Pendred's Syndrome: A Study of Patients and Relatives

1995 ◽  
Vol 104 (12) ◽  
pp. 957-962 ◽  
Author(s):  
Musa N. Jamal ◽  
Mohammed A. Arnaout ◽  
Ribhi Jarrar
Keyword(s):  
Hearing Loss ◽  
Low Frequency ◽  
Sensorineural Hearing ◽  
The Other ◽  
Test Results ◽  
Profound Hearing Loss ◽  
Low Frequencies ◽  
Pendred’S Syndrome ◽  
Perchlorate Discharge Test ◽  
Discharge Test

Four families, 29 members, with Pendred's syndrome were studied to clarify hearing loss and hormonal status. The ages ranged fro 3 to 50 years. Complete Pendred's syndrome was found in 9 patients. They had bilateral profound hearing loss with residual hearing low frequencies. Goiter was diagnosed at the age of 1 to 14 years with a positive perchlorate discharge test. Twelve of the patient relatives showed partial Pendred's syndrome. Mild sensorineural hearing losses occurred in the low- and medium-range frequencies wi normal perchlorate discharge test results in 6 cases. The other 6 had a slight drop in the perchlorate discharge test results with norm hearing. Five subjects were normal and 3 had normal hormonal and normal perchlorate discharge test results, but were not teste audiologically. This paper shows that patients with Pendred's syndrome may have goiter at birth or develop it between 8 and 14 year that their deafness is bilateral and profound, and that their perchlorate discharge tests are positive. Relatives of Pendred's syndrorr patients showed mild low-frequency sensorineural hearing loss without goiter and normal perchlorate discharge test results in half tl cases, and a slight drop in the perchlorate discharge test results with normal hearing and without goiter in the other half. A correlatic between these findings and genetic studies needs further investigation.

Hearing Rehabilitation in a Patient with Sudden Sensorineural Hearing Loss in the Only Hearing Ear

2008 ◽  
Vol 19 (03) ◽  
pp. 267-274 ◽  
Author(s):  
David B. Hawkins
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
The Other ◽  
Profound Hearing Loss ◽  
Hearing Rehabilitation ◽  
Aural Rehabilitation ◽  
Product Manager ◽  
Software Product

A case report is presented of a 62-year-old software product manager who had normal hearing in one ear and a congenital profound hearing loss in the other ear and then sustained a sudden sensorineural hearing loss in the only hearing ear. The approach to amplification decisions, cochlear implant evaluation, and rehabilitation options are discussed. Providing aural rehabilitation and continually updating and providing new amplification options and accessories are described. Se presenta un reporte de caso de un gerente de productos de software de 62 años de edad quien tenía audición normal en un oído y un sordera congénita profunda en el otro, y quién súbitamente sufrió una sordera sensorineural súbita en el único oído con audición. Se discute el enfoque de decisiones de amplificación, la evaluación para implante coclear, y las opciones de rehabilitación. Se describen las pautas para proveer rehabilitación aural y para actualizar continuamente y aportar nuevas opciones de amplificación.

TYPICAL AND ATYPICAL CASES OF PENDRED'S SYNDROME IN ONE FAMILY

1966 ◽  
Vol 53 (2) ◽  
pp. 264-270 ◽  
Author(s):  
G. M. Bax
Keyword(s):  
Hearing Loss ◽  
Two Generations ◽  
Complete Form ◽  
Pendred’S Syndrome ◽  
Perchlorate Discharge Test ◽  
Loss Of Hearing ◽  
Discharge Test

ABSTRACT Pendred's syndrome is a condition which, in its complete form, is characterized by congenital deaf-mutism, goitre, and defective organic binding of iodide in the thyroid. However, there are less typical cases, e. g. without a goitre, with only limited hearing loss, or a normal perchlorate discharge test. A family was studied in which Pendred's syndrome was found in two generations. The complete form was present in two members; two other members were considered to show less typical forms of the same disorder. All were euthyroid. Three out of five sons were deaf-mute and goitrous. Perchlorate caused a discharge of radioiodine from the thyroid in two of them, and also in the father who had no goitre and a slight loss of hearing only detectable by audiometry. Peripheral deiodination of radioactive diiodotyrosine was normal in all patients, but had been abnormal in one son while he was hypothyroid.

Preferred Low- and High-Frequency Compression Ratios among Hearing Aid Users with Moderately Severe to Profound Hearing Loss

2007 ◽  
Vol 18 (01) ◽  
pp. 017-033 ◽  
Author(s):  
Gitte Keidser ◽  
Harvey Dillon ◽  
Ole Dyrlund ◽  
Lyndal Carter ◽  
David Hartley
Keyword(s):  
Hearing Loss ◽  
High Frequency ◽  
Hearing Aid ◽  
Low Frequency ◽  
Fine Tuning ◽  
Adaptive Procedure ◽  
Profound Hearing Loss ◽  
Low Frequencies ◽  
Frequency Compression ◽  
Fast Acting

This study aimed to determine the low- and high-frequency compression ratios of a fast-acting device that were preferred by people with moderately severe to profound hearing loss. Three compression ratios (1:1, 1.8:1, and 3:1) were combined in the low and high frequencies to produce nine schemes that were evaluated pair-wise for three weeks in the field using an adaptive procedure. The evaluation was performed by 21 experienced hearing aid users with a moderately severe to profound hearing loss. Diaries and an exit interview were used to monitor preferences. Generally, the subjects preferred lower compression ratios than are typically prescribed, especially in the low frequencies. Specifically, 11 subjects preferred linear amplification in the low frequencies, and 14 subjects preferred more compression in the high than in the low frequencies. Preferences could not be predicted from audiometric data, onset of loss, or past experience with amplification. The data suggest that clients with moderately severe to profound hearing loss should be fitted with low-frequency compression ratios in the range 1:1 to 2:1 and that fine-tuning is essential. Este estudio trató de determinar las tasas de compresión de alta y baja frecuencia de un dispositivo de acción rápida, que resultara preferido por personas con hipoacusias moderadamente severas a profundas. Se combinaron tres tasas de compresión (1:1, 1.8:1, y 3:1) en las frecuencias graves y agudas para producir nueve esquemas que fueron evaluados en el campo, en pares, durante tres semanas, utilizando un procedimiento de adaptación. La evaluación fue realizada por 21 usuarios experimentados de audífono con hipoacusias moderadamente severas a profundas. Se usaron diarios y un cuestionario final para monitorear las preferencias. Generalmente, los sujetos prefirieron menores tasas de compresión de lo que típicamente se prescribe, especialmente en las bajas frecuencias. Específicamente, 11 sujetos prefirieron la amplificación lineal en las frecuencias graves y 14 sujetos prefirieron más compresión en las frecuencias altas. Las preferencias no podían predecirse a partir de los datos audiométricos, del inicio de la pérdida, o por experiencias anteriores con amplificación. Los datos sugieren que los clientes con hipoacusias moderadamente severas a profundas, deberían adaptarse con tasas de compresión en las frecuencias graves en el rango de 1:1 a 2:1, y que un ajuste fino es esencial.

scholarly journals Association Between Hearing Characteristics/Prognosis and Vestibular Function in Sudden Sensorineural Hearing Loss With Vertigo

2020 ◽  
Vol 11 ◽  
Author(s):  
Yixu Wang ◽  
Le Wang ◽  
Yuanyuan Jing ◽  
Lisheng Yu ◽  
Fanglei Ye
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Low Frequency ◽  
Vestibular Function ◽  
Vestibular Dysfunction ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Potential Association ◽  
Hearing Recovery

Sudden sensorineural hearing loss (SSNHL) patients with vertigo have a poorer prognosis. However, the factors associated with hearing recovery remain uncertain. This retrospective study was to evaluate the association between hearing characteristics/hearing recovery and the patterns of vestibulocochlear lesions in SSNHL patients with vertigo. Patients were classified into groups according to the patterns of vestibular dysfunction. We not only compared hearing characteristics and prognosis among subgroups but also determined the potential association between vestibular lesion location and hearing recovery. The shapes of the audiogram differed significantly between patients with normal vestibular function and patients with vestibular dysfunction (p = 0.022). Patients whose audiogram indicated profound hearing loss were 3.89 times more likely to have vestibular dysfunction than those whose audiogram shape indicated low-frequency hearing loss (95% CI, 1.02–14.86, p = 0.047). Patients who had saccule dysfunction were 0.11 times as likely to have hearing recovery than those who had normal saccule function (95% CI, 0.11–0.31, p = 0.001). When adjusted for sex and age, patients who had saccule dysfunction were 0.07 times as likely to have hearing recovery than those who had normal saccule function (95% CI, 0.02–0.22, p = 0.001). Abnormal results following cVEMP testing may be a potential predictive factor for poor hearing recovery.

Clinical characteristics and prognosis of low frequency sensorineural hearing loss without vertigo

2015 ◽  
Vol 136 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Ah Ra Jung ◽  
Myung Gu Kim ◽  
Sung Su Kim ◽  
Sang Hoon Kim ◽  
Seung Geun Yeo
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Clinical Characteristics ◽  
Low Frequency ◽  
Sensorineural Hearing

Outcome of Cochlear Implantation in Postmeningitis Profound Sensorineural Hearing Loss at Rajavithi Hospital

2021 ◽  
Vol 104 (2) ◽  
pp. 260-263
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Preoperative Imaging ◽  
Profound Hearing Loss ◽  
Language Status ◽  
Auditory Performance ◽  
Electrode Insertion ◽  
One Year

Background: Bacterial meningitis is one of the major factors in the etiology of acquired sensorineural hearing loss in children and adults. Cochlear implantation in these patients is challenging because of inner ear ossification and fibrosis, and this procedure sometimes achieves poorer outcomes in this scenario than with other causes of sensorineural hearing loss. There has been little research into the factors affecting the outcomes of this procedure. Objective: To evaluate the outcomes of cochlear implantation in patients with postmeningitis profound sensorineural hearing loss and to evaluate the factors that affect the results. Materials and Methods: A retrospective review was conducted of thirty patients who were diagnosed with post meningitis profound hearing loss and underwent cochlear implantation at Rajavithi Hospital between 2001 and 2016. Preoperative language status, duration of deafness, preoperative imaging, and degree of electrode insertion were recorded. Categories of auditory performance-II test (CAP-II) was evaluated in all cases, one year postoperative. Results: Thirty postmeningitis deafness patients underwent cochlear implantation. The median age at diagnosis of meningitis and age at implantation were 41 years (range 1 to 75) and 49.50 years (range 3 to 75), respectively. The median duration of deafness was 12 months (range 4 to 300), and the overall mean CAP-II at one year after surgery was 5.47±2.21. The postlinguistic group had a significantly higher CAP-II score than the prelinguistic one (p=0.006). Electrodes were successfully totally inserted in 19 patients (63.3%) and partially inserted in 11 (36.7%). The average CAP-II score in the group with fully-inserted electrodes was significantly higher than in the group with partially-inserted electrodes (p=0.045). There was no correlation between CAP-II score and age at meningitis diagnosis (p=0.069), age at time of surgery (p=0.105), duration of deafness (p=0.506), or preoperative CT (p=0.228) or MRI abnormality (p=0.078). Conclusion: Cochlear implantation in patients with postmeningitis profound hearing loss had high success rates and favorable outcomes. Preoperative language status and degree of electrode insertion were factors that affected auditory performance results. Keywords: Cochlear implantation, Postmeningitis hearing loss, Sensorineural hearing loss, Meningitis, Rajavithi Hospital

Present Audiologic Status of Subjects Implanted with Auditory Prostheses

1977 ◽  
Vol 86 (3_suppl) ◽  
pp. 40-48 ◽  
Author(s):  
N. T. Hopkinson ◽  
R. C. Bilger ◽  
F. O. Black
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Implant Surgery ◽  
Auditory Prostheses

Audiological testing indicated that all of the subjects had profound sensorineural hearing losses. In 12 of the 13 subjects, this profound hearing loss was bilateral. The 13th subject, however, had useable hearing in his unimplanted ear. Tympanometric testing gave no evidence that implant surgery had disrupted middle ear function.

Significance of 1 kHz Pure-tone Threshold in Acute Low-frequency Sensorineural Hearing Loss

2018 ◽  
Vol 39 (10) ◽  
pp. e950-e955
Author(s):  
Chi Kyou Lee ◽  
Jong Bin Lee ◽  
Kye Hoon Park ◽  
Ho Yun Lee ◽  
Mi-Jin Choi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Pure Tone ◽  
Low Frequency ◽  
Sensorineural Hearing ◽  
Pure Tone Threshold

scholarly journals Effects of Various Extents of High-Frequency Hearing Loss on Speech Recognition and Gap Detection at Low Frequencies in Patients with Sensorineural Hearing Loss

2017 ◽  
Vol 2017 ◽  
pp. 1-9
Author(s):  
Bei Li ◽  
Yang Guo ◽  
Guang Yang ◽  
Yanmei Feng ◽  
Shankai Yin
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Sensorineural Hearing Loss ◽  
High Frequency ◽  
Hearing Threshold ◽  
Sensorineural Hearing ◽  
Gap Detection ◽  
Detection Thresholds ◽  
Low Frequencies ◽  
Compressed Speech

This study explored whether the time-compressed speech perception varied with the degree of hearing loss in high-frequency sensorineural hearing loss (HF SNHL) individuals. 65 HF SNHL individuals with different cutoff frequencies were recruited and further divided into mildly, moderately, and/or severely affected subgroups in terms of the averaged thresholds of all frequencies exhibiting hearing loss. Time-compressed speech recognition scores under both quiet and noisy conditions and gap detection thresholds within low frequencies that had normal thresholds were obtained from all patients and compared with data from 11 age-matched individuals with normal hearing threshold at all frequencies. Correlations of the time-compressed speech recognition scores with the extents of HF SNHL and with the 1 kHz gap detection thresholds were studied across all participants. We found that the time-compressed speech recognition scores were significantly affected by and correlated with the extents of HF SNHL. The time-compressed speech recognition scores also correlated with the 1 kHz gap detection thresholds except when the compression ratio of speech was 0.8 under quiet condition. Above all, the extents of HF SNHL were significantly correlated with the 1 kHz gap thresholds.

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