Indian Journal of Otology

CASE REPORT
Year
: 2019  |  Volume : 25  |  Issue : 4  |  Page : 213--215

A rare entity in a child: Unilateral sensorineural hearing loss with an ipsilateral vascular loop – Is there any correlation? A case report and literature review


Ramaprabah Kandiah1, Nik Adilah Nik Othman2,  
1 Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, Kubang Kerian, Kelantan, Malaysia
2 Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus; Audiology and Speech Pathology Program, School of Health Sciences, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia

Correspondence Address:
Dr. Ramaprabah Kandiah
1294, Lorong KK 8/4, Bandar Baru Kubang, Kerian, Kota Bharu 16150, Kelantan
Malaysia

Abstract

Unilateral sensorineural hearing loss (USNHL) in children is multifactorial, which includes the inner ear malformations, cochlear nerve deficiency, perinatal cytomegalovirus infections, and Waardenburg syndromes. The exact cause of unexplained sensorineural hearing loss (SNHL) in children is explained via findings from imaging such as high-resolution computer tomography and magnetic resonance imaging (MRI). There are some evidence reported showing correlation between vascular loop and unilateral hearing loss in adults. Association between vascular loop and USNHL in children is yet to be published. A 12-year-old girl presented to otorhinolaryngology clinic with left ear reduced hearing since the age of 6 years. Pure tone audiometry yielded unilateral severe-to-profound SNHL. MRI of brain revealed ipsilateral second-degree vascular loop which projected into porus acusticus (medial end) of the internal auditory canal. She was counseled for hearing aid to improve her hearing. As there was no other possible abnormality noted, we are correlating the hearing loss with the presence of vascular loop in the MRI.



How to cite this article:
Kandiah R, Othman NA. A rare entity in a child: Unilateral sensorineural hearing loss with an ipsilateral vascular loop – Is there any correlation? A case report and literature review.Indian J Otol 2019;25:213-215


How to cite this URL:
Kandiah R, Othman NA. A rare entity in a child: Unilateral sensorineural hearing loss with an ipsilateral vascular loop – Is there any correlation? A case report and literature review. Indian J Otol [serial online] 2019 [cited 2023 Feb 5 ];25:213-215
Available from: https://www.indianjotol.org/text.asp?2019/25/4/213/272228


Full Text



 Introduction



Unilateral sensorineural hearing loss (USNHL) is a common deficit and occurs in 0.1%–3% of newborns and in more than 5% of school-aged children.[1],[2],[3],[4] Etiologies for USNHL differ from bilateral sensorineural hearing loss (SNHL). Paul et al. concluded that the inner ear malformations, cochlear nerve deficiency, perinatal cytomegalovirus infections, and Waardenburg syndromes were some of the common causes of pediatric USNHL. USNHL may pose challenges to clinicians in both timely diagnosis and effective treatment as it is known to impact on school performance and social skills during childhood.[5],[6],[7],[8] We report a rare case of a child with a unilateral severe-to-profound hearing loss, who has no other clinical abnormalities but only an ipsilateral vascular loop in the magnetic resonance imaging (MRI) of the brain.

 Case Report



A 12-year-old Malay girl presented to our otorhinolaryngology clinic in July 2017 with progressively reducing left ear hearing since the age of 6 years. She had no other ear complaints such as otorrhea, itchiness, tinnitus, and vertigo and had no complaints on the contralateral ear. The patient denies any ear infections in the past and history of either fall or trauma. She was born term with body weight of 3 kg. She had neonatal jaundice but was not admitted for phototherapy. There is no history of intubation or neonatal intensive care unit admission. There is absence of family history related to deafness. There was no delay in speech and language development. Otologic examinations were unremarkable bilaterally. Nasal examination only revealed mildly enlarged adenoids on rigid nasoendoscopy. Oropharyngeal examination showed bilateral grade 2 tonsillar enlargement without evidence of chronicity. Pure tone audiometry findings yielded left severe-to-profound SNHL with right normal hearing. The stapedial reflexes of left ipsilateral and right contralateral were absent. She was then subjected to MRI brain to rule out retrocochlear pathology. MRI brain revealed a left vascular loop, which projected into the porus acusticus of the internal auditory canal (IAC) [Figure 1]. No other significant findings were reported. She was counseled for hearing aid.{Figure 1}

 Discussion



USNHL in children has variety of etiologies. It can be classified according to nongenetic and genetic causes. Specific cause of auditory impairment in children with unexplained SNHL was able to be identified via radiologic studies such as high-resolution computed tomography and MRI.[9] High-resolution magnetic resonance gives excellent imaging of lesions of the central auditory pathway in the brain, e.g., in the IAC, cerebellopontine angle (CPA), brainstem, and cerebral cortex.

