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 Diagnostic Yield of MRI for Sensorineural Hearing Loss – An Audit

2020 ◽  
Vol 47 (5) ◽  
pp. 656-660
Author(s):  
Helen Wong ◽  
Yaw Amoako-Tuffour ◽  
Khunsa Faiz ◽  
Jai Jai Shiva Shankar
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Diagnostic Yield ◽  
Internal Auditory Canal ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Vestibular Evoked Myogenic Potentials ◽  
Clinical Indication ◽  
Presenting Symptoms ◽  
Brainstem Response

ABSTRACT:Purpose:Contrast-enhanced magnetic resonance imaging (CEMRI) of the head is frequently employed in investigations of sensorineural hearing loss (SNHL). The yield of these studies is perceptibly low and seemingly at odds with the aims of wise resource allocation and risk reduction within the Canadian healthcare system. The purpose of our study was to audit the use and diagnostic yield of CEMRI for the clinical indication of SNHL in our institution and to identify characteristics that may be leveraged to improve yield and optimize resource utilization.Materials and methods:The charts of 500 consecutive patients who underwent CEMRI of internal auditory canal for SNHL were categorized as cases with relevant positive findings on CEMRI and those without relevant findings. Demographics, presenting symptoms, interventions and responses, ordering physicians, and investigations performed prior to CEMRI testing were recorded. Chi-squared test and t-test were used to compare proportions and means, respectively.Results:CEMRI studies revealed relevant findings in 20 (6.2%) of 324 subjects meeting the inclusion criteria. Pre-CEMRI testing beyond audiometry was conducted in 35% of those with relevant positive findings compared to 7.3% of those without (p < 0.001). Auditory brainstem response/vestibular-evoked myogenic potentials were abnormal in 35% of those with relevant CEMRI findings compared to 6.3% of those without (p < 0.001).Conclusion:CEMRI is a valuable tool for assessing potential causes of SNHL, but small diagnostic yield at present needs justification for contrast injection for this indication. Our findings suggest preferred referral from otolaryngologists exclusively, and implementation of a non-contrast MRI for SNHL may be a better diagnostic tool.

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.

Effect of Sensorineural Hearing Loss on Loudness Discomfort Level and Most Comfortable Loudness Judgments

1978 ◽  
Vol 21 (4) ◽  
pp. 668-681 ◽  
Author(s):  
Candace Kamm ◽  
Donald D. Dirks ◽  
Max R. Mickey
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Threshold ◽  
Sensorineural Hearing ◽  
Intersubject Variability ◽  
Adaptive Procedure ◽  
Speech Stimuli ◽  
Wide Range ◽  
Sensorineural Hearing Impairment ◽  
Comfortable Loudness

A simple up-down adaptive procedure was used to estimate the 50% point on the psychometric function for loudness discomfort level (LDL) and the two functions describing the range of most comfortable loudness (MCL) for listeners with sensorineural hearing impairment. For pure tone and speech stimuli, median LDL and MCL levels were observed at relatively constant SPLs for subjects with hearing loss ≤ 50 dB HL and at progressively higher SPLs with further increase in hearing loss. Correlation analysis verified a statistically significant relationship between LDL and magnitude of hearing loss. The nonlinear relationship between LDL and hearing loss together with the large intersubject variability in the data suggest that prediction of LDL from hearing threshold would often be highly inaccurate. These results also demonstrate that averaging LDL data across a group of subjects with a wide range of hearing loss may lead to inaccurate conclusions regarding the effects of sensorineural hearing loss on LDL.

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 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 Acute vertigo and sensorineural hearing loss from infarction of the vestibulocochlear nerve

Medicine ◽  
2018 ◽  
Vol 97 (41) ◽  
pp. e12777 ◽  
Author(s):  
Seonghwan Byun ◽  
Ji Ye Lee ◽  
Bo Gyung Kim ◽  
Hyun Sook Hong
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Vestibulocochlear Nerve

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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