Speech Perception and Production in Cochlear Implant Recipients with Pendred Syndrome.

AIMS: Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients. METHODS: Ten patients with Pendred syndrome were analyzed for speech perception and production. The control group consisted of 41 non-syndromic implanted patients. All implantees were scored according to speech perception, speech production, and the sum of both. The data were statistically analyzed. RESULTS: No statistical difference was found in language acquisition and production in implantees with Pendred syndrome when compared to non-syndromic patients with cochlear implants. Nor there was any difference in speech production and acquisition between the 2 compared groups regarding surgical age, time elapsed after surgery, or age during the testing. CONCLUSION: In this study evaluating language and speech production and acquisition, patients with Pendred syndrome who underwent cochlear implants show comparable results to their implanted peers with deafness of a different etiology.

Cochlear implantation in patients with Pendred syndrome.

OBJECTIVE: To examine the outcomes of cochlear implantation in children with Pendred Syndrome. MATERIAL AND METHODS: A retrospective case series of nine pediatric patients with Pendred syndrome undergoing cochlear implantation at a tertiary academic medical center from 2003 to 2017. RESULTS: All patients were diagnosed with bilateral mild-to-profound to severe-to-profound sensorineural hearing loss and used hearing aids prior to implantation. Preoperative imaging results revealed that all patients exhibited bilateral enlarged vestibular aqueducts and 8 of 9 had cochlear dysplasia equivalent to Incomplete Partition II. Despite inner ear malformations, all electrodes were successfully implanted with minimal complications and favorable post-operative audiological outcomes. CONCLUSIONS: Cochlear implantation is an effective and successful treatment for severe-to-profound hearing loss in children with Pendred syndrome and for whom traditional amplification aids provide limited benefit. With early intervention, cochlear implantation allows speech and cognitive development in Pendred children comparable to that of normal hearing individuals, allowing patients to learn in a mainstream educational setting.

Endoscopic versus Conventional Thyroid Surgery: A Comparison of Quality of Life, Cosmetic Outcomes and Overall Patient Satisfaction with Treatment.

BACKGROUND: Our aim was to investigate whether the surgical approach, i.e. endoscopic (ET) versus conventional/open thyroidectomy (ConT), contributes towards the improvement in quality of life (QoL), cosmetic outcomes and overall patient satisfaction with treatment. METHODS: We conducted a prospective study (October 2016-August 2018) consisting of patients undergoing hemithyroidectomy for euthyroid, non-malignant solitary thyroid nodules (STNs). Patients were divided into two groups: Group I (ET)-41 patients and Group II (ConT)-52 patients. ET was performed via bilateral-axillo breast approach (BABA). A thyroid disease-specific questionnaire "ThyPRO-39hin" was used to evaluate pre- and post-operative QoL. Visual analogue scale (VAS) was used for post-operative pain. Cosmetic satisfaction, paraesthesia and overall satisfaction with treatment were evaluated using Likert-type verbal response scales. Pre- and post-operative scores (>6 months) were compared, and p value < 0.05 was considered significant. RESULTS: Mean age (p = 0.26), gender distribution (p = 0.07), mean tumour size (p = 0.74) and preoperative scores of QoL were comparable between the two groups. Post-operatively scores of symptom domain (p = 0.03), tiredness (p = 0.03), impaired social life (p = 0.03), cosmetic complaints (p = < 0.001) and overall QoL (p = < 0.001) were significantly better in Group I. Also, post-operative pain perception at first follow-up visit (p = 0.001) was lower in patients undergoing ET/Group I, with higher scar satisfaction scores (p = < 0.001) and overall satisfaction with treatment (p = < 0.001). Post-operative paraesthesia perception was comparable (p = 0.06) amongst the two groups. CONCLUSION: In this cohort study, patients undergoing endoscopic thyroid surgery reported superior post-operative QoL, cosmetic and overall satisfaction with treatment as compared to conventional/open thyroidectomy.

Receptive language acquisition in a pediatric population with Pendred syndrome and non-syndromic enlarged vestibular aqueduct.

