Search results for "Hearing loss."

Nitric oxide--a versatile key player in cochlear function and hearing disorders.

2012

Nitric oxide (NO) is a signaling molecule which can generally be formed by three nitric oxide synthases (NOS). Two of them, the endothelial nitric oxide synthase (eNOS) and the neural nitric oxide synthase (nNOS), are calcium/calmodulin-dependent and constitutively expressed in many cell types. Both isoforms are found in the vertebrate cochlea. The inducible nitric oxide synthase (iNOS) is independent of calcium and normally not detectable in the un-stimulated cochlea. In the inner ear, as in other tissues, NO was identified as a multitask molecule involved in various processes such as neurotransmission and neuromodulation. In addition, increasing evidence demonstrates that the NO-dependent…

Agalsidase alpha and hearing in Fabry disease: data from the Fabry Outcome Survey.

2006

Fabry disease is an X-linked lysosomal storage disorder characterized by multi-organ dysfunction, including hearing loss - mainly sensorineural. The recent introduction of enzyme replacement therapy (ERT) has resulted in improvements in renal and cardiac function, pain and quality of life. One study has also suggested small improvements in high-frequency hearing. In this paper, we study the effect of ERT on hearing in patients in the Europe-wide database - the Fabry Outcome Survey (FOS). Twenty-six patients in FOS had pure-tone audiometry performed up to 6 months before starting ERT with agalsidase alpha and after a median of 12 months of treatment. We assessed changes in hearing thresholds…

BIOACTIVE GLASS CERAMIC

1983

The practicability of the bioactive glass ceramic Cernvitnl® in ear surgery was tested in animals. The histological findings are presented. Over the last 3 years implants of bioactive glass ceramic were used in humans. Prostheses for the total or partial reconstruction of the ossicular chain and the reconstruction of the bony wall of the outer ear canal were fashioned. We have conducted about 300 tympanoplasties and 60 total or partial reconstructions of the bony wall of the outer ear canal. The otoscopic and functional results were satisfactory.

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.

Do deaf children use phonological syllables as reading units?

2004

This study aimed at examining whether deaf children process written words on the basis of phonological units. In French, the syllable is a phonologically and orthographically well-defined unit. French deaf children and hearing children matched on word recognition level were asked to copy written words and pseudo-words. The number of glances at the item, copying duration, and the locus of the first segmentation (i.e., after the first glance) within the item were measured. The main question was whether the segments copied by the deaf children corresponded to syllables as defined by phonological and orthographic rules.The results showed that deaf children, like hearing children, used syllables…

Congenital adrenal hypoplasia and hearing loss. A case report

1995

We report on the diagnostics, the therapeutics and the follow-up (to 3 years and 5 months) of a newborn affected by X-linked congenital adrenal hypoplasia. After the beginning of substitute hormonal therapy, the patient underwent periodical clinical examinations, with particular attention to the growth, and laboratory tests, which monitored the hormonal pattern. This experience points out the diagnostic role of low maternal levels of urinary estriol during pregnancy and the importance of a prolonged follow-up, so as to discover associated pathologies as early as possible. In our case, in fact, the study of auditory brainstem responses enabled us to recognize a mild transmission hearing loss…