scholarly journals Auditory dysfunction in type 2 Stickler Syndrome

2020 ◽  
Author(s):  
Philip Alexander ◽  
Philip Gomersall ◽  
Jack Stancel-Lewis ◽  
Gregory Scott Fincham ◽  
Arabella Poulson ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Spatial Information ◽  
Self Report ◽  
Direct Result ◽  
Stickler Syndrome ◽  
Hearing Sensitivity ◽  
Central Processing ◽  
Auditory Dysfunction ◽  
The Impact

Abstract Purpose To present the extent and site of lesion of auditory dysfunction in a large cohort of individuals with type 2 Stickler Syndrome. Type 2 Stickler Syndrome results from a mutation in the gene coding for α-1 type XI pro-collagen, which has been identified in the human vitreous, cartilage and the cochlea of the mouse. The condition is characterised by classic ocular abnormalities, auditory dysfunction, osteoarthropathy and oro-facial dysplasia. Methods This is a population study which used a combination of audiometric, tympanometric, and self-report measures on a series of 65 individuals (mean age 29.2 years, range 3–70, female 63.1%) with genetically confirmed type 2 Stickler Syndrome. Results Hearing impairment was identified in at least one ear for 69% of individuals. Analysis against age-matched normative data showed that reduced hearing sensitivity was present across all test frequencies. Sensorineural hearing loss was most common (77% of ears), with conductive (3%), mixed (7%) and no hearing loss (13%), respectively. The proportion of hypermobile tympanic membranes (24%) was less than previously documented in type 1 Stickler Syndrome. When present, this appears to arise as a direct result of collagen abnormalities in the middle ear. Self-report measures of speech and spatial hearing in sound were comparable to a non-syndromic cohort with similar audiometric thresholds. Conclusions Auditory impairment in type 2 Stickler Syndrome is predominantly associated with cochlear hearing loss of varying severities across affected individuals. The impact on hearing thresholds can be seen across the frequency range, suggesting a contribution of defective collagen throughout the cochlea. Self-report questionnaires showed that difficulties understanding speech, and spatial information in sound (such as that used for localisation), were worse than a young, normal-hearing population but comparable to a non-syndromic cohort with similar audiometric thresholds. Therefore, it is likely that hearing loss in type 2 Stickler Syndrome arises in the auditory periphery, without significant central processing deficits.

scholarly journals The Relationship between Severity of Hearing Loss and Subjective Tinnitus Loudness among Patients Seen in a Specialist Tinnitus and Hyperacusis Therapy Clinic in UK

2019 ◽  
Vol 30 (08) ◽  
pp. 712-719 ◽  
Author(s):  
Hashir Aazh ◽  
Richard Salvi
Keyword(s):  
Hearing Loss ◽  
Regression Model ◽  
Pure Tone ◽  
Cross Sectional Study ◽  
Emotional Disturbances ◽  
Self Report ◽  
Strongly Correlated ◽  
Cross Sectional ◽  
The Impact ◽  
The Relationship

AbstractHearing loss is often associated with the phantom sound of tinnitus. However, the degree of the association between severity of hearing loss and tinnitus loudness taking into account the impact of other variables (e.g., emotional disturbances) is not fully understood. This is an important question for audiologists who are specialized in tinnitus rehabilitation as patients often ask whether the loudness of their tinnitus will increase if their hearing gets worse.To explore the relationship between tinnitus loudness and pure tone hearing thresholds.This was a retrospective cross-sectional study.445 consecutive patients who attended a Tinnitus and Hyperacusis Therapy Specialist Clinic in UK were included.The results of audiological tests and self-report questionnaires were gathered retrospectively from the records of the patients. Multiple-regression analysis was used to assess the relationship between tinnitus loudness, hearing loss and other variables.The regression model showed a significant relationship between the pure tone average (PTA) at the frequencies 0.25, 0.5, 1, 2, and 4 kHz of the better ear and the tinnitus loudness as measured via visual analogue scale (VAS), r (regression coefficient) = 0.022 (p < 0.001). Other variables significantly associated with tinnitus loudness were tinnitus annoyance (r = 0.49, p < 0.001) and the effect of tinnitus on life (r = 0.09, p = 0.006). The regression model explained 52% of the variance of tinnitus loudness.Although increased tinnitus loudness was associated with worse PTA, the relationship was very weak. Tinnitus annoyance and impact of tinnitus on life were more strongly correlated with tinnitus loudness than PTA.