Background: Children with Pendred syndrome (PS) and non-syndromic enlarged vestibular aqueduct (NSEVA) represent a group of pre-lingual hearing-impaired individuals with rehabilitation challenges.Objective: To evaluate receptive language capabilities in a pediatric cohort with PS and NSEVA.Materials and methods: Twenty-four (24) children diagnosed with either PS or NSEVA, were examined using the Peabody Picture Vocabulary Test (PPVT) and compared to a cohort of 55 Danish children with normal hearing, as well as to a mixed group of 29 children with hearing impairment of congenital and hereditary non-syndromal type. In addition, test results were compared to normative data (PPVT-4 US standard scores).Results: PS/NSEVA children's PPVT-4 test scores fall within the normative data for the PPVT-4 (US) but proved to be significantly lower statistically, when compared to Danish children with normal hearing (p<.0001) or to children with mixed non-syndromic hereditary hearing impairment (p=.006). Implantation age was significantly later for the PS/NSEVA group (median = 43 months), compared to the mixed non-syndromic hereditary congenial hearing impairment group (median = 11 months).Conclusions and significance: Children with PS/NSEVA perform below age equivalent for receptive vocabulary outcome when compared to both children with normal hearing, and children with non-syndromic mixed hereditary congenital hearing impairment who receive cochlear implants earlier.

Hipotireoidismo congênito como fator de risco para os transtornos do processamento auditivo central / Congenital hypothyroidism as a risk factor for central hearing process disorders

RESUMO Objetivo: Investigar a manifestação de sintomas do transtorno do processamento auditivo central em crianças com hipotireoidismo congênito. Métodos: Estudo de caráter exploratório, descritivo e transversal com 112 pacientes com hipotireoidismo congênito com idade ≥5 anos. Realizou-se entrevista com os pais/cuidadores no momento da espera da consulta médica. Portadores de outras afecções médicas foram excluídos. Como instrumento de pesquisa utilizou-se o protocolo estruturado de anamnese para avaliação do processamento auditivo rotineiramente empregado por audiologistas. A análise estatística utilizou o teste Qui-quadrado. Resultados: A distribuição por sexo foi semelhante (meninas: 53,3%). Os casos não-disgenesia constituíram a forma fenotípica mais prevalente para o hipotireoidismo congênito (88,4%), e verificou-se que 65,3% das crianças apresentavam algum episódio de níveis séricos irregulares de hormônio tireoestimulante. Dentre as manifestações mais frequentes dos sintomas do transtorno do processamento auditivo central, as queixas relaciondas às funções cognitivas auditivas, como: figura-fundo (83,0%), atenção auditiva (75,9%) e memória auditiva (33,0%) foram as mais evidentes. Reclamações relacionadas ao rendimento escolar foram reportadas em 62,3%. Conclusões: Os dados obtidos evidenciaram altas frequências de sintomas de defasagem nas funções cognitivas relacionadas ao processamento auditivo central, em especial na atenção auditiva, figura-fundo e memória auditiva nos portadores do hipotireoidismo congênito.

ABSTRACT Objective: To investigate the presence of central auditory processing disorder symptoms in children with congenital hypothyroidism. Methods: An exploratory, descriptive, cross-sectional study of 112 patients with congenital hypothyroidism aged ≥5 years old. An interview was held with the parents/caregivers at the time of the medical consultation. Patients with other medical conditions were excluded. As a research instrument, the structured protocol of anamnesis was used to evaluate the auditory processing routinely used by audiologists. For statistical analysis, the chi-square test was used. Results: Sex distribution was similar in both boys and girls (girls: 53.3%). The most prevalent phenotypic form of congenital hypothyroidism was no dysgenesis (88.4%), and 65.3% of the children had an episode of irregular serum thyroid-stimulating hormone (TSH) levels. Among the manifestations of the most frequent central auditory processing disorder symptoms, problems were reported with regard to cognitive functions, as they related to hearing, such as figure-background ability (83.0%), auditory attention (75.9%) and auditory memory (33.0%). Complaints related to school performance were reported in 62.3% of the cases. Conclusions: The data obtained show a high frequency of lag symptoms in cognitive functions related to central auditory processing, particularly with regard to auditory attention, figure-background ability and auditory memory in patients with congenital hypothyroidism.

