Idiopathic sudden sensorineural hearing loss in children
Introduction
Idiopathic sudden sensorineural hearing loss (ISSHL) is a frequent disease in adults and is estimated to occur with an incidence of 20/100,000 in Germany [1]. It is characterized by sudden hearing impairment up to deafness within a short period of time and is possibly associated with vestibular dysfunction, tinnitus and pressure sensation in the affected ear. In a majority of cases, ISSHL is unilateral. The underlying mechanism remains unclear. Several causes of ISSHL have been discussed in literature including disturbed perfusion of the cochlear artery [2], viral infection [3] and autoimmunologic response [4], [5]. Furthermore, a large variety of treatment options have been tried out. Drug therapy of ISSHL includes oral or intravenous application of vasodilatators, plasma expanders, antiviral therapy and corticosteroids as single medication or in combination. HELP–apharesis, carbogen inhalation and hyperbaric oxygen therapy have been evaluated. Prospective, placebo-controlled, double-blind clinical trials have been performed on treatment with hydroxyethyl starch + pentoxifyllin and hydroxyethyl starch + pentoxifyllin + naftidrofuryl [6] hydroxyethyl starch + lidocaine [7], oral prednisone [8] and carbogen inhalation [9], hydroxyethyl starch and pentoxyfyllin [10]. Except for one study, which showed a positive treatment effect with steroid therapy [8], all of the above-mentioned clinical trials were unable to detect any positive effect in comparison to placebo treatment. The spontaneous complete hearing recovery is reported to range from 31% [11] to 68% [12] in all cases of ISSHL. Although several studies are focused on epidemiology and etiology of hearing-impaired children, less is known about the incidence and effective treatment of ISSHL in children. The positive effect of treatment with a combination of prednisolone, pentoxifyllin and hydroxyethyl starch for progressive hearing loss in hearing-impaired children has been reported [13]. In comparison to untreated children, a higher recovery rate is observed. Initial severe hearing loss associated with vertigo and a downward audiometry curve are coupled with poor prognosis for hearing recovery in a small group of children under the age of 15 years [14].
The aim of this study was to observe the incidence of ISSHL in children and the outcome in hearing improvement under prednisolone treatment.
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Patients and methods
We performed a retrospective chart analysis of patients under 18 years of age from January 2000 to December 2003, who presented to our department with unilateral sudden hearing loss. The interval between the sudden onset of complaints and the initiation of treatment was limited to 2 weeks. The level of sudden hearing loss was measured by pure-tone audiometry at frequencies between 0.125 and 8 kHz and had to be at least more than 20 dB in three consecutive frequencies in comparison to the
Results
Fourteen patients under 18 years of age with ISSHL were included in the study. The mean age of these patients was 15 years (9–18 years). The interval between onset of complaints and treatment ranged between 1 and 14 days (mean, 3.2 days). Hearing loss was pantonal in 10 (71%) patients and the thresholds ranged from 30 to 100 dB. In two cases, hearing loss was located in low frequencies between 0.125 and 1 kHz with thresholds between 25 and 30 dB and in the two remaining cases hearing loss was
Discussion
As it has been stated before, little is known about treatment of ISSHL in children, while the treatment of ISSHL in adults is discussed controversially. In one study on medical treatment for progressive hearing loss in hearing-impaired children [13], a total recovery in 57% and a partial recovery in 28% of patients was achieved by prednisolone in combination with HAES 6% and pentoxifyllin. In an untreated group of 13 children, 3 (23%) had a spontaneous recovery. From another study a rate of
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Analysis of hearing prognosis risk factors in pediatric unilateral sudden sensorineural hearing loss
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2022, Pediatric Clinics of North AmericaCitation Excerpt :If genetic testing fails to reveal an underlying cause and there is no prior imaging workup done, patients will benefit from having dedicated CT temporal bone imaging to look for potential inner ear anomalies (see Fig. 2). If the results of both genetic and imaging workups are negative, the congenital hearing loss is considered idiopathic, which accounts for up to 25% to 30% of cases undergoing the workup.39 Ophthalmology consultation and electrocardiography together with renal ultrasonography are recommended to evaluate for potential significant morbidities associated with hearing loss.
Pediatric sudden sensorineural hearing loss: Experience in a pediatric ENT emergency care center
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Ascending type audiogram, early treatment, identifiable ABR waves and DPOAEs were shown to be positive prognostic factors in univariate analysis. Chen et al. found that younger patients have better audiological outcomes [19]. Qian et al. in a series of 75 children, showed the type of audiometric curve and the interval from onset to intervention were two independent risk factors that correlated with the prognosis of SSHNL [25].
Declining prevalence of pediatric sudden deafness during the past two decades
2019, International Journal of Pediatric OtorhinolaryngologySide-related differences in sudden sensorineural hearing loss in children
2018, International Journal of Pediatric Otorhinolaryngology
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