scholarly journals Looking beyond the audiogram in ototoxicity associated with platinum-based chemotherapy

2019 ◽  
Vol 85 (2) ◽  
pp. 245-250 ◽  
Author(s):  
David M. Baguley ◽  
Pattarawadee Prayuenyong
Keyword(s):  
Hearing Loss ◽  
Real Life ◽  
Pure Tone Audiometry ◽  
Self Report ◽  
Surveillance Program ◽  
Speech Comprehension ◽  
High Frequency Audiometry ◽  
Platinum Based Chemotherapy ◽  
Monitoring And Surveillance ◽  
The Impact

Abstract Introduction Ototoxicity associated with platinum-based chemotherapy is highly prevalent and can cause detrimental consequences among cancer survivors. Discussion In this article, we highlight important aspects of the evaluation of ototoxicity with the aim to increase awareness of Oncologists in this regard. Standard pure tone audiometry alone is inadequate for this context. Comprehensive and consistent hearing tests should be implemented in a monitoring and surveillance program. High-frequency audiometry (10–16 kHz) is a sensitive tool in the detection of ototoxic hearing loss at onset. In addition to threshold audiometry, measures of speech comprehension (both in quiet and in noise) can add useful information in the evaluation of hearing in real-life situations. Not only hearing loss, but also tinnitus and imbalance are common in patients who receive platinum-based chemotherapy, and can cause debilitating effects upon quality of life in this population. Moreover, self-report measures associated with cochlear and vestibular handicaps can provide valuable information regarding the impact of ototoxicity. Conclusions It is vital to build awareness about the variety and impact of the symptoms of ototoxicity. Comprehensive evaluation of hearing status along with self-reported impact of the cochlear and vestibular handicap should be implemented in a monitoring and surveillance program for appropriate investigation and management.

scholarly journals Modern Drugs in the Treatment of Type 2 Diabetes Mellitus

2021 ◽  
Vol 6 (4) ◽  
pp. 15-21
Author(s):  
I. O. Marazha ◽  
◽  
D. I. Nazarova ◽  
S. B. Kramar ◽  
◽  
...  
Keyword(s):  
Physical Activity ◽  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
Cardiovascular Diseases ◽  
World Health ◽  
Direct Result ◽  
Management Protocol ◽  
The Impact

The purpose of the study is to analyze the impact of type 2 diabetes on the skin, to establish the pathogenesis and analysis of modern treatments. Materials and methods. The materials and methods of research were theoretical analysis of scientific literature; statistical methods of literature data analysis, comparative analysis and classification according to the protocol of the international prospective study International Prevalence and Treatment of Diabetes and Depression (INTERPRET-DD). The emphasis is placed on the relevance of research in ways to treat diabetes as a serious disease with high mortality. The statistical indicators of the disease are given and the percentages of disability are indicated. The pathogenesis of type 2 diabetes mellitus is considered and risk factors for the disease are listed. Some diabetic-associated skin symptoms are said to be a direct result of metabolic changes such as hyperglycemia and hyperlipidemia. Progressive damage to the vascular, nervous or immune systems also greatly contributes to the development of skin manifestations. Early medication of insulin should be considered in case of continuous weight loss, symptoms of hyperglycemia or HbA1c in blood > 10% (86 mmole/mole) or plasma glucose ≥300 mg/dL (≥16.7 mmole/l). In patients with type 2 diabetes mellitus with cardiovascular diseases of atherosclerotic etiology or high risk, diabetic kidney disease or heart failure, it is recommended to use inhibitors of the sodium-dependent glucose cotransporter of the type 2 or agonists of the glucagon-1 peptide receptor, which are effective with cardiovascular diseases, regardless of the level of HbA1c in the blood, taking into account individual factors. Conclusion. It was found that the initial treatment, according to the World Health Organization in 2020, should begin with the appointment of metformin, which is a first-line drug for the treatment of patients, that does not cause weight gain, does not provoke hypoglycemia and is recommended for the treatment of people who do not reach the desired level of glycemia through diet and physical activity. It is emphasized that diabetes should be treated comprehensively: diet, physical activity, metformin. Metformin drugs are the basic therapy and should be prescribed in any situation in the presence of disorders of carbohydrate metabolism and if there are no contraindications to their use. It is important to increase the dosage of metformin gradually according to the diabetes management protocol: all hypoglycemic drugs should be titrated from the lowest dosage to that necessary to ensure good compensation of carbohydrate indices. Intensification of treatment of patients with type 2 diabetes mellitus in case of failure to achieve the main goals of therapy should be timely