CONGENITAL HYPOTHYROIDISM AS A RISK FACTOR FOR CENTRAL HEARING PROCESS DISORDERS. / HIPOTIREOIDISMO CONGÊNITO COMO FATOR DE RISCO PARA OS TRANSTORNOS DO PROCESSAMENTO AUDITIVO CENTRAL.

OBJECTIVE: To investigate the presence of central auditory processing disorder symptoms in children with congenital hypothyroidism. METHODS: An exploratory, descriptive, cross-sectional study of 112 patients with congenital hypothyroidism aged ≥5 years old. An interview was held with the parents/caregivers at the time of the medical consultation. Patients with other medical conditions were excluded. As a research instrument, the structured protocol of anamnesis was used to evaluate the auditory processing routinely used by audiologists. For statistical analysis, the chi-square test was used. RESULTS: Sex distribution was similar in both boys and girls (girls: 53.3%). The most prevalent phenotypic form of congenital hypothyroidism was no dysgenesis (88.4%), and 65.3% of the children had an episode of irregular serum thyroid-stimulating hormone (TSH) levels. Among the manifestations of the most frequent central auditory processing disorder symptoms, problems were reported with regard to cognitive functions, as they related to hearing, such as figure-background ability (83.0%), auditory attention (75.9%) and auditory memory (33.0%). Complaints related to school performance were reported in 62.3% of the cases. CONCLUSIONS: The data obtained show a high frequency of lag symptoms in cognitive functions related to central auditory processing, particularly with regard to auditory attention, figure-background ability and auditory memory in patients with congenital hypothyroidism.

OBJETIVO: Investigar a manifestação de sintomas do transtorno do processamento auditivo central em crianças com hipotireoidismo congênito. MÉTODOS: Estudo de caráter exploratório, descritivo e transversal com 112 pacientes com hipotireoidismo congênito com idade ≥5 anos. Realizou-se entrevista com os pais/cuidadores no momento da espera da consulta médica. Portadores de outras afecções médicas foram excluídos. Como instrumento de pesquisa utilizou-se o protocolo estruturado de anamnese para avaliação do processamento auditivo rotineiramente empregado por audiologistas. A análise estatística utilizou o teste Qui-quadrado. RESULTADOS: A distribuição por sexo foi semelhante (meninas: 53,3%). Os casos não-disgenesia constituíram a forma fenotípica mais prevalente para o hipotireoidismo congênito (88,4%), e verificou-se que 65,3% das crianças apresentavam algum episódio de níveis séricos irregulares de hormônio tireoestimulante. Dentre as manifestações mais frequentes dos sintomas do transtorno do processamento auditivo central, as queixas relaciondas às funções cognitivas auditivas, como: figura-fundo (83,0%), atenção auditiva (75,9%) e memória auditiva (33,0%) foram as mais evidentes. Reclamações relacionadas ao rendimento escolar foram reportadas em 62,3%. CONCLUSÕES: Os dados obtidos evidenciaram altas frequências de sintomas de defasagem nas funções cognitivas relacionadas ao processamento auditivo central, em especial na atenção auditiva, figura-fundo e memória auditiva nos portadores do hipotireoidismo congênito.

[Syncope due to giant goitre].

We present a case report of a 70-year-old male patient with a giant goitre. The patient experienced recurrent episodes of syncope/fainting, when he turned his head towards to the right, and he experienced discomfort when lying on his back. A CT scan showed compression of the right carotid artery and carotid sinus from a thyroid mass. After a mass of 1,473 g of thyroid tissue had been removed by a right thyroid lobectomy, the patient experienced no further episodes of syncope. It is well known, that giant goitres can bring a wide range of symptoms including compression of arteries, veins, nerves and related structures on the neck and in the thorax.

Quality of life after thyroidectomy in patients with nontoxic nodular goiter: A prospective cohort study.