The most common imaging abnormality detected in children with SNHL is the large vestibular aqueduct.[9] Correlation of vascular loop and hearing impairment in children has not been reported. However, Janetta first proposed that vascular loops located at the adult patients' CPA might interfere with the eighth cranial nerve resulting in symptoms of vertigo, tinnitus, and auditory neural loss.[10] McDermott et al.[11] found a correlation between the presence of a vascular loop and the unilateral hearing loss. SNHL, tinnitus, and vertigo might be caused by the compression of the auditory and vestibular nerve by vascular loops.[12],[13]

Vascular loops first reported by neurosurgeon Walter Dandy in 1925 are anatomical abnormalities that arise from branches of the basilar artery (98%) or from the vertebral artery (2%).[14] Vascular loops formed by the anterior inferior cerebellar artery (AICA) in the IAC, is a common finding in the MRI.[11],[12]

McDermott et al. classified the loops based on their nerve trajectory. They found that 62% were located in the patients' CPA (Degree I), 30% were located in their porus acusticus (Degree II), and 8% were located completely in their IAC (Degree III) after studying 667 patients with vascular loops.[13] Lee et al. also had similar findings and concluded that third-degree vascular loop is the least found findings in MRIs.

The nerves (facial and vestibulocochlear nerve) and AICA loop are located within the internal auditory meatus which has minimum space and hence the nerve is susceptible to vascular compression dysfunction. Chadha and Weiner proposed that vascular loops are twice more likely to be found in symptomatic ear, in cases of USNHL.[15] Similar to our case, the vascular loop was also found at the symptomatic ear.

 Conclusion



Vascular loops in the IAC have correlations with hearing loss, especially in cases where there are absence of other abnormalities or pathologies. High-resolution MRI brain is the best modality of choice to detect vascular loops. Reporting this case report is important as finding of unilateral hearing loss with ipsilateral vascular loop in a child although is a rare entity, it is an important finding that can be explained to the parents of the child, and this will lead to a better parents' expectation and management of the child.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Bess FH, Dodd-Murphy J, Parker RA. Children with minimal sensorineural hearing loss: Prevalence, educational performance, and functional status. Ear Hear 1998;19:339-54.
2Gravel J. Prevalence and screening in newborns. In: National Workshop on Mild and Unilateral Hearing Loss: Workshop Proceedings 2005. Breckenridge, CO.: Centers for Disease Control and Prevention; 2005. p. 15-7.
3Bess FH, Tharpe AM. Unilateral hearing impairment in children. Pediatrics 1984;74:206-16.
4Bess FH, Tharpe AM. Case history data on unilaterally hearing-impaired children. Ear Hear 1986;7:14-9.
5Lieu JE, Karzon RK, Ead B, Tye-Murray N. Do audiologic characteristics predict outcomes in children with unilateral hearing loss? Otol Neurotol 2013;34:1703-10.
6Lieu JE, Tye-Murray N, Karzon RK, Piccirillo JF. Unilateral hearing loss is associated with worse speech-language scores in children. Pediatrics 2010;125:e1348-55.
7Fischer C, Lieu J. Unilateral hearing loss is associated with a negative effect on language scores in adolescents. Int J Pediatr Otorhinolaryngol 2014;78:1611-7.
8Lieu JE, Tye-Murray N, Fu Q. Longitudinal study of children with unilateral hearing loss. Laryngoscope 2012;122:2088-95.
9Mafong DD, Shin EJ, Lalwani AK. Use of laboratory evaluation and radiologic imaging in the diagnostic evaluation of children with sensorineural hearing loss. Laryngoscope 2002;112:1-7.
10Jannetta PJ. Neurovascular cross-compression in patients with hyperactive dysfunction symptoms of the eighth cranial nerve. Surg Forum 1975;26:467-9.
11McDermott AL, Dutt SN, Irving RM, Pahor AL, Chavda SV. Anterior inferior cerebellar artery syndrome: Fact or fiction. Clin Otolaryngol Allied Sci 2003;28:75-80.
12Lee AD, Djalilian H. Radiology case Quiz 2. Left vascular loop. Arch Otolaryngol Head Neck Surg 2007;133:1163, 1165-6.
13Irving RM, Chavda S. Re: Vascular loops causing otological symptoms: A systematic review and meta-analysis. Clin Otolaryngol 2008;33:498.
14Dandy WE. Trigeminal neuralgia and trigeminal tic douloureux. In: Lewis D, editor. Practice of Surgery. Hagerstown, MD: WF Prior CO.; 1932. p. 177-200.
15Chadha NK, Weiner GM. Vascular loops causing otological symptoms: A systematic review and meta-analysis. Clin Otolaryngol 2008;33:5-11.