scholarly journals Hearing loss among patients with type 2 diabetes mellitus: a cross-sectional study

2021 ◽  
Vol 41 (3) ◽  
pp. 171-178
Author(s):  
Khalid Al-Rubeaan ◽  
Murad AlMomani ◽  
Aisha Khalaf AlGethami ◽  
Jamal Darandari ◽  
Abdulaziz Alsalhi ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Risk Factors ◽  
Type 2 Diabetes ◽  
Hearing Loss ◽  
Comorbid Condition ◽  
Multivariate Logistic Regression ◽  
Cross Sectional ◽  
Hearing Sensitivity ◽  
Factors Associated

BACKGROUND: Hearing loss is an underestimated comorbid condition in type 2 diabetes. OBJECTIVES: Investigate hearing loss as a comorbidity associated with type 2 diabetes mellitus and evaluate the factors associated with hearing loss. DESIGN: Cross-sectional. SETTING: Tertiary care center, diabetes clinic. PATIENTS AND METHODS: Patients with type 2 diabetes, aged 30 to 60 years, were randomly selected to participate. All patients underwent clinical ear examinations and were referred for full audiological evaluation. Otoacoustic emission was used to assess inner function, tympanometry to assess middle-ear function, and pure tone air/bone audiometry to assess hearing sensitivity. Risk factors for hearing loss were assessed by multivariate logistic regression. MAIN OUTCOME MEASURE: Frequency, severity and risk factors for hearing loss. SAMPLE SIZE: 157 RESULTS: Of the 157 patients, 77 had hearing loss in both ears (49.0%), 13 in the right ear only (8.3%), 14 in the left ear only (8.9%), and 53 (33.8%) had normal hearing. In the 181 ears with sensorineural hearing loss, 90 had mild loss (49.7%), 69 moderate loss (38.2%), 16 severe loss (8.8%) and 6 had profound loss (3.3%). Disabling hearing loss was observed in 46 (29%) patients. A higher frequency of hearing loss was present in patients with glycated hemoglobin levels ≥8%. In the multivariate logistic regression analysis, the most important factors associated with hearing loss were longer diabetes duration, poor glycemic control and the presence of hypertension. CONCLUSIONS: Hearing loss is an underestimated comorbid condition in type 2 diabetes that warrants frequent hearing assessments and management. Strict glycemic and hypertension control is essential for the minimization of the effects of diabetes on hearing sensitivity. LIMITATIONS: Small sample size, limited age window (30-60 years), which was chosen to eliminate the natural aging effect on hearing. Cross-sectional nature was not ideal for the assessment of causality. CONFLICT OF INTEREST: None.

scholarly journals Inherited and de novo biallelic pathogenic variants in COL11A1 result in type 2 Stickler syndrome with severe hearing loss