BACKGROUND: Using the thoroughly validated Thyroid-Related Quality-of-Life Patient-Reported Outcome (ThyPRO) questionnaire, the purpose of this study was to investigate changes in disease-specific quality of life (QOL) after surgical treatment in patients with benign nontoxic multinodular goiters. METHOD: Patients with goiters scheduled for thyroid surgery (n = 106) and individuals from the general population (n = 739) were studied. The ThyPRO data before, 3 months, and 6 months after surgery were compared with normative scores from the general population using a linear mixed model and t tests. RESULTS: Before surgery, patients with goiters experienced poorer scores on all scales compared to the general population. After surgery, moderate to large improvements were seen in goiter symptoms, tiredness, anxiety, and overall QOL. After surgery, all scales returned to values equal to the general population. The degree of anxiety was, in fact, lower than in the general population. CONCLUSION: Thyroid surgery leads to significant benefit among patients with benign nontoxic goiters by restoring QOL equal to that in the general population.

Quality-of-Life Impairments Persist Six Months After Treatment of Graves' Hyperthyroidism and Toxic Nodular Goiter: A Prospective Cohort Study.

BACKGROUND: The treatment of hyperthyroidism is aimed at improving health-related quality of life (HRQoL) and reducing morbidity and mortality. However, few studies have used validated questionnaires to assess HRQoL prospectively in such patients. The purpose of this study was to assess the impact of hyperthyroidism and its treatment on HRQoL using validated disease-specific and generic questionnaires. METHODS: This prospective cohort study enrolled 88 patients with Graves' hyperthyroidism and 68 with toxic nodular goiter from endocrine outpatient clinics at two Danish university hospitals. The patients were treated with antithyroid drugs, radioactive iodine, or surgery. Disease-specific and generic HRQoL were assessed using the thyroid-related patient-reported outcome (ThyPRO) and the Medical Outcomes Study 36-item Short Form (SF-36), respectively, evaluated at baseline and six-month follow-up. The scores were compared with those from two general population samples who completed ThyPRO (n = 739) and SF-36 (n = 6638). RESULTS: Baseline scores for patients with Graves' hyperthyroidism and toxic nodular goiter were significantly worse than those for the general population scores on all comparable ThyPRO scales and all SF-36 scales and component summaries. ThyPRO scores improved significantly with treatment on all scales in Graves' hyperthyroidism and four scales in toxic nodular goiter, while SF-36 scores improved on five scales and both component summaries in Graves' hyperthyroidism and only one scale in toxic nodular goiter. In Graves' hyperthyroidism, large treatment effects were observed on three ThyPRO scales (Hyperthyroid Symptoms, Tiredness, Overall HRQoL) and moderate effects on three scales (Anxiety, Emotional Susceptibility, Impaired Daily Life), while moderate effects were seen in two ThyPRO scales in toxic nodular goiter (Anxiety, Overall HRQoL). However, significant disease-specific and generic HRQoL deficits persisted on multiple domains across both patient groups. CONCLUSIONS: Graves' hyperthyroidism and toxic nodular goiter cause severe disease-specific and generic HRQoL impairments, and HRQoL deficits persist in both patient groups six months after treatment. These data have the potential to improve communication between physicians and patients by offering realistic estimates of expected HRQoL impairments and treatment effects. Future studies should identify risk factors for persistent HRQoL deficits, compare HRQoL effects of the various therapies, and thereby aid in determining the optimal treatment strategies.

Effect of stressful life events on the initiation of graves' disease.

