Speech Perception in Noise by Listeners With Hearing Impairment and Simulated Sensorineural Hearing Loss

2019 ◽  
pp. 461-473 ◽  
Author(s):  
Alyssa R. Needleman ◽  
Carl C. Crandell
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Sensorineural Hearing ◽  
Speech Perception In Noise

Evaluation of The Speech Perception in Noise (Spin) Test

1979 ◽  
Vol 87 (2) ◽  
pp. 239-245 ◽  
Author(s):  
Robert W. Hutcherson ◽  
Donald D. Dirks ◽  
Donald E. Morgan
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Sensorineural Hearing Loss ◽  
Clinical Utility ◽  
Normal Hearing ◽  
Sensorineural Hearing ◽  
Speech Communication ◽  
Speech Discrimination ◽  
Speech Perception In Noise ◽  
Presentation Level

Several investigations were performed with normal hearing subjects to determine the effects of presentation level and signal-to-babble ratio on the speech perception in noise (SPIN) test. The SPIN test contains sentences that simulate a range of contextual situations encountered in everyday speech communication. Findings from several representative patients with sensorineural hearing loss demonstrate the possible clinical utility of the test to measure the effects of context on speech discrimination.

scholarly journals Novel USH1G homozygous variant underlying USH2-like phenotype of Usher syndrome

2019 ◽  
pp. 112067211987939
Author(s):  
Fabiana D’Esposito ◽  
Viviana Randazzo ◽  
Gilda Cennamo ◽  
Nicola Centore ◽  
Paolo Enrico Maltese ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Usher Syndrome ◽  
Age At Onset ◽  
Vestibular Dysfunction ◽  
Sensorineural Hearing ◽  
Type I ◽  
Missense Variation

Purpose: Usher syndrome (USH) is an autosomal recessive disorder characterized by congenital sensorineural hearing impairment and retinitis pigmentosa. Classification distinguishes three clinical types of which type I (USH1) is the most severe, with vestibular dysfunction as an added feature. To date, 15 genes and 3 loci have been identified with the USH1G gene being an uncommon cause of USH. We describe an atypical USH1G-related phenotype caused by a novel homozygous missense variation in a patient with profound hearing impairment and relatively mild retinitis pigmentosa, but no vestibular dysfunction. Methods: A 26-year-old female patient with profound congenital sensorineural hearing loss, nyctalopia and retinitis pigmentosa was studied. Audiometric, vestibular and ophthalmologic examination was performed. A panel of 13 genes was tested by next-generation sequencing (NGS). Results: While the hearing loss was confirmed to be profound, the vestibular function resulted normal. Although typical retinitis pigmentosa was present, the age at onset was unusually late for USH1 syndrome. A novel homozygous missense variation (c.1187T>A, p.Leu396Gln) in the USH1G gene has been identified as causing the disease in our patient. Conclusions: Genetic and phenotypic heterogeneity are very common in both isolated and syndromic retinal dystrophies and sensorineural hearing loss. Our findings widen the spectrum of USH allelic disorders and strength the concept that variants in genes that are classically known as underlying one specific clinical USH subtype might result in unexpected phenotypes.

Relation between Frequency Selectivity and Speech Perception Following Glycerol-Induced Cochlear Changes

1985 ◽  
Vol 50 (4) ◽  
pp. 372-377 ◽  
Author(s):  
Patricia G. Stelmachowicz ◽  
Dawna E. Johnson ◽  
Lori L. Larson ◽  
Patrick E. Brookhouser
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Sensorineural Hearing Loss ◽  
Frequency Selectivity ◽  
Auditory Threshold ◽  
Sensorineural Hearing ◽  
Frequency Resolution ◽  
Intersubject Variability ◽  
Tuning Curves ◽  
Clear Relation

Changes in auditory threshold, psychophysical tuning curves, and speech perception (in both quiet and noise) were monitored over a 3-hr period following the ingestion of glycerol. All listeners had sensorineural hearing loss secondary to Menière's disease. Findings were characterized by large intersubject variability and in general did not show a clear relation between changes in threshold, frequency resolution, and speech perception.

Acupuncture and Sensorineural Hearing Loss: A Review

1975 ◽  
Vol 40 (4) ◽  
pp. 427-433 ◽  
Author(s):  
Harvey A. Taub
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Placebo Effect ◽  
Sensorineural Hearing ◽  
Hearing Ability ◽  
Sensorineural Hearing Impairment

Recent reports concerning the use of acupuncture as a possible treatment for sensorineural hearing impairment were reviewed and evaluated. It was concluded that acupuncture has no effect upon the hearing ability of individuals with sensorineural losses. Further, it was suggested that subjective feelings of improvement represent a placebo effect resulting from the application of a treatment that patients believe might work and not from acupuncture itself.

scholarly journals Sensorineural Hearing Loss: What Lies Beneath? Neurovascular Conflict Secondary to a Dural Arteriovenous Malformation

2014 ◽  
Vol 29 (2) ◽  
pp. 37-38
Author(s):  
Ian C. Bickle
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Sensorineural Hearing ◽  
Assessment Process ◽  
External Carotid ◽  
Neurovascular Conflict ◽  
Vestibulocochlear Nerve ◽  
Presenting Symptoms ◽  
Wide Range

This middle-aged gentleman with no previous medical history presented to the local ENT outpatient clinic complaining of right-sided hearing loss.  No history of trauma or previous head and neck surgery was elicited. Following clinical and auditory assessment a right sensorineural hearing loss was confirmed.  A right-sided facial palsy was additionally identified on examination. A MRI of the internal auditory meati was performed (Figure 1a & 1b).  Following radiologist review, MRI and MRA of the brain was undertaken.   DISCUSSION   Auditory impairment is a condition with a legion of potential causes. One of the routine aspects of the assessment process for those with sensorineural hearing loss is MR imaging (MRI) of the internal auditory meati (IAMS).   The vast majority of MRI studies are normal, however one of the more commonly identified pathologies are cerebrovascular abnormalities. The most well recognised is neurovascular conflict of the vestibulocochlear nerve by a vascular loop at the root entry zone (REZ), however a broader range of potential responsible structural abnormalities are known. A wide range of processes for auditory dysfunction have been outlined.1 These include; cerebral ischaemia events, subarachnoid haemorrhage, cerebrovascular malformations and rarely dural arteriovenous fistulas (dAVFs).   Dural AVF's are abnormal vascular communications between the dural venous sinuses and an arter(ies) - most frequently branches of the external carotid artery. Sensorineural hearing impairment is one of the rarer presenting symptoms. The mechanism for hearing impairment is believed to result from either direct vascular compression on the vestibulocochlear nerve from an enlarged aberrant draining vein or from a vascular steal phenomenon (Figures 2a & 2b). An engorged draining vein from the dAVF causing mechanical compression on the nerve is the most well recognized.2 A single prior case has been reported of compression from an intraossesous dAVF of the skull base.3   The arteriovenous fistula may be directed identified (Figure 3) along with the associated signs of enlarged cerebral cortical veins and white matter change of venous hypertension (Figure 4).  

scholarly journals Characterization of Sensorineural Hearing Loss in Children with Alport Syndrome

Life ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 360
Author(s):  
Jan Boeckhaus ◽  
Nicola Strenzke ◽  
Celine Storz ◽  
Oliver Gross ◽  
◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Alport Syndrome ◽  
Age Groups ◽  
Hearing Impaired ◽  
Sensorineural Hearing ◽  
Pure Tone Average ◽  
Hearing Thresholds

Most adults with Alport syndrome (AS) suffer from progressive sensorineural hearing loss. However, little is known about the early characteristics of hearing loss in children with AS. As a part of the EARLY PRO-TECT Alport trial, this study was the first clinical trial ever to investigate hearing loss in children with AS over a timespan of up to six years Nine of 51 children (18%) had hearing impairment. Audiograms were divided into three age groups: in the 5–9-year-olds, the 4-pure tone average (4PTA) was 8.9 decibel (dB) (n = 15) in those with normal hearing and 43.8 dB (n = 2, 12%) in those with hearing impairment. Among the 10–13-year-olds, 4PTA was 4.8 dB (healthy, n = 12) and 41.4 dB (hearing impaired, n = 6.33%). For the 14–20-year-olds, the 4PTA was 7.0 dB (healthy; n = 9) and 48.2 dB (hearing impaired, n = 3.25%). On average, hearing thresholds of the hearing impaired group increased, especially at frequencies between 1–3 kHz. In conclusion, 18% of children developed hearing loss, with a maximum hearing loss in the audiograms at 1–3 kHz. The percentage of children with hearing impairment increased from 10% at baseline to 18% at end of trial as did the severity of hearing loss.

scholarly journals Audiological and otologic manifestations of glutaric aciduria type I

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Yen-Chi Chen ◽  
Chii-Yuan Huang ◽  
Yen-Ting Lee ◽  
Chia-Hung Wu ◽  
Sheng-Kai Chang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Impairment ◽  
Past History ◽  
Glutaric Aciduria Type ◽  
Sensorineural Hearing ◽  
Neurological Deficits ◽  
Type I ◽  
Glutaric Aciduria ◽  
Icu Admission

Abstract Background Glutaric aciduria type 1 (GA-1) is a rare disease connected with speech delay and neurological deficits. However, the audiological and otologic profiles of GA-1 have not yet been fully characterized. To our knowledge, this is the largest study of comprehensive audiological and otologic evaluation in patients with GA-1 to date. Methods Thirteen patients diagnosed with GA-1 between January 1994 and December 2019 with audiological, radiological and genetic manifestations were retrospectively analyzed. Hearing tests were performed in all patients. MRI was performed for radiological evaluation. Results Hearing loss was found in 76.9% (10/13) of GA-1 patients, including slight hearing loss in 46.1% (6/13) of patients, mild hearing loss in 15.4% (2/13) of patients, and moderate hearing loss in 7.7% (1/13) of patients. Normal hearing thresholds were seen in 23% (3/13) of patients. Patients with intensive care unit (ICU) admission history showed significantly worse hearing than those without (29.17 ± 12.47 vs 13.56 ± 3.93 dB HL, 95% CI 2.92–24.70, p = 0.0176). One patient had moderate sensorineural hearing loss and a past history of acute encephalopathic crisis. No usual causative gene mutations associated with hearing loss were found in these patients. MRI showed a normal vestibulocochlear apparatus and cochlear nerve. One patient with extensive injury of the basal ganglia on MRI after acute encephalopathic crisis was found to have moderate sensorineural hearing loss. Two patients with disability scores above 5 were found to have mild to moderate hearing impairment. No obvious correlation between macrocephaly and hearing loss was found. Conclusion A high prevalence of hearing impairment is found in GA-1 patients. Adequate audiological evaluation is essential for these patients, especially for those after encephalopathic crises or with ICU admission history.

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