2020 ◽  
Vol 8 (9) ◽  
Author(s):  
Thomas Nixon ◽  
Allan J. Richards ◽  
Adrian Lomas ◽  
Stephen Abbs ◽  
Pradeep Vasudevan ◽  
...  
Keyword(s):  
Hearing Loss ◽  
De Novo ◽  
Stickler Syndrome ◽  
Severe Hearing Loss ◽  
Pathogenic Variants

scholarly journals Prevalence of Hearing Loss in Type 2 Diabetes Mellitus and Its Association with Severity of Diabetic Neuropathy

2022 ◽  
Author(s):  
Abin M Abraham ◽  
Ashish Varghese ◽  
Jubbin Jagan Jacob
Keyword(s):  
Type 2 Diabetes ◽  
Hearing Loss ◽  
Diabetic Neuropathy ◽  
Hearing Threshold ◽  
Self Report ◽  
Increased Risk ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Purpose This study assessed the prevalence of hearing loss (HL) in patients with Type 2 Diabetes (T2DM) and its relationship with the presence and severity of diabetic neuropathy. Methods Patients between the ages of 30 to 60 years (both ages inclusive) with T2DM were recruited and divided into three groups. Group 1 included patients without neuropathy. Group 2 had patients with mild neuropathy. Group 3 had patients with moderate and severe neuropathy. After informed consent hearing threshold was assessed using pure tone audiometry (PTA). Results Of the 200 patients recruited, the prevalence of hearing loss was overall 81%. The prevalence was 66.7% in group 1, 80.9% in group 2 and 87.6% in group 3 (p=0.009). Among patients with moderate to severe neuropathy (group 3) 33.3% had clinically significant hearing loss (p=0.015). Age, gender, presence of neuropathy and severity of neuropathy were associated with increased risk of developing hearing loss. Severity of hearing loss worsened with increase in severity of neuropathy. Conclusions Age, gender and severity of neuropathy were associated with increased risk of developing hearing loss. Screening for hearing loss in patients with moderate to severe diabetic neuropathy using self-report questionnaires can help in timely diagnosis and treatment.

Epidemiological studies of physical activity and diabetes risk, and implications for diabetes preventionThis paper was originally part of the Symposium “Exercise, insulin sensitivity and diabetes—what’s new?”, which appeared in the June issue of Appl. Physiol. Nutr. Metab.

2007 ◽  
Vol 32 (4) ◽  
pp. 778-782 ◽  
Author(s):  
Nicholas J. Wareham
Keyword(s):  
Physical Activity ◽  
Physical Inactivity ◽  
Objective Assessment ◽  
Epidemiological Studies ◽  
Self Report ◽  
Metabolic Risk ◽  
Activity Levels ◽  
Preventive Interventions ◽  
The Impact

The evidence linking physical inactivity to the future risk of type 2 diabetes is strong, and modification of behaviour is a critical and effective element of strategies aimed at the prevention of this increasingly prevalent disorder. Two key unresolved epidemiologic issues relate to the type of activity that is likely to be maximally effective in preventing diabetes and the amount of activity that is required. Resolution of both these issues is likely to require a change in the way activity is measured, with a move away from self-report instruments, toward objective assessment of activity and the pattern and overall level of energy expenditure. It is also unclear whether the impact of physical activity on metabolic risk is homogenous across the population. Subgroups that might respond differently could be defined on the basis of characteristics such as age, degree of obesity, family history, ethnicity, and genetic risk, but the literature on effect modification is limited by study design issues. The identification of such subgroups could aid in the targeting of preventive interventions. An appropriate balance between individually tailored approaches aimed at those at high risk and interventions aimed at trying to shift physical activity levels in entire populations remains to be determined.