OBJECTIVE: The aim of this study was to investigate the relationship between stressful life events (LESs) and its effect on the initiation of Graves' disease (GD) and toxic nodular goitre (TNG). PATIENTS AND METHOD: Forty-five patients with GD, 24 patients with TNG and 36 healthy control (CG) were included to the study. Graves and TNG patients had diagnosed within the last 12 months, with clinical and biochemical confirmation in Endocrinology Metabolism Outpatient Clinic of Cerrahpasa Medical School. The Holmes-Rahe Stress Scale and the Life Experience Survey (LES) was the psychological evaluation instrument used in this study. RESULTS: There was no significant difference according to Holmes-Rahe scale (Graves & TNG P = 0.329, Graves & Control P = 0.115, TNG and control P = 0.571). According to LES scale when negative event number, positive event number, neutral events and their effects are considered, between Graves and TNG groups no statistically difference was observed (P = 0.139, P = 0.083, P = 0.167, P = 0.162, P = 0.861). The number and impact of negative SLEs were significantly higher in GD compared to CG (P = 0.015, P < 0.001). CONCLUSION: According to LES scale GD patients has significant difference with respect to CG when negative event number and impact are considered.

[Smoking among thyroid patients]. / Palenie wsród pacjentów tyreologicznych.

UNLABELLED: The effects of cigarette smoking on the thyroid disease (Graves disease, toxic nodular goiter, toxic adenoma) have been studied for years. However, the effect of smoking on thyroid function is still controversial. OBJECTIVES: To determine the impact of cigarette smoking on the development of above-mentioned thyroid disease. PATIENTS METHODS: Study of 405 individuals with different thyroid disease. Information on thyroid disease and smoking habits was gathered by Fagerström questionnaire, and the patients' endocrinologist or general practitioner verified the diagnosis. RESULTS: The effect of smoking was more pronounced in Graves' patients than in other thyroid patients. We found out significant number of patients with toxic nodular goiter. CONCLUSIONS: Smoking is associated with an developing clinically thyroid disease--especially Graves disease. We also suggest the connection between psychological traits and smoking in this group of patients, corresponding with our previous examinations.

[Psychological evaluation of patients with a nodular goiter before and after surgical treatment]. / Psiholoska procena bolesnika sa nodoznom strumom pre i posle hirurske intervencije.

INTRODUCTION: Association between endocrine and mental disorders has been recognized a long time ago, as well as their mutual dependence. The aim of this study was to evaluate the psychological structure of patients with thyroid nodule before and after surgical treatment. MATERIAL AND METHODS: In order to establish the type and degree of psychological disorders, we have examined 60 patients with thyroid nodule before and after surgical treatment by using DSM-IV classification of mental disorders and psychological instruments: semistructured psychiatric interview, MMPI, Zung Depression Scale and a list of panic symptoms. Patients with nonautonomous ("cold") nodules presented as euthyroid, and those with autonomous ("hot") nodules (after a period of drug therapy if they were hyperthyroid), required surgery. After a period of drug therapy two groups of patients were compared: group 1--euthyroid with "cold" nodules and group 2--euthyroid with "hot" nodules. Before surgical treatment both groups underwent psychological evaluation. Group 2 presented with: anxiety, depression and panic disorder with or without agoraphobia (72%, 46%, 28%) while group 1 presented with following results: 48%, 23%, 14% respectively. After surgical treatment patients from group 1 presented with psychologic disorders in less than 1%. DISCUSSION AND CONCLUSION: In some physical disorders, psychologic factors contribute directly or indirectly to the etiology, in others, psychologic symptoms are the direct results of a lesion affecting neural or endocrine organs. One of the key etiological factors is often a short-time or long-time stress and its direct consequence is altered functioning of various hormonal systems. Unexpected high percentage of psychological disorders in patients with thyroid disorders suggests that psychological evaluation before and after surgical treatment is unavoidable for good assessment and choice of treatment.

Relationship between the number and impact of stressful life events and the onset of Graves' disease and toxic nodular goitre.

