Search results for " Hearing Loss"

Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome

2017

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic va…

Medikamentöse Therapie progredienter kindlicher Hörstörungen

1991

In 1987 and 1988 Nickisch et al. discussed the effect of infusions of prednisolone, pentoxifylline and, partially, piracetam in Ringer lactate solvent on progressive sensorineural hearing loss in childhood and adolescence. Time of observations was 4 years. Unfortunately long-term investigations could not confirm the initially good therapeutic results of 1987/88. In 35% were found permanently and in 12.5% temporarily better auditory thresholds after infusion. The loss of progressive sensorineural hearing in childhood usually takes a fateful course that can hardly be influenced. As is the case with adults, good therapeutic results can most probably be achieved by infusions starting close to t…

Association between obstructive apnea syndrome during sleep and damages to anterior labyrinth: Our experience

2019

The obstructive sleep apnea syndrome is a chronic condition characterized by frequent episodes of collapse of the upper airways during sleep. It can be considered a multisystem disease. Among the districts involved, even the auditory system was seen to be concerned. It was enrolled a population of 20 patients after polysomnographic diagnosis of OSAS (Apnea Hypopnea Index > 10) and a control group of 28 healthy persons (Apnea Hypopnea Index < 5). Each patient has been subjected to Pure Tone Audiometry, Tympanometry, study of Acoustic Reflex, Otoacoustic Emissions and Auditory Brainstem Response. Moreover they were submitted to endoscopy of upper airway with Muller Maneuver and Epworth …

Soluble Coenzyme Q10 in Chronic Hearing Loss.

2011

Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6

2013

Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis an…

Psychiatric Disorders in Otoneurology Patients

2005

Nearly half the patients who are in specialized otoneurologic units exhibit psychiatric disorders. The disorders are most significant in patients who have the chief symptoms of dizziness, complex forms of tinnitus, or sudden and relapsing sensorineural hearing loss. If not properly diagnosed, these disorders can lead to improper treatment, particularly when the illness is a somatoform otoneurologic disorder. Patients who have comorbid symptoms, such as reactive anxiety or depressive disorders, may experience difficulties in coping with the illness. In many instances, reactive psychologic disorders can lead to severe impairment of daily life and work activities.

Oxidative stress neuroinflammation and cellular stress response in sensorineural hearing loss: novel nutritional therapeutical approaches

2016

This study is intended to validate the hypothesis that changes in the redox state of glutathione, the major endogenous antioxidant, associated with the abnormal expression and activity of cytoprotective vitagenes, which in normal conditions are expressed only at low level may represent a critical factor, involved in the physiopathological changes associated to degenerative damage occurring in cochlear diseases. Moreover modulation of stress responsive vitagenes by nutritional antioxidants can be an effective therapeutic strategy to minimize consequences of oxidative stress associated to the pathogenesis and course of sensorineural hearing loss. One therapeutic approach can be antioxidant su…

Genetic analysis of 2299delG and C759F mutations (USH2A) in patients with visual and/or auditory impairments

2004

The most common mutation in the USH2A gene (Usherin), 2299delG, causes both typical Usher (USH) syndrome type II and atypical USH syndrome, two autosomal recessive disorders, characterised by moderate to severe sensorineural hearing loss and retinitis pigmentosa (RP). Furthermore, the C759F mutation in the USH2A gene has been described in 4.5% of patients with nonsyndromic recessive RP. We have investigated the presence of the 2299delG and/or the C759F mutations in 191 unrelated Spanish patients with different syndromic and nonsyndromic retinal diseases, or with nonsyndromic hearing impairment. The 2299delG mutation was observed in patients with clinical signs of USHII or of atypical USH sy…

Pharmacological treatment of sensorineural hearing loss

2019

Sudden sensorineural hearing loss is a common and alarming symptom of about 360 million people that suffer from hearing impairment worldwide. The sudden sensorineural hearing loss usually arises unilaterally and it is habitually described as greater than 30dB hearing reduction, attributable to lesions of the cochlea, cranial nerve VIII, brainstem and temporal lobe. There are many factor that promote the onset of this lesions such us infections, circulatory diseases, inner ear neoplasia and neurological disorders. This pathology is characterized by primary symptoms such as the impairment of the comprehension of spoken language and the struggling to listen to music. Subsequently, secondary sy…

Das Cogan-I-Syndrom

2003

Wir berichten uber 2 Patienten, die seit dem Sauglings- und Kleinkindesalter unter einem Cogan-I-Syndrom leiden.Bei diesem seltenen Krankheitsbild handelt es sich um eine akute oder schubweise Verschlechterung der Horleistung des Innenohrs mit vestibularer Symptomatik und einer nichtsyphilitischen interstitiellen Keratitis oder anderen entzundlichen okularen Symptomen beim atypischen Cogan-I-Syndrom. Die erste Patientin erkrankte im 5.Lebensjahr an verschiedenen okularen Entzundungen, sodass ein Auge im 8.Lebensjahr erblindete.Zu dieser Zeit begann die Horverschlechterung. Im Alter von 17 Jahren litt die Patientin beidseits unter einer an Taubheit grenzenden Schwerhorigkeit. Bei dem zweiten…