Association Between Tinnitus Pitch and Consonant Recognition in Noise

2020 ◽  
Vol 29 (4) ◽  
pp. 916-929
Author(s):  
Yihsin Tai ◽  
Fatima T. Husain
Keyword(s):  
Hearing Loss ◽  
Word Recognition ◽  
Pure Tone Audiometry ◽  
Error Rates ◽  
Self Report ◽  
Hearing Sensitivity ◽  
Sentence Recognition ◽  
Speech In Noise ◽  
Consonant Recognition ◽  
Noise Test

Purpose Difficulties in speech-in-noise understanding are often reported in individuals with tinnitus. Building on our previous findings that speech-in-noise performance is correlated with subjective loudness of tinnitus, this study aimed to investigate the effect of tinnitus pitch on consonant recognition in noise. Method Pure-tone audiometry and the Quick Speech-in-Noise Test were conducted on 66 participants categorized into four groups by their hearing sensitivity and self-report of tinnitus. Consonant recognition scores at various frequency ranges were obtained at the 5 dB SNR condition of the Quick Speech-in-Noise Test. Participants with tinnitus also completed a tinnitus pitch-matching procedure. Correlation analyses were conducted between tinnitus pitch and the frequency of the worst consonant recognition, and the error rates based on word and sentence position were compared. Results Regardless of hearing sensitivity, tinnitus pitch did not correlate with the frequency of the worst consonant recognition. Sentence-initial word recognition was affected by hearing loss, whereas sentence-final word recognition was not affected by hearing loss or tinnitus. In contrast to individuals with normal hearing, participants with hearing loss varied in full-sentence recognition, with those reporting tinnitus exhibiting significantly higher error rates. Conclusions The findings suggest that the effect of tinnitus on consonant recognition in noise may involve higher level functions more than perceptual characteristics of tinnitus. Furthermore, for individuals with speech-in-noise concerns, clinical evaluation should address both hearing sensitivity and the presence of tinnitus. Future speech-in-noise studies should incorporate cognitive tests and, possibly, brain imaging to parse out the contribution of cognitive factors, such as cognitive control, in speech-in-noise in tinnitus.

scholarly journals Alternative splicing modifies the effect of mutations in COL11A1 and results in recessive type 2 Stickler syndrome with profound hearing loss

2013 ◽  
Vol 50 (11) ◽  
pp. 765-771 ◽  
Author(s):  
Allan J Richards ◽  
Gregory S Fincham ◽  
Annie McNinch ◽  
David Hill ◽  
Arabella V Poulson ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Alternative Splicing ◽  
Stickler Syndrome ◽  
Profound Hearing Loss ◽  
Recessive Type

The Relationship Between Hearing Loss Self-Management and Hearing Aid Benefit and Satisfaction

2019 ◽  
Vol 28 (2) ◽  
pp. 274-284 ◽  
Author(s):  
Elizabeth Convery ◽  
Gitte Keidser ◽  
Louise Hickson ◽  
Carly Meyer
Keyword(s):  
Decision Making ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Clinical Decision Making ◽  
Hearing Aid ◽  
Well Being ◽  
Self Report ◽  
Self Management ◽  
Hearing Aid Benefit ◽  
The Relationship

Purpose Hearing loss self-management refers to the knowledge and skills people use to manage the effects of hearing loss on all aspects of their daily lives. The purpose of this study was to investigate the relationship between self-reported hearing loss self-management and hearing aid benefit and satisfaction. Method Thirty-seven adults with hearing loss, all of whom were current users of bilateral hearing aids, participated in this observational study. The participants completed self-report inventories probing their hearing loss self-management and hearing aid benefit and satisfaction. Correlation analysis was used to investigate the relationship between individual domains of hearing loss self-management and hearing aid benefit and satisfaction. Results Participants who reported better self-management of the effects of their hearing loss on their emotional well-being and social participation were more likely to report less aided listening difficulty in noisy and reverberant environments and greater satisfaction with the effect of their hearing aids on their self-image. Participants who reported better self-management in the areas of adhering to treatment, participating in shared decision making, accessing services and resources, attending appointments, and monitoring for changes in their hearing and functional status were more likely to report greater satisfaction with the sound quality and performance of their hearing aids. Conclusion Study findings highlight the potential for using information about a patient's hearing loss self-management in different domains as part of clinical decision making and management planning.

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