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High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss.
Int J Pediatr Otorhinolaryngol. 2013 Feb; 77(2):228-32.IJ

Abstract

OBJECTIVE

Radiological and genetic examination has recently advanced for diagnosis of congenital hearing loss. The aim of this study was to elucidate the prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss (USNHL) for better management of hearing loss and genetic and lifestyle counseling.

METHODS

We conducted a retrospective study of charts and temporal bone computed tomography (CT) findings of 69 consecutive patients 0-15 years old with USNHL. In two cases, genetic examination was conducted.

RESULTS

Of these patients, 66.7% had inner-ear and/or internal auditory canal malformations. The prevalence of malformations in infants (age <1 year) was 84.6%, which was significantly higher than that in children 1-15 years old (55.8%; p<0.01). Almost half of the patients (32; 46.4%) had cochlear nerve canal stenosis; 13 of them had cochlear nerve canal stenosis alone, and in 19 it accompanied other malformations. Internal auditory canal malformations were observed in 22 subjects (31.8%), 14 (20.3%) had cochlear malformations, and 5 (7.2%) had vestibular/semicircular canal malformations. These anomalies were seen only in the affected ear, except in two of five patients with vestibular and/or semicircular canal malformations. Two patients (2.9%) had bilateral enlarged vestibular aqueducts. Mutations were found in SLC26A4 in one of the two patients with bilateral large vestibular aqueducts. The prevalence of a narrow internal auditory canal was significantly higher in subjects with cochlear nerve canal stenosis (50.0%) than in subjects with normal cochlear nerve canals (11.1%; p<0.01). There were no correlations between the type and number of malformations and hearing level.

CONCLUSIONS

The prevalence of inner-ear and/or internal auditory canal malformations detected by high-resolution temporal bone CT in children with USNHL was very high. Radiological and genetic examination provided important information to consider the pathogenesis and management of hearing loss. Temporal bone CT should be recommended to children with USNHL early in life. SLC26A4 mutation also should be examined in cases with bilateral enlarged vestibular aqueduct.

Authors+Show Affiliations

Department of Otorhinolaryngology, Institute for Clinical Research, National Mie Hospital, Tsu, Mie, Japan. masudas@mie-m.hosp.go.jpNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

23200870

Citation

Masuda, Sawako, et al. "High Prevalence of Inner-ear And/or Internal Auditory Canal Malformations in Children With Unilateral Sensorineural Hearing Loss." International Journal of Pediatric Otorhinolaryngology, vol. 77, no. 2, 2013, pp. 228-32.
Masuda S, Usui S, Matsunaga T. High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 2013;77(2):228-32.
Masuda, S., Usui, S., & Matsunaga, T. (2013). High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss. International Journal of Pediatric Otorhinolaryngology, 77(2), 228-32. https://doi.org/10.1016/j.ijporl.2012.11.001
Masuda S, Usui S, Matsunaga T. High Prevalence of Inner-ear And/or Internal Auditory Canal Malformations in Children With Unilateral Sensorineural Hearing Loss. Int J Pediatr Otorhinolaryngol. 2013;77(2):228-32. PubMed PMID: 23200870.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss. AU - Masuda,Sawako, AU - Usui,Satoko, AU - Matsunaga,Tatsuo, Y1 - 2012/11/30/ PY - 2012/08/14/received PY - 2012/10/29/revised PY - 2012/11/03/accepted PY - 2012/12/4/entrez PY - 2012/12/4/pubmed PY - 2013/7/19/medline SP - 228 EP - 32 JF - International journal of pediatric otorhinolaryngology JO - Int J Pediatr Otorhinolaryngol VL - 77 IS - 2 N2 - OBJECTIVE: Radiological and genetic examination has recently advanced for diagnosis of congenital hearing loss. The aim of this study was to elucidate the prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss (USNHL) for better management of hearing loss and genetic and lifestyle counseling. METHODS: We conducted a retrospective study of charts and temporal bone computed tomography (CT) findings of 69 consecutive patients 0-15 years old with USNHL. In two cases, genetic examination was conducted. RESULTS: Of these patients, 66.7% had inner-ear and/or internal auditory canal malformations. The prevalence of malformations in infants (age <1 year) was 84.6%, which was significantly higher than that in children 1-15 years old (55.8%; p<0.01). Almost half of the patients (32; 46.4%) had cochlear nerve canal stenosis; 13 of them had cochlear nerve canal stenosis alone, and in 19 it accompanied other malformations. Internal auditory canal malformations were observed in 22 subjects (31.8%), 14 (20.3%) had cochlear malformations, and 5 (7.2%) had vestibular/semicircular canal malformations. These anomalies were seen only in the affected ear, except in two of five patients with vestibular and/or semicircular canal malformations. Two patients (2.9%) had bilateral enlarged vestibular aqueducts. Mutations were found in SLC26A4 in one of the two patients with bilateral large vestibular aqueducts. The prevalence of a narrow internal auditory canal was significantly higher in subjects with cochlear nerve canal stenosis (50.0%) than in subjects with normal cochlear nerve canals (11.1%; p<0.01). There were no correlations between the type and number of malformations and hearing level. CONCLUSIONS: The prevalence of inner-ear and/or internal auditory canal malformations detected by high-resolution temporal bone CT in children with USNHL was very high. Radiological and genetic examination provided important information to consider the pathogenesis and management of hearing loss. Temporal bone CT should be recommended to children with USNHL early in life. SLC26A4 mutation also should be examined in cases with bilateral enlarged vestibular aqueduct. SN - 1872-8464 UR - https://www.unboundmedicine.com/medline/citation/23200870/High_prevalence_of_inner_ear_and/or_internal_auditory_canal_malformations_in_children_with_unilateral_sensorineural_hearing_loss_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0165-5876(12)00619-2 DB - PRIME DP - Unbound Medicine ER -