BACKGROUND: In the last few decades, several studies have suggested a possible association between Stressful Life Events (SLEs) and the onset of Graves' Disease (GD). However, others have criticised this association and it has not yet been possible to prove it unequivocally. At present, we are not aware of studies correlating SLE and non autoimmune thyrotoxicosis. OBJECTIVE: To assess possible associations between SLEs, the onset of GD and the onset of non autoimmune thyrotoxicosis (toxic nodular goitre, TNG). DESIGN: A case-control retrospective study. PATIENTS: This study included 93 subjects, divided into three groups of 31 each: GD, TNG and control (CG). The GD and TNG patients had thyroid disease diagnosed within the last 12 months, with clinical and biochemical confirmation. In the CG, psychopathological and endocrine disturbances had been ruled out. All three groups consisted of nine males (29%) and 22 females (71%). The mean age was 38.4 +/- 10.9 years in the GD group, 48.3 +/- 11.1 years in the TNG group and 41.1 +/- 11.8 years in the CG group. SLEs were evaluated (number and impact) for the 12 months preceding the onset of symptoms of thyroid disease. MEASUREMENTS: SLE occurrences and their impact on each group of cases were measured. To assess SLEs, we used the Life Experiences Survey (LES). Our statistical analysis included descriptive techniques and parametric and/or nonparametric comparative tests. P < 0.01 was considered statistically significant. Odds ratios were also calculated. RESULTS: Patients with GD had a significantly greater number of SLEs compared to the TNG group and the CG (P < 0.001). The number and impact of negative SLEs were significantly higher in GD compared to TNG and CG (P < 0.001). The difference between TNG and CG was not significant (P > 0.01). GD had a higher impact of positive SLEs than TNG (P = 0.004), and no significant differences were found between the GD group and CG. Neutral SLEs were similar in the three groups. CONCLUSIONS: These results suggest that SLEs are a precipitating factor of the onset of GD. We also demonstrated that SLEs do not seem to have any conclusive relationship with the onset of TNG.

Psychoses associated with thyrotoxicosis - 'thyrotoxic psychosis.' A report of 18 cases, with statistical analysis of incidence.

OBJECTIVE: To report a series of newly diagnosed thyrotoxic patients with concurrent acute psychosis, and to assess the association between the two disorders. DESIGN: Retrospective study of thyrotoxic patients with associated psychosis ('thyrotoxic psychosis'; TP) requiring inpatient psychiatric care. New Zealand thyrotoxicosis annual incidence figures and first psychiatric admission rates for affective psychosis were utilised to statistically assess the co-occurrence of thyrotoxicosis and affective psychosis. PATIENTS AND METHODS: During the 20-year study period, 18 inpatients (16 women and 2 men), mean age 54 years, with TP were identified. No patient had a past history of thyrotoxicosis, but four had required psychiatric inpatient care many years earlier. Thyrotoxicosis was documented by radioimmunoassay of thyroid hormone levels, and thyroid scintiscan. Psychiatric manifestations were classified using ICD9 criteria. RESULTS: Thyroid hormone levels were markedly elevated in more than half of our TP patients. All younger patients had Graves' disease, and most older patients toxic nodular goitre. All patients were treated with antithyroid drugs, and all but one subsequently received (131)I therapy. Two patients were not mentally ill when thyrotoxicosis was diagnosed, but suffered major mood swings when thyroid hormone levels were falling. There was no specific psychiatric clinical picture but affective psychoses were commonest - seven depression, seven mania. The other diagnoses were two schizophreniform, one paranoid, and one delirium. Initially, neuroleptic medication was used in all but one patient, and during long-term follow-up (median 11 years) more than half our series had remained well with no further psychiatric problems. Statistical analysis was restricted to thyrotoxic patients with first psychiatric hospital admission for affective psychosis. During the 20-year period, there were nine thyrotoxic patients (95% confidence interval 4.5-17.1) with concurrent affective psychosis requiring first admission, and the calculated expected number was only 0.36. These findings indicate a clear association well above chance co-occurrence. CONCLUSION: TP is not a specific clinical picture, but affective psychoses are commonest. Statistical analysis of thyrotoxic patients with concurrent affective psychoses showed an incidence well above chance co-occurrence. It appears that thyrotoxicosis may be a precipitant of acute affective psychosis.

Depression and anxiety in an 85-year-old woman with a toxic nodular goiter.

Depression is commonly encountered in medical settings and often may be a symptom of an underlying medical illness. This case report illustrates the disabling symptoms of anxiety and depression that can accompany a toxic nodular goiter, and underscores the importance of both accurate psychiatric diagnosis and appropriate medical evaluation in the assessment of psychiatric disorders in the elderly.