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Original Investigation
July 6, 2017

Etiologic and Audiologic Characteristics of Patients With Pediatric-Onset Unilateral and Asymmetric Sensorineural Hearing Loss

Author Affiliations
  • 1Department of Otolaryngology, National Taiwan University Hospital Yunlin Branch, Yunlin, Taiwan
  • 2Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan
  • 3Department of Otolaryngology, Taichung Tzu-Chi Hospital, Taichung, Taiwan
  • 4Graduate Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan
  • 5Graduate Institute of Medical Genomics and Proteomics, National Taiwan University College of Medicine, Taipei, Taiwan
  • 6Department of Medical Genetics, National Taiwan University Hospital, Taipei, Taiwan
JAMA Otolaryngol Head Neck Surg. Published online July 6, 2017. doi:10.1001/jamaoto.2017.0945
Key Points

Question  What are the etiologic and audiologic features of patients with pediatric-onset unilateral and asymmetric hearing loss?

Findings  In this cohort study of 133 patients, inner ear malformations were detected at a higher rate in patients with unilateral hearing loss than in those with asymmetric hearing loss, whereas common genetic mutations were detected at a higher rate in patients with asymmetric hearing loss than in those with unilateral hearing loss.

Meaning  During the general etiologic workup, imaging studies might be prioritized for patients with unilateral hearing loss; genetic testing might be prioritized for patients with asymmetric hearing loss.

Abstract

Importance  Pediatric-onset unilateral and asymmetric sensorineural hearing loss (SNHL) is a common condition, but in most patients, the cause remains unclear; thus, determination of the hearing outlook is difficult.

Objective  To analyze the etiologic and audiologic characteristics of pediatric-onset unilateral and asymmetric SNHL.

Design, Setting, and Participants  In this retrospective cohort study performed from January 1, 2008, through December 31, 2016, patients at a tertiary referral center who were diagnosed with pediatric-onset unilateral or asymmetric SNHL were divided into 3 groups according to their hearing levels: unilateral hearing loss with scaled-out levels (UHL-SO), unilateral hearing loss with residual hearing (UHL-RH), and asymmetric hearing loss (AHL).

Main Outcomes and Measures  Basic demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and compared among patients of the 3 groups.

Results  A total of 133 patients (mean [SD] age, 9.1 [10.9] years; 63 [47.4%] male and 70 [52.6%] female), including 50 with UHL-SO, 42 with UHL-RH, and 41 with AHL, were enrolled for analyses. Of 50 patients with UHL-SO, 49 (98.0%) had stable hearing levels with time, whereas 10 of 42 patients with UHL-RH (23.8%) and 18 of 41 patients with AHL (43.9%) revealed progressive or fluctuating hearing loss. Inner ear malformations detected with temporal bone high-resolution computed tomography, particularly cochlear aperture stenosis, were detected at higher rates in patients with UHL-SO (9 of 31 [29.0%]) and UHL-RH (6 of 24 [25.0%]) than in those with AHL (1 of 30 [3.3%]). In contrast, screening for mutations in 3 common deafness genes—GJB2, SLC26A4, and MTRNR1—achieved definite diagnosis in a higher percentage of patients with AHL (10 of 37 [27.0%]) than patients with UHL-SO (0 of 33) and UHL-RH (1 of 25 [4.0%]).

Conclusions and Relevance  The UHL-SO and UHL-RH conditions share a common or similar etiopathogenesis different from that of AHL. Imaging studies and genetic testing might be prioritized during the respective general etiologic workups for patients with UHL and AHL. Regular hearing checkups are warranted for patients with UHL and AHL because a certain proportion of patients might sustain progression in SNHL.

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