Idiopathic sudden sensorineural hearing loss in children

doi:10.1016/j.ijporl.2005.01.015

International Journal of Pediatric Otorhinolaryngology

Volume 69, Issue 6, June 2005, Pages 817-821

https://doi.org/10.1016/j.ijporl.2005.01.015 Get rights and content

Summary

Objective:

Although idiopathic sudden sensorineural hearing loss (ISSHL) is a frequent disease in adults, less is known about incidence and treatment of ISSHL in children.

Method:

A retrospective chart analysis was performed to evaluate the frequency of ISSHL in children aged under 18 years between 2000 and 2003, who were treated in our department. Children received prednisolone intravenously at an initial dose of 3 mg/kg bodyweight. Prednisolone dose was reduced to half every second day. The medication was given for a maximum of 14 days or finished 2 days after the hearing normalized in pure-tone audiometry. The follow-up was continued between 3 and 14 months.

Results:

The complete recovery rate was 57%, and the partial recovery was 36%. Initial hearing loss of 50 dB and more was predictive for poor outcome in children (p = 0.028). Presence of tinnitus was without relevance for the outcome. The incidence of ISSHL in the local area of about 250,000 inhabitants was 1/10,000 in children.

Conclusion:

ISSHL seems to be a less frequent disease in children than in adults. Severe initial hearing loss is coupled with poor outcome. Under treatment with prednisolone hearing improvement was found in 13 of 14 patients.

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.

Section snippets

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
2023, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Pediatric idiopathic sudden hearing loss (PISSNHL) is a rare disease with no established factor affecting its prognosis. In this study, we investigate the risk factors affecting the prognosis of PISSNHL.
Among the patients who visited our hospital from January 2010 to December 2021, the characteristics associated prognosis of 54 patients with unilateral PISSNHL were retrospectively confirmed.
Patients' recovery was determined by applying Siegel's criteria (SC) and AAO-HNS criteria (AC). Twenty-seven (50 %) and 29 patients (54.3 %) recovered for SC and AC, respectively. Age, sex, side, duration between onset and treatment, administration of intra-tympanic steroid injection, accompanying symptoms (tinnitus and dizziness), BMI, serum creatinine level, neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte count (PLR), lymphocyte count, and platelet count were not significantly different between the recovery group and the poor recovery group (P > 0.05). The patients were divided into five groups according to the initial hearing of the affected ear and again according to their audiogram type. The initial hearing levels, hearing level severity, and the audiogram type were significantly different between the deaf group (>100 dB HL) and the non-deaf group (P < 0.05).
The prognosis of PISSNHL is closely related to the initial hearing at the onset. If the initial hearing level is <100 dB, the recovery rate is approximately 50 %, therefore requiring active treatment and emotional support. It may also be related to the type of audiometric curve.
Practice trends in pediatric sudden sensorineural hearing loss management: An unresolved diagnosis
2023, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Assess practice patterns amongst pediatric otolaryngologist for the management of children with SSNHL.
A cross-sectional online survey of members of the American Society of Pediatric Otolaryngology (ASPO) was performed; 135 responded. Patterns in treatment modalities, ancillary tests, and timing of treatment and follow-up were evaluated. These patterns were compared between respondents with different characteristics (number of years in practice, clinic location, and number of pediatric SSNHL cases within the last year) using ordered logistic regression, Kruskal-Wallis, Wilcoxon rank-sum, and Fisher's exact tests.
Mean time from onset of hearing loss to presentation to a pediatric otolaryngologist was 10 days (range 1–60 days). The most cited reasons for delay in care were ‘patient not seeking any healthcare evaluation’ (65 %) and ‘lack of access to obtain an audiogram’ (54 %). The most ordered blood work was complete blood count (14 %) and herpes simplex testing (15 %). Complete blood count was ordered more frequently by physicians in practice for >10 years compared with those in practice 1–10 years, P = 0.03. Most respondents reported treating with systemic steroids (86/92, 93 %), including intratympanic steroids (32/92, 35 %). Treatment with systemic steroids was more common in academic compared with private practice, P = 0.03. Antivirals were the most common additional agent prescribed (14/89, 16 %). Most patients were seen in follow-up 1–4 weeks after diagnosis (63/85, 74 %).
Most pediatric otolaryngologists treat SSNHL with systemic steroids. The remainder of the diagnostic and management paradigm varies significantly, highlighting the need to systematically define which treatment optimizes outcomes in this population.
Congenital Sensorineural Hearing Loss
2022, Pediatric Clinics of North America
Citation 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 Otorhinolaryngology
Citation 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].
Sudden sensorineural hearing loss (SSNHL) is relatively rare and its physiopathology remains unclear, particularly in children. Our goal was to evaluate clinical characteristics, etiologies, management, treatment outcomes and prognostic factors in the pediatric population.
We performed a retrospective chart review of all children registered for SSNHL between August 2004 and September 2017 in a tertiary care pediatric hospital. We analysed data regarding clinical symptoms, audiological characteristics, diagnostic investigations and treatment outcomes.
Thirty-five patients were included. Mean age was 12 years (range 4–18 years). Male:female ratio was 15:20. Hearing loss was left-sided for 18 patients, right-sided for 12 patients and bilateral for 5 patients. Degree of hearing loss varied from mild to profound across frequencies in the 40 ears studied. Thirty-four patients had associated otologic symptoms: the most frequent was tinnitus (28 ears), followed by vertigo (23 ears), otalgia (5 ears) and sensation of blocked ear (5 ears). Twenty-nine patients received systemic steroids and 3 intra-tympanic steroids. In the treated group, 69% had improvement on the audiograms (14% total, 55% partial). Vestibular tests were performed in 16 patients and were abnormal in 10 patients. Radiological examination included computed tomography scan (n = 16) and/or magnetic resonance imaging (n = 33). They revealed 2 bilateral enlarged vestibular aqueducts, 1 labyrinthitis, 1 intra-cochlear haemorrhage.
SSNHL can affect speech and language development in children. There are differences among the pediatric population, including inner ear malformation and immune disease. Specific work up is proposed. Appropriate diagnosis and therapeutic management are discussed.
Declining prevalence of pediatric sudden deafness during the past two decades
2019, International Journal of Pediatric Otorhinolaryngology
This study compared the prevalence of sudden deafness (SD) in children to investigate the evolution of pediatric SD during the past two decades.
From 1996 to 2005, totaling 358 SD patients were experienced. Of them, 25 patients (7%) aged <15 years were assigned to Group A. In contrast, 5 patients (2%) aged <15 years of 242 SD patients encountered during the period 2006–2015 were assigned to Group B. All patients underwent audiovestibular function testing.
The measles-mumps-rubella (MMR) vaccination reached to 95% vaccination rate in Taiwan after 1994. As 1994 (MMR vaccination years) + 11 (mean age of pediatric SD) equals 2005, this study found that declining prevalence of pediatric SD was from 7% (1996–2005) to 2% (2006–2015) at our hospital, consistent with declining annual cases of SD in Taiwan during the past decades. A significantly higher abnormality rate of mean hearing level (93%) than abnormal caloric responses (20%) was identified indicating that pediatric SD predominately affected the cochlear partition.
The prevalence of pediatric SD cases has significantly declined during the past 20 years, probably due to global vaccination policy. Other causal factors such as growing numbers of hospital and advancement in radiological diagnostic technique may also contribute to the declining prevalence.
Side-related differences in sudden sensorineural hearing loss in children
2018, International Journal of Pediatric Otorhinolaryngology
Most studies on sudden sensorineural hearing loss (SSNHL) do not differentiate the outcomes within varied affected ears in children. The present study was designed to determine the clinical differences between unilateral and bilateral SSNHL in children.
The clinical data, from a total of 101 pediatric patients with SSNHL, was retrospectively analyzed from January 2003 to December 2016. The main outcome measures included basic characteristics, etiology, clinical symptoms and treatment courses.
When the bilateral group (n = 28) was compared to the unilateral group (n = 73), neither gender nor onset of SSNHL was significantly different (p > 0.05 each); However, bilateral SSNHL tended to occur in younger ages (8.1 ± 4.0 yrs), with higher percentages of suspected etiologies (50%) and proportion of profound deafness (55.4%, p < 0.05 each). The short-term recovery rate was superior in the unilateral cases over the bilateral cases (37.0% vs. 12.5%, p < 0.05). Milder initial hearing threshold, early onset of treatment (5.6 ± 4.8 days) with unilateral involvement and an older age (11.3 ± 3.0 yrs) in bilaterally affected cases were associated with a better prognosis in this cohort. In addition, the unilateral group showed comparable outcomes, when sub-analyzed by comparison to that in either left- (n = 42) or right-sided (n = 31) SSNHL.
Although bilateral and unilateral pediatric SSNHL could cause partial to complete cochlear lesion, they may be relevant to distinct backgrounds. Our data also provides valuable information about demographics and outcomes of SSNHL in children.

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Idiopathic sudden sensorineural hearing loss in children

Summary

Objective:

Method:

Results:

Conclusion:

Introduction

Section snippets

Patients and methods

Results

Discussion

Lancet

Int. J. Pediatr. Otorhinolaryngol.

Int. J. Pediatr. Otorhinolaryngol.

Hear. Res.

Int. J. Pediatr. Otorhinolaryngol.

Int. J. Pediatr. Otorhinolaryngol.

Int. J. Pediatr. Otorhinolaryngol.

Int. J. Pediatr. Otorhinolaryngol.

Randomized double-blind study of therapy of sudden deafness: initial results

HNO

Diagnosis and treatment of idiopathic sudden sensorineural hearing loss (ISSHL): a survey in The Netherlands and Flanders

Acta Otorhinolaryngol. Belg.

Autoimmune identification of sudden hearing loss

Acta Otolaryngol.

Autoimmune sensorineural hearing loss in young patients: an exploratory study

Laryngoscope