating-polyneuropathy-causing PMP22 duplication and SOX10 gene mutation producing severe hypertrophic neuropathy

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nozomu Matsuda ◽  
Koushi Ootsuki ◽  
Shunsuke Kobayashi ◽  
Ayaka Nemoto ◽  
Hitoshi Kubo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Neuromuscular Disorders ◽  
Whole Body ◽  
Congenital Hearing Loss ◽  
Severe Phenotype ◽  
Demyelinating Neuropathy ◽  
Magnetic Resonance Neurography ◽  
Peripheral Myelin Protein 22 ◽  
Hypertrophic Neuropathy

Abstract Background Hereditary motor and sensory neuropathy, also referred to as Charcot–Marie–Tooth disease (CMT), is most often caused by a duplication of the peripheral myelin protein 22 (PMP22) gene. This duplication causes CMT type 1A (CMT1A). CMT1A rarely occurs in combination with other hereditary neuromuscular disorders. However, such rare genetic coincidences produce a severe phenotype and have been reported in terms of “double trouble” overlapping syndrome. Waardenburg syndrome (WS) is the most common form of a hereditary syndromic deafness. It is primarily characterized by pigmentation anomalies and classified into four major phenotypes. A mutation in the SRY sex determining region Y-box 10 (SOX10) gene causes WS type 2 or 4 and peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, WS, and Hirschsprung disease. We describe a 11-year-old boy with extreme hypertrophic neuropathy because of a combination of CMT1A and WS type 2. This is the first published case on the co-occurrence of CMT1A and WS type 2. Case presentation The 11-year-old boy presented with motor developmental delay and a deterioration in unstable walking at 6 years of age. In addition, he had congenital hearing loss and heterochromia iridis. The neurological examination revealed weakness in the distal limbs with pes cavus. He was diagnosed with CMT1A by the fluorescence in situ hybridization method. His paternal pedigree had a history of CMT1A. However, no family member had congenital hearing loss. His clinical manifestation was apparently severe than those of his relatives with CMT1A. In addition, a whole-body magnetic resonance neurography revealed an extreme enlargement of his systemic cranial and spinal nerves. Subsequently, a genetic analysis revealed a heterozygous frameshift mutation c.876delT (p.F292Lfs*19) in the SOX10 gene. He was eventually diagnosed with WS type 2. Conclusions We described a patient with a genetically confirmed overlapping diagnoses of CMT1A and WS type 2. The double trouble with the genes created a significant impact on the peripheral nerves system. Severe phenotype in the proband can be attributed to the cumulative effect of mutations in both PMP22 and SOX10 genes, responsible for demyelinating neuropathy.

scholarly journals The Effects of Round Window Membrane Injury and the Use of a Model Electrode Application on Hearing in Rats

2021 ◽  
pp. 014556132199018
Author(s):  
Murat Koc ◽  
Abdullah Dalgic ◽  
Mehmet Ziya Ozuer
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Round Window ◽  
Mechanical Trauma ◽  
Round Window Membrane ◽  
Albino Rats ◽  
Topical Steroids ◽  
Distortion Product ◽  
Significant Difference ◽  
Group 3

Objective: To investigate the effects of the mechanical trauma to the round window, a model electrode inserted into the scala tympani on the cochlear reserve, and the efficacy of topical steroids in preventing hearing loss. Materials and Methods: 21 male Wistar Albino rats were equally categorized into three groups. In all groups an initial mechanical injury to round window was created. Only subsequent dexamethasone injection was administrated into the cochlea in the subjects of group 2 while a multichannel cochlear implant guide inserted into the cochlea prior to dexamethasone administration for group 3. Distortion product otoacoustic emissions (DPOAEs) were obtained prior to and immediately after the surgical injury, eventually on postoperative seventh day (d 7). Mean signal/noise ratios (S/Ns) obtained at 2000, 3000, and 4000 Hz were calculated. Data sets were compared with non-parametric statistical tests. Results: The early intraoperative mean S/Ns were significantly less than preoperative measurements for group 1 and 2; however, preoperative and postoperative d 7 average S/Ns did not differ. There was statistically significant difference between preoperative, intraoperative and postoperative d 7 average S/Ns for group 3. Conclusion: We observed that hearing was restored approximately to the preoperative levels following early postoperative repair. However, an electrode insertion into the cochlea via round window subsequent to mechanical trauma seems to cause a progressive hearing loss. Therefore, a special care must be taken to avoid the injury to the round window membrane in the course of the placement of a cochlear implant electrode and surgery for the chronic otitis media.

scholarly journals IGF-1 Haploinsufficiency Causes Age-Related Chronic Cochlear Inflammation and Increases Noise-Induced Hearing Loss

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1686
Author(s):  
Adelaida M. Celaya ◽  
Lourdes Rodríguez-de la Rosa ◽  
Jose M. Bermúdez-Muñoz ◽  
José M. Zubeldia ◽  
Carlos Romá-Mateo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inflammatory Cytokines ◽  
Noise Exposure ◽  
Sensorineural Deafness ◽  
Serum Levels ◽  
Genetic Syndromes ◽  
Expression Ratio ◽  
Postnatal Maturation ◽  
Age Related ◽  
Underlying Mechanisms

Insulin-like growth factor 1 (IGF-1) deficiency is an ultrarare syndromic human sensorineural deafness. Accordingly, IGF-1 is essential for the postnatal maturation of the cochlea and the correct wiring of hearing in mice. Less severe decreases in human IGF-1 levels have been associated with other hearing loss rare genetic syndromes, as well as with age-related hearing loss (ARHL). However, the underlying mechanisms linking IGF-1 haploinsufficiency with auditory pathology and ARHL have not been studied. Igf1-heterozygous mice express less Igf1 transcription and have 40% lower IGF-1 serum levels than wild-type mice. Along with ageing, IGF-1 levels decreased concomitantly with the increased expression of inflammatory cytokines, Tgfb1 and Il1b, but there was no associated hearing loss. However, noise exposure of these mice caused increased injury to sensory hair cells and irreversible hearing loss. Concomitantly, there was a significant alteration in the expression ratio of pro- and anti-inflammatory cytokines in Igf1+/− mice. Unbalanced inflammation led to the activation of the stress kinase JNK and the failure to activate AKT. Our data show that IGF-1 haploinsufficiency causes a chronic subclinical proinflammatory age-associated state and, consequently, greater susceptibility to stressors. This work provides the molecular bases to further understand hearing disorders linked to IGF-1 deficiency.

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