A case of severe hearing loss in action myoclonus renal failure syndrome resulting from mutation in SCARB2

2012 ◽  
Vol 27 (9) ◽  
pp. 1200-1201 ◽  
Author(s):  
Claudia Perandones ◽  
Federico E. Micheli ◽  
Luis A. Pellene ◽  
Marta A. Bayly ◽  
Samuel F. Berkovic ◽  
...  
Keyword(s):  
Renal Failure ◽  
Hearing Loss ◽  
Severe Hearing Loss ◽  
Action Myoclonus

Otoacoustic Emissions in an Adult With Severe Hearing Loss

1991 ◽  
Vol 34 (3) ◽  
pp. 703-703
Author(s):  
B. A. Prieve ◽  
M. Gorga ◽  
S. T. Neely
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Severe Hearing Loss

scholarly journals Recessive mutations of TMC1 associated with moderate to severe hearing loss

Neurogenetics ◽  
2016 ◽  
Vol 17 (2) ◽  
pp. 115-123 ◽  
Author(s):  
Ayesha Imtiaz ◽  
Azra Maqsood ◽  
Atteeq U. Rehman ◽  
Robert J. Morell ◽  
Jeffrey R. Holt ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Severe Hearing Loss ◽  
Recessive Mutations

scholarly journals Speech Sound Production in 2-Year-Olds Who Are Hard of Hearing

2014 ◽  
Vol 23 (2) ◽  
pp. 91-104 ◽  
Author(s):  
Sophie E. Ambrose ◽  
Lauren M. Unflat Berry ◽  
Elizabeth A. Walker ◽  
Melody Harrison ◽  
Jacob Oleson ◽  
...  
Keyword(s):  
Socioeconomic Status ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Hard Of Hearing ◽  
Sound Production ◽  
Speech Sound ◽  
Linguistic Variables ◽  
Vowel Production ◽  
Severe Hearing Loss ◽  
Children's Speech

Purpose The purpose of the study was to (a) compare the speech sound production abilities of 2-year-old children who are hard of hearing (HH) to children with normal hearing (NH), (b) identify sources of risk for individual children who are HH, and (c) determine whether speech sound production skills at age 2 were predictive of speech sound production skills at age 3. Method Seventy children with bilateral, mild-to-severe hearing loss who use hearing aids and 37 age- and socioeconomic status–matched children with NH participated. Children's speech sound production abilities were assessed at 2 and 3 years of age. Results At age 2, the HH group demonstrated vowel production abilities on par with their NH peers but weaker consonant production abilities. Within the HH group, better outcomes were associated with hearing aid fittings by 6 months of age, hearing loss of less than 45 dB HL, stronger vocabulary scores, and being female. Positive relationships existed between children's speech sound production abilities at 2 and 3 years of age. Conclusion Assessment of early speech sound production abilities in combination with demographic, audiologic, and linguistic variables may be useful in identifying HH children who are at risk for delays in speech sound production.

scholarly journals The Physiologic and Psychophysical Consequences of Severe-to-Profound Hearing Loss

2018 ◽  
Vol 39 (04) ◽  
pp. 349-363 ◽  
Author(s):  
Eric Hoover ◽  
Pamela Souza
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Treatment Options ◽  
Sufficient Information ◽  
Noisy Environments ◽  
Cochlear Function ◽  
Profound Hearing Loss ◽  
Sound Perception ◽  
Severe Hearing Loss ◽  
Tuning Curves

AbstractSubstantial loss of cochlear function is required to elevate pure-tone thresholds to the severe hearing loss range; yet, individuals with severe or profound hearing loss continue to rely on hearing for communication. Despite the impairment, sufficient information is encoded at the periphery to make acoustic hearing a viable option. However, the probability of significant cochlear and/or neural damage associated with the loss has consequences for sound perception and speech recognition. These consequences include degraded frequency selectivity, which can be assessed with tests including psychoacoustic tuning curves and broadband rippled stimuli. Because speech recognition depends on the ability to resolve frequency detail, a listener with severe hearing loss is likely to have impaired communication in both quiet and noisy environments. However, the extent of the impairment varies widely among individuals. A better understanding of the fundamental abilities of listeners with severe and profound hearing loss and the consequences of those abilities for communication can support directed treatment options in this population.

scholarly journals Complications of cochlear implant surgery

2004 ◽  
Vol 132 (9-10) ◽  
pp. 302-305 ◽  
Author(s):  
Rade Kosanovic ◽  
Zoran Ivankovic ◽  
Sandra Stojanovic
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Fatal Outcome ◽  
Modern Technology ◽  
Hearing Impairments ◽  
Severe Hearing Loss ◽  
Cochlear Implant Surgery ◽  
Coding Strategies ◽  
The World

During the last several decades, cochlear implant has been fully recognized in treatment of severe hearing loss. Development of modern technology enabled inconceivable possibilities of technical qualities of the device as well as development of usable coding strategies, which led to extraordinary results in patient rehabilitation. Although cochlear implantation has become one of the routine operative procedures throughout the world nowadays, it gives rise to certain complications. These complications, though rare, can sometimes be very serious, even with fatal outcome. If cochlear implantation is performed by experienced and well-educated team of experts, the possibility of complications is minimal and is certainly not the argument against cochlear implantation as a method of treatment of severe hearing impairments.

scholarly journals Evaluating the Prevalence of Sensorineural Hearing Loss in Chronic Renal Failure Patients Undergoing Hemodialysis in Imam Khomeini Hospital, Ahvaz, 2012-2014

2015 ◽  
Vol 12 (Special-Edn2) ◽  
pp. 91-95
Author(s):  
Mozafar Sarafraz ◽  
Seyed Seifollah Beladi Musavi ◽  
Somayeh Araghi ◽  
Samaneh Azizi Manesh
Keyword(s):  
Renal Failure ◽  
Hearing Loss ◽  
Chronic Renal Failure ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing

scholarly journals Otosclerosis Revisited

2019 ◽  
Vol 27 (1) ◽  
Author(s):  
C V Srinivas
Keyword(s):  
Hearing Loss ◽  
Current Treatment ◽  
P Value ◽  
College Hospital ◽  
Severe Hearing Loss ◽  
Medical College ◽  
Mixed Hearing Loss ◽  
Operative Outcome ◽  
Conductive Component ◽  
Indian Scenario

Introduction: Otosclerosis usually manifests as a progressive conductive or mixed hearing loss occurring clinically to varying degrees in 0.5% - 1% of the general population. Stapedectomy /stapedotomy is the current treatment of choice for conductive component of Otosclerosis. Materials and Methods: Sixty patients attending the department of ENT of a Medical College Hospital in Bangalore between October 2012 and October 2017 were included in the study.  Results: The incidence of otosclerosis is highest in the 3rd and 4th decade. Bilateral symptoms were present in 25% and 75% had unilateral symptoms, right side being more common in our study. Tinnitus and vertigo with deafness are seen in 60% and 25 % of the patients respectively.  Ninety percent of the patients presented with moderate to moderately severe hearing loss. Twenty four (40%) out of 60 audiograms studied showed Carhart's notch. Postoperativeclosure of a-b gap to within 10dB could be achieved in 65% cases. The p value calculated comparing pre and post op results is <0.0001 and significant. Vertigo was found to be most common post-operative complication (35%) followed by sensorineural hearing loss (6.6%). Other complications observed during the study include -  tympanic membrane tears, foot plate extrusion, lax long process of incus, perilymph leak. Conclusion: From the assessment of the postoperative hearing gain and postoperative complications, it can be concluded that small fenestra stapedotomy gives satisfactory post-operative outcome and may be appropriatein the Indian scenario.

scholarly journals Novel mutations of MYO15A associated with profound deafness in consanguineous families and moderately severe hearing loss in a patient with Smith-Magenis syndrome

2001 ◽  
Vol 109 (5) ◽  
pp. 535-541 ◽  
Author(s):  
Nikki Liburd ◽  
Manju Ghosh ◽  
Saima Riazuddin ◽  
Sadaf Naz ◽  
Shaheen Khan ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Novel Mutations ◽  
Severe Hearing Loss ◽  
Profound Deafness
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