Positive family history of idiopathic sudden sensorineural hearing loss

2010 ◽  
Vol 267 (12) ◽  
pp. 1843-1848 ◽  
Author(s):  
A. Gäckler ◽  
A. K. Eickelmann ◽  
D. Brors ◽  
S. Dazert ◽  
J. T. Epplen ◽  
...  
2008 ◽  
Vol 123 (3) ◽  
pp. 359-362 ◽  
Author(s):  
E Kunstmann ◽  
A Eickelmann ◽  
H Sudhoff ◽  
M Pearson ◽  
D Brors

AbstractObjective:This case is reported in order to demonstrate the importance of detailed clinical analysis, including evaluation of personal and family history, in the differential diagnosis of sudden sensorineural hearing loss.Case report:A 50-year-old woman presented with a sudden onset of sensorineural hearing loss in her right ear. She had experienced three previous episodes of sudden sensorineural hearing loss in her left ear, at the ages of 35, 48 and 50 years. She also reported suffering two strokes with left hemiparesis due to fibromuscular dysplasia of her right internal carotid artery. A positive family history of stroke among maternal relatives suggested autosomal dominant inheritance. The patient's personal and family history suggested a rare cause of sudden sensorineural hearing loss, for which alternative therapeutic modalities may be applicable in selected cases.Conclusions:Careful follow up of any patient with sudden sensorineural hearing loss and evaluation of their personal and family history is essential, in order to uncover evidence of rare underlying causes of sudden sensorineural hearing loss. For patients with such rare diagnoses, alternative therapy and surveillance modalities may be useful in disease management, depending on pre-existing pathology. Those patients should be managed via a multidisciplinary approach, including genetic counselling, in order to achieve the best possible outcome.


2015 ◽  
Vol 11 (1) ◽  
pp. 30-32
Author(s):  
Adem Binnetoglu ◽  
Ali Cemal Yumusakhuylu ◽  
Berat Demir ◽  
Tekin Baglam ◽  
Ufuk Derinsu ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Benjamin J. Wycherly ◽  
Jared J. Thompkins ◽  
H. Jeffrey Kim

Objective. To review our experience with intratympanic steroids (ITSs) for the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL), emphasizing the ideal time to perform follow-up audiograms.Methods. Retrospective case review of patients diagnosed with ISSNHL treated with intratympanic methylprednisolone. Injections were repeated weekly with a total of 3 injections. Improvement was defined as an improved pure-tone average ≥20 dB or speech-discrimination score ≥20%.Results. Forty patients met the inclusion criteria with a recovery rate of 45% (18/40). A significantly increased response rate was found in patients having an audiogram >5 weeks after the first dose of ITS (9/13) over those tested ≤5 weeks after the first dose of ITS (9/27) ().Conclusions. Recovery from ISSNHL after ITS injections occurs more frequently >5 weeks after initiating ITS. This may be due to the natural history of sudden hearing loss or the prolonged effect of steroid in the inner ear.


2005 ◽  
Vol 119 (2) ◽  
pp. 148-151 ◽  
Author(s):  
John S Phillips ◽  
Jacquelyn A King ◽  
Siddharthan Chandran ◽  
Peter R Prinsley ◽  
David Dick

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an autosomal dominant angiopathy characterized by recurrent cerebrovascular events, migraine and dementia. We describe a case of sensorineural hearing loss as the presenting feature of this condition. We have found no previous reports in the world literature of CADASIL presenting with a sudden sensorineural hearing loss. The significance of questioning a patient with regard to family history is exemplified in this case.


1995 ◽  
Vol 112 (5) ◽  
pp. P150-P150
Author(s):  
Frederick Byl

Educational objectives: To better understand the etiology and natural history of sudden sensorineural hearing loss and to develop a practical, cost-effective management plan consistent with sound medical judgment.


2019 ◽  
Vol 21 (1) ◽  
pp. 56-63
Author(s):  
Juan C. Ospina-García ◽  
Irene C. Pérez-García ◽  
Diana M. Guerrero-González ◽  
Nataly J. Sánchez-Solano ◽  
Juan D. Salcedo-Betancourt

Objective This study aimed to determine the prevalence of nonsyndromic congenital sensorineural hearing loss at the Hospital Universitario San Ignacio, Bogotá, Colombia, and to describe the risk factors associated with this condition.Materials and Methods A prospective, observational cross-sectional study with bivariate analysis was conducted. A three-phase process using the Otoacoustic Emissions test screened all live newborns between June 2013 and June 2014. Negative cases were confirmed by Automated Auditory Brainstem Response test.Results A total of 962 newborns were screened with Otoacoustic Emissions test bilaterally: 401 males (46.36%), 464 females (53.64%). The mean weight was 2 798.10 g (95%CI: 2 766.51 - 2 839.76). The mean height was 48.60 cm (95%CI: 48.38 - 48.79). The mean age was 16.24 days (95%CI: 15.47 - 17.01). The mean maternal age was 27.37 years (95%CI: 26.76 - 27.98). There was a family history of hearing loss in 9.48% of the cases (n=90), and a family history of genetic diseases in 100 cases (10.56%). There were 14 cases of TORCH infections (1.45%), 375 admissions to the NICU (39.06%), 160 cases of neonatal jaundice (20.1%), and 79 cases of postpartum infections (8.21%). One live newborn presented with microtia.Conclusions The prevalence of congenital sensorineural hearing loss was 0.31% in both ears, and 0.11% in one ear. Currently, Colombia lacks a public universal newborn hearing screening program, and its future implementation faces great challenges. 


2020 ◽  
Vol 25 (4) ◽  
pp. 209-214
Author(s):  
Junfang Xue ◽  
Xin Ma ◽  
Yunjuan Lin ◽  
Haijun Shan ◽  
Lisheng Yu

Introduction: The aim of this study was to investigate and compare the auditory findings in migraine, vestibular migraine (VM), and healthy controls. Methods: Twenty-eight migraine patients (56 ears), 18 VM (36 ears), and 25 healthy controls (50 ears) were included. Audiometry, speech discrimination scores, distortion product optoacoustic emission (DPOAE), and auditory brainstem response were tested. Results: The pure tone in the VM group showed higher thresholds at lower frequencies (250, 500, 1,000, 2,000 Hz) than the control group, with statistical differences observed (P250 Hz = 0.001, P500 Hz = 0.003, P1,000 Hz = 0.016, P2,000 Hz = 0.002). Compared with the healthy controls, the patients with VM had significantly lower amplitudes of DPOAE at 1 kHz (p < 0.001) and 2 kHz (p = 0.020), and the patients with migraine had lower amplitudes at 2 kHz (p = 0.042). Compared with the control group, the patients with migraine reported prolonged latency of wave V (p = 0.016) and IPL I–V (p = 0.003). The patients with VM had significant prolongation of IPL I–V (p = 0.024). Conclusion: Not only the peripheral, but also the central auditory system was involved in patients with migraine and VM. In particular, lower frequencies of the auditory system were more likely to be involved in VM. The history of migraine may be a cause of low-tone sudden sensorineural hearing loss.


2019 ◽  
Vol 99 (8) ◽  
pp. 528-531 ◽  
Author(s):  
Changling Sun ◽  
Xiaolin Xuan ◽  
Zhi Zhou ◽  
Yuan Yuan ◽  
Fei Xue

To date, no specific biomarkers for idiopathic sudden sensorineural hearing loss (ISSHL) have been used. The aim of this study is to investigate whether prestin, the motor protein of cochlear outer hair cells, could be used as a biomarker candidate for the diagnosis and prognosis judgement of ISSHL. Blood samples of 14 ISSHL patients and 28 control patients without history of hearing loss were collected. Plasma prestin concentration was measured using Human Prestin (SLC26A5) ELISA Kit. The results showed that prestin was detectable in the plasma of all patients and the concentration of prestin was significantly higher in ISSHL patients with about half being above the average range of control patients. Moreover, in treatment responsive group, 6 of 10 patients had decreased prestin levels after treatment compared to those of before treatment, while the prestin levels of all the 4 patients in treatment unresponsive group increased in varying degrees. Our promising preliminary results suggest that prestin has the potential to serve as a biomarker to assist diagnosis and judge response to pharmacological treatments.


Sign in / Sign up

Export Citation Format

Share Document