Diagnosis of Neurofibromatosis Type 2 in a Patient with Long-standing Bilateral Sensorineural Hearing Loss

2001 ◽  
Vol 30 (06) ◽  
pp. 368
Author(s):  
Steven E. Sobol ◽  
Jamie M. Rappaport ◽  
Khalid Al-Abdulhadi ◽  
Gerard Mohr
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Sensorineural Hearing ◽  
Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

2020 ◽  
pp. 624-624
Author(s):  
S. I. Ereniev ◽  
O. V. Plotnikova
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Biological Age ◽  
Biological Aging ◽  
Vibration Disease ◽  
Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Skin Lesions ◽  
Sudden Hearing Loss ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Urticarial Vasculitis ◽  
Wells Syndrome ◽  
Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

scholarly journals Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

2021 ◽  
Author(s):  
Suat Kılıç ◽  
Malek H. Bouzaher ◽  
Michael S. Cohen ◽  
Judith E. C. Lieu ◽  
Margaret Kenna ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Medical Evaluation ◽  
Bilateral Sensorineural Hearing Loss

Is simultaneous bilateral mastoidectomy ever advisable?

2007 ◽  
Vol 121 (11) ◽  
pp. 1041-1047 ◽  
Author(s):  
J J Klemens ◽  
E Mhoon ◽  
M Redleaf
Keyword(s):  
Hearing Loss ◽  
Decision Tree ◽  
Sensorineural Hearing Loss ◽  
Chart Review ◽  
Historical Data ◽  
Sensorineural Hearing ◽  
Opinion Survey ◽  
Potential Patient ◽  
Simultaneous Procedures ◽  
Bilateral Sensorineural Hearing Loss

AbstractIntroduction:We report our experience with bilateral, simultaneous tympanomastoidectomies and the results of an opinion survey of otologists.Methods:A chart review of 116 tympanomastoidectomies revealed 12 patients who underwent bilateral, simultaneous tympanomastoidectomies. An opinion survey generated 121 responses.Results:Of the 12 patients, none suffered any outcome which would have been avoided by staging the procedures. Twenty-three of 24 operated ears had the same or better hearing post-operatively. Of the survey respondents, 74 felt that performing bilateral, simultaneous tympanomastoidectomies was unsafe, largely because of the risk of bilateral sensorineural hearing loss.Discussion:Although bilateral, simultaneous tympanomastoidectomies carry double the risk of unilateral sensorineural hearing loss, compared with the unilateral procedure, the risk of bilateral sensorineural hearing loss is only 0.006–0.2 per cent, as derived mathematically from historical data. Respondents to the survey were mostly opposed to bilateral, simultaneous tympanomastoidectomies, but even those opposed gave indications for simultaneous procedures. This finding probably reflects an ambivalence about the theoretical risks of the operation versus the potential patient benefits. A decision tree for proceeding to the second case is presented.

Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

2020 ◽  
pp. 1-4
Author(s):  
K Pollaers ◽  
A Thompson ◽  
J Kuthubutheen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Bilateral Sensorineural Hearing Loss ◽  
Single Sided Deafness ◽  
Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

scholarly journals Characteristics of sensorineural hearing loss secondary to inner ear acoustic trauma

2008 ◽  
Vol 136 (5-6) ◽  
pp. 221-225
Author(s):  
Slobodan Spremo ◽  
Zdenko Stupar
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Threshold ◽  
Acoustic Trauma ◽  
Sensorineural Hearing ◽  
Cochlear Damage ◽  
Type 3 ◽  
Trauma Group

INTRODUCTION Cochlear damage secondary to exposure to acoustic trauma is the consequence of the acoustic energy effects on the hearing cells in Korti's organ. OBJECTIVE The objective was to assess the correlation between the degree of sensorineural hearing loss and the type of audiogram registered in acoustic trauma exposed patients. METHOD We analyzed 262 audiograms of patients exposed to acoustic trauma in correlation to 146 audiograms of patients with cochlear damage and hearing loss not related to acoustic trauma. "A" group consisted of acoustic trauma cases, while "B" group incorporated cases with hearing loss secondary to cochlear ischaemia or degeneration. All audiograms were subdivided with regard to the mean hearing loss into three groups: mild (21-40 dB HL), moderate (41-60 dB HL) and severe (over 60 dB HL) hearing loss. Based on audiogram configuration five types of audiogram were defined: type 1 flat; type 2 hearing threshold slope at 2 kHz, type 3 hearing threshold slope at 4 kHz; type 4 hearing threshold notch at 2 kHz; type 5 notch at 4 kHz. RESULTS Mild hearing loss was recorded in 163 (62.2%) ears in the acoustic trauma group, while in 78 (29.8%) ears we established moderate hearing loss with the maximum threshold shift at frequencies ranging from 4 kHz to 8 kHz. The least frequent was profound hearing loss, obtained in 21 (8%) audiograms in the acoustic trauma group. Characteristic audiogram configurations in the acoustic trauma patient group were: type 1 (N=66; 25.2%), type 2 (N=71; 27.1%), and type 3 (N=68; 25.9%). Audiogram configurations were significanly different in the acoustic trauma group in comparison to the cochlear ischaemia group of patients (p=0.0005). CONCLUSION Cochlear damage concomitant to acoustic trauma could be assessed by the audiogram configuration. Preserved hearing acuity at low and mild frequency range indicates the limited damage to the hearing cells in Korti's organ in the apical cochlear